(1) Francis Fontan performed the Fontan operation first in 1968. The Fontan operation is a heart operation used to treat complex congenital heart defects (birth defects of the heart) like tricuspid atresia, hypoplastic left heart syndrome (HLHS), pulmonary atresia and single ventricle.
(2) ICD-9 code: 35.94 MeSH D018729
(Francois Maurice Fontan, 20th century, French heart surgeon) operation to divert systemic venous blood flow into the pulmonary artery without passage through the right ventricle. The initial Fontan procedure involved an anastomosis of the right atrial appendage to the pulmonary artery. Several modifications have been devised including direct connection of the superior vena cava to the superior aspect of the right pulmonary artery and construction of a conduit from the inferior vena cava to the undersurface of the right pulmonary artery or into the main pulmonary artery. Construction of the conduit from the inferior vena cava has been done both inside and outside of the right atrium. Frequently, the operation is a staged procedure starting with a superior vena caval right pulmonary arterial connection. The operation is performed for the correction of tricuspid atresia, hypoplastic right heart, hypoplastic left heart syndrome and single ventricle. It is employed in any complex anomaly in which there is only one adequate morphological or functional ventricle.
Chest X-ray after Fontan procedure may demonstrate persistent or recurrent pleural effusions. Cardiac catheterization and pulmonary arteriography are necessary preoperatively in order to exclude pulmonary arterial hypertension and pulmonary arterial stenoses. Such impediments to pulmonary blood flow produce a poor outcome. Cardiac angiography, echocardiography and MRI are used to depict the Fontan connection and to evaluate blood flow through this circuit into the pulmonary arteries. These imaging studies demonstrate severe enlargement of the right atrium and/or inferior vena cava and stasis of blood in this structure in patients with a poor result (Fig.1).
(3) The Fontan is an open-heart surgery used to treat a variety of cardiac defects that are present at birth (congenital heart defects). It is commonly used on children who have been born with either no separation between the right and left ventricles of the heart (a ventricular septal defect), with only one functional ventricle or the lack of development of one or more heart valves. The common factor is that the patient has only one functioning ventricle or, because of the nature of the defect, the patient is best served by being left with only one functioning ventricle.
During this operation, surgeons create a passageway that allows blood to bypass the right ventricle, which is usually responsible for pumping oxygen–poor blood to the lungs for fresh oxygen. By routing blood around the right ventricle, surgeons are able to adapt the right ventricle to pump oxygen-rich blood to the body if the left ventricle is unable to. In other cases in which the left ventricle is healthy, but the right ventricle is too small to pump blood or is totally missing, the Fontan procedure may be performed simply to bypass circulation around the under-performing right ventricle. “Your total health”
Intestinal Lymphangiectasia Secondary intestinal lymphangiectasia is a documented possible complication of the Fontan procedure
ICD-9 code: 35.94 MeSH D018729