Single Ventricle Heart Disease
Single Ventricle Heart Disease is a rare disease impacting the two lower chambers of the heart.
Single Ventricle Heart Disease is a congenital heart disease where a person is born with a heart in which one of the two lower chambers or ventricles does not develop properly. In a normal heart, each ventricle does a separate job—the right ventricle pumps blood without oxygen (blue blood) to the lungs and the left ventricle pumps blood with oxygen (red blood) around the body.
People with Single Ventricle Heart Disease have just one ventricle large enough to pump all the blood and do its job. Because of this, the blue blood and red blood, which are supposed to stay separate, mix together. This means there is a smaller amount of oxygenated blood getting to the rest of the body causing the person’s skin to appear blue.
Single Ventricle Heart Disease are not all the same.
Hypoplastic left heart syndrome (HLHS)
The left ventricle, mitral valve, aortic valve and aorta (a blood vessel) are all too small.
Double outlet right ventricle
Both the aorta and the pulmonary artery (blood vessels) come out of the right ventricle. This means the left ventricle doesn’t develop properly.
Tricuspid atresia
The tricuspid valve fails to develop. This means that the right ventricle doesn’t develop properly.
Double inlet left ventricle
Both of the top chambers of the heart connect to the left ventricle. This means that the right ventricle doesn’t develop properly.
Single Ventricle Heart Disease is rare and in most cases, causes are unknown.
Single ventricle heart defects are rare— they occur in about five of every 100,000 children born and are usually diagnosed at birth. The cause is unknown in most cases, and they don’t tend to run in families, but rather are random.
Single Ventricle Heart Disease comes with serious long-term effects.
Every child and adult with Single Ventricle Heart Disease are affected differently, but there are lots of similarities in how they will be treated and managed, most often with a Fontan Procedure. Some of the long-term effects of Fontan circulation include difficulty exercising, cyanosis (blue skin), complications like liver problems, blood clotting and heart failure, and overall, a lower life expectancy.