CSC Congenital Heart Disease Surgery 3

Question 1

The Rastelli procedure is specifically designed to repair which combination of congenital defects?

Accepted Answer

D-TGA with VSD and left ventricular outflow tract obstruction

Answer

The Rastelli procedure repairs D-TGA with VSD and LVOT obstruction by creating an intracardiac LV-to-aorta tunnel through the VSD and placing an extracardiac RV-to-PA conduit.

Question 2

What is the primary long-term consequence of leaving a large, unrestrictive VSD untreated beyond early childhood?

Accepted Answer

Eisenmenger syndrome with irreversible pulmonary vascular obstructive disease

Answer

A large unrestrictive VSD causes sustained high-pressure, high-flow pulmonary hypertension, eventually leading to irreversible pulmonary vascular obstructive disease and shunt reversal (Eisenmenger syndrome).

Question 3

In a complete atrioventricular septal defect (AVSD), how many leaflets does the common AV valve typically contain?

Accepted Answer

Five leaflets spanning both the tricuspid and mitral annuli

Answer

Complete AVSD features a common five-leaflet AV valve (two bridging leaflets and three mural leaflets) that spans both ventricles, associated with a primum ASD and inlet VSD.

Question 4

Which chromosomal abnormality is most strongly associated with complete atrioventricular septal defect?

Accepted Answer

Trisomy 21 (Down syndrome)

Answer

Trisomy 21 (Down syndrome) is present in approximately 70% of patients with complete AVSD, making it by far the most common chromosomal association with endocardial cushion defects.

Question 5

The Konno-Rastan procedure (aortoventriculoplasty) is indicated for which condition?

Accepted Answer

Severe tunnel-type subaortic stenosis with an annulus too small for adequate prosthesis

Answer

The Konno-Rastan procedure enlarges the aortic annulus and LVOT by incising the interventricular septum, indicated when severe subaortic obstruction is accompanied by a small annulus inadequate for a standard prosthesis.

Cardiac Surgery Certification Exam Practice Test

Cardiac Surgery Certification Exam Practice Test

CSC Congenital Heart Disease Surgery 3