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Part 20. Cardiovascular System

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PART 20. The Cardiovascular System

Section 1. Evaluation of the Cardiovascular System

Chap. 380. History and Physical Examination

# cardiovascular disorders°¡ ÀǽɵǴ ȯ¾Æ¿¡ À־ history & physical examinationÀÇ Á߿伺À» ¾Æ¹«¸® °­Á¶Çصµ Áö³ªÄ¡Áö ¾Ê´Ù.

History

# history takingÇØ¾ß ÇÒ »çÇ×

  : details of the perinatal period - cyanosis, respiratory distress, or prematurity

  : maternal complication, such as gestational diabetes, medication exposure, or

    substance use

  : during infancy, the timing of 1st presentation

# *Sx of congestive heart failure - age specific

# Infant

    ; *feeding diffculities

           - *¡ãcommon

        - less volume per feeding

           - dyspneic or diaphoretic while sucking

           - awaken after brief period of time

    ; frequent G-E reflux

    ; respiratiory distress

           - rapid brething, nasal flaring, chest retraction

# older children

    ; exercise intolerance

           - *initially Sx

    ; fatigue

    ; orthopnea, nocturnal dyspnea

; cyanosis

   -Á¤»óÀο¡ ÀÖ¾î deep coloring

   -¿îµ¿½Ã blueness·Î Àß °üÂûµÊ

; chest pain

    - *usually not Sx of cardiac disease

¡ÚTable 380-1

# cardiac disease may be a congenital malformation syndrome

¡ÚTable 380-2

# cardiac disease may be a generalized disorder affecting the heart and other organ system

¡ÚTable 380-3

# Congenital HD

    ; *25%- extracardic malformation

    ; *10%- chromosomal abnormality

# family history·Î¼­ ¾Ë ¼ö ÀÖ´Â Áúȯ

1) early coronary a.ds(familial hypercholesterolemia)

2) generalized m.ds(muscular dystrophy,dermatomyositis)

3) prior congenital heart ds.

General Physical Examination

; ¡Ø96°´ begin with a general assessment of the patient

; height and weight

; cyanosis

    - best observed over the nail beds, lip, tongue, and mucus membranes.

; difference cyanosis

    - right-to-left shunting across a ductus arterious in the presence of a coraction of aorta.

; circumoral cyanosis or blueness about forehead

    - by *prominant venous plexus than arterial oxygen saturation

; heart failure

  - failure to thrive, tachypnea, liver and less so spleen enlargement, pulmonary rales, peripheral edema.

; Heart rate

¡ÚTable 380-4

    1) newborn infants¿¡¼­´Â rapid & wide fluctuation

    2) average range ; 120-140beats/min

    3) crying & activity¶§´Â 170±îÁö Áõ°¡Çϰí ÀáÀÚ´Â µ¿¾ÈÀº 70-90±îÁö °¨¼ÒÇÑ´Ù.

    4) persistent tachycardia

           - neonate > 200 beats/min

           - infant > 150 beats/min

           - old child > 120 beats/min

# character of pulse

    1) congenital heart ds.ÀÇ physical Dx¿¡ ÀÖ¾î early stepÀ¸·Î¼­ Áß¿ä.

    2) *wide pulse Pr.with bounding pulse(Water-Hammer pulse)

       - aortic runoff lesion

               ; *PDA, aortic insufficiency, A-V communication

       - increased cardiac output secondary to anemia, anxiety

       - increased catecholamine secretion

    3) *diminished pulse

       - heart failure

       - pericardial tamponade

       - left ventricular outflow obstruction

       - cardiomyopathy

# ¡Úblood pressure

    1) arm & leg

    2) decreased femoral and/or dorsalis pedis pulse

           - not reliable finding of coarctation

    3) 2/3 covered cuff of upper arms & legs

    4) small cuff -> high reading, large cuff -> low reading

    5) use popliteal a. in measuring legs pressure

    6) legs B.P - *10mmHg higher than upper arms

    7) in infant, ausculation, palpation, ultrasonic(doppler), oscillometric(dinamap)devices, flush method

    8) ³ªÀÌ¿¡ µû¶ó º¯ÇÏ¸ç ½ÅÀå,üÁß°úµµ ¹ÐÁ¢ÇÑ °ü°è

    9) exercise,excitment,coughing,strainingµîÀº Á¤»óº¸´Ù ¼öÃà±â Ç÷¾ÐÀ» 40-50mmHg ¿Ã¸°´Ù.

# normal jugular phlebogram- information about CVP, RA pressure

  ; three positive component

1) a wave; atrial systole

2) v wave; atrial diastole

3) c wave; early ventricular systole

  - external jugular vein should not be visible above the clavicle unless venous

    pressure is elevated.

Cardiac Examination

# Precardial bulge to the left of the sternum with increased precordial activity

  ; cardiac enlargement

# *Substernal thrust

  ; *right ventricular enlargement

# Apical heave

  ; left ventricular hypertrophy

# Hyperdynamic precordiun

  ; volume load like that found with a large left to right shunt

# Silent precordium with a barely detectable apical impulse

  ; pericardial effusion or severe cardiomyopathy

# Right sided apical impulses

  ; dextrocardia, tension pneumothorax, left sided thoracic space occupying lesions

    ; e.g.diaphragmatic hernia

# Thrills

  ; areas of maximum intensity of the auscultatory murmurs

# *Aortic bruits

    ; palpate suprasternal notch and neck

  ; *aortic stenosis

    ; less prominent pulmonary stenosis

# *apical systolic thrills on Rt. lower sternal border

  ; VSD, mitral insufficience

# diastolic thrills

  ; A-V vavular stenosis

# Stethoscopy

  ; diaphragm-high pitched sounds, bell-low pitched sounds

# 1st heart sound

  ; A-V valves closure¿¡ ÀÇÇØ »ý¼º, heart apex¿¡¼­ °¡Àå Àß µé¸²

# 2nd heart sound

  ; semilunar valves closure¿¡ ÀÇÇØ »ý¼º, left upper sternal border¿¡¼­ °¡Àå Àß µé¸²

# Inspiration°ú heartÀÇ right side¿¡ fillingÀÌ Áõ°¡ÇÏ´Â µ¿¾È¿¡´Â rt.vent.ejection timeÀÌ Áõ°¡Çϰí pul.valve closure´Â delayed

# heart soundÀÇ intensity¿¡ ¿µÇâÀ» ÁÖ´Â ¿ä¼Ò

  ; age if pt.,thickness of chest wall,cardiac output

# ¢¾ Wide splitting of S2

    1) PS

    2) Ebstein anomaly

    3) total anomalous venous rturn

    4) ASD

    5) TOF

    6) RBBB

¡Ø96 single S2

    ; pulmonary or aortic atresia

    ; severe stenosis

    ; truncus arteriosus

    ; TGA

# Gallop rhythm

    ; sign of congestive heart failure and tachycardia

    ; poor compliance of ventricle

¡Ø96ÁÖ Accentuated 2nd sound with narrow splitting

  ; pul.hypertension

# 3rd heart sound

    ; mid-diastolic¶§ bell·Î¼­ ½É÷ºÎ¿¡¼­ °¡Àå Àß µé¸²

  ; narrow in adolescent with a relatively slow heart rate

  ; CHF and tachycardia ÀÓ»óÁõ»óÀ» °¡Áø ȯ¾Æ¿¡¼­µµ ³ªÅ¸³²

# 4th heart sound

    ; late diastole¶§ 1st heart sound ¹Ù·Î Á÷Àü¿¡ µé¸²

# ¢¾ Ejection clicks

    1) heard in early systole

    2) related to *dilation of or hypertension in the aorta and pul.a.

    3) heard so close to 1st heart sound

           - mistaken for a split 1st sound

    4) Aortic systolic clicks

           - left mid to right upper sternal border

           - constant

           - AS,TOF,truncus arterious

    5) pulmonary ejection clicks

           - left mid to upper sternal border

           - vary with respiration, disappearing with inspiration

    6) midsystolic click at apex - mitral valve prolapse

# description of murmur

  ; intensity, pitch, timing(systolic and diastolic), area of maximal intensity, radiation to other areas

# Systolic murmurs

    1) ¡ÚIntensity

        1-barely audible

    2-medium intensity

    3-loud but no thrill

    4-loud with a thrill

    5-very loud but still requires the stesthoscope to be on the chest

    6-so loud the murmur can be heard with the stethoscope off the chest

    2) ºÐ·ù

           ; ejection

                - 1st heart soundÀÌÈÄ¿¡ ½ÃÀÛÇÏ¿© 2nd sound ÀÌÀü¿¡ ³¡³­´Ù.

                   - increased flow or stenosis across semilunar valve

                   - 1st heart sound appreciated --> ejection in nature

             - severe aortic or pulmonary stenosis

           ; pansystolic

                - 1st sound¿Í µ¿½Ã¿¡ ½ÃÀÛÇÏ¿© systoleµ¿¾È¿¡ Áö¼ÓµÈ´Ù

             - VSD or A-V valve insufficience

       ; late systolic

               - 1st soundÀÌÈÄ¿¡ ½ÃÀÛÇÏ´Â bruit·Î¼­ end systole±îÁö Áö¼ÓµÈ´Ù

               - mitral valve prolapse

    3) continuous murmur

        ; ¼öÃà±âºÎÅÍ ½ÃÀÛÇÏ¿© 2nd sound¸¦ Áö³ª¼­ È®Àå±â±îÁö µé¸°´Ù

    ; PDA, A-P communication

           ; DDx

                   - to and fro mummur

                   - aortic or pulmonary stenosis with insufficiency

¡Ø°´ Diastolic murmurs

    1) high pitched, blowing, decrescendo diastolic murmur along left sternal border

       ; *aortic insuffiency

           ; pul. insuffiency(if high pul. pressure)

    2) early, short, lower-pitched diastolic murmurs along left mid & upper sternal border

       ; pul. insuffiency

           ; after surgical repair of pul. outflow defect such as TOF

    3) rumbling mid-diastolic murmur at the left mid and lower sternal border

       ; increased blood flow across TV

           ; ASD

           ; tricuspid valve stenosis

    4) ¡Úrumbling mid-diastolic murmur at the apex follow the 3rd heart sound

       ; *increased transmitral flow

           ; *large right to left shunts

           ; mitral insuffiency

    5) longer diastolic rumbling murmur at apex

       ; anatomic mitral stenosis

# *absence of precordial mummur --> no rule-out congenital or acquired heart disease

    ; pulmonary or tricuspid valve atresia & TGA

# insignificant mummur

  ; severe AS, ASD, anomalous pul.venous return, A-V septal defects, COA

# loud mummur in absence structual anomaly

  ; large noncardiac arteriovenous malformation, myocarditis, severe anemia, hypertension

# ¢¾ Innocent(functional,normal,insignificant) murmur

    1) over 30% of children during routine random ausculation

    2) increase percentage under nonbasal circumstances

        ; high cardiac output due to fever, infection, anxiety

    3) *¡ãcommon innocent murmur

           ; *still murmur

           ; from 3 to 7yr of age

        ; *medium-pitched,vibratory, or "musical" relatively short systolic ejection murmur along left lower and midsternal border

           ; *attenuated in sitting or prone position

    4) innocent pulmonic murmurs

       - common in children,adolescents

       - normal turbulence during ejection into the pulmonary artery

       - high pitched, blowing, brief, early systolic murmurs(grade 1-2)

       - best detected in 2nd left parasternal space in supine position

    5) *venous hum

           - *turbulence of blood in the jugular venous system

       - no pathologic significance

       - heared in neck or ant.part of upper chest

       - soft huming sound in both systole and diastole

       - head position¿¡ µû¶ó ¾ÇÈ­µÇ°Å³ª »ç¶óÁü

       - *jugular venous systemÀ» °¡º±°Ô ¾Ð¹ÚÇÔÀ¸·Î¼­ ¾àÈ­µÊ

               /organic cardiovascular ds¿¡ ÀÇÇØ ¹ß»ýÇÏ´Â murmur¿Í ±¸ºÐ ÇÒ ¼öÀÖ´Ù.

Chap. 381. Laboratory Evaluation

381.1 Radiologic Assessment

# ÈäºÎ»çÁø¿¡¼­ ¾Ë ¼ö ÀÖ´Â »çÇ×

 ; cardiac size and shape, pul.blood flow(vascularity), pul.edema, lung and thorax anomaly(skeletal dysplasia,extra or deficient numbers of ribs)

# ÈäºÎ»çÁø¿¡¼­ variationÀÌ »ý±â´Â ¿øÀÎ

  1) difference in body build

  2) phase of respiration or cardiac cycle

  3) abnormality of thoracic cage

  4) position of diaphragm

  5) pul.ds

# cardiac size ÃøÁ¤¿¡ °¡Àå ÈçÈ÷ »ç¿ëÇÏ´Â ¹æ¹ý

 ; PA»çÁø¿¡¼­ midinsperation¶§ cardiac shadowÀÇ maximal width

# maximal cardiac width

 ; sternumÀÇ Áß°£À» ¿¬°áÇÏ´Â ¼öÁ÷¼±À» ±ß°í, ÀÌ ¼öÁ÷¼±¿¡¼­ Á÷°¢À¸·Î ½ÉÀåÀÇ extreme

   right and left border·Î ¼±À» ±×¾î ±× µÎ ¼±ÀÇ ÇÕÀ¸·Î Ç¥½ÃÇÑ´Ù.

# maximal chest width

 ; right diaphragmÀÇ top level¿¡¼­ rib cageÀÇ right and lett inner border¸¦ ¿¬°áÇÑ

   horizontal line

# ¡ÚCardiac Enlargements

    ; maximal cardiac width°¡ maximal chest widthÀÇ 1/2ÀÌ»óÀÌ¸é ½ÉÀåÀº enlarged

    ; *evaluation in upright and inspiration

    ; *less useful index in infancy

           - ¢¾why ?

                   / horizontal position of heart

                           --> increase ratio more than 50%

                   / thymus overlap entire mediasternum & heart base

# Chest PA View

   Fig 381-1

    ; heartÀÇ left border¸¦ ±¸¼ºÇÏ´Â ¿ä¼Ò

       - aortic knob, main and left pul.arteries, left ventricle

    ; heartÀÇ right border¸¦ ±¸¼ºÇÏ´Â ¿ä¼Ò

        - superior vena cava, ascending aorta, right atrium

# ECG

  ; more sensitive and accurate index of ventricular hypertrophy

# pl. overcirculation

   1) left to right shunts

   2) stenosis or atresia of the outflow tract of the right ventricle or of the pul.valve

381.2  Electrocardiogram(ECG)

# Ãâ»ý½Ã¿¡´Â pulmonary vascular resistance¿Í systemic vascular resistance´Â °ÅÀÇ

  µ¿ÀÏÇÏ´Ù. Ãâ»ýÀÌÈÄ¿¡´Â systemic vascular resistance°¡ Áõ°¡ÇÏ°Ô µÈ´Ù.

# ECG´Â QRS and T wave morphology·Î¼­ anatomic and hemodynamic feature¸¦

  ¹¦»çÇÏ°Ô µÈ´Ù

# right precordial lead(V3R or V4R)

  - RVH Æò°¡¿¡ Áß¿ä

  - dominant R or S patternÀ» ¹Ý¿µÇÔ

# ¢¾»ýÈÄ Ã¹³¯ÀÇ ECG Ư¡

    1) RAD

  2) large R wave

  3) upright T wave in the right precordial leads(V3R or V4R and V1)

# »ýÈÄ 48½Ã°£³»¿¡ ´ëü·Î pulmonary resistance´Â °¨¼ÒµÇ°í right ventricular pressure     ´Â Á¤»ó¿¡ µµ´ÞÇÏ°Ô µÇ¾î right precordial T wave´Â negative°¡ µÈ´Ù. ¸¸ÀÏ 1ÁÖÀÏÀÌ ³Ñ¾î¼­µµ V3R and/or V1¿¡ upright T wave°¡ Áö¼ÓµÇ¸é ºñÁ¤»óÀÌ´Ù.

# newborn¿¡ ÀÖ¾î mean QRS axis;+110 to +180

# infancy¿¡ À־´Â ¿ì½É½ÇÀÌ »ó´ëÀûÀ¸·Î ¶Ñ²®±â ¶§¹®¿¡ ¼ö°³¿ù ȤÀº ¼ö³âµ¿¾ÈÀº right

   sided chest leads´Â larger positive (R) than negative (S) wave¸¦ ³ªÅ¸³»°Ô µÈ´Ù.

# ¼¼¿ùÀÌ È帣¸é QRS axis´Â ¿ÞÂÊÀ¸·Î À̵¿ÇÏ°Ô µÇ°í right ventricular forces´Â °¨¼Ò

   ÇÏ°Ô µÈ´Ù.

# 6°³¿ù¿¡¼­ 8¼¼±îÁö´Â lead V1 and V4R¿¡¼­ ÇöÀúÇÑ R wave¸¦ ³ªÅ¸³½´Ù.

  4¼¼±îÁö´Â lead V4R¿¡¼­ RS ratio´Â 1ÀÌ»óÀ» ³ªÅ¸³½´Ù

# infancy µ¿¾È¿¡´Â V4R,V1,V2,V3¿¡¼­ inverted T wave¸¦ ³ªÅ¸³»¸ç, À̰ÍÀº 10´ëÁß¹Ý

  ȤÀº ±× ÀÌÈıîÁö Áö¼ÓµÉ ¼ö ÀÖ´Ù.

