Abstract
The atrioventricular (AV) valves are complex anatomical structures which perform sophisticated functions. 1 A wide spectrum of malformations of these valves can occur in patients with AV septal defects. We here describe the anatomic and functional abnormalities of a rare form of the disease, where two valves connected the right atrium to both the right and left ventricles, in addition to a third valve that connected the left atrium to the left ventricle, with no evidence of regurgitation or cyanosis in spite of the relatively large communication between the right atrium and the left ventricle. In addition, the patient had severe subaortic stenosis. The pathophysiology, hemodynamics and method of repair of the condition are discussed.
Highlights
A 12-year-old female patient, presented with dyspnea on moderate effort
Echocardiography revealed the diagnosis of atrioventricular septal defect (AVSD) with two adequate ventricles, closed atrial component and almost closed ventricular component, with what seemed to be aneurysmal tissue that had a tiny leak (Figures 1, 2)
The atrial septum was incised at the fossa ovalis, through which the left AV valve was seen opening to the left ventricle with “a cleft”
Summary
A 12-year-old female patient, presented with dyspnea on moderate effort. She had no history of cyanosis. Echocardiography revealed the diagnosis of atrioventricular septal defect (AVSD) with two adequate ventricles, closed atrial component and almost closed ventricular component, with what seemed to be aneurysmal tissue that had a tiny leak (Figures 1, 2).
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