congenital heart disease

Question 1

What are the right-to-left shunts?

Answer 1

5 Ts:
truncus arteriosus (1 vessel), transposition (2 switched vessels), tricuspid atresia (tri), tetralogy of fallot (4), TAPVR (total anomalous pulmonary venous return)

Question 2

persistant truncus arteriosus

Answer 2

failure of the truncus to divide into pulm trunk and aorta. most pts also have a VSD

Question 3

tx for most right to left shunts; coloring

Answer 3

surgical correction and/or maintenance of a PDA

these babies are cyanotic.

Question 4

transposition of the great vessels

Answer 4

Associated with maternal diabetes!!!
aorta leaves the RV (anterior) and the pulmonary trunk leaves the LV (posterior). this separates the systemic and pulmonary circulations. it is incompatible with life unless a shunt is present to allow mixing of blood (VSD, PDA, patent forament ovale). due to failure of the aorticopulmonary septum to spiral. most infants die in first few months without surgical intervention

Question 5

tricuspid atresia

Answer 5

absence of the tricuspid and hypoplastic RV. requires both ASD and VSD for viability

Question 6

tetrology of fallot

Answer 6

1. pulmonary artery stenosis
2. right ventricular hypertrophy- looks like a boot.
3. overriding aorta
4. ventricular septal defect
   forces right to left flow across the VSD and causes early cyanotic spells.

Question 7

how does squatting affect the tetrology of fallot

Answer 7

squatting: increased systemic vascular resistance, decreases right to left shunt, and improves cyanosis.

Question 8

what is TAPVR

Answer 8

total anomalous pulmonary venous return.
pulmonary vein drains to right heart. this is associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO.

Question 9

causes of late cyanosis

Answer 9

VSD>ASD>PDA

Question 10

eisenmenger syndrome

Answer 10

1. left-to-right shunt causes increased pulmonary flow –> pulm remodeling and pulm arteriole HTN. Right ventricular HTN occus to compensate, and the shunt becomes right to left. this causes late cyanosis, clubbing, and polycythemia.

Question 11

VSD

Answer 11

most common left to right defect. associated with fetal alcohol syndrome. asymmptomatic at birth, and may or may not manifest later. if very large, it may lead to LV overload and heart failure.

Question 12

ASD

Answer 12

defect in inter-atrial septum. loud S1. wide, fixed, split S2. usually occurs at septum secundum; septum primum defects occur with other abnormalities (and are associated with Down syndrome). distinct from PFO in that the septa tissue is missing, not unfused.

Question 13

PDA

Answer 13

associated with a continuous machine like murmur. patency is maintained by PGE synthesis and low O2. it can eventually result in late cyanosis of the lower extremities. can cause progressive RVG and/or LVH and/or heart failure

Question 14

What other conditions are associated with coarctation of the aorta?

Answer 14

bicuspid aortic valve

Question 15

infantile coarctation of the aorta

Answer 15

narrowing proximal to the insertion of the ductus arteriousus. associated with Turner syndrome. may present with closure of the ductus arteriosus.

Question 16

adult coarctation of the aorta

Answer 16

aortic narrowing distal to the ligamentum arteriosum. associated with NOTCHING of the ribs (d/t development of collateral circulation through the intercostal vasculature), HTN in the upper extremities, and weak, delayed pulses in the lower extremities.