Congenital Heart Disease Flashcards
Right to left shunt: Truncus arteriosus fails to divide into pulmonary trunk and aorta due to lack of aorticopulmonary septum formation; most Px have accompanying VSD. What is this called?
Persistent truncus arteriosus.
Right to left shunt: Aorta leaves RV and pulmonary trunk leaves LV –> separation of systemic and pulmonary circulations. Not compatible with life unless a shunt is present. *Associated with maternal diabetes. Que es?
D-transposition of great vessels.
Right to left shunt: Absence of tricuspid valve and hypoplasic RV. What treatment is needed for survival?
Tricuspid atresia
Requires both ASD and VSD for viability.
Left to right shunt: Most common congenital cardiac defect. Asymptomatic at birth, may manifest weeks later or remain asymptomatic throughout life. Most self resolve; larger lesions may lead to LV overload and HF.
Ventricular septal defect.
- Holosystolic murmur
- Assoc. with FAS
Left to right shunt: defect in interatrial septum. Loud S1; wide, fixed split S2. Ostium secundum defects most common and usually occur as isolated findings. Ostium primum defects rarer yet usually occur with other cardiac anomalies. Symptoms range from none, to HF. Distinct from patent foramen foramen ovale in that septa are missing tissue rather than infused.
Atrial Septal Defect (ASD)
In fetal period, shunt is R–>L (normal). In neonatal period, decreased lung resistance –> shunt becomes L–>R; progressive RVH and/or LVH and HF.
Assoc. with machine-like murmur. Patency is maintained by PGE synthesis and low O2 tension. Uncorrected ___ can eventually result in late cyanosis in the lower extremities (differential cyanosis).
Patent Ductus Arteriosus (PDA)
Tx- Indomethacin… PGE keeps it open.
PDA normal in utero, and normally closes only after birth.
Left to right shunt: Uncorrected L–>R shunt causes increased pulmonary blood flow. This causes remodeling of vasculature–> pulmonary arterial HTN. RVH occurs to compensate –> shunt becomes R–>L. Late cyanosis, clubbing, and polycythemia. Age of onset varies.
Eisenmenger Syndrome
Tx- indomethacin
Hypertension in upper extremities and weak delayed pulse in lower extremities (brachial femoral delay). Eventually collateral arteries erode ribs (notched appearance in CRX). Aortic narrowing near insertion of ductus arteriosus (justaductal). Assoc. with bicuspid aortic valve, other heart defects, and Turner syndrome.
Coarction of the aorta
-infantile form assoc. with a PDA.
Caused by anterosuperior displacement of the infundibular septum. Most common cause of childhood cyanosis.
1-pulmonary infundibular stenosis
2-R ventricular hypertrophy - boot shaped heart
3-Overriding aorta
4-VSD
Improves with squatting, Tx-early surgical correction
Tetralogy of Fallot
Pulmonary veins drain to SVC, Coronary sinus, or into the right heart circulation (SVC, coronary sinus, etc.); without a shunt patient will die because circulation is a closed loop. What pathology is at hand? What needs to be done to maintain life?
Total anomalous pulmonary venous return (TAPVR)
You need an ASD and sometimes PDA to flow from R–>L shunting to maintain some CO.
Defects with cardiac congenital associations: VSD, PDA, ASD, tetralogy of fallot
Disorder: Alcohol exposure in utero (FAS)
Defects with cardiac congenital associations: Septal defects, PDA, pulmonary artery stenosis.
Disorder: Congenital Rubella
Defects with cardiac congenital associations: AV Septal defect (endocardial cushion defect), VSD, ASD
Disorder: Down Syndrome
Defects with cardiac congenital associations: Transposition of great vessels.
Disorder: Infant of diabetic mother
Defects with cardiac congenital associations: MVP, thoracic aortic aneurysm and dissection, aortic regurgitation.
Disorder: Marfan Syndrome
