Single Ventricle Heart Defects (SVD)

Also known as: single ventricle defect, SVD, hypoplastic left heart syndrome (HLHS), double outlet left ventricle (DOLV), tricuspid atresia and others.

What are Single Ventricle Heart Defects?

The ventricles are two of the hearts’ four chambers (the other two are called atria). The ventricles are responsible for pumping blood out of the heart. When a baby is born with only one of the ventricles functioning  properly (or in some cases where a heart valve may be missing ), this group of heart defects are called “ single ventricle defects “. These include a group of quite different cardiac abnormalities.

What causes single ventricle heart defects? 

These are rare heart defects that are present at birth. The exact cause is not known but is probably related to a genetic abnormality. Some Single ventricle heart defects are often present with other congenital heart defects.

What are the symptoms of single ventricle heart defects? 

The reduction in blood flow caused by single ventricle heart defects typically leads to extreme fatigue or even unresponsiveness. The baby usually has trouble breathing and feeding, and may have a blue tint to the skin or lips that indicates low oxygen levels. Some infants will need early treatment depending on how much or how little oxygen the heart can provide.

What are single ventricle heart defects care options? 

These are often serious heart defects that require cardiac catheterization, surgical intervention and cardiac intensive care,  shortly after birth. Nicklaus Children's hospital has the most modern facilities and expert professionals to manage these children.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 6/12/2018 2:01:27 PM

From the Newsdesk

Infant flown from Puerto Rico days after Hurricane Maria for Lifesaving Surgery
Naialee Perez had just given birth to her first child, a baby boy named Liam, when a category five hurricane was making its way towards her hometown in the island of Puerto Rico. Liam was on a ventilator and undergoing treatment for a congenital heart defect in Hospital del Niño in San Juan while those on the island prepared for what would become one of the most catastrophic hurricanes in its history.
August Patient of the Month: Luife
While he was still inside his mother’s womb, Luife was diagnosed with transposition of the great arteries, a congenital heart defect. Shortly after birth, Luife was taken by ambulance to the cardiac team at Nicklaus Children’s. The pediatric cardiology team took Luife’s heart apart, piece by delicate piece, and successfully, put it back together. Today, Luife is a healthy, active and outgoing 8-year-old boy who wears his “Scar of Honor” with pride.