Cardiology>>>>>Adult Congenital Heart Disease and Pregnancy
Question 13#

One of the medical students asks you what a Fontan operation consists of.

What is your answer?

A. A palliative procedure when a biventricular surgical repair is not possible: the systemic venous blood is directly routed into the pulmonary arteries bypassing the ventricle
B. When a biventricular surgical repair is not possible the systemic venous blood is directly routed into the pulmonary arteries bypassing the ventricle; life expectancy is near normal
C. It is a procedure for the treatment of transposition of the great arteries but is no longer performed; the systemic venous blood is routed via baffles to the morphological left ventricle (subpulmonary ventricle) and the pulmonary venous blood is routed to the morphological RV (systemic ventricle)
D. It consists of an SVC-to-PA shunt to increase pulmonary blood flow in congenital cyanotic heart disease when pulmonary flow is low
E. It consists of a subclavian-to-PA shunt to increase pulmonary blood flow in congenital cyanotic heart disease when pulmonary flow is low

Correct Answer is A

Comment:

1. A describes the Fontan operation for complex congenital cyanotic heart disease when a biventricular repair is not possible. There are a number of modifications. The modern technique is the total cavopulmonary connection which routes blood directly to the PAs avoiding the atrium.
2. B: the procedure is palliative, i.e. life expectancy is reduced.
3. C describes an atrial switch (Senning or Mustard) operation for TGA.
4. D describes a Glenn shunt. This can be part of a staged Fontan operation which would require IVC blood rerouting to the PA for completion (either via an extracardiac conduit or a lateral tunnel).
5. E describes the Blalock–Tausigg shunt.