There are several different forms of congenital heart disease which are collectively referred to as a "single ventricle." Patients with this entity have mixing of both unoxygenated and oxygenated blood within the single ventricle. Surgical repair is ultimately achieved with a procedure known as the Fontan operation. The Fontan directs unoxygenated blood into the pulmonary artery and lungs without the need for a right sided pumping chamber (ventricle). Oxygenated blood from the lungs returns to the single ventricle and is ejected into the aorta.
Most adults with a Fontan operation have required early palliative operations such as a shunt, banding of the pulmonary artery, or a Glenn anastomosis. While most Fontan procedures are performed in children, an occasional adult with a single ventricle may still be a candidate for a Fontan repair.
The Fontan operation corrects cyanosis and eliminates the right to left shunt which is present in patients with a single ventricle. Since the number of Fontan operations in children has steadily increased since the 1980s, many more adult patients with Fontan operations are being seen today.
The late problems following a Fontan operation include:
1. Heart rhythm disturbances, such as atrial tachycardia, atrial flutter, atrial fibrillation, sinus node and AV conduction problems,
2. Fluid retention
3. Protein losing enteropathy.
The Fontan operation has undergone several modifications over the years in order to improve long term results. The early operations consisted of an atriopulmonary anastomosis in which the right atrium is connected directly to the pulmonary artery. This may lead to marked right atrial enlargement and the development of arrhythmias. In more recent years, this operation has been replaced by the "cavopulmonary anastomosis" or "lateral tunnel." This procedure directs both inferior and superior vena caval blood directly into the pulmonary arteries. In some patients, an extracardiac conduit may be used to connect the inferior vena cava to the pulmonary artery. These modifications to the Fontan operation will hopefully reduce the incidence of late atrial arrhythmias.
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