Congenital Heart Disease

Question 1

what congenital heart defect is most commonly seen with Ebstein’s anomaly?

Answer 1

ASD/PFO (seen in over 50% of patients with Ebstein’s anomaly), for this reason exercise testing with O2 monitoring is recommended because desaturation with exercise is indication for ASD/PFO closure

Question 2

what is the Glenn procedure?

Answer 2

the SVC is disconnected from the RA and directly anastomosed to the right PA

Question 3

what is the Blalock-Taussig procedure?

Answer 3

a Gortex shunt is placed connecting the right subclavian or right carotid artery to the right pulmonary artery

Question 4

what are the three consecutive surgeries done for hypoplastic left heart and similar single ventricle physiology conditions?

Answer 4

1. Norwood: ASD is created with atrial septectomy, RV to aorta shunt is created, aorta to PA shunt is created
2. Glenn: aorta-pulmonary shunt is removed and replaced with bidirectional SVC to PA shunt
3. Fontan: IVC is connected to branch pulmonary arteries