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