# ¿ì½É½ÇÀÌ ¾ã¾ÆÁö°í Á½ɽÇÀÌ ¶Ñ²¨¿öÁö´Â °úÁ¤Àº right precordial leadsÀÇ QRS-T

   pattern¿¡¼­ Àß º¼ ¼ö ÀÖ´Ù.

# ventricular hypertrophyµÇ¸é chest leadsÀÇ R and S waveÀÇ voltage°¡ Áõ°¡ÇÏ°Ô µÈ´Ù

# physiologic right ventricular hypertrophy´Â Á¤»ó¼Ò°ßÀ̹ǷΠ»ýÈÄ ÀÏÁÖÀϱîÁö´Â

  pathologic  right ventricular hypertrophyÀÇ Áø´ÜÀº ¾î·Æ´Ù

# Pathologic RVH

    ; physiologic RVH in neonate

           --> serial tracingsÀÌ ÇÊ¿äÇÏ´Ù.

    ; adult ECG pattern in neonate

           --> left ventricular enlargement

           - ¡Úexception) premature infant - mature ECG pattern

                   / *result of lower pulmonary vscular resistance due to underdevelopment of medial muscular layer of pulmonary arterioles

¡Ø97¦Áþ±â P wave

1. tall (2.5mmÀÌ»ó), narrow, spiked P wave (P-pulmonale)

    ; *congenital pul. stenosis, Ebstein anomaly of the tricuspid valve, tricuspid atresia, cor pulmonale, thyrotoxicosis

  ; Right atrial hypertrophy and/or dilatation

  ; *obvious in lead II, V4R, V3R, V1

2. widened P wave(bifid) (P-mitrale)

  ; *large VSD with communication between the aorta and pul.circulation, severe MS

  ; Left atrial enlargement

3. flat P wave

  ; ¡Úhyperkalemia

# normal P wave

    ; upright in lead I, AVF

    ; inverted in AVR

# ¡Úinverted P wave in lead I, AVF

  ; atrial inversion(situs inversus) in lead I

  ; *nodal or junctional rhythm in lead I and AVF

QRS Complex

Right Ventricular Hypertrophy

# ¢¾RVH Criteria

    ; at least two of following

   - qR pattern in right ventricular surf. leads

   - positive T wave in leads V3-4R and V1-3 between 6days and 6yr

   - *monophasic R wave in V3-4R and/or V1

   - *rsR' in right precordial leads, often with a tall secondary R wave

   - age-related increased voltage criteria of R wave in V3-4R and/or of S wave in V6-7

   - marked RAD(>*120degrees)

   - *complete reversal of the normal adult precordial RS pattern

   - RAE

2) Right vent.systolic overload pattern

   - PS¿¡¼­ º¼ ¼ö ÀÖ´Ù.PS¿¡¼­ rsR' in right precordial lead º¸ÀÓ

   - right precordial lead¿¡¼­ tall pure  R waveº¸À̸ç óÀ½¿¡´Â upright T wave ³ª

     Áß¿¡´Â inverted T waveº¸ÀÓ

3) Right vent.diastolic overload pattern

   - ASD¿¡¼­ º¼ ¼ö ÀÖ´Ù

   - rsR' pattern and right ventricular conduction delay

Left Ventricular Hypertrophy

# ¢¾LVH Criteria

    ; S-T segment depression and T wave inversion in left precordial leads (V5-7)

         - left ventricular strain pattern

    - severe lesion and significant myocardial abnormality

    ; *increase in magnitude of initial forces to the right (i.e., deep Q in left precordial leads)

    ; voltage criteria in V3R & V1(S) and/or V6-7(R)

2) severe systolic overload of the left ventricles ¼Ò°ß

 a) straightening of the ST segment

 b) inverted T wave over the left precordial leads

3) diastolic overload ¼Ò°ß

 a) tall R wave,large Q wave,normal T waves over the left precordium

Q-T Interval

1) cardiac rate¿¡ µû¶ó º¯È­

2) normal Q-TC < 0.45 sec

# ¢¾Prolonged Q-Tc Interval

  ; hypokalemia

  ; hypocalcemia

    ; *Jervell-Lange-Nielsen syndrome or Romano-Ward syndrome

        - high risk of ventricular arrhythmia such as torsade de pointes

        - sudden death

           - *mutation in Harvey ras-1 gene, encoding G protein

ST Segment And T Wave Abnormalities

1) normal teenengers¿¡¼­ ST segment elevationÀº ½ÉÀåÀÇ repolarizationÀ» À¯¹ß

2) pericarditis¿¡¼­ superficial epicardial involvement´Â abnormal T wave inversionÈÄ¿¡

   ST segment elevationÀ» À¯¹ßÇÑ´Ù

3) Digitalis Åõ¿© È¿°ú

  - sagging of ST segment,abnormal T wave inversion

4) Depression of ST segment

  - muocardial damage¸¦ À¯¹ßÇÏ´Â »óȲ¿¡¼­ ¹ß»ý

  - anemia, CO poisoning, endocardial fibroelastosis, aberrant origin of the left 

    coronary a. from the pul.a., glycogen storage disease of the heart, myocardial

    tumors, mucopolysaccharidoses.

5) carditis¿¡¼­´Â T wave inversionÀ» º¸ÀÓ

6) hypothyroidism

   - flat or inverted T waves,generalized low voltage

7) hyperkalemia(Fig.381-14)

   - high voltage,tent shaped T waves

381.3 Hematologic Data

# acyanotic infants with large Lt-to-Rt shunts

    ; CHF coincides with nadir of normal physiologic anemia of infancy

           --> Tx : increasing Hct > 40%

# Persistent Polycythemia

    ; *¡ãfrequent abnormalities

       - accelerated fibrinolysis

       - thrombocytopenia

       - abnormal clot retraction

       - hypofibrinogenemia

       - prolonged PT,PTT

    ; Complication

           - *vascular thrombosis esp. cerebral veins

           - ¢¾Predisposings Factors

                   / dehydration

                   / IDA

    ; Treatment

           - *epsilon-aminocaproic acid

                   / suppress fibrinolysis

           - correction of predisposing factors of CVA

           - phlebotomy

# Hematologic F/U of cyanotic patients

    ; increasing polycythemia, asso. with headache, fatigue and/or dyspnea

           --> indication of palliative or corrective surgical intervention

    ; *phlebotomy, if Hct 65-70%   

           - replacement of fresh frozen plasma or albumin

           - desired level : 60%

           - a week until desired level and then at interval of only 3-5wks

381.4 Echocardiography

# ¡Ø82,87 ±â´É

  1) cardiac contractility(performance)

  2) gradients across stenotic valves

  3) direction of flow across a shunt

  4) patency of coronary arteries

  5) prescence of vegetations due to endocarditis

  6) prescence of pericardial fluid,cardiac tumors,chamber thrombi

  7) prosthetic valve function

  8) septal hypertrophy

  9) aortic root dimensions

  10) affects of cardiotonic or cardiotoxic drugs

  11) assist in performance of pericardiocentesis

M Mode Echocardiography

; ¡ÚUse

    - *identifies dimensions & motion of intracardiac structure

           /opening, closing of valves, movement of septa

    - anatomy of valves

    - *presence of endocarditis vegetations larger than 2-3mm

    - presence or absence of individual structure and their relationships

    - cardiac function 

Two-Dimensional Real-Time Imaging

; better,more coherent,realistic image of cardiac structures

; technique of choice for diagnosing structural heart disease

; superior to angiography in several areas

    - *imaging AV valves and their chordal attachments

Doppler Echocardiography

; ¡ÚUse

    - identifies blood flow than morphology

    - *estimate systemic or pulmonary blood flow, pressure

; color doppler

    - *presence & direction of intracardiac shunt

Transesophageal Echocardiography

; ¡ÚUse

    - *clearer view of smaller lesion such as vegetation in endocarditis

    - *visualize posteriorly located structures

           / atria, aortic root, atrioventricular valves

    - intraoperative monitoring of cardiac function

    - intraoperative screening for residual cardiac defects after cardiopulmonary bypass

Fetal Echocardiography

Overview

; *ASD or PDAµîÀº cardiac cath.¸¦ ÇÏÁö ¾Ê°í 2-D and doppler echo.¸¸À¸·Îµµ ¼ö¼ú

381.5 Exercise Testing

# ±â´É

  1) evaluating symptoms

  2) quantitating the severity of cardiac abnormality

  3) assisting in the management of patients

# Bruce protocol·Î ½ÃÇà

# exercise¿¡ ´ëÇÑ ÁÖ ¹ÝÀÀÀº heart rate,stroke volume,systemic venous return,pul.pr.ÀÇ

  Áõ°¡·Î ÀÎÇØ cardiac outputÀÌ Áõ°¡Çϸç, ¶ÇÇÑ systemic vascular resistance°¡ °¨¼Ò

  ÇÑ´Ù

# Á¤»ó¾Æ¿¡ ÀÖ¾î exerciseµ¿¾È¿¡ ECGÀÇ º¯È­´Â P-R intervalÀÇ °¨¼Ò(½É¹Ú¼ö Áõ°¡)ÀÌ´Ù

# ¡ØAbnormal Exercise ECG

    ; *ST segment depression > 2mm & extends for at least 0.06sec after J point in conjunction with a horizontal-, upward- or downward-sloping ST segment

# ¡Ø87 Indication

    1) LV outflow obstruction

    - valvular,subvalvular,supravalvular aortic stenosis

    - hypertrophic cardiomyopathy

    - CoA

    2) chronic vol. overload of the left or right ventricle

       - atrioventricular or semilunar valve incompetence

       - left to right shunts

  3) arrhythmia

  4) hypertension

    5) patients who have undergone open heart surgical correction of complex congenital heart lesions

           - fontan op.

# ¢ÀIndication For Termination Of A Exercise Test

  1) failure or inadequacy of the ECG monitoring

  2) onset of serious arrythmia

  3) premature beats(more than 25% of beats) precipitated or aggrevated by exercise

  4) development of heart block

  5) precipitation of pain,headache,dizziness,syncope

  6) ST segmental depression or elevation of 3mm or more

  7) inappropriate hypertension

    - syst. pr. > 230mmHg

       - dias. pr. > 120mmHg

  8) inappropriate fall of BP

  9) development of cutaneous vascular insufficiency(e.g.,pallor)

  10) severe fatigue

381.6 Magnetic esonance Imaging(MRI) and Radionuclide Studies

# gay-scale intensity¿¡ °ü°èÇÏ´Â ¿ä¼Ò

  ; concentration,motion,and chemical microenvironment of hydrogen nuclei

# excellent contrast resolution of fat,myocardium,lung,moving blood from blood vessel

  wallÀ» ¾òÀ» ¼ö°¡ ÀÖ´Ù.

# Áø´ÜÀû °¡Ä¡°¡ ÀÖ´Â Áúȯ

  1) malformation of great vessels

   - COA, proximal brench pul.a.stenosis, TGA

  2) simple and complex cardiac malformations

   -AS, PS, ASD, VSD, TOF, single ventricle,inversion of the ventricles

# Á¾·ù

  1) cine MRI

   - wall thickening,chamber volume,valve functionÀÇ º¯È­¸¦ ³ªÅ¸³¿

  2) phosphorous MR spectroscopy

   - high-energy metabolites(ATP,ADP,Pi,phosphocreatine)ÀÇ »ó´ëÀû ³óµµ¸¦ ¹¦»çÇÔ

     À¸·Î½á ¿µ»óÀ» ¾ò°ÔµÈ´Ù

# Radionuclide angiography

  ; detect and quantify shunts

  ; analyze distribution of blood flow to each lung

# ¡ÚGated Blood Pool Scanning

  ; calculate hemodynamic measurements

  ; quantify valvular regurgitation

  ; detects regional wall motion abnormalities

# ¡ÚThalium Imaging

  ; cardiac muscle perfusion

381.7 Cardiac Catheterization

# ¢¾Major Indication

    1) presurgical evaluation of cardiac anatomy and shunt size

    2) evaluation of pulmonary vascular resistance and its reactivity to vasodilators or oxygen

    3) F/U after surgical repair or palliation of complex CHD

    4) myocardial biopsy for diagnosis of cardiomyopathy or screening for cardiac rejection after trnsplantation

    5) interventional cardiac catherization

    6) electrophysiologic study and/or transcatheter ablation

# *avoidance of deep anesthesia

    ; distort calculation of hemodynamic measurements

           - cardiac output, pul. and systemic resistance, shunt ratio

# postangiographic care

    ; thermally neutral environment, correction of hypothermia, acidemia, excess blood loss

# ¢¾Complication

    1) severe arrhythmia

    2) cardiac perforation

    3) intramyocardial injection of contrast material

           ; soft, flow-directed ballon-tipped cathetersÀÇ °³¹ß·Î ºóµµ°¡ °¨¼ÒµÊ

    4) anaphylaxis

           ; nausea, generalized burning sensation, CNS symptoms, allergic rashs

Indicator Dilution and Appearance Technique

# indicator meterialÀ» heartÀÇ right side ȤÀº I.V·Î ÁÖÀÔÇϸé pul.circulationÀ» °ÅÃÄheart left side·Î µé¾î°¡ arterial circulationÀ» ÇÏ°Ô µÇ´Âµ¥ ÀÌ·¯ÇÑ indicator materialÀº arterial circulation¿¡¼­ detectµÈ´Ù

# A contious record of the circulation of indicator in normal subjects shows two peaks

    a. 1st peak´Â passage of indicator past the arterial detectors¿¡ ÀÇÇØ ¹ß»ý

  b. 2nd peak´Â recirculation through the systemic arterial and venous system,the pul. circulation, reappearance in the arterial tree¿¡ ÀÇÇØ ¹ß»ý

# thermodilution method

  ; cardiac outputÃøÁ¤À» À§ÇØ °¡Àå ÈçÈ÷ »ç¿ëÇÏ´Â indicator dilution technique

  ; dye dilution°ú °°ÀÌ »ç¿ëÇÏ°Ô µÇ¸é diseased mitral or aortic valves¸¦ °¡·ÎÁö¸£´Â regurgitant volumeÀ» ÃøÁ¤ÇÒ ¼ö ÀÖ´Ù

¡ÚTable 381-2

Fig. 381-23

Fig. 381-24

Angiocardiography

1) intramyocardial injectionÀº ÇÇÇÑ´Ù

2) hypertonic contrast medium ºÎÀÛ¿ë

    ; tansient myocardial depression, drop in BP, tachycardia, cadiac outputÁõ°¡, shift of interstitial fluid into the circulation

3) idealized diagrams of the normal angiocardiogram

Interventional Catheterization

# balloon angioplasty·Î Ä¡·á°¡ °¡´ÉÇÑ Áúȯ

    ; valvular PS, AS, restenotic of CoA after early surgery, amelioration of MS of subaortic stenosis, dilatation of surgical conduits(atrial baffles), relief of branch pul. a. narrowing, dilatation of venous obstruction, long utilized balloon atrial septostomy for TGA, obliteration of temporary A-V shunts as well as pul. collateral vessels, PDA, secundum ASD

section 2. the transitional circulation

Chap 382. Fetal And Neonatal Circulation

Fetal Circulation

# three CV sturcture for maintaining parallel circulation

    ; ductus venosus, foramen ovale, ductus arteriosus

# placental oxygenation ; *PO2 30-50mmHg

    --> umbilical vein --> IVC via ductus venosus (*PO2 26-28mmHg) --> RA

    --> hepatic circulation (*50%)

    --> RA

    --> foramen ovale

    --> LA

    --> LV

    --> ascending aorta

# fetal SVC blood ; *PO2 12-14mmHg

    --> RA

    --> RV

    --> pulmonrary artery --> pulmonary circulation (*10%)

    --> ducturs arteriosus : PO2 18-22mmHg

    --> descending arota --> umbilical artery (*65%) --> placenta

    --> fetal organ

4) Rt ventricular outputÀº Lt ventricular output ÀÇ 1.3¹è.

   Total cardiac output(combined both ventricular output) : 450ml/kg/min 

5) effective fetal cardiac output

   ; sum of left ventricular output and the ductal flow

   ; 220mL/kg/min

   ; 65%´Â placenta·Î returnÇϰí, ³ª¸ÓÁö 35%´Â fetal organ and tissues·Î perfusionµÈ´Ù.

6) left ventricular output ±¸¼º¿ä¼Ò

   ; mixture of venous return from IVC,foramen ovale,left atrium,minimal pul.venous          return

7) fetal life µ¿¾È¿¡´Â right ventricleÀÌ dominantÇϱ⠶§¹®¿¡ right ventricle outputÀÌ left

   ventricular outputº¸´Ù 50%Á¤µµ Å©´Ù.

