CHD

Question 1

PDA

Answer 1

Patent Ductus Arteriosus

Connects L PA with Descending Aorta

Functional Closure: 1st day of life with spontaneous ventilation, rise in O2, decreased PVR, decreased endogenous prostaglandin

Anatomic Closure: 2-3 weeks of life. Fibrosis of ductal tissue. Permanent closure of lumen –> Ligamentum Arteriosum

Question 2

ASD

Answer 2

Atrial Septal Defect

Communication in atrial septum from improper embryologic formation of septal wall

Question 3

VSD

Answer 3

Ventricular Septal Defect

Communication in ventricular septal wall

Most common form of CHD** (20% of all CHD)

Question 4

Truncus Arteriosus (TA)

Answer 4

Failure of primitive arterial trunk to separate and divide into distinct aorta and PA

A single arterial vessel override the ventricular septum and supplies the coronary, pulm, and systemic circulations through a single truncal valve

VSD always present (perimembranous, infudibular below truncal valve)

DiGeorge Syndrome

Question 5

Total Anomalous Pulmonary Venous Return (TAPVR)

Answer 5

Drainage of the pulmonary veins into a systemic venous structure or into the RA (instead of LA)

All 4 P. veins drain into systemic venous system or the RA

ASD necessary for survival**

Question 6

Coarctation of the Aorta

Answer 6

Narrowing of the aorta with pressure elevation proximally (before coarctation) and pressure decrease distally (after coarctation)

Genetic influence (siblings, cousins)

Turner Syndrome

Berry aneurysm in Circle of Willis

Ductal Dependent Lesion! PDA

Question 7

Interrupted Aortic Arch

Answer 7

Complete lack of luminal continuity between the ascending and descending aorta

DiGeorge Syndrome (Hypocalcemia)
Microdeletion of Chormosome 22.q.11.2

Ductal Dependent Lesion! PDA

Question 8

Aortic Stenosis

Answer 8

Above, below, or at the level of the valve

Autosomal dominant inheritance

20% have other CHD

William Syndrome & Supravalvar AS

PDA Dependent (Severe AS)

Diagnosis beyond neonatal period holds better prognosis

Question 9

Pulmonary Stenosis

Answer 9

Rise in ventricular pressure proportional to severity of obstruction

Pulmonary obstruction can occur above, below, or at level fo the valve

Question 10

Tetralogy of Fallot

Answer 10

1. Outlet Type VSD (Anterior malalignment of outlet portion of infundibular septum)
2. R Ventricular Outflow Tract Obstruction
3. Overriding aorta
4. RVH

Most common CYANOTIC congenital heart defect (10% CHD) **

Question 11

Absent Pulmonary Valve

Answer 11

Total absence of pulmonary valve leaflets or rudimentary leaflets that lack cusp-like anatomy, have limited mobility, and are centrally deficient

Question 12

Single Ventricle

Answer 12

Only 1 ventricle present or defects with 2 ventricles but either one ventricle is “dominant” due to hypoplasia of the other or the AV valve alignment precludes successful partitioning of the heart into 2 functioning ventricles

Question 13

Pulmonary Atresia with Intact Ventricular Septum

Answer 13

Ductal dependent PBF

Question 14

Transposition of the Great Arteries

Answer 14

Aorta arising from anatomic RV and PA arising from anatomic LV

2 separate, parallel circulations

Question 15

Ebstein Anomaly of the Tricuspid Valve

Answer 15

Failure of the TV leaflets to detach from the ventricular septum, which causes displacement of the septal hinge-point and effective TV orifice

Question 16

Vascular Ring/Pulmonary Artery Sling

Answer 16

Anomaly of the aortic arch which causes compression of the esophagus , trachea, or both structures