Part 20. Cardiovascular System
PART 20. The Cardiovascular System
Section 1. Evaluation of the Cardiovascular System
Chap. 380. History and Physical Examination
#
cardiovascular disorders°¡ ÀǽɵǴ ȯ¾Æ¿¡ ÀÖ¾î¼ 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 ¾Çȵǰųª »ç¶óÁü
-
*jugular venous systemÀ»
°¡º±°Ô ¾Ð¹ÚÇÔÀ¸·Î¼
¾àȵÊ
/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
ù³¯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־ ¿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 output˼ Lt ventricular output ˂
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 ¾ÇȵȴÙ.
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, ÁÖ·Î 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¿¹ÝÇÏ¸é¼ 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» ¾ÇȽÃŰ´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
vessel˼ 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 pulsation˼ 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°¡ ½ÉÇϰÔ
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 saturation˼
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 lung˼ 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¸¦ ¾ÇȽÃÅ´
#
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