Transitional Circulation

Neonatal Circulation

# ¢¾Neonatal Circulation°ú Older Infancy¿ÍÀÇ Â÷ÀÌÁ¡

    ; Rt-to-Lt or Lt-to-Rt shunting across foramen ovale

    ; in presence of cardiopulmonary disease, Lt-to-Rt, Rt-to-Lt or bidirectional shunting across ductus arteriosus

    ; neonate pul. vasculature - more vigorouly constriction in response to hypoxemia, hypercapnea, acidosis

    ; almost equal of Rt. and Lt.ventricle muscular mass & wall thickness

    ; high oxygen consumption asso. with high cardiac output

           - *350ml/kg/min

           - cf.) 150 ml/kg/min by 2mo, gradually 75 ml/kg/min of adult level

# ¡ÚFunctional Closure

  ; foramen ovale

           - 3rd month

  ; ductus arteriosus

           - 10-15hrs

# ductus arteriosus closure¿¡ ¿µÇâÀ» ¹ÌÄ¡´Â ÀÎÀÚ

    ; ¡ÚOxygen

           - ¡ãimportant

    - *PO2 > 50mmHg

           - direct or mediated by its effect on PG synthesis

    ; gestational age

           - less response to oxygen

Chapter 383. Persistence Of Fetal Circulatory Pathways

# pul.a.pr.= pul.blood flow # pul.vascular resistance (P=F#R)

Persistent Fetal Circulation

(=Persistent Pulominary Hypertension Of The Newborn)

 ; hypoxemiaÈÄ¿¡ pul.vasoconstriction and hypertensionÀÌ ¿À¸é Rt. to Lt. patent foramen

   ovale and ductus arteriosus shuntingÀÌ ¹ß»ý

Pul.Venous Hypertension

À» À¯¹ßÇÏ´Â Áúȯ

  (»ýÈÄ Ã¹ ¼öÀϳ»¿¡ pul.venous obstructionÀ» À¯¹ßÇÒ ¼ö ÀÖ´Â Áúȯ)

 ; stenosis of pul.veins,cor triatriatum,congenital MS,supravalvular webs.

Pul. A. Hypertension

À» À¯¹ßÇÏ´Â Áúȯ

 ; Lt.ventricular failure,COA,aortic valve disease,cardiomyopathy

Hyperviscosity Syndrome

 ; maternal-fetal or fetal-fetal transfusion ȤÀº perinatal hypoxemia¿¡ ÀÇÇÑ polycythemia

  °¡Áø ȯ¾Æ¿¡¼­ Àß ¹ß»ý

Persistence Of Fetal Circulation

 ; pul.vascular constrictionÀÌ ÀÖÀ¸¸é¼­ parenchymal pul.disease or cardiac lesionÀÌ ¾ø´Â      °æ¿ì

# decreased pul.vascular bed

 1) elevated pul.resistance and persistent pul.hypertension of newbornÀ» À¯¹ß   

 2) À¯¹ßÇÒ ¼ö ÀÖ´Â °æ¿ì

  - congenital pul.hypoplasia

  - diaphragmatic hernia

  - space occupying intrathoracic masses

# systemic Rt.ventricles or single ventriclesÀ» °¡Áø ȯ¾Æ´Â pul.hypertension°ú medial

   muscular hypertrophy of small pul.vesselsÀ» À¯¹ßÇÏ°Ô µÈ´Ù.

# anatomic and physiologic abnormalitiesÀÌ µ¿¹ÝµÈ perinatal hypoxemia´Â º¹ÇÕÀû ¿øÀÎÀÇ    persistent pul.hypertensionÀ» À¯¹ßÇÏ°Ô µÈ´Ù. ¿¹¸¦ µé¾î diaphragmatic hernia¸¦ °¡Áø ȯ    ¾Æ´Â ipsilateral pul.hypoplasia and contralateral pul.vasoconstrictionÀ» Áö´Ï°Ô µÇ´Âµ¥ ÀÌ     µÎ °¡Áö´Â  high pul.resistance,hypertension,Rt.to Lt.shuntingÀ» À¯¹ßÇÏ°Ô µÈ´Ù.

# severe RDS¸¦ °¡Áø ¹Ì¼÷¾Æ°¡ cyanoticÇÑ ÀÌÀ¯

  1) pul.vasoconstriction

  2) pul.hypertension

  3) Rt.to Lt.ductus arteriosus

  4) foramen ovale shunting in the first few days of life

section 3. CONGENITAL HEART DISEASE

Chapter 384. Epidemiology Of Congenital Heart Disease

 

Incidence

    ; 8/1000 live births

    ; stillborns(2%), abortuses(10-25%), premature infants (about 2% including VSD, excluding tansient PDA)¿¡¼­ ³ô´Ù.

3) 1000¸í´ç 2-3¸íÀº 1³â³»¿¡ Áõ»óÀ» ³ªÅ¸³½´Ù.

4) Áø´ÜÀº 1ÁÖÀϰ µÇ´Â °æ¿ì°¡ 40-50%, 1´Þ   µÇ´Â °æ¿ì°¡ 50-60%ÀÌ´Ù.

5) pul.vascular resistance´Â »ýÈÄ 1ÁÖÀÏ   °¨¼ÒÇϰí,Lt.to Rt.shunts´Â º¸´Ù ÇöÀúÇØÁø´Ù. ¿©     ·¯°¡Áö defects´Â ¼ºÀå¿¡ µû¶ó º¯È­°¡ »ý±â´Âµ¥, ¿¹¸¦  µé¾î large VSD´Â ¼ºÀå¿¡ µû¶ó      small communicationÀÌ µÇ°í, aortic or pul.valve stenosis´Â ¼ºÀå¿¡ µû¶ó valve orifice°¡     Ä¿ÁöÁö ¾Æ´ÏÇÏ¸é ¿ÀÈ÷·Á ´õ ¾ÇÈ­µÈ´Ù.

Etiology

1) genetic factors

  ; supracristal VSD´Â µ¿¾çÀο¡ ¸¹´Ù

2) single gene defect

  ; 3%

  ; Marfan or Noonan syndrome

3) chromosome anomalies

  ; 5-8%

4) environmental or adverse maternal conditions and teratogenic influences

  ; 2-4%

    ; maternal DM, phenylketonuria, SLE, congenital rubella syndrome, drugs (lithium, ethanol, thalidomide, anticonvulsant agents)

5) ¡ÚPolygenetic Or Multifactorial inheritance

  ; mostly

¢ÞÇ¥ 15-3, 15-4, 15-5 (p540-541)

Genetic Counseling

Chapter 385. Evaluation Of The Infnat Or Child With Congenital Heart Disease

Acynotic Congenital Heart Lesions

- most common lesion : volume overload¿¡ ÀÇÇÑ °Í, ÁַΠ Lt to Rt shunt lesion

- second most common lesion : pressure load¿¡ ÀÇÇÑ °Í,

   1) PS, aortic valve steenosis ( ventricular outflowobstruction¿¡ ÀÇÇÑ °Í )  

   2) coactation of aorta ( great vesselÀÇ Çѱºµ¥°¡ Á¼¾ÆÁø °Í¿¡ ÀÇÇÑ °Í)

Lesions Resulting In Increased Volume Overload

- m/c : Lt to Rt shunt¸¦ À¯¹ßÇÏ´Â º´º¯ --> ASD, VSD, AVSD, PDA

Lesions Resulting In Increased Pressure Overload

- m/c : obstruction to ventricular outflow --> valvar pulmonary stenosis, valvar aortic stenosis, CoA

- less common lesion --> tricuspid stenosis, mitral stenosis, cor triatrium

- ventricular outflow obstructionÀÇ level¿¡ µû¶ó

  ¨ç below the valve : double-chambered Rt ventricle, subaortic menbane

  ¨è above the valve : branch pulmonary stenosis, supravalvular aotic stenosis       

Cyanotic Congenital Heart Lesions

Cyanotic Lesions With Decreased Pulmonary Blood Flow

- obstruction to pulmonary blood flow(at the tricuspid valve, Rt. ventricle, pulmonary       valve level) : tricuspid atresia, single ventricle with pulmonary stenosis, TOF

- Rt to Lt shunt lesions : PFO, ASD, VSD

Cyanotic Lesions With Increased Pulmonary Blood Flow

- pulmonary blood flow obstructionÀº ¾øÀÌ

  ¨ç ºñÁ¤»óÀûÀÎ ventricular-arterial connectionÀÌ ÀÖ´Â °æ¿ì : TGA

  ¨è heart³»¿¡¼­ systemic venous, pulmonary venous bloodÀÇ systemic venous,

     pulmonary  venous bloodÀÇ total mixingÀ¸·Î ÀÎÇØ cyanosis°¡ ÀϾ´Â °Í

     : cadiac defect with a common atria or ventricle

       TAPVR, truncus arteriosus

Chapter 386. Acynotic Congenital Heart Disease

THE LEFT-TO-RIGHT SHUNT LESIONS

386.1 ASD

; occur in any portion of atrial septum

    - secundum, primum, sinus venosus

386.2 Ostium Secundum Defect

# ³²:¿©=1:3

# associated lesion

    ; *partial anomalous venous return

Pathophysiology

# ¡Ø81¢¾Lt-to-Rt shunt °áÁ¤ ¿ä¼Ò

    ; size of the defect

    ; relative compliances of the Rt. and Lt. ventricles

    ; relative vascular resistances in the pulmonary and systemic circulations

# large defects

    ; pulmonary blood flow 2-4 times systemic blood flow

    ; in infancy, paucity of symptom

           - why ?

                   / *RV is thick and less compliant

  <5> infant°¡ ¼ºÀåÇÒ¼ö·Ï right ventricular wallÀÌ ¾ã¾ÆÁ® left to right shunt°¡ Áõ°¡ÇÑ´Ù

  <6> large pul.blood flow¿¡µµ ºÒ±¸Çϰí pul.a.pr.´Â normal·Î À¯ÁöÇÑ´Ù. ¿Ö³ÄÇϸé pul.vascular

      resistance°¡ ±ØÈ÷ ³·±â ¶§¹®ÀÌ´Ù

  <7> LV and aorta;normal size

  <8> cyanosis;±ØÈ÷ µå¹°°í pul.vascular disease°¡ µ¿¹ÝµÈ ¼ºÀο¡¼­ º¼ ¼ö ÀÖ´Ù.

Clinical Manifestations

; *most often aymptomatic

    - discovered inadvertently during P/E

; normal pulse

; right ventricular systolic lift

    - palpable from left sternal border to midclavicular line

# ¡ÊûÁø¼Ò°ß

    ; loud 1st heart sound & sometimes pulmonary ejection click

    ; ¢Þwidely fixed splitting 2nd heart sound

    ; ejection type systolic murmur

        - medium pitched, without harsh qualities, seldom thill

           - best heard in left mid. and upper sternal border

           - *produced by increased blood flow across RVOT

    ; short rumbling mid-diastolic murmur

           - *produced by increased volume of blood flow across TV

           - audible at lower left sternal border

           - best with bell of stethoscope

           - *excellent diagnostic sign of shunt ratio of at least 2:1

Diagnosis

# X-ray

  ; Shunt ¾ç¿¡ µû¶ó RA, RVÀÇ enlargement°¡ ´Ù¾çÇÏ´Ù.

  ; LV and aorta ; normal size

  ; *large pulmonary artery & increased pulmonary vascularity

  ; RV cardiomegaly

           - best seen on lat.view

       - due to ant. protrusion of RV enlargement

# EKG

  1) volume overload of RV with RAD or normal axis

  2) minor right ventricular conduction delay (rsR' in right precordial leads)

# ¡ÚEchcardiogram

    ; right ventricular volume overload

           - increased right ventricular end-diastolic dimension

           - *paradoxical septal motion

# catheterization

    ; O2 content

           - *RA >SVC

           - not diagnostic

       - ¡ÚDDx

                   / partial anomalous pul.venous return to RA

             / VSD and tricuspid insufficiency

             / atrioventricular septal defect associated with left ventricular-right atrial shunts

             / aortic-right atrial communication (eg.ruptured sinus of Valsalva)

    ; indicator dilution curves

       - left to right shuntÀÇ À§Ä¡¿Í anomalous pul.v.ÀÇ Á¸À縦 ¹àÈù´Ù.

 3) right sided heart pr.;normal

 4) pul.a.resistance;normal or lower than normal

 5) shunt volume ; as high as 20 l/min/m2

Prognosis And Complications

<1> childhood¶§¿¡´Â well tolerable

<2> Áõ»óÀº 3rd decade ȤÀº ±× ÀÌÈÄ¿¡ ³ªÅ¸³­´Ù.

<3> late manifestation

     1) pul.hypertension

     2) atrial dysrhythmias

     3) tricuspid or mitral incompetence

     4) heart failure

<4> ÀӽŠÁß¿¡µµ Áõ»óÀÌ ³ªÅ¸³¯ ¼ö ÀÖ´Ù.

<5> infectious endocarditis;extremely rare

<6> 20¼¼ ÀÌÈÄ¿¡ ¼ö¼úÇß¾ú´ø ȯÀÚ¿¡¼­ post op. Cx.À¸·Î¼­ late heart failure¿Í atrial fibrillation

     ÀÌ more common

<7> µ¿¹ÝµÇ´Â Áúȯ

     1) partial anomalous pul.venous return

     2) pul.valvular stenosis

     3) VSD

     4) pul. a. branch stenosis

     5) persistent left SVC

     6) mitral valve prolapse and insufficiency

Treatment 

# surgical operation

    ; *symptomatic or shunt ratio > 2:1

    ; op. mortality < 1%

    ; prior to entry into school

 <3> pregnancy½Ã riskÁõ°¡

 <4> early surgical repair·Î¼­ ¿îµ¿½Ã¿¡ ¹ß»ýÇÏ´Â mild symptomÀ» ¿¹¹æÇÒ ¼ö ÀÖ´Ù.

 <5> large shunt¶óµµ ¼ö¼úÈÄ °á°ú´Â ÁÁ´Ù.

 <6> ¼ö¼úÈÄ °á°ú

      1) Áõ»óÀÌ ½Å¼ÓÈ÷ »ç¶óÁü

      2) physical development°¡ ÁÁ¾ÆÁü

      3) heart size´Â Á¤»óÀûÀ¸·Î °¨¼ÒµÊ

      4) EKG;decreased right ventricular forces

 <7> late arrhythmias;less frequent and less important

386.3 Sinus Venosus Defect

  # SVC ÀÔ±¸¿¡ ±ÙÁ¢ÇÏ¿© atrial septumÀÇ upper part¿¡ À§Ä¡ÇÑ´Ù

  # 1°³ ÀÌ»óÀÇ pul.v.(usually from right lung) SVC·Î drainµÈ´Ù

  # ¶§¶§·Î SVC´Â defect¿¡ °ÉÃļ­ À§Ä¡ÇÏ¿© systemic venous blood´Â LA·Î µé¾î°¡°Ô µÈ´Ù.

  # abnormal hemodynamics(RVÀÇ volume overload)´Â secundum ASD¿Í À¯»ç

  # DX. ; two-dimensional echocardiography

  # catheter´Â SVC¿¡¼­ pul.v.À¸·Î À¯ÀÔ

  # Áõ»ó,EKG,X-ray´Â secundum ASD¿Í À¯»ç

386.4 Partial Anomalous Pulmonary Venous Return

# more often right lung origin pulmonary vein

# associated ASD

    ; *sinus venosus type

# ¡ÚScimtar syndrome

    ; *anomalous pul. v. draining into IVC

           --> crescentric shadow of vascular density along the right border of cardiac silhouette

    ; usually not present ASD

# prognosis ; excellent

386.5  Atrioventricular Septal Defect (Ostium Primum And Av Canal Or  Endocardial Cushion Defects)

# Classification

    1) ostium primum defect

        ; lower portion of atrial septum & overlies MV and TV

           ; *mostly MV ant. leaflet cleft

    ; TV - functionally normal

       ; intact ventricular septum

    2) A-V septal defect (=AV canal defect, endocardial cushion defect)

       ; contiguous atrial and ventricular septal defect with markedly abnormal A-V valves

       ; variable valve abnormalities

            - *complete form : single AV valve

                           / common to both ventricle

                           / *ant. post. bridging leaflet with lat. leaflet

                     / *common in Down syndrome

                           / *occasionally with PS

    3) Transitional Varieties

           ; Á¾·ù

    - ostium primum defect with clefts in the ant. mitral and septal tricuspid valve leaflets, mild ventricular septal deficiencies

        - ostium primum defects with normal A-V valves

           - atrial septum ; intact

Pathophysiology

# ostium primum defect

    1) left to right shunt across the atrial defect with mitral incompetence

    2) shunt;moderate to large

    3) degree of mitral insufficiency; mild to moderate

    4) pul.a.pr.;normal or only mildly increased

# A-V canal

    1) left to right shunt

      ; both transatrial and transventricular

    2) pul.hypertension and increased pul.vascular resistance

    3) A-V valvular,incompetence·Î ÀÎÇØ ventricle¿¡¼­ both atriumÀ¸·Î blood°¡ regurgitated

    4) ¶§·Î both atrial and ventricular level¿¡¼­ right to left shunt°¡ ¹ß»ý

    5) mild,significant arterial unsaturation

    6) progressive pul.vascular disease´Â right to left shunt¸¦ Áõ°¡½ÃÄÑ cyanosis¸¦ À¯¹ßÇÑ´Ù

Clinical Manifestations

# ostium primum defect

    1) ´ë°³´Â asymptomaticÇϰí anomaly´Â P/E¶§ ¹ß°ßµÈ´Ù

    2) moderate shunt and trivial mitral incompetence°¡ ÀÖÀ¸¸é physical signÀº secundum

       type°ú À¯»ç

    3) apical systolic murmur

    4) large left to right shunts and severe mitral incompetence°¡ ÀÖ´Â °æ¿ì

      (1) effort intolerance, easy fatigability, recurrent pneumonitis ³ªÅ¸³²

      (2) cardiomegaly

      (3) hyperdynamic precordium

    5) left to right shunt¿¡ ÀÇÇÑ Ã»Áø¼Ò°ß

      (1) normal or accentuated S1

      (2) wide,fixed splitting S2

      (3) pul.ejection systolic murmur

      (4) low-pitched early diastolic murmur at LLSB or apex

    6) mitral incompetence°¡ ÀÖÀ¸¸é left axilla·Î radiationÇÏ´Â apical pansystolic murmur ¹ß»ý

