Single ventricle

Symptoms

Clinical symptoms and subsequent treatment are determined by the amount of flow that reaches the lungs. Depending on this, defects can be separated into two groups.

• Decreased pulmonary flow. A blueish cast to the skin called “cyanosis”. This is serious and progressive from the first days of life and is present in over 50 % of cases.
• Increased pulmonary flow. Most of these babies show signs of congestive heart failure a few days after being born.

Diagnosis

Echocardiogram is the most important tool to define the anatomy of the heart and the large vessels in patients with single ventricle.

Typical treatment        

Total caval-pulmonary deviation, or Fontan circulation, is achieved through a series of procedures in several stages:

• First stage or neonatal palliation: pulmonary circulation is protected (pulmonary artery band) or pulmonary flow is increased (systemic-pulmonary fistula).

• Second stage (Glenn procedure): the  superior vena cava is connected to the right pulmonary artery. This is carried out during the first three months to one year after a child is born, to decompress the single ventricle.

• Third stage or Fontan procedure: completion of the caval-pulmonary connection carried out between one and five years old.

Evolution following this treatment is very good. The survival rate is around 90% after 10 years and 85% after 15 years.

In some cases, a heart transplant may be required in the long term due to improper functioning.