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