# A-V canal

    ; CHF, intermittent pul. infection

    ; minimal cyanosis

  ; epatomegaly, prominent neck vein, failure to thrive, cardiomegaly, palpable systolic thrill

    ; ûÁø¼Ò°ß

      (1) S1 ; normal or accentuated

      (2) S2 ; pul.flow°¡ massiveÇϸé widely split

      (3) low pitched mid diastolic rumbling murmur(lower sternal edge)

      (4) pul.systolic ejection murmur; large pul.flow¿¡ ÀÇÇØ ¹ß»ý

      (5) mitral insufficiency½Ã apical holosystolic murmur¹ß»ý

Diagnosis

# X-ray

    1) cardiomegaly;both ventricles and RAÀÇ prominence¿¡ ÀÇÇØ ¹ß»ý

    2) pul.a.;large

    3) pul.vascularity;Áõ°¡

# ¡ØEKG

    1) sup. orientation of the mean frontal QRS axis with LAD to the left or right upper quadrant

           ; *¡ãimportant

    2) counter-clockwise inscription of the superiorly oriented QRS vector loop

  3) signs of biventricular hypertrophy or isolated RVH

    4) *right ventricular conduction delay (RSR¡¯ in leads V3R and V1)

    5) normal or tall P waves

  6) occasional prolongation of the P-R interval

# echocardiogram

    1) RVH

    2) encroachment of the MV echo on the left ventricular outflow

# catheterization and angiocardiography

    ; selective left ventriculography

        1) deformity of mitral or common A-V valve

    2) distortion of the outflow of the left ventricle

    3) *goose-neck deformity

    4) MVÀÇ abnormal ant.leaflet;serrated

    5) mitral incompetence

    6) regurgitation of blood to both the left and right atrium

Prognosis

# ¡Ú°áÁ¤ ¿ä¼Ò

    1) magnitude of left and right shunt

    2) degree of pul.vascular resistance

    3) severity of mitral incompetence

# infancy¶§ op.ÇÏÁö ¾ÊÀ¸¸é CHF·Î Á¾Á¾ »ç¸ÁÇÔ

# op.ÇÏÁö ¾Ê°í »ýÁ¸Çϰí Àִ ȯÀÚ´Â pul.vascular obstructive disease°¡ Àß ¹ß»ý

# ´ë°³ ȯÀÚ´Â ¹«Áõ»óÀ̰ųª 3rd-4th decade°¡ µÉ ¶§±îÁö minor, nonprogressive symptomÀÌ ³ªÅ¸³­´Ù.

Treatment

<1> surgical mortality; low

<2> CHF and pul. hypertensionÀÌ ÀÖÀ¸¸é ¼ö¼úÀÌ º¸´Ù ¾î·Æ´Ù.

<3> Pul. a. bending

386.6  Ventricular Septal Defect

; *¡ãcommon cardiac malformation - 25%

# ´ë°³´Â membranous type

  µ¿¾çÀο¡¼­´Â supracristal type(30%)ÀÌ ÈçÇÏ´Ù

# defects between the crista supraventricularis and the papillary muscle of conus

  ; PS and TOF¿Í µ¿¹ÝµÊ

# defects superior to the crista supraventricularis

  <1> less common

  <2> pul.valve ¹Ù·Î ¹Ø¿¡ À§Ä¡

  <3> aorta sinus¿¡ ħ¹üÇÏ¿© aortic insufficiency À¯¹ß

# defects in the midposition or apical region of the ventricular septum or apical area

  <1> muscular type

  <2> single or multiple (Swiss-cheese type)

Pathophysiology

; small defect (restrictive)

    - *< 0.5 cm2

    - *pul. to systemic flow ratio < 1.75:1

    - normal cardiac chamber and pul.vascular bed

; large defect (nonrestrictive)

    - *> 1.0cm2

    - *flow ratio > 2.5:1

    - LV volume overload, RV and pul. a. hypertension

    - LA and LV enlargement & large pul.a.trunk

; Ãâ»ýÈÄ large VSD°¡ ÀÖ´Â °æ¿ì

  - óÀ½¿¡´Â normal infant¿¡ ºñÇØ pul.resistance°¡ ºñ±³Àû ³ô¾Æ left to right shunt°¡ limited

  - ¼öÁÖÀ̳»¿¡ pul.a.& arteriolesÀÇ muscular media°¡ normal involutionÇÔ¿¡ µû¶ó large left to right shunt and clinical symptomÀÌ ³ªÅ¸³²

<4> large VSDÀÖ´Â some patients¿¡¼­´Â medial thickness°¡ ½Ã°£ÀÌ È帧¿¡ µû¶ó intimal

     arteriolar pathologic change°¡ ¹ß»ýÇÏ¿© right to left shunt°¡ ¹ß»ý ( Eisenmenger

     syndrome ) ±×·¯³ª large VSD ÀÖ´Â ´ëºÎºÐÀÇ È¯ÀÚ¿¡¼­´Â massive left to right shunt°¡ ¹ß»ý

# Hemodynamics

  ; ¡Úleft to right shuntÀÇ Á¤µµ¸¦ °áÁ¤ÇÏ´Â ¿ä¼Ò

        - size of defect

    - systemic resistance¿Í ºñ±³ÇÏ¿© pul.vascular resistanceÀÇ Á¤µµ

  2> ´ë°³ pul.resistance´Â ¾à°£ Áõ°¡

  3> pul. hypertension À¯¹ß¿ä¼Ò

      ; pul.a. and heart right side¸¦ ÅëÇÑ extremely large blood flow

  4> small communicationÀÎ °æ¿ì

      1) defects;restictive

      2) RV ; normal

Clinical Menifestation

; varies according to defect size and pul.blood flow and pressure

# small defects with trival left to right shunts and normal pul. a. pr.

    ; *¡ãcommon

  ; asymptomatic

  ; ûÁø ¼Ò°ß

           - *loud, harsh, or blowing left parasternal holosystolic murmur

           - *frequently accompanied by thrill

    ; prematurity¿¡¼­ murmur°¡ »¡¸® µé¸®´Â ÀÌÀ¯

    - pul.vascular resistance°¡ more rapidly decrease

    ; X-ray

        - normal or minimal cardiomegaly

        - border increase in pul. vasculature

  ; EKG

     - normal

# Large Defects With Excessive Pul. Blood Flow And Pul. Hypertention

    ; dyspnea, feeding difficulty, poor growth, profuse perspiration, recurrent pul. infection, cardiac failure

    ; *cyanosis (-)

         - sometimes duskiness during infetion or crying

    ; *prominent left precordium and sternum --> cardiomegaly

    ; palpable parastenal lift, apical thrust, systolic thrill

    ; ûÁø ¼Ò°ß

           - holosystolic murmur

                   / less harsh more blowing

           - accentuated P2

           - *mid-diastolic low-pitched rumble at apex

                   / increased blood flow across MV

                   / *indicate Lt to Rt shunt 2:1 or greater

    ;  X-ray

       - gross cardiomegaly

       - prominence of both ventricles, LA, pul.a.

    ; EKG

        - *biventricular hypertrophy

       - P wave ; norched or peaked

Diagnosis

# two dementional echocardiogram

  ; volume overload of LA and LV

# catheterizaton

  ; RV°¡ RAº¸´Ù O2 content°¡ ³ô´Ù.

     2) small defect associated with

        ; normal Rt. sided heart pressure and pul. vascular resistance

     3) µ¿ÀÏÇÑ pul. and systemic pr. °¡Áø large VSD ȯ¾Æ¿¡¼­ pul. bl. flow´Â systemic blood

        flowº¸´Ù 3¹è ÀÌ»óÀÌ´Ù.

Prognosis And Complication

# Small VSD

    ; *30-50% spontaneous closure during 1st yr

           - *´ëºÎºÐ 4¼¼ÀÌÀü¿¡ closure

    ; asymptomatic without evidence of a increase in heart size, pul. artery pressure, resistance

    ; infective endocarditis

           - fewer than 2%

           - *rare in 2yr, more common in adolescent

           - *independent of VSD size

# Moderate to large VSD

    ; less common spontaneous closure

    ; *repeated episodes of respiratory infection & CHF

    ; pulmonary hypertension

           - result of high pulmonary blood flow

           - *risk of developing pul. vscular disease if not repaired

# Acquired infundibular stenosis

Treatment

# Small defects

# Large defects

    1) medical management

           ; *control of CHF, prevention of pul. vascular disease

    2) surgical management

           ; symptomatic infants

           ; *asso. with pul. hypertension

                   - *surgery electively 6 - 12mo or earlier if symptom

left to right shunt°¡ »ç¶óÁö°í ³ª¸é

 ¨ç quiet heart

 ¨è heart size´Â Á¤»óÀûÀ¸·Î °¨¼Ò

 ¨é thrills and murmurs »ç¶óÁü

 ¨ê pul. a. hypertension regress

 ¨ë ÀÓ»ó Áõ»óÀÇ ÇöÀúÇÑ °³¼±

¢ÞTable 386-1

386.7  VSD With Aortic Insufficiency

 # VSD ȯÀÚÀÇ 5%¿¡¼­ ¹ß»ýµÊ. µ¿¾çÀο¡°Ô¼­ ¸¹´Ù.

 # VSD ; small and moderate size ant. and subpulmonary ( outlet septum )

         ¶§¶§·Î infracristal

 # AI´Â 1st decade¸» ȤÀº ±× ÀÌÈÄ¿¡µµ Àß ¹ß°ßµÇÁö ¾Ê´Â´Ù.

 # early CHF´Â Àß ¹ß»ýÇÏÁö ¾ÊÀ¸³ª ¼ö¼úÇÏÁö ¾ÊÀ¸¸é severe AI and LV failure°¡ ¹ß»ýÇÑ´Ù

 # DDx

    ¨ç PDA

    ¨è other defects associated with aortic runoff

 # Clinical manifestations

    ¨ç asymptomatic child¿¡¼­ trivial AR and small left to right shunt

    ¨è symptomatic adolescent¿¡¼­ floride aortic incompetence and massive cardiomegaly

Treatment

    ; supracristal VSD(=subarterial juxta-arterial VSD)

           - *Áø´Ü ´ç½Ã ¼ö¼ú

386.8 PDA

; pul.vascular resistance°¡ °¨¼ÒÇÑ ÀÌÈÄ¿¡µµ Ãâ»ý ÈÄ ductus°¡ ´ÝÈ÷Áö ¾Ê´Â °æ¿ì,

   aortic blood°¡ pul. a.·Î shuntingµÊ

; congenital rubella syndrome¶§ È£¹ß

; ³²:¿© = 1:2

; ¡ÚTerm Infant

    - deficiency of mucoid endocardial layer and muscular media of ductus

           --> *no spontanous closure if persisting beyond 1wks

; premature infant

    - normal structural anatomy of ductus

           --> *mostly spontaneous closure if not early pharmacologic or surgical intervention

; CHFÀÇ 10%

; isolated PDA´Â high altitude¿¡¼­ Ãâ»ýÇÑ ¾î¸°ÀÌ¿¡ ÈçÇÏ´Ù

Pathophysiology

  <1> high aortic pr.ÀÇ °á°ú·Î¼­ aorta¿¡¼­ ductus¸¦ ÅëÇØ pul.a.·Î blood flow°¡ ¹ß»ýÇÑ´Ù

  <2> shunt extent¸¦ °áÁ¤ÇÏ´Â ¿ä¼Ò

       1) ductus size

       2) ratio of pulm. to systemic vascular resistance

  <3> ½ÉÇÑ °æ¿ì¿¡´Â LV outputÀÇ 70%°¡ diastoleµ¿¾È¿¡ ductus¸¦ ÅëÇØ pul.circulationÀ¸·Î

       shunting -> wide pulse pressure°¡ ¹ß»ý

   <4> small PDAÀÎ °æ¿ì¿¡´Â pul. a., RV, RAÀÇ pr.´Â Á¤»ó. ±×·¯³ª large PDAÀÎ °æ¿ì¿¡ pulm.

       a. pr.´Â  Áߵ  ȤÀº systemic level±îÁö Áõ°¡ÇÏ°Ô µÈ´Ù.

  <5> total blood volume Áõ°¡

Clinical Manifestations

# small PDA

    ; asymptomatic

# large PDA

       1) retardation of physical growth( ÁÖÁõ»ó )

       2) left ventricular failure

       3) wide pulse pr. -> µÎµå·¯Áø bounding arterial  pr.

       4) moderately or grossly enlarged heart

# ûÁø ¼Ò°ß

    ;  apical impulse;prominent

  ; thrill

       1) 2nd left intercostal space¿¡¼­ Àß ´À²¸Áü

       2) radiate toward left clavicle,down left sternal border,toward apex

  ; classic murmur

        - machinery,a humming top,a mill wheel,rolling thunder in quality·Î ¹¦»çµÊ

       -> S1ÀÌ onsetÇÏ°í ³­ ÈÄ¿¡ ½ÃÀ۵Ǿî end-systole¶§ maximal intensity¿¡ µµ´ÞµÈ ÈÄ late diastole¶§ ÀÛ¾ÆÁø´Ù

       - localized to 2nd left intercostal space ¶Ç´Â radiate down the left sternal border or left clavicle

    ; *increased pul. vascular resistance

           --> *less prominent or absent diastolic component of murmur

cf) large left to right shunt°¡ ÀÖÀ¸¸é MV¸¦ °¡·ÎÁö¸£´Â large blood flow¿¡ ÀÇÇØ

          low-pitched mitral diastolic murmur°¡ ¹ß»ý

# EKG

       1) small PDA;normal

       2) large;left ventricular or biventricular hypertrophy

# X-ray

       1) prominent pul.a.

       2) intrapul.vascular marking;Áõ°¡

       3) involved chamber;LA and LV

       4) aortic knob;normal or prominent

# echocardiography

       1) small PDA ; cardiac chamber´Â normal

       2) left atrial and ventricular dimension;Áõ°¡

       3) isovolumic contraction time;°¨¼Ò

       4) aortic runoff in diastole

# catheterization

       1) RV and pul.a.pr.;Á¤»ó ȤÀº Áõ°¡

       2) pul. a.·Î oxygenated blood°¡ shuntingµÇ¸é left to right shunt°¡ ÀÖÀ½À» Áõ¸í

       3) vena cava,RA,RV;normal oxygen content

       4) catheter´Â ductus¸¦ ÅëÇØ descending aorta·Î °£´Ù

       5) ascending aorta·Î dye¸¦ ÁÖÀÔÇϸé aorta·ÎºÎÅÍ pul.a.ÀÇ opacificationÀ» º¼ ¼ö ÀÖ´Ù.

Diagnosis

# ¡ÚDDx

    ; venous hum

    ; aorticopulmonary window defect

    ; sinus of valsalva aneurysm that has ruptured into the Rt. side of heart or pulmonary artery

    ; coronary artery fistulas

    ; aberrant left coronary artery with massive collaterals from Rt. coronary artery

    ; Truncus arteriosus with torrential pulmonary flow, pul. branch stenosis

           - normal pulse pressure

    ; peripheral arteriovenous fistula

           - distinctive murmur from PDA

    ; VSD with AI, combined rheumatic aortic and mitral insufficiency

           - distinctive murmur from PDA

Prognosis & Complication

  <1> small PDA´Â Á¤»ó ¼ö¸íÀ» »ì ¼ö ÀÖ´Ù

  <2> infancy¸¦ Áö³ª¼­ spontaneous closure´Â ±ØÈ÷ µå¹°´Ù

# ¡ÚComplications

    ; CHF

           - *¡ãoften in early infancy

  ; infective endocarditis

           - *at any age

    ; pul. or systemic emboli

    ; rare

           - aneurysmal dilatation of pul. artery or ductus, calcification of ductus, non-infective thrombosis of ductus with embolization, paradoxic emboli, pul. hypertension( Eisenmenger syndrome )

Treatment

# surgical operation

    ; irrespective of age

    ; *case fatality < 1%

           --> *operation before 1yr

  ; *pul.hypertensionÀÌ À־ reverse shunt°¡ ¾øÀ¸¸é ¼ö¼úÇÒ ¼ö ÀÖ´Ù.

