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Congenital heart disease in the adults

1. ¿øÀÎ

10%¹Ì¸¸¿¡¼­ chromosomal abnormality¿Í °ü·Ã:Down's, Turner's, trisomy 13-15, 17-18

Hurler's syndrome, homocystinuria, or type II glycogen storage ds

´ëºÎºÐ multifactorial and environmental

2. º´Å»ý¸®

1) pul. hypertension

* Eisenmenger syndrome

high-resistance & obstructive pul. hypertension¶§¹®¿¡ bidirectional or

predominantly Rt-to-Lt shunt¹ß»ý.

Ä¡·á¹æ¹ý ¾ø´Ù.

À¯ÀÏÇÑ Ä¡·á ; single lung transplantation with intracardiac defect repair

or total heart-lung transplantation

2) erythrocytosis

¨ç chronic hypoxemia -> erythropoietin»ý»êÁõ°¡ -> erythrocytosis(polycythemia)

¨è compensated erythrocytosis with iron-replete equilibrium hematocrit

: Hct < 65%¿¡¼­ hyperviscosityÁõ»óÀ» °ÅÀÇ ÀÏÀ¸Å°Áö ¾ÊÀ¸¸ç 70%À̻󿡼­µµ

°¡²û¾¿ ÀÏÀ¸Å²´Ù.

therapeutic phlebotomy°¡ ÇÊ¿äÇÑ °æ¿ì´Â µå¹°´Ù.

¨é decompensated erythrocytosis

: Hct»ó½Â, recurrent hyperviscosity sxÀ¯¹ß.

therapeutic phlebotomy°¡ ÇÊ¿äÇÒ ¼öµµ ÀÖÀ¸³ª iron depletion½Ãų¼ö ÀÖÀ¸¹Ç·Î

ÁÖÀǰ¡ ÇÊ¿äÇÏ´Ù.

¨ê hemostasis¿¡ ÀÌ»óÀÌ ¿À´Âµ¥ ±× ÀÌÀ¯´Â

i) blood volumeÀÌ Áõ°¡ÇÏ¿© capillary°¡ engorgeµÇ°í

ii) platelet fx¿¡ ÀÌ»óÀÌ ¿À°í, aspirin or NSAID¿¡ ´ëÇÑ sensitivity¿¡ ÀÌ»óÀÌ ¿À¸ç

iii) extrinsic & intrinsic coagulation system¿¡ Àå¾Ö¸¦ ÃÊ·¡Çϱ⠶§¹®ÀÌ´Ù.

¨ë oral contraceptive´Â vascular thrombosis risk¸¦ Áõ°¡½ÃŰ¹Ç·Î ±Ý±âÀÌ´Ù.

¨ì stroke risk : 4¼¼ ¹Ì¸¸¿¡¼­´Â ³ôÀ¸³ª ¼ºÀο¡¼­´Â ³·´Ù.

4¼¼ ¹Ì¸¸¿¡¼­ÀÇ stroke- cyanotic heart disease + iron depletion¿¡¼­

³ô´Ù. dehydrationÀÌ À¯¹ßÀÎÀÚ°¡ µÈ´Ù.

¼ºÀο¡¼­ : phletobomy¸¦ ½ÉÇÏ°Ô Çϰųª aspirin & anticoagulant¸¦ ºÎÀûÀý

ÇÏ°Ô Åõ¿©ÇÏÁö ¾ÊÀ¸¸ç atrial arrhythmia or infective endocarditis

°¡ ¾ø´Ù¸é ³ôÁö ¾Ê´Ù.

¨í ¿Ü·¡ ȯÀÚ¿¡°Ô¼­ phlebotomy½ÃÇà½Ã 500 ml blood over 45min + isovolumetric

replacement with isotonic saline(CHF¶§´Â 5% D/W)

3) pregnancy

»ê¸ð : pul. hypertension(Eisenmenger's physiology or MS) or LVOT

obstruction(AS)¿¡¼­ À§ÇèÀÌ Áõ°¡Çϰí heart failure or hemodynamically

important arrhythmia¸¦ ÀÏÀ¸Å°´Â malformation¿¡¼­ »ç¸ÁÀ§ÇèÀÌ ÀÖ´Ù.

ÅÂ¾Æ : maternal cyanosis, heart failure, pul. hypertension¶§ À§ÇèÀÌ Áõ°¡ÇÑ´Ù.

aortic coarctation, Marfan syndrome¶§ dissection À§ÇèÀÌ Áõ°¡ÇÑ´Ù.

µû¶ó¼­ cyanotic heart disease, pul. hypertension, Marfan syndrome¶§´Â ÀÓ½ÅÀÌ

±Ý±âÀÌ´Ù. ±×·¯¹Ç·Î À̶§´Â ÇÇÀÓÇØ¾ß ÇÑ´Ù. => tubal ligation

4) infective endocarditis

dental procedure, GI & GU surgery, diagnostic procedure(proctosigmoidoscopy &

cystoscopy)¶§ recommend

5) exercise

LVOT obstruction or pul. vascular resistance -> syncope, sudden death

TOF, isotonic exercise-induced decrease in systemic vascular resistance

=> RV outflow obstruction¿¡¼­ Rt-to-Lt shunt¸¦ Áõ°¡½ÃÄÑ

hypoxiaÁõ°¡ -> subjective breathlessness

Specific cardiac defects * Tab 234-3

(1) Acyanotic with Lt-to-Rt shunt

¨ç ASD, ASD with MS(Lutembacher's syndrome)

PAPVC(partial anomalous pul. venous connection)

¨è VSD, VSD with AR, VSD with LV to RA shunt

¨é Aortic root to Rt heart shunt:

valsalva sinus aneurysm rupture, coronary AV fistula,

anomlous origin of the Lt coronary a. from the pul. trunk

¨ê Aortopul. level shunt : A-P shunt, PDA

¨ë multiple level shunt

(2) Acyanotic without shunt

Lt heart malformation ; congenital obstruction to LA inflow

pul. vein stenosis, MS, cor triatriatum

MR, primary dilated endocardial fibroelastosis, AS, AR, COA

(3) Cyanotic

¨ç pul. blood flowÁõ°¡ : TGA, double-outlet RV, total anomalous pul. venous

connection, TOF with pul. atresia

¨è pul. blood flow Á¤»ó ȤÀº °¨¼Ò : Ebstein's anomaly with Rt-to-Lt shunt

Acyanotic congenital heart disease with a Lt-to-Rt shunt

1. ASD F>M

1) Type

¨ç sinus venosus type : SVC¿Í RA junction±Ùó¿¡ »ý±è

Rt lung¿¡¼­ ³ª¿À´Â pulmonary vein°ú anomalous connection

¨è ostium primum type : AV septal defect, AV valve±Ùó¿¡ ÀÌ»ó

Down syndrome°ú °ü·Ã

¨é ostium secundum type : most common

fossa ovalisħ¹ü, midseptum

30´ë ÀÌÈÄ¿¡ Áõ»óÀÌ ³ªÅ¸³­´Ù: atrial arrhythmia, pul. a. hypertension, bidirectional &

Rt-to-Lt shunt, heart failure

2) P/E

midsystolic pul. ejection murmur

S2 : widely split, fixed

middiastolic rumbling murmur : 4th intercostal space, Lt sternal border