What is a Fontan, TAPVR and HRHS?

Hi there, my name is Melinda! Welcome to my blog. Here you will find my upcoming health journey and how I am navigating life with heart failure.

About me:

I was born with TAPVR (Total anomalous pulmonary venous return), which is a rare congenital heart defect that occurs when the pulmonary veins do not connect properly to the heart. My condition also consists of HRHS (Hypoplastic Right Heart Syndrome), which is also a rare congenital heart defect where the right side of the heart is underdeveloped. This can cause issues with the tricuspid valve, right ventricle, and or pulmonary valve. Several open-heart surgeries and cardiac catheterizations were done throughout childhood (1994-2017) for correction and revision. 

The corrective surgery I have had is called a FONTAN procedure. The Fontan procedure is a type of heart surgery used to treat children born with complex congenital heart defects, especially those where only one functional ventricle is available to pump blood (like hypoplastic left heart syndrome or tricuspid atresia).

How it works:

Normally, the heart has two ventricles: The right ventricle pumps blood to the lungs. The left ventricle pumps blood to the body. In some congenital defects, only one ventricle is working properly. The Fontan procedure helps by rerouting blood flow so that oxygen-poor blood from the body bypasses the heart and flows directly into the lungs.

Basic steps of the Fontan: Connect the inferior vena cava (IVC) (and previously the superior vena cava via a Glenn shunt) directly to the pulmonary arteries. This lets blue (deoxygenated) blood from the body flow passively into the lungs without being pumped by the heart.

The single working ventricle now only pumps oxygen-rich blood to the body.


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