# ligation and diversion

386.9  Aorticopulmonary Window Defect

; ascending aorta¿Í main pul.a.»çÀÌÀÇ communication

; *pul. and aortic valve°¡ Á¸ÀçÇϸç ventricular septumÀº intact

    - DDx with truncus arteriosus

# Symptom

  <1> large VSD and PDA appearing early infant¿Í À¯»ç

  <2> recurrent pul.infection,CHF,minimal cyanosis

# defect;large

# ûÁø ¼Ò°ß

  <1> systolic with a mid-diastolic rumbling murmur

      - MVÀ» °¡·ÎÁö¸£´Â increased blood flow¿¡ ÀÇÇØ ¹ß»ý

# Sign

  <1> PDA¿Í À¯»ç

  <2> wide pulse pr.,cardiac enlargement,continuous right and left upper sternal border

       systolic murmur

# EKG

  ; left or biventricular hypertrophy

# X-ray

  <1> cardiac enlargement

  <2> prominence of pul.a. and intrapul.vascularity

# echocardiogram

  ; large volume left sided heart chamber, and the window

# catheterization

  <1> pul.a.level¿¡¼­ left to right shunt

  <2> hyperkinetic pul.hypertension

  ; large defect¿¡ ÀÇÇØ ¹ß»ý

# Tx.

  ; infant¶§ cardiopul.bypass

386.10 Coronary Artery Fistula

# coronary a.¿Í atrium,ventricle(esp.right),pul.a.»çÀÌ¿¡ congenital fistula°¡ Á¸ÀçÇÏ´Â °Í

# coronary a.¿¡¼­ blood°¡ right sided heart·Î ÇâÇÒ ¶§ atrial or ventricular level¿¡¼­ ´ÜÁö

  small left to right shunt°¡ Á¸ÀçÇÑ´Ù

# involved coronary a.;dilated or aneurysmal

386.11 Ruptured Sinus Of Valsalva

# aortaÀÇ valsalva sinusÁßÀÇ Çϳª°¡ congenital or acquired disease¿¡ ÀÇÇØ ¾àÇØÁ³À» ¶§

  aneurysmÀÌ ruptureµÇ¾î RA ȤÀº RV·Î ÇâÇÑ´Ù

# extremely rare in childhood, sudden onset

# Dx.

  ; ȯÀÚ°¡ °©ÀÚ±â new loud to-and-fro murmur¸¦ µ¿¹ÝÇϸ鼭 acute CHF°¡ ¹ß»ýÇßÀ» ¶§

# catheterization

  ; atrial or ventricular level¿¡¼­ left to right shunt

# Tx.

  ; urgent surgical repair

THE OBSTRUCTIVE LESIONS

386.12 Pulmonary Valve Stenosis With Intact Ventricular Septum

# ¿©·¯ ÇüÅÂÀÇ right ventricular outflow obstructionÀÌ ÀÖÀ¸¸é¼­ ventricular septumÀº intactÇÏ´Ù

# the most common;valvular pulmonary stenosis

# systoleµ¿¾È¿¡ dome-like obstructionÀÌ ¹ß»ý

# ¶§¶§·Î PS¿Í ASD°¡ µ¿½Ã¿¡ ³ªÅ¸³²

Pathophysiology

  <1> right ventricular outflow obstructionÀº systolic pr.À» Áõ°¡½Ã۰í RVHÀ» À¯¹ß½ÃŲ´Ù

  <2> severity °áÁ¤¿ä¼Ò

       ; size of restricted valvular opening

  <3> severe case;RV pr.> systemic systolic pr.

       mild case ; RV pr.°¡ °æµµ ȤÀº Áߵ·Î Áõ°¡

  <4> pul. a. pr. ; Á¤»ó ȤÀº °¨¼Ò

  <5> arterial oxygen saturation

       ; RV compliance °¨¼Ò¿Í intra-cardiac communication¿¡ ÀÇÇØ right to left shuntingÀÌ »ý±â´Â ½ÉÇÑ °æ¿ì ( neonate ȤÀº small infant¿¡¼­ º¼¼ö ÀÖÀ½ )¸¦ Á¦¿ÜÇϰí´Â Á¤»óÀÌ´Ù

Clinical Manifestaitions

  <1> °æµµ ȤÀº ÁߵÀÇ stenosis´Â ¹«Áõ»ó

  <2> severe stenosis´Â exercise intolerance

  <3> critical pulm. stenosis¸¦ °¡Áø neonate¿Í young infant¿¡¼­ÀÇ obstuction; RV failure°¡ ´õ

      ÇöÀúÇϰí foramen ovale¿¡¼­ÀÇ shunting ¶§¹®¿¡ cyanosis À¯¹ß

  <4> PSȯ¾Æ

       ; growth and development´Â Á¤»ó

  <5> valve dysplasia¿¡ ÀÇÇÑ PS;Noonan syndromeÀÇ °¡Àå ÈçÇÑ cardiac anomaly

  <6> mild PS½Ã

       1) venouse pressure and pulse ; Á¤»ó

       2) not enlarged heart

       3) apical impulse ;  Á¤»ó

       4) RV ; not palpable

       5) pulmonic area¿¡¼­ relativly short pul systolic ejection murmur°¡ µé¸²

       6) S2 ; split (pul. element´Â delayed)

       7) EKG ; mild RVH

       8) X-ray ; pul.a.ÀÇ poststenotic dilatation

       9) two-dimentional echocardiography ; domed valve

  <7> Moderate PS

       1) venouse pressure ; ¾à°£ Áõ°¡

       2) jugular pulse¿¡ intrinsic "a"°¡ ÇöÀú

       3) palpable RV sternal lift

       4) systolic ejetion murmur ; prolonged later into systolic

       5) S2 ; split (pul. component´Â delayed and diminished)

       6) EKG

         (1) RVH (systolic over load)

         (2) prominent spiked P wave

       7) X-ray

         (1) heart ; Á¤»óÀ̰ųª ȤÀº RV prominence¿¡ ÀÇÇØ¼­ ¾à°£Áõ°¡

         (2) pul. vascularity ; °¨¼Ò

  <8> severe PS

       1) interarterial communicationÀÌ ÀÖÀ¸¸é mild to moderate cyanosis

       2) RV failure½Ã hepatomegalry and peripheral edema

       3) elevation of venouse pr.

          ; large presystolic jugular "a" wave¿¡ ÀÇÇØ ¹ß»ý

       4) heart

          ; moderatly or greatly enlarged

       5) loud systolic ejection murmur

          (1) trill°ú µ¿¹Ý

          (2) pul. area¿¡¼­ Àß µé¸²

          (3) entire precordium¿¡¼­ neck°ú backÀ¸·Î radiation

          (4) late systolic accentuationÀ» µ¿¹ÝÇÔ

       6) S2ÀÇ pul. element°¡ µé¸®Áö ¾Ê´Â´Ù.  

       7) EKG

          (1) RVH

          (2) tall spiked P wave

       8) two-dimensional echocardiogram

          (1) severe pul.valve deformity

          (2) intact ventricular septum

          (3) RVH

       9) X-ray

          (1) cardiomegaly

          (2) prominence of RV and RA

          (3) prominence of pul.a.segment

              ; poststenotic dilatation¿¡ ÀÇÇØ ¹ß»ý

          (4) pul.vascularity´Â °¨¼Ò

      10) catheterization

          (1) pul. a. pr. ; Á¤»ó ȤÀº °¨¼Ò

          (2) RV pr. ; mild case¿¡¼­´Â 30-50mmHg

                      moderate case¿¡¼­´Â 30-50mmHg

                      severe case¿¡¼­´Â systemic systolic pr.º¸´Ù Å©´Ù

          (3) severe or moderate case¿¡¼­´Â RA.pr.´Â prominentÇϸç giant "a" wave°¡ ³ªÅ¸³²

Prognosis And Complication

# Complication

  <1> CHF

       1) the most common

       2) severe case¿¡¼­¸¸ ¹ß»ýÇÏ¸ç »ýÈÄ Ã¹ 1°³¿ù¿¡ ¹ß»ý

  <2> cyanosis

       1) foramen ovale¸¦ °¡·ÎÁö¸£´Â right to left shunt¿¡ ÀÇÇØ ¹ß»ý

       2) infancy and severe case¿¡¼­¸¸ ¹ß»ý

  <3> infective endocarditis

        ; not common

# Course and prognosis

  <1> mild to moderate case

      1) Á¤»ó »ýȰ °¡´É

      2) Á¤±â °ËÁø ¹Þ¾Æ¾ß ÇÔ

  <2> small gradient°¡Áø ȯÀÚ

      ; º´ÀÌ ÁøÇàÇÏÁö ¾ÊÀ¸¸ç Ä¡·áÇÒ ÇÊ¿ä ¾ø´Ù

  <3> moderate case

      ; ¼ºÀåÇÔ¿¡ µû¶ó more significant gradient

  <4> obstructionÀ» ¾ÇÈ­½ÃŰ´Â ¿ä¼Ò

      ; subvalvular muscular and fibrous tissue hypertrophy

  <5> severe case¸¦ Ä¡·áÇÏÁö ¾ÊÀ¸¸é RV dysfunction and cardiac failure ¹ß»ý

  <6> severe case Tx.

      ; urgent catheter balloon valvuloplasty or surgical valvotomy

Treatment

# ¡ÚBalloon valvuloplasty

 ; *moderate or severe isolated PSÀÎ °æ¿ì Tx. of choice

# Emergency Closed or Open Valvotomy

 ; obstructionÀÌ ½ÉÇÑ neonate or infant¿¡ ½Ç½Ã

386.13 Infundibular Pulmonary Stenosis And Double Right Ventricle

# infundibular pul. stenosis

  <1> RV outflow tractÀÇ muscular or fibrous obstruction¿¡ ÀÇÇØ ¹ß»ý

  <2> obstruction site;pul.valve ±Ùó ȤÀº ¹Ù·Î ¹Ø

  <3> infundibular chamber´Â RV cavity and pul.valve»çÀÌ¿¡ ¹ß»ý

  <4> Ãʱ⿡ VSD ³ªÅ¸³µ´Ù°¡ ³ªÁß¿¡ spontaneously closed

  <5> hemodynamics and ÀÓ»óÁõ»ó

       ; valvular PS¿Í À¯»ç

# double right ventricle

  <1> pul.valveÀÇ ¹Ø¿¡ À§Ä¡ÇÏ´Â RV outflow obstructionÀÇ more common variation

  <2> mid right ventricular region¿¡ muscular band°¡ ÀÖ¾î chamber¸¦ two part·Î ³ª´©°í

       inlet¿¡¼­ outletÀ¸·Î obstructionÀ» À¯¹ß½ÃŲ´Ù

  <3> VSD°¡ Àß µ¿¹ÝµÇ¸ç À̰ÍÀº spontaneously closed

  <4> obstructionÀº Ãʱ⿡´Â Àß ³ªÅ¸³ªÁö ¾ÊÀ¸¸ç ºü¸£°Ô ÁøÇà

386.14 PS In Combination With An Intracardiac Shunt

# valvular or infundibular PS´Â ASD or VSD¸¦ °¡·ÎÁö¸£´Â left to right shunt°¡ Àß µ¿¹ÝµÊ

# ÀÓ»óÁõ»ó °áÁ¤¿ä¼Ò

  <1> degree of stenosis

  <2> magnitude of left to right shunt

# left to right shunt°¡ ÀÖÀ¸¸é PS´Â mildÇϰí Áõ»óÀÌ ½ÉÇØÁö¸é (severe PS) right to left

   shunt°¡ ¹ß»ý

386.15 Peripheral Pulmonary Arterial Stenosis

# ¢¾Associated Other Type Congenital Heart Diseases

    ; pul. valvular stenosis

  ; TOF

  ; PDA

  ; VSD

  ; ASD

  ; supravalvular aortic stenosis

; *familial tendency

# high risk condition

    ; ¡Úcongenital rubella syndrome

# Williams syndrome

    ; supravalvular AS with pulmonary arterial branch stenosis

    ; idiopathic hypercalcemia

# multiple severe constriction½Ã RV and obstruction ±ÙÀ§ºÎÀ§ pul.a.pr.´Â Áõ°¡ÇÑ´Ù

# systolic, continuous murmur

# EKG

  ; RVH and RAH (severe caseÀÎ °æ¿ì)

# X-ray

  <1> cardiomegaly and prominence of main pul.a.

  <2> pul.vascularity;Á¤»ó

386.16  Aortic Stenosis

Pathophysiology

; *³²:¿©=3:1

; *mostly valvualr stenosis

# Type

    1) valvular

       ; *¡ãcommon

    ; thickened leaflet

       ; fused commissure

    2) subvalvular(subaortic)

       ; LV ourflow obstructionÀÇ important form

       ; rapid progression in severity

    ; ´Ù¸¥ CHD(COA,PDA,VSD)ÀÇ successful surgeryÈÄ¿¡ ³ªÅ¸³²

  3) supravalvular

       ; less common type

       ; sporadic,familial or ass. with williams syndrome

    ; ¡ÚWilliams Syndrome

                   - mental retardation, elfin facies (full face, broad forehead, flattened bridge of nose, long upper lip, rounded cheek)

                   - idiopathic hypercalcemia in infancy

Clinical Manifestations

  <1> early infancy¶§ critical aortic stenosis and severe left ventricular failure ³ªÅ¸³¿.

    -> low cardiac output signÀ¸·Î¼­, congestive heart failure,  cardiomegaly, pulm edema°¡

       ½ÉÇϰí,  »çÁö¿¡¼­ pulse°¡ ¾àÇØÁö¸ç, urine outputÀÌ ÀÛ¾ÆÁü.  

       ´ë°³ childrenÀº ¹«Áõ»óÀ¸·Î¼­ normal growth and development

  <2> murmur ; routine P/E¿¡¼­´Â ³ªÅ¸³ªÁö ¾ÊÀ½

  <3> sudden death

       ; severe left ventricular outflow obstruction½Ã º¼ ¼ö ÀÖ´Ù.

  <4> mild or moderate case

       ; heart size and apical impulseÀº Á¤»ó

  <5> severe case

       ; cardiomegaly with left ventricular apical thrust

  <6> ûÁø¼Ò°ß

      1> rough systolic ejection murmur

         (1) right upper sternal border¿¡¼­ Àß µé¸²

         (2) suprasternal notch  thrillÀ» µ¿¹Ý

         (3) neck and down the left sternal border·Î radiate

      2> diastolic murmur

         (1) mild aortic insufficiency ÀǹÌ

         (2) subvalvular obstruction ȤÀº bicuspid aortic valve½Ã ³ªÅ¸³²

      3> apical short mid-diastolic rumbling murmur

         ; normal MV½Ã¿¡µµ ³ªÅ¸³²

      4> S2

        (1) mild case½Ã normal splitting

        (2) severe case½Ã aortic valve closure´Â diminished, paradoxic splitting S2³ªÅ¸³²

      5> S4

          ; severe case½Ã ³ªÅ¸³²

      6> subvalvular type½Ã murmur´Â left sternal border or apex¿¡¼­ maximal intensity

      7> valvular type½Ã aortic ejection clickÀÌ ¼±ÇàµÊ

  <7> infancy½Ã critical ASÀÇ Áõ»ó

      1> CHF ; cardiomegaly,pul.edema;severe

      2> pulse ; weak

Diagnosis

  <1> EKG

      1) severe case½Ã normal

      2) ¿À·§µ¿¾È severe stenosis°¡ Áö¼ÓµÇ¸é LVH and strainÀÌ ³ªÅ¸³²(Lt precordial lesd¿¡¼­

         inverted T-wave ³ªÅ¸³¿.)

  <2> X-ray

      1) prominent ascending aorta

      2) aortic knob;mormal

      3) heart size;normal

      4) valvular calcification;old children ½Ã

  <3> M-mode echocardiography

      1) multiple diastolic echoes of aortic valve

      2) eccentric aortic valve closure

      3) increased thickness of ventricular septum and free wall of LV

  <4> Graded exercise testing

      1) old children¿¡¼­ left ventricular outflow obstructionÀÇ severity¸¦ Æò°¡

      2) gradient severity°¡ Áõ°¡ÇÒ¼ö·Ï

        (1) working capacity;°¨¼Ò

        (2) systolic pr.;fails to rise adequately

        (3) diastolic pr.;Áõ°¡

        (4) ST segment;depression

  <5> left cardiac catheterization

      1) obstructionÀÌ ½ÉÇϸé abnormal aortic pr.curve ³ªÅ¸³¿

        (1) early-appearing anacrotic notch

        (2) slow,prolonged,delayed systolic upstroke

        (3) narrow pulse pr.

        (4) delayed dicrotic notch

Prognosis

  <1> mild to moderate ½Ã good

  <2> severe ½Ã sudden death;gross LVHÀÇ evidence°¡ º¸ÀÓ

  <3> neonate¿¡¼­ÀÇ »ç¸Á¿øÀÎ

       ; LVÀÇ endocardial fibroelastosis¸¦ µ¿¹ÝÇÑ CHF

Treatment

  <1> progressive left ventricular dysfunctionÀ» ¿¹¹æÇϱâ À§ÇØ severe valvular AS°¡Áø ¾î¸°ÀÌ

      ´Â surgical valvotomy ½Ç½ÃÇÑ´Ù   

  <2> Balloon valvuloplasty

      ; infant and older children¿¡ ¼º°øÀû ½Ç½Ã

  <3> OP Ix.

      1> LVHÀÇ definitive evidence

      2> significant gradient ( rest½Ã normal cardiac outputÀÌ ÀÖÀ¸¸é¼­ Á½ɽǰú ´ëµ¼¸·°úÀÇ

         ¾Ð·ÂÂ÷°¡ 60mmHg ÀÌ»ó ) across the aortic valve

         => surgery or balloon dilatationÀ» ½Ç½ÃÇÔ

   <4> careful follow-upÀÌ ÇʼöÀûÀÌ´Ù

        ¿Ö³ÄÇϸé ventricular obstructionÀÇ severe recurrence°¡ early symptom°ú °ü°è°¡ ¾ø±â

        ¶§¹®ÀÌ´Ù.

   <5> infective endocarditis¿¡ ´ëÇØ prophylaxis°¡ ÇÊ¿ä

 386.17 Coarctation Of The Aorta

; constriction of aorta

    - any point from transverse aorta to iliac bifurcation

    - *98% just below origin of left subclavian a. at origin of ductus arteriosus(=justaductal coarctation)

; ³²:¿©=2:1

; Turner syndrome(XO)¿¡ ¸¹´Ù

; ¡Úµ¿¹Ý anomaly

  - *bicuspid aortic valve(70%)

  - *MV anomaly : supravalvular mitral ring, parachute mitral valve

  - *subaortic stenosis

Pathology

# type

    1) preductal segmental tubular hypoplasia

    ventricular hypertension and hypertrophy¡æ PDA widening¡æ relief of

    obstruction¡æ acyanotic

    2) discrete juxtaductal obstruction

    RV blood¡æ ductus¡æ descending aorta¡æ lower body¡æ femoral pulse are

    palpable¡æ severe pulmonary hypertension and vascular rssistence¡æ signs of heart failure 

# hypertensionÀÇ ¿øÀÎ

  ; mechanical obstruction

  ; renal mechanism

 COA´Â extensive collateral circulationÀ» À¯¹ßÇÑ´Ù

 1> branches of subclavian a.

