Atrioventricular canal defect is a combination of heart problems resulting in a defect in the center of the heart. The condition occurs when there's a hole between the heart's chambers and problems with the valves that regulate blood flow in the heart. The condition is often associated with Down syndrome. Atrioventricular canal defect allows extra blood to flow to the lungs. Untreated, atrioventricular canal defect can cause heart failure and high blood pressure in the lungs.
The natural history of ostium primum ASD without surgery carries a 50% mortality before 20 years of age with atrial fibrillation an important cause of morbidity. Following surgery the Mayo Clinic reports a 40 year survival of 76%. The reoperation rate in this large series was 11%. These increased with increasing age at primary operation. Bergin reports low mortality and good long?term survival in patients with ostium primum ASD presenting for surgical repair in later life (>?40 years old).
Patients with incomplete atrioventricular septal defects (AVSDs) present with signs and symptoms similar to those of secundum atrial septal defects (ASDs) and, as such, rarely require medical therapy. Medical therapy in patients with complete atrioventricular septal defects consists of aggressive anticongestive treatment for the signs and symptoms of congestive heart failure (CHF). The mainstays of medical therapy are furosemide (for diuresis for the volume-overloaded heart), digoxin (as a mild inotrope), and ACE inhibitors (for afterload reduction).