Conditions We Treat

We treat all types of single ventricle anomalies. Common ones include:

Atrioventricular canal defects (AVCD)/atrioventricular septal defects (AVSD). A type of congenital heart defect where the endocardial cushions (cells that help form the heart) do not develop properly when a growing baby is still an embryo. The result is often a hole in the center of the heart where the upper and lower chambers do not come together correctly.

Double inlet left ventricle (DILV). In this complex defect the upper two chambers, or atriums, the heart’s two major arteries connect to one ventricle (the left) rather than to both ventricles in the heart. This leaves the right ventricle small and unable to function. A result can be an oversized left ventricle.

Double outlet right ventricle (DORV). This rare, complex heart defect is the counterpart of DILV. The heart’s two major arteries connect to one ventricle (the right) rather than to both. This leaves the left ventricle underdeveloped and unable to function. Children who have DORV always have a condition called ventricular septal defect, which creates a hole in the septum—the wall of tissue that separates the two ventricles.

Heterotaxy syndrome of the heart (right atrial isomerism, left atrial isomerism). Heterotaxy syndrome can affect many different organs. It simply means “different arrangement” of organs when a baby is an embryo. With the heart, this can result in a number of defects, including heart or pulmonary valves that do not work properly, or holes in the heart.

Hypoplastic left heart syndrome (HLHS). With this congenital heart defect, the left side of the heart doesn’t develop properly, affecting the mitral valve, which controls blood flow between the left atrium and left ventricle. One treatment for HLHS is the Norwood procedure.

Mitral atresia. The mitral valve, which connects the left atrium with the left ventricle, does not develop correctly, causing the left ventricle to be underdeveloped. Mitral atresia is often associated with other heart defects.

Pulmonary atresia. The pulmonary valve, which lies between the right ventricle and the main pulmonary artery, does not form properly. When this happens, blood flow to the lungs is restricted.

Tricuspid atresia. Valves exist in the heart to let blood flow from one chamber to another. With this heart defect, the tricuspid valve between the right ventricle and the right atrium fails to form, essentially cutting off blood flow to the right ventricle, leaving it underdeveloped and unable to function correctly.

Conditions associated with single ventricle anomalies

Our pediatric Single Ventricle Program team is here to support children in dealing with all the aspects of single ventricle problems. Children with a single ventricle may experience anxiety disorders or other mental health needs. They may struggle with learning disorders, including attention deficit hyperactivity disorder (ADHD) and behavioral disorders. Others may fail to grow properly or maintain a healthy weight. Our interdisciplinary team works closely with your family to address whatever mental health or developmental challenges your child faces by putting in place a full spectrum of support and resources.