 2> sup.intercostal a.

 3> internal mammary a.

 4> axillary a.ÀÇ thoracic and subscapular branches

 infancyÀ» Áö³ª¸é ´ë°³´Â ¹«Áõ»ó

Clinical Manifestations

# *After infancy, mostly asymptomatic even severe coarctation

# Older Children

    ; *hypertensive on routine P/E

           --> frequently brought to cardiologist

# classic sign

  ; disparity in pulsations and blood pressures of arms and legs

           - *radial-femoral delay

                   / normal¿¡¼­´Â femoral before radial pulse

           - lower BP in legs

                   / normal¿¡¼­´Â lower BP in arms 10-20mmHg

                   / common over 1yr

           - *exercise¿¡ ¹ÝÀÀÇÏ¿© systemic blood prÀÇ Áõ°¡°¡ ÇöÀúÇÏ´Ù

# *90% systolic hypertension in upper extremities

# higher pressure in right arm

    - suggest involvement of left subclavian artery

# ûÁø ¼Ò°ß

    ; usually normal

    ; *apical systolic ejection click or thrill in suprasternal notch

           - suggest *bicuspid aortic valve

    ; short systolic murmur

       - along left sternal border at 3rd and 4th ICS

    - *well transmitted to left infrascapular area & neck

    ; typical murmur of mild AS in 3rd Rt. intercostal space

    ; *low-pitched mid-diastolic murmur at apex

           - *suggest MS

    ; *systolic or continuous murmur over left & right side of chest

           - *well developed collarteral blood flow

Diagnosis

# X - Ray

    ; cardiac enlargement

           - CHF or LV prominence¿¡ ÀÇÇØ ¹ß»ý

    ; prominence shadow in left sup. mediastinum

       - enlarged left subclavian a.¿¡ ÀÇÇØ ¹ß»ý

    ; ¡Únotching of inf. border of ribs

       - pressure erosion by enlarged collateral vessel

           - *except upper and lower 2 to 3 ribs

           - *8¼¼ÀÌÈÄ¿¡ ³ªÅ¸³­´Ù.

    ; displacement of barium filled esophagus and discontinuity of lateral margin of aorta below the arch

           - post stenotic dilatation of descending aorta ¿¡ ÀÇÇØ ¹ß»ý ( E sign or inverted 3 sign. )

# EKG

           ¨ç NEONATE AND INFANT ¶§¿¡´Â RVH or BVH

           ¨è Young child¶§¿¡´Â normal

           ¨é Old child¶§¿¡´Â LVH

Prognosis And Complicatoin

# ¢ÞAssociated Anomalies

    ; *aortic valve anormality

           - *most patients

           - *biscuspid valve is ¡ãcommon

           - no clincal sign in mostly

    ; PDA

    ; VSD and ASD

        - left to right shunt ÀÖÀ» ¶§ ÀǽÉ

    ; mitral valve abnormality

  ; *congenital aneurysm of wilis circle, defective elastic & medial tissue of vessels

           --> *subarachnoid or intracerebal hemorrage

                   / secondary to hypertensive state

    ; subclavian a. abnormality

       - involvement of left subclavian a. in the area of coarctation

    - *stenosis of orifice of Lt. subclavian a., anormalous origin of Rt.subclavian a.

# *Ä¡·á ¾ÊÀ¸¸é 20-40¼¼ »çÀÌ¿¡ »ç¸Á

# ¡ÚCommon Serious Complication

    ; related hypertensive state

           - *premature coronary a. disease

           - CHF    

           - hypertensive encephalopathy

           - intracranial hemorrage

    ; infective endocarditis or endarteritis

    ; *aneurysm of the descending aorta or of the enlarged collateral vessels

Treatment

  in neonate severe COA + ductal closure¡æ hypoperfusion¡æ acidosis rapid

  deterioration¡æ PGE1

# ¼ö¼ú ½Ã±â

    ; *2-4¼¼ (mortality rate´Â 1% ¹Ì¸¸)

    ; 2nd decade½Ã ¼ö¼úÀÌ ºñ¼º°øÀûÀÎ ÀÌÀ¯

    1> decreased LV function

    2> degenerative change

# Choice OP

  ; excision of the area of coarctation and primary anastomosis

# ¡ÚPostop. Complication

    ; striking increase in amplitude of pulsation in lower extremities

    ; rebound hypertension

    ; residual murmur

           - due to asso. cardiac anomalies

    ; rare operative problem

           - spinal cord injury, chylothorax, diaphragm injury, laryngeal nerve injury

           - diminished or absent pulse if left subclavian flap

           - aortic aneurysm if balloon angioplasty

    ; recoarctation

           - *common in end-to-end anastomosis within 1st mo

           - *balloon angioplasty°¡ choice procedure

    ; premature cardiovascular disease

           - early onset adult hypertension

           - *repair of 2nd decade or beyond½Ã Áõ°¡ÇÑ´Ù.

    ; postcoarctectomy syndrome

Postcoarctectomy Syndrome 

; *postoperative mesenteric arteritis

    --> *hypertension, abdominal pain

1) Sx and Sg

  - anorexia, nausea, vomiting, leukocytosis, intestinal hemorrhage, small bowel obstruction, bowel necrosis. 

2) Tx

   ¨ç antihypertensive drug ; nitroprusside, labetalol

   ¨è intestinal decompression

   ¨é corticosteroid

386. 18 Coarctation with VSD

       infancy   isolated COA´Â µå¹°°Ô CHF¸¦ À¯¹ßÇÑ´Ù.

         <1> LVÀÇ preload and afterload¸¦ Áõ°¡½ÃÅ´

         <2> »ýÈÄ Ã¹ 1°³¿ù¿¡ ¹ß»ý

         <3> intractable cardiac failureÀ» À¯¹ß

      Sx.

         <1> tachypnea, failure to thrive, heart failure

         <2> cardiac output°¡ ³·¾Æ »óÇÏÁö ¾Ð·ÂÂ÷À̰¡ ÇöÀúÇÏ´Ù

386. 19 Coarctation With Orher Cardiac Anomaly

µ¿¹Ý Anomaly

         ¨ç hypoplastic Lt Ht

         ¨è severe aortic valvular disease

         ¨é TGA

         ¨ê Variation of single ventricle

         ¨ë endocardial fibroelastosis

      ductus¸¦ °¡·ÎÁö¸£´Â blood flow¸¦ °áÁ¤ÇÏ´Â ¿ä¼Ò 

         ¨ç positipon

         ¨è severity of obstruction at the site of coarctation

         ¨é pul. vascular resistance

386. 20 Congenital MS

# associated defects

  ; AS

  ; CoA

      MV

         <1> funnel shape

         <2> leaflet;thickened

         <3> chordae tendineae;shortened and deformed

         <4> parachute MV and double orifice MV

      Sx.

         <1> ù 2³â ³»¿¡ ³ªÅ¸³²

         <2> infant : underdevelopment, dyspnea secondary to CHF, cyanosis, pallor

      ûÁø¼Ò°ß

         <1> rumbling diastolic murmur

         <2> S2 : loud and split

         <3> opening snap of MV

      EKG

         <1> RVH

         <2> normal, bifid, or spiked P waves

      X-ray

         <1> LA and RV enlargement

         <2> pul. congestion

      echocardiogram : Ư¡Àû

         <1> thickened MV leaflets

         <2> diminished E-F slope

         <3> enlarge LA with normal or small LV

         <4> two-dimensional (short axis)

               significant reduction of MV orifice in diastole

 

      catheterization

              RV, pul.a., pul. capillary wedge pr. : Áõ°¡

      Doppler study : pressure gradient across the mitral orifice

      PROGNOSIS

         <1> ´ë°³ Ä¡·áÇÏÁö ¾ÊÀ¸¸é ¿¹Èİ¡ poor

         <2> ´ë°³ »ýÈÄ Ã¹ 2³â ³»¿¡ »ç¸Á

386.21 Pulmonary Venous Hypertension

  chronic pul.venous hypertensionÀ» À¯¹ßÇϰí À̰ÍÀÌ ½ÉÇØÁö¸é pul. a. hypertension and

      right sided heart failure¸¦ À¯¹ßÇÑ´Ù

      pul.venous hypertensionÀ» À¯¹ßÇÏ´Â Áúȯ

         <1> congenital MS

         <2> MI

         <3> some varieties of total anomalous pul.venous return with obstruction

         <4> left atrial myxomas

         <5> cor triatriatum (stenosis of common pul.v.)

         <6> indivisual pul.venous stenosis

         <7> supravalvular mitral ring or web

      EKG

         <1> RVH

         <2> spiked P waves

      X-ray

         <1> cardiomegaly

         <2> pul. v. , RV, RA, main pul. a. : prominent

         <3> LA : normal size or slightly enlarged

      Echocardiogram

         <1> left atrial myxoma

         <2> cor triatriatum

         <3> mitral valve abnormality

      Catheterization

         <1> pul. hypertension

         <2> pul. a. wedge pr.°¡ Áõ°¡

         <3> left atrial pr. : lesionÀÌ proximal sideÀ̸é normal

      DDx.

        pul.veno-occlusive disease

         <1> children°ú young adult¿¡¼­ pul.v.¿¡ obstructive lesionÀ» À¯¹ßÇÏ´Â idiopathic

              process

         <2> toxin or viral agent¿¡ ÀÇÇØ local injury ¹ÞÀº ÈÄ¿¡ obstruction¹ß»ý

         <3> Ãʱ⿡ pul.edema°¡ ÀÖÀ¸¸é¼­ left sided heart failure

         <4> dyspnea, fatigue, pleural effusion : common

              cyanosis, digital clubbing, syncope, hemoptysis : variable

         <5> LA pr. : normal

              pul. a. wedge pr. : normal or elevated

              pul. venous returnÀÇ anatomic abnormality´Â ¾ø´Ù

      ¿¹ÈÄ

         <1> infant : ¼ö ÁÖ¿¡¼­ ¼ö °³¿ù°£ »ýÁ¸

         <2> adult : ¼ö °³¿ù¿¡¼­ ¼ö ³â°£ »ýÁ¸

THE REGURGETANT LESIONS

386.22 Pulmonary Valvular Insufficiency And Congenital  Absence Of The Pulmonary Valve

     

      ¿©·¯ cardiovascular disease¸¦ Àß µ¿¹ÝÇϸç severe pul.hypertension¿¡ ÀÌÂ÷ÀûÀ¸·Î ¿Â´Ù

      ¼ö¼ú ÈÄ ÇÕº´Áõ

         incompetence of valve

         prominent physical sign

         diastolic murmur

          <1> upper and mil left sternal border¿¡¼­ Àß µé¸²

          <2> AI¶§º¸´Ù lower pitch

      X-ray

         prominence of main pul. a.

    

      EKG

         normal or minimal RVH

      catheterization

         pul. a. diastolic pr. : °¨¼Ò

      isolated pul.valvular incompetence

         well tolerableÇÏ¸ç ¼ö¼úÀÌ ÇÊ¿ä¾ø´Ù

      absence of pul.valve

        <1>µ¿¹ÝÁúȯ

           1> VSD

           2> TOF

        <2> neonate or infant¿¡¼­ pul. a.´Â widely dilated and compress the bronchiÇÏ¿©

           1> recurrent episodes of wheezing

           2> pul. collapse

           3> pneumonitis¸¦ Àß ¹ß»ý½ÃŲ´Ù

        <3> »ç¸Á¿øÀÎ

           1> bronchial compression

           2> hypoxemia

           3> heart failure

        <4> Ä¡·á

              plication of massive pul.arteries along with intracardiac correction

386. 23 Congenital MI

      Associated anomaly

         PDA, COA, VSD, corrected TOGV

         anomalous origin of the LT coronary a from the pul. a

         endocardial fibroelastosis

         Marfan syndrome

         atrioventricular septal defect

      auscultation

         high pitched apical holosystolic m

         apical low pitched mid-diastolic rumbling m

            increased diastolic flow

      EKG

         bifid p wave, LVH or RVH

      X-ray

         LV is prominent

         pul. vascularity is normal or prominent

      Echo

         enlarge of LA and LV

         motion of MV is excessive with a steep E-F slope on M mode

      Cath

         elevated LA pres

         pul. a hypertension

      Tx

         mitral valvuloplasty

         instillation of prosthetic valve 

386. 24 Mitral Valve Prolapse

¿øÀÎ

       - Çϳª ȤÀº ¾çÂÊÀÇ mitral leafletsÀÇ billowing(¼Ò¿ëµ¹ÀÌ) ƯÈ÷, post. cuspÀÌ end systole

         ½Ã left atriumÀ¸·Î ÇâÇÏ°Ô µÇ´Â abnormal mitral valve mechnism¿¡ ÀÇÇØ ¹ß»ýÇÑ´Ù

      Ç×»ó congenital ( autosomal dominant ) adolescence or adulthood¶§ ±îÁö´Â ÀÎÁöµÇÁö           ¸øÇÔ

      girls¿¡ ¸¹´Ù

      Àß ¹ß°ßµÇ´Â °æ¿ì

        ¨ç Marfan syndrome

        ¨è straight back syndrome

        ¨é pectus excavatum

        ¨ê scoliosis

        ¨ë congenital rheumatic

        ¨ì viral myocarditis

        ¨í secundum ASD

      ûÁø¼Ò°ß : Ư¡Àû

        <1> apical murmur

           1) late systolic in timing

           2) clickÀÌ ¼±ÇàµÊ

           3) standing or sitting position¿¡¼­ clickÀº early systole¶§ murmurÀº late systole¶§ 

               ÇöÀúÇÔ

        <2> arrhythmia

        <3> primarily unifocal or multifocal premature ventricular contraction

      EKG

        <1> usually normal

        <2> diphasic T wave (lead II, III, AVF, V6)

      Chest X-ray : normal

      Echocardiogram

        <1> mid or late systole¶§ post. mitral leafletÀÇ post. movement

        <2> ant. and post. mitral leafletsÀÇ pansystolic prolapse

        <3> Two-dimensional real time echocardiography

               mitral leafletÀÇ free edge and body°¡ systole   LA¸¦ ÇâÇØ post.·Î move

      infective endocarditisÀÇ À§Ç輺ÀÌ ³ôÀ¸¹Ç·Î surgery or dental procedure antibiotic

      prophylaxis°¡ ÇÊ¿ä

      thickened and redundant MV leafletsÀÇ Á¸ÀçÇÏ¿¡¼­ MV prolapse¸¦ °¡Áø ¼ºÀÎ(³²ÀÚ¿¡¼­

      ÈçÇÔ)¿¡¼­

      ¹ß»ýÇÒ¼ö ÀÖ´Â cardiovascular complication

         <1> sudden death

         <2> arrhythmia

         <3> CVA

         <4> progressive valve dilatation

         <5> heart failure

         <6> endocarditis

386.25 TR

      Associated with Ebstein anomaly of TV

      Older children : acyanotic form

      newborn : severe cyanosis

      accompanies RV dysfunction

      encountered in newborn with perinatal asphyxia

ADDITIONAL CONGENITAL HEART LESIONS

386. 26 Anomalous Of The Aortic Arch

Right Aortic Arch

1) aorta´Â right·Î curve

   ¸¸ÀÏ aorta°¡ vertebral columnÀÇ right side·Î descendingÇϸé other cardiac

   malformation°ú °ü°è

2) ¹ß»ýµÇ´Â °æ¿ì

   (1) TOFÀÇ 20%¿¡¼­ ¹ß»ý

   (2) TA

3) trachea´Â midlineÀÇ left side·Î deviated

4) barium filled esophagusÀÇ Rt.border°¡ idented

Vascular Rings

1) vascular ringÀ» ³ªÅ¸³»´Â common anomaly

  (1) double aortic arch (Fig. 386-7 and 386-8)

  (2) right aortic arch with left ligamentum arteriosum

  (3) anomalous innominate a. arising further to the left on the arch than usual

  (4) anomalous left carotid a.arising further to the right than usual and passing

     ant. to the trachea

  (5) anomalous left pul.a. (vascular sling)

       abnormal vesselÀº elongated main pul. a. or right pul. a.¿¡¼­ arise

2) associated congenital heart disease

    5-20%

3) vascular ringÀÌ infancy¶§ trachea¿Í esophagus¸¦ ¾Ð¹ÚÇϸé symptom

  (1)wheezing respiration : crying, feeding, neck flexion ½Ã ½ÉÇØÁü

                          neck extension½Ã °æ°¨

  (2) vomiting

  (3) brassy cough and pneumonia

  (4) sudden death from aspiation

4) X-ray (Fig.386-8)

5) aberrant right subclavian a.°¡ common ÇÏ°Ô º¸ÀÌÁö¸¸ trachea¸¦ compressionÇÏÁö¾Ê´Â´Ù

6) Tx. and Px.

  (1) surgery

      trachea compressionÀÇ X-ray ¼Ò°ßÀÌ º¸ÀÌ´Â Áõ»óÀÌ Àִ ȯ¾Æ¿¡ ´ëÇØ¼­ ½Ç½Ã

  (2) severe tracheomalacia°¡ ³ªÅ¸³ª¸é poor Px.

7) Dx.

  (1) 2-D echocardiography

  (2) MRI

  (3) digital subtraction angiography

  (4) angiography during cardiac cath.

386.27 Anomalous Origin Of Coronary Arteries

Anomalous Origin Of The Left Coronary A. From The Pul. A.

# decreased pul. a. pr. after birth

    --> inadequate left coronary artery perfusion

    --> *reversed left coronary artery flow

    --> myocardial infarction and fibrosis

# occasionally interarterial collateral anastomosis

# Myocardial steal syndrome

3) ÇÕº´Áõ

  (1) mitral incompetence

     papillary m.ÀÇ infarction¿¡ ÀÇÇØ ¹ß»ý

  (2) localized aneurysm in LV

Clinical Manifestation

; *CHF within 1st few months

; often precipitated by respiratory infection

; recurrent attacks of discomfort, restlessness, irritability, sweating, dyspnea, pallor with or without cyanosis

; ûÁø¼Ò°ß

  ¨ç murmur : ejection type, non-specific

  ¨è Gallop rhythm

  ¨é continuous murmur : intercoronary anastomosis ÀÖ´Â old pt.¿¡ ¹ß»ý

Diagnosis

; X-ray

  - cardiomegaly(contour and pulsationÀº nonspecific)

; EKG

   ¨ç QR pattern followed by inverted T wave (lead I and aVL)

 ¨è deep Q wave and elevated ST segments and inverted T wave (V5 and V6)

  ¨é old pt.¿¡¼­´Â exercise study°¡ µµÀ½ÀÌ µÈ´Ù

     ST-T wave changes or symptomÀÌ ¹ß»ý

; aortography

  ¨ç diagnostic

  ¨è immediate opacification of right coronary a.

Treatment and Prognosis

  ¨ç ù 6°³¿ù³»¿¡ heart failure·Î »ç¸Á

  ¨è medical Tx.

     a. for heart failure

        diuretics, digoxin, captopril

     b. for controlling ischemia

        nitrates, calcium channel blochers, beta blocking agent

  ¨é surgical Tx.

     a. pul. a.¿¡¼­ anomalous coronary a.¸¦ ºÐ¸®

     b. anomalous coronary a.¸¦ aorta¿¡ anastomosis

Anomalous Origin Of The Right Coronary A. From The Pul. A.

1) infancy¿Í early childhood¿¡¼­´Â Áõ»óÀ» ³ªÅ¸³»Áö ¾Ê´Â´Ù

2) left coronary a. : enlarged

     right coronary a. : thin-walled and midly enlarged

3) right coronary a.ÀÇ early infancy perfusion : pul. a.¿¡¼­ origin

   right coronary a.ÀÇ later perfusion : collaterals of the left coronary vessel¿¡¼­origin

4) angina & sudden death

      adolescence or adult¿¡¼­ ¹ß»ý

5) Tx.

      right coronary a.¸¦ aorta¿¡ reanastomosis

Ectopic Origin of Coronary A. from The Aorta with Aberrant Proximal Course

1) aberrant a. : left, right, or major branch coronary a.

2) origin site

   ¨ç wrong sinus of Valsalva

   ¨è proximal coronary a.

3) ostium

       hypoplastic, slit-like, normal caliber

4) ostia hypoplasia¿¡ ÀÇÇÑ obstructionÀº aorta & RV outflow tract or

   interventricular septum »çÀÌ¿¡ tunnelÀ» Çü¼ºÇϰí acute angulationÀº

   myocardial fibrosis or myocardial infarctionÀ» À¯¹ßÇÔ

5) Áõ»ó

   ¨ç myocardial infarction

   ¨è ventricular arrhythmia

   ¨é sudden death

   ¨ê angina pectoris

   ¨ë syncope                    

386.28 Pul. Vascular Disease (=Eisenmenger Syndrome)

     # reversed or bidirectional shunt through a VSD as a result of pul. vascular obstructive            disease

     # assiciated with

         1) ASD

         2) A-V canal

         3) PDA

         4) other communication between the aorta and pul.a

     # pul.vascular resistance´Â early infancy¶§ Á¤»óÀûÀ¸·Î °¨¼ÒµÇ°í ³­ ÀÌÈÄ¿¡  high or rise            µÈ´Ù

       -> ÀÌ·¯ÇÑ Çö»óÀº ÀÌÂ÷ÀûÀ¸·Î prolonged elevated pul.pr.ÀÇ °á°ú·Î ¹ß»ýÇÏ¿© vessel¿¡

           severe obliterative intimal lesionÀ» ¾ß±â½ÃŲ´Ù.

     # pul. vascular disease  ÀÇ factor

        1) pul, a presÀÇ Áõ°¡

        2) pul. blood flowÀÇ Áõ°¡

        3) hypoxia

        4) hypercarbia

     # pul hypertension due to pul, blood flowÀÇ Áõ°¡

     # Eisenmenger¿¡¼­ pul. hypertensionÀº  pul. vascular disease ¶§¹®

Pathology And Pathophysiology

# occurs in small pul. arterioles and muscular arteries (*< 300um)

# ¡ÚHeath-Edwards Classification

    ; type I - medial thickening

  ; type II - medial and intimal thickening

    ; type III - type I, II plus plexiform lesions secondary to hypoplasia of medial layer of small muscular arteries

# ¢¾Physiologic Definitions

    ; absolute elevation of pul. artery resistance > 12 wood units/m2

    ; Rp/Rs > 1.0

# pul. vascular hypertension

         <1> trisomy 21ȯÀÚ¿¡¼­´Â Ãâ»ý ÈÄ Áï½Ã ¹ß»ý°¡´É

         <2> ÇÕº´Áõ

              1) pul. vascular pr.°¡ ³ôÀº ȯÀÚÀÇ natural history¸¦ complicated

              2) transmission of systemic pr. to the pul.circulation

              3) exposure to low PO2 (high altitude)

              4) high pul. blood flow from birth

Clinical Manifestation

2nd or 3rd decade±îÁö´Â Áõ»óÀÌ ³ªÅ¸³ªÁö ¾Ê´Â´Ù.

irreversible pul. vascular obstructionÀº high pul. vascular resistanceÀ» À¯¹ßÇÔ

pul. resistance°¡ systemic resistanceº¸´Ù Ŭ ¶§´Â Á¤»óÀûÀÎ left to right

 shunt°¡ right to left shunt·Î º¯ÇÒ ¼ö ÀÖ´Ù.

cyanosis, dyspnea, fatogue, dysrhythmia

late stage : heart failure, chest pain, syncope, hemoptysis

physical examination : right ventricular loud, narrowly split S2, soft ejection

 pul. a. pulsationÀÌ left upper sternal border¿¡¼­ palpable

  Graham Steel murmur (pul. valveÀÇ functional incompetence¿¡ ÀÇÇØ left sternal

              border¿¡¼­ µé¸®´Â blowing diastolic murmur)

Diagnosis

 cyanosis with polycythemia

 X-ray

 1) heart size : normal to enlarged

 2) main pul. a. : prominent

 3) hilar areaÀÇ pul. vessels : enlarged

    pul. vesselÀÇ peripheral branchÀÇ caliber : diminished

 4) RV and RA : prominent

 EKG

 1) RVH

 2) tall, spiked P wave

 echocardiography

 1) thick walled RV

 2) communication between the systemic and pul. circulation

 3) right sided systolic time interval : Áõ°¡

 catheterization

 1) defect site¿¡¼­ bidirectional rhunt

 2) systemic and pul.circulationÀÇ systolic pr.´Â equal

 3) pul. capillary wedge pr. : normal

 4) arterial oxygen saturation : °¨¼Ò

   #cf) vasodilator theraphy¿¡ ¹ÝÀÀÀ» º¸À̸é reversible pul. hypertensionÀ» ÀǹÌ

Treatment

¢ÞTable 386-2

Chapter 387. Cyanotic Congenital Heart Disease

387.1 Evaluation Of The Critically Ill Neonate With Cyanosis And Respiratory Distress

      Cardiac disease : CHD is responsible for cyanosis

                         obstruction to RV outflow ( RT to LT shunt )

                         anatomic defect

                         pul. edema

                         persistence of fetal pathway

      CNS disease : CNS depression¡æ irregular shallow breathing

                     ¡æ alveolar ventilation¡é

                     ¡æ lower alveolar oxygen tension

                     ¡æ PCO2¡é

                        ex) ICH

      pulmonary disease

         HMD, atelectasis, pneumonitis

         ¡æ inflammation, collapse, fluid accumulation in alveoli

         ¡æ incomplete oxygenation

      hemoglobinopathy

         methemoglobinemia

      DDx

         careful observation of infant breathing pattern : CNS

         hyperoxia test : pul. disease

         murmur : cardiac disease

         2-D echo : cardiac disease    

CYANOTIC LESIONS ASSOCIATED WITH DECREASED PUL. BLOOD FLOW

387.2 TOF

# ¡ÚConsist Of

    1) obstruction to right ventricular outflow(PS)

    2) VSD

    3) dextroposition of the aorta

    4) RVH

Pathophysiology

1) pul. valve : small ring (bicuspid), site of stenosis

2) crista supraventricularisÀÇ hypertrophy´Â infundibular stenosis¸¦ À¯¹ßÇÏ¿© 

   variable size and contourÀÇ infundibular chamber¸¦ Çü¼ºÇÏ°Ô µÈ´Ù.

3) PS°¡ ÀÖ´Â °æ¿ì pul. blood flow´Â aortaÀÇ collateral vessel·ÎºÎÅÍ °ø±Þ¹ÞÀ½

4) VSD ; non-restrictive, large, aortic valve ¹Ù·Î ¹Ø¿¡ Á¸Àç, related to the post.

         and  right aortic cusps

5) mitral and aortic valveÀÇ normal continuity´Â À¯Áö

6) aorta arches to the right(20%)

   aortic root : large, overrides VSD

 

(1) RA and RV·ÎÀÇ systemic venous returnÀº Á¤»ó

         (2) PS°¡ ÀÖ´Â °æ¿ì RV°¡ contractionÇϸé blood´Â VSD¸¦ ÅëÇØ aorta·Î °¡°Ô µÈ´Ù.

            ÀÌ °á°ú·Î persistent arterial desaturation and cyanosis°¡ ¹ß»ýÇÑ´Ù.

         (3) PS¿¡ ÀÇÇØ pul. blood flow°¡ ½ÉÇÏ°Ô restictionµÇ¸é, pul. blood flow´Â bronchial  

             collateral circulation and PDA¿¡ ÀÇÇØ °ø±Þ¹Þ°Ô µÈ´Ù.

         (4) RV outflow obstrctionÀÇ Á¤µµ¸¦ °áÁ¤ÇÏ´Â ¿ä¼Ò

             1) severity of cyanosis

             2) presence of RVH

             3) acyanotic or pink TOF : RV flow obsructionÀÌ moderateÇÒ °æ¿ì VSD¸¦

               ÅëÇØ balanced shunt°¡ ÀÖÀ¸¸é ȯ¾Æ´Â û»öÁõÀ» º¸ÀÌÁö ¾Ê´Â´Ù.

Clinical Manifestation

Cyanosis

1) °¡Àå ÇöÀúÇÑ Áõ»ó

2) Ãâ»ý½Ã¿¡´Â º¸Åë ³ªÅ¸³ªÁö ¾ÊÀ½. infant´Â RV outflow obstrctionÀÌ ½ÉÇÏÁö ¾Ê°í large left to right shunt and CHF¸¦ ³ªÅ¸³»°³ µÈ´Ù.

3) º¸Åë 1¼¼¸» °æ ³ªÅ¸³² : lips and mouthÀÇ mucous membrane, fingernails, toenails¿¡ ÇöÀúÇÔ

Dyspnea

          - occur on exercise

            physical effort¿¡ ÀÇÇÑ dyspneaÇØ¼Ò¸¦ À§ÇØ squatting positionÀ» ÃëÇÔ.

Paroxysmal Hypercyanotic Attacks(Hypoxic Or Blue Spells)

    ; particular problem *during 1st 2yr of life

    ; Áõ»ó

           - hyperpneic and restless, cyanosis increase, gasping respirations ensure, syncope

           - if severe spells

                   / unconsciousness, convulsion, hemiparesis

    ; *¡ãfrequently in the morning upon first awakening or following episodes of vigorous crying

    ; disappearance or decrease in systolic murmur due to decreased RVOT blood flow

    ; last a few minutes to a few hours

    ; *rarely fatal

    ; only mildly cyanotic infant --> more prone to develop hypoxic spell

    ; ¢ÞÄ¡·á

         - knee chest position with no constricting clothing

         - administration of oxygen

         - morphine sc

                   / *not in excess of 0.2mg/kg

         - correction of metabolic acidosis

             / *PaO2 below 40mmHg¡æ immediately bivon iv

         - *beta-adrenergic blockade(propranolol)

                   / *0.1-0.2mg/kg iv

                   / in severe spell, esp. with tachycardia

           - *methoxamine, phenylephrine

                 / increase of systemic vascular resistance

Delayed Growth And Development

Physical Examination

    ; pULSE : normal

    ; SYSTOLIC THRILL ; 3rd and 4th parasternal spaceÀÇ left sternal border¸¦

                          µû¶ó Àß ´À²¸Áü

    ; ûÁø¼Ò°ß

systolic murmur

 (1) left sternal border¿¡¼­ Àß µé¸²

 (2) RV outflow tractÀ» ÅëÇÑ turbulence¿¡ ÀÇÇØ ¹ß»ý

 (3) severe obsruction and large right to left shunts½Ã less prominent

S2 ; single, aortic valve closure¿¡ ÀÇÇØ ¹ß»ý

continuous murmur

 ¹ß»ý¿øÀÎ (1) enlarged bronchial collateral vessels

         (2) persistent PDA(rarely)

# X-ray

A-P view

 (1) narrow base

 (2) pul. a. areaÀÇ left border°¡ concavity

 (3) normal heart size

   (4) RVH¿¡ ÀÇÇØ diaphragmÀÇ º¸´Ù À§ÂÊ¿¡ À§Ä¡ÇÑ rounded apical shadow

 (5) coeur en sabot;cardiac silhouetteÀÌ wooden shoe¸ð¾çÀ» ³ªÅ¸³»´Â °Í

Lat.view

 (1) hilar areas and lung fields°¡ relatively clear

      ¿øÀÎ 1) pul.blood flowÀÇ °¨¼Ò

           2) small size of pul. a.

        3) aorta : large, 20%¿¡¼­ right side·Î arches

                  -> indentation of the leftward positioned air-filled

                     tracheobronchial shadow°¡ ¹ß»ýÇÔ

        4) poststenotic dilatation of pul. a.

             valvular pul.stenosis¸¦ ÀǹÌÇÔ

2> EKG

     1) RAD

     2) RVH

     3) P wave;tall and peaked,sometimes bifid

3> Two-dimensional echocardiography

4> cardiac cath.

     1) RV=LV(systolic Pr.)

     2) mean pul. a. Pr. : 5-10mmHg

     3) RA Pr. : normal

     4) VSD¸¦ ÅëÇØ ¿ì½É½Ç¿¡¼­ ½±°Ô ´ëµ¿¹éÀ¸·Î µé¾î°¥ ¼ö ÀÖ´Ù.

     5) a.oxygen saturationÀº right to left shuntÀÇ ¾ç¿¡ µû¶ó °áÁ¤µÈ´Ù; moderate                     cyanosis ȯ¾Æ°¡ È޽Ľà PAO2´Â 75-85%(Á¤»óÀº 95%)]

        <5> selective right ventriculography

             TOFÀÇ anatomy¸¦ Àß º¼ ¼ö ÀÖ´Ù.

        <6> Left ventriculography

             ventricle size, VSD À§Ä¡, overriding aorta

        <7> Aortography or coronary arteriography

Complications

Cerebral Thrombosis

   1) site ; º¸Åë cerebral veins or dural sinuses, ¶§·Î´Â cerebral arteries

   2) extreme polycythemia, dehydration½Ã¿¡ Àß ¹ß»ý

cerebral ishemia

   1) 2¼¼ ÀÌÇÏ¿¡¼­ ÈçÈ÷ ¹ß»ý

   2) Hb.and Hct.´Â Á¤»ó¹üÀ§À̳ª ¶§  ·Î iron deficiency anemia¸¦ µ¿¹ÝÇÔ

   3) Ä¡·á

     (1) adequate hydration

     (2) supportive measures

     (3) phlebotomy and volume replacement with FFP : extremely polycythemia¿¡

                                                       Àû¿ë

     (4) heparinÀº °ÅÀÇ È¿°ú°¡ ¾ø´Ù.

     (5) physical theraphy : affected extremity¿¡ °¡´ÉÇÑ »¡¸® ½Ç½Ã

Brain Abscess

    ; less common than cerebral vascular events

    ; over 2yr

    ; insidious with low-grade fever and/or behavior change

    ; some acute onset of headache, nausea, vomiting

    ; epileptiform seizures

    ; elevated  ESR & WBC

    ; Ä¡·á

           - massive antibiotic theraphy

    - *surgical drainge

                   / *almost necessary

Bacterial Endocarditis

 1) ¼ö¼úÇÏÁö ¾ÊÀº ȯ¾ÆÀÇ pul., aortic, or rarely tricuspid valveÀÇ infundibulum¿¡¼­ ¹ß»ý

   2) dental or surgical procedures ÀüÈÄ¿¡ Ç×»ýÁ¦ ¿¹¹æ¿ä¹ýÀÌ ÇʼöÀû.

CHF

   1) pul. atresia and large collateral blood flow¸¦ °¡Áø young infant¿¡¼­ ¹ß»ý

       -> »ýÈÄ Ã¹ 1´Þ³»¿¡ »ç¶óÁü

       -> ȯ¾Æ´Â pul.blood flow °¨¼Ò¿Í ÇÔ²² cyanosis¸¦ ³ªÅ¸³¿  

   2) TOFÀÇ ÈçÇÑ Áõ»óÀº ¾Æ´Ï´Ù.

Associated Cardiovascular Anomalies

    ; PDA

    ; ASD

    ; absence of the pul.valve

   1) mild cyanosis

   2) heart;large and hyperdynamic

   3) loud to-and-fro murmurµé¸²

   4) neonatal period¿¡ Ä¡¸íÀûÀ̳ª, ¶§·Î spontaneous regressionÇÏ´Â °æ¿ìµµ ÀÖ´Ù.

    ; absence of a pul.a.

   1) left pul.a.°¡ ¾ø¾î right lungÀº more vascularized

     -> X-ray»ó ¾çÂÊ pul.vasculature°¡ ´Ù¸¦ ¶§ ¹Ýµå½Ã ÀǽÉÇØ¾ßÇÔ

   2) affected lungÀÇ hypoplasia¸¦ µ¿¹ÝÇÔ

    ; right aortic arch     

    ; multiple VSD

    ; atrioventricular canal;Down syndrome½Ã Àß µ¿¹ÝµÊ

    ; absence of branch pul. a

Treatment

         depends on the severity the RV outflow

1>¸ñÀû ; Áï°¢ÀûÀ¸·Î pul. blood flow¸¦ Áõ°¡½ÃÄÑ severe hypoxiaÀÇ sequelea        

         ( shock, resiratory failure, intractable acidosis )¸¦ ¹æÁöÇÔ

2> medical treatment

  1) PEG1 (0.05-0.20ug/kg/min)

    (1) ductal smooth m.ÀÇ potent and specific relaxant·Î¼­ ductus arteriosus¸¦

       È®Àå½ÃÅ´->adeqate pul. blood flow

    (2) long-term theraphy·Î´Â »ç¿ëÄ¡¾ÊÀ½

  2) dehydrationÀÇ ¿¹¹æ ¹× Áﰢġ·á

      hemoconcentration and possible thrombotic episodes¸¦ ¹æÁöÇÔ

  3) iron deficiency anemia¸¦ Ä¡·á

        Hct.´Â 55-65%·Î À¯Áö

  4) oral propranolol(1mg/kg every 6hrs)

      dyspneic spellsÀÇ frequency and severity¸¦ °¨¼Ò½ÃÅ´ 

3> surgical treatment

    1 ) 1st month of life³»¿¡ severe cyanosis°¡ ÀÖÀ» ¶§ systemic-pul. a .shunt·Î¼­

        pul. a. blood flow¸¦ Áõ°¡½ÃÄÑ hypoxia¸¦ ÇØ¼Ò½Ã۰í small pul. vesselÀÇ ¼ºÀå À» µµ¿î´Ù

  (1) modified Blalock-Taussing shunt

      most common aorto-pul shunt procedure

      side to side anastomosis subclavian a. to branch of pul. a

  (2) Blalock-Taussing shunt

      subclavian a.-pul. a.ÀÇ homolat.branchÀÇ direct anastomosis

  (3) Waterson shunt

      1.ascending aorta.-right pul. a.

  (4) Potts shnt

      1. upper descending aorta-left pul. a.

      2. rarely done

      3. complication (CHF, late-onset pul. hypertension)ÀÌ Àß ¹ß»ý

  (5)¼º°øÀûÀ¸·Î shunt op.µÇ¾úÀ» °æ¿ì

      1. cyanosis°¨¼Ò

      2. machinery type murmur¹ß»ý

  (6) systemic to pul.a.shunt½Ã infective endocarditis°¡ Ä¡¸íÀûÀ̹ǷΠ¿¹¹æ¿ä¹ýÀÌ ÇÊ¿äÇÏ´Ù

  (7) thoracotomyÈÄÀÇ ÇÕº´Áõ

      1. chylothorax

      2. diaphragmatic paralysis

      3. Horner syndrome

      4. cardiac failure

 2 ) corrective surgery

     (1) ¹æ¹ý

         1. RV outflow tract obstructionÀ» ÇØ¼Ò

         2. closure of VSD

     (2) risk´Â 5%ÀÌÇÏ

     (3) ¼ö¼úÀÇ ¼º°ø·üÀ» ³ôÀÌ´Â ¿ä¼Ò

         1. optimal total body perfusion

         2. adequate myocardial protection during bypass

         3. RV outflow obstructionÇØ¼Ò

         4. air embolism ¹æÁö

         5. meticulous postoperative care

     (4) ÇÕº´Áõ

         1. pul. valve insufficiency

         2. working capacity, maximal heart rate, cardiac output °¨¼Ò

         3. conduction disturbance

Prognosis

immediate postoperative problems

1) RV failure

2) transient heart block

3) residual VSD with left to right shunting

4) myocardial infarction from manipulation of an aberrant coronary a.

5) residual collateals¿¡ ÀÇÇØ ºÒ±ÕÇüÀûÀ¸·Î LA Pr.Áõ°¡

TOF repairÈÄ¿¡´Â premature ventricualr beats°¡ ¹ß»ýÇÒ ¼ö Àִµ¥ À̰ÍÀº

 benign and nonprogressiveÇÏ´Ù

 È޽Ľà ³ªÅ¸³ªÁö ¾Ê´Â ºÎÁ¤¸ÆÀº exercise study·Î¼­ ã¾Æ³¾ ¼ö ÀÖ´Ù

ventricular ectopyÄ¡·á

  quinidine, propranolol, dilantin, or combinations of these agents

387.3 Pulmonary Atresia With Ventricular Septal Defect

; extreme form of TOF

; pul. valve

    - atretic, rudimentary, or absent

; pul. trunk

    - atretic or hypoplastic

Clinical Manifestation

; cyanosis within 1st few hr or days

    - *mostly severe cyanotic

           --> *require urgent PGE1 infusion and palliative surgicl intervention

# ûÁø¼Ò°ß

    ; *absent systolic murmur

    ; S1

           - followed by an ejection click by enlarged aortic root

    ; S2

           - *moderately loud and single

    ; continuous murmur

           - audible over entire precordium

# X-Ray

    ; small or enlarged heart

       - depending on pul. blood flow

    ; concavity at the position of the pul.arterial segment

  ; often reticular pattern of bronchial collateral flow

# Electrocardiogram

    ; *RVH

# Echocardiogram

    ; aortic override

  ; thick right ventricular wall

  ; pul. atresia

# Cardiac Catheterization

    ; right ventriculography

    ; large aorta opacified immediately by passage of the contrast medium through the septal defect

    ; no dye entering the lungs through the right ventricular out flow tract

    ; pul. blood flow from aorta to lungs

Treatment

; surgical procedure depend on there is adequate main pul. a segment and on the size of the branch pul. a

; option

    - aortopulmonary shunt

    - connection from the RV to pul. a

387. 4 Pulmonary Atresia With Intact Ventricualr Septum

; pul. valve leaflets

    - completely fused to form a membrane

; pul. blood flow

    - only supply via PDA

; Rt. ventricle

    - *usually hypoplastic

    - small right ventricular cavity

           / small TV annulus

           / sinusoid channel within Rt. ventricular wall

                   --> coronary arterial circulation communication

    - intermediate size or large ventricle cavity

           / tricuspid insufficiency

Clinical Manifestation

; markedly cyanosis

    - ductus arteriosus°¡ ´ÝÈú ¶§

# ûÁø ¼Ò°ß

    ; *loud single S2

    ; *no murmurs

    ; *sometimes systolic or continuous murmur due to ductal blood flow

# electrocardiogram

  ; *QRS axis - 0 ~ +90 degree

    ; tall, spiked P waves due to right atrial enlargement

    ; *left ventricular dominance or hypertrophy

# X-Ray Findings

    ; variable heart size with *marked decreased pul.vascularity

# Cardiac Catheterization

    ; right atrial and right ventricular hypertension

  ; ventriculography

           - size of the ventricular cavity

        - atretic right ventricular outflow tract

         - the degree of ticuspid regurgitation

           - intramyocardial sinusoid filling the coronary vessel

Treatment

1> urgent medical and surgical managementÇÏ¿© ¿¹ÈÄ´Â °³¼±µÇ¾ú´Ù.

2> PGE1;interventionÀü¿¡ Åõ¿©Çϸé ductus openingÀ» À¯ÁöÇÏ¿© ¼ö¼ú Àü¿¡

    hypoxemia and acidemia¸¦ °¨¼Ò½ÃŲ´Ù

3> pul. valvotomy

    outflow obstructionÀ» ÇØ¼Ò

    ÀÌ ½Ã¼ú±â°£µ¿¾È¿¡ adequate pul.blood flow¸¦ À¯ÁöÇϱâ À§ÇØ systemic

        - pul. arterial anastomosis¸¦ ½ÃÇàÇÑ´Ù.

4> unroofing the outflow tract and patch grafting

     ¼ö¼ú ¸ñÀûÀº right ventricular chamber ¼ºÀåÀ» µµ¿Í forward flow¸¦ ÁÁ°ÔÇϴ°ÍÀÌ´Ù.

5> more extensive valvutomy & shunt op.

     ³ªÁß¿¡ ½Ç½ÃÇÔ

6> Fontan procedure

    - right ventricular chamber°¡ ÀÛÀº °æ¿ì¿¡ ½Ç½ÃÇÏ¿© right atrium¿¡¼­ pul. a.·Î Á÷Á¢ blood flow¸¦ È帣°Ô ÇÏ´Â ¹æ¹ý

7> myocardial sinusoids¸¦ ÅëÇØ right ventricleÀ» °ÅÃÄ coronary perfusionÀÌ ÀÖÀ¸¸é ¿¹ÈÄ´Â ÁÁÁö ¾Ê´Ù.

387.5 Tricuspid Atresia

Pathophysiology

; entire systemic venous return

    --> foramen ovale or ass. with ASD

    --> left heart

; pul.blood flow

    - *depend on size of VSD and presence & severity of PS

    - *maybe depend on PDA

; *if intact ventricular septum, right ventricle is completely hypoplastic with pul. atresia

; decreased pul. blood flow, cyanosis in early months

; less often *VSD in absence of right ventricular outflow obstruction

    - *high pul. flow

    - mild cyanosis & congestive heart failure

; one variant

    - *ass. with TGA(30%)

Clinical Manifestations

    ; cyanosis at birth

    ; polycythemia, easy fatigability, exertional dyspnea, occasional hypoxic episodes

    ; 85% diagnosis before 2mo

    ; spontaneous VSD closure

           --> cyanosis¸¦ ¾ÇÈ­½ÃÅ´

# ûÁø¼Ò°ß

  ; *holosystolic murmurs along audible the left sternal border

  ; *single S2

# X-ray finding

    ; pul. undercirculation - normal related great vessels

    ; overcirculation - TGA

# Electrocardiogram

    ; ¢ÞLAD, LVH

    ; prominent R wave --> rS complex in right precordial leads

    ; qR complex followed by normal flat diphasic or inverted T wave in left precordial leads

    ; RV6 is normal or tall

    ; SV1 is generally deep

    ; P wave

           - biphasic with the initial component tall and spiked in lead II

# ¡ÚCyanosis with LAD

    --> *highly suggestion of TA

# Two-dimensional echocardiogram

¨ç absence of the tricuspid valve

¨è the small right ventricle

¨é large left ventricle and aorta

# Cardiac Catheterization

 right atrial pr.´Â Á¤»ó ȤÀº ¾à°£ Áõ°¡µÇ¾î ÀÖÀ¸¸é¼­ prominent "a" wave¸¦ º¸ÀÓ

Treatment

# PGE1

# Surgical Aorto-Pulmonary Shunt Procedure

    ; *Blalock-Taussing procedure(or its variations)

         - preferred

    ; Rashkind balloon atrial septostomy(BAS)

# Palliative Surgery

    ; Glenn anastomosis(right sup.vena cava to right pul. a.)

           - *¡ãoften performed after outgrowing sign of previous aorto-pulmonary shunt

           - *4-12mo

           - benefits

                   / reduces volume work on the Lt ventricle

                   / lessen chances of developing LV dysfunction

           - disadvantages

                   / SVC syndrome

                   / spontaneous closure of shunt

# Later surgical management

    ; modified Fontan operation

           - *1.5-3yr

           - caval-pulmonary isolation procedure

           - advantages

                   / decrease possibility of RA dilatation

                   / reduce incidence of postoperative pleural effusion

           - ¢¾Contraindication

                   / very young infants

           / elevated pul. vascular resistance( >4 wood units/m2)

           / pul. a. hypoplasia

           / LV dysfunction

           - *patients´Â ¹Ýµå½Ã sinus rhythm, no MIÀ̾î¾ß ÇÑ´Ù.

    ; ¡ÚPostoperative Problem After Fontan Procedure

    - marked elevated systemic venous pr.

    - fluid retention

           - pleural or pericardial effusion

         / pleural effusion persist more than 3wks 30-40% of patients

    ; modified procedureÀ» »ç¿ëÇϸé, 5%·Î °¨¼ÒµÈ´Ù.

    ; ¢¾Late Complication Of Fontan Procedure

       - residual obstruction

            / sup. or inf. vena caval syndrome

           - vena caval or pul. a. thromboembolism

           - protein-loosing enteropathy

    - supraventricular arrhythmias

            / atrial flutter, paroxysmal atrial tachycardia

           - sudden death

387. 6 DORV With PS

     Ư¡ : aorta and pul.a.°¡ right ventricle¿¡¼­ origin

             left ventricleÀÇ outlet´Â VSD

       aortic and mitral valve´Â ¿¬°áµÇ¾î ÀÖÁö ¾Ê´Ù.

       VSD´Â crista supraventricularis ¹Ø¿¡ À§Ä¡ÇÔ

       physiology´Â TOF¿Í À¯»çÇÔ

       two-dimensional echocardiography : anatomy, double outlet right ventricle,                              mitral-aortic valve discontinuity¸¦ ³ªÅ¸³¿

       operation : intraventricular channelÀ» ¸¸µé¾î left ventricle¿¡¼­ VSD¸¦ ÅëÇØ aorta¸¦

                  ÅëÇØ blood¸¦ aorta·Î ejectionÇϰÔÇÔ.

                  pul. obstructionÀº pul. or aortic homograft·Î½á ÇØ¼Ò½Ãų ¼ö ÀÖ´Ù.

                  aortic pul. shunt·Î½á Áõ»óÀ» °³¼±½Ãų ¼ö ÀÖ´Ù.

387. 7 TGA With VSD And PS

      TOF¿Í À¯»ç

      obstruction site

         <1> valvular

         <2> subvalvular

                successful atrial septostomy or pul.arterial bandingÈÄ¿¡ Àß ¹ß»ý

      clinical manifestation

          cyanosis, decreased exercise tolerance, poor physecal develpment

 

      enlarged heart

      pul. vascularity : normal

      EKG

         <1> RAD 

         <2> RVH and LVH

         <3> tall spiked P waves

      cardiac catheterization

         <1>low pul. a. pr.

         <2> oxygenation saturation : pul. a.> aorta

      selective right and left ventriculography

         <1> aorta´Â RV¿¡¼­ origin

         <2> pul. a.´Â LV¿¡¼­ origin

         <3> VSD

         <4> PS

      Tx.

        <1> systemic-pul.arterial shunt ÇÊ¿äÇϸé ÈÄ¿¡ neonatal BAS

        <2> Rastelli operation

              1) 2-6¼¼ »çÀÌÀÇ ¾î¸°ÀÌ¿¡ ½ÃÇà

              2) ¹æ¹ý

               (a) VSD¸¦ patch closure½ÃÄÑ left ventricular flow¸¦ aorta·Î ÇâÇÏ°Ô ÇÑ´Ù.

               (b) proximal pul. a. ligation°ú RV°ú distal pul. a. »çÀÌ¿¡ extracardiac

                   homograft¸¦ ÇØ¼­ RV°ú pul.a.¸¦ ¿¬°á½ÃŲ´Ù

387. 8 Ebstein Disease

; downward displacement of an abnormal tricuspid valve into the RV

    - ant. cusp of valve to valve ring

    - other leaflets to RV wall

; RV divided two part by abnormal valve

    - first

           / thin walled atrialized portion

           / continuous with cavity of RA

    - second

           / normal ventricular myocardium

; RA huge

; TV usually regurgitant

# *Decreased Right Heart Output(=Functional Pulmonary Atresia)

    ; poorly functioning small RV

  ; TV regurgitation

  ; obstruction due to large, sail-like, ant.tricuspid leaflet

; *right to left shunting through foramen ovale

Clinical Manifestations

; severity depend on degree of displacement of TV & Rt. outflow tract obstruction

; *mostly

    - mild symptom only fatigue

; ¡Úcardiac dysrhythmia

    - extrasystole

           / *¡ãcommon

    - *paroxysmal tachycardia (usually supraventricualr)

; cyanosis and polycythemia

; normal or increased venous pr.

; quiet precordium

# ûÁø ¼Ò°ß

    ; holosystolic murmur

           - audible over ant. left side of chest due to tricuspid regurgitation

    ; gallop rhythm

    ; scratchy diastolic murmur

; *some aymptomatic until well into adults

; often cya