A Functional Univentricle: Large Ventricular Septal Defect, Atrioventricular Discordance, Malposition of the Great Arteries, Pulmonary Stenosis, and Major Aorto Pulmonary Collaterals

We report a case of a 25 year-old male with a functionally univentricular heart. He underwent consult for clearance prior to employment and has been asymptomatic since childhood with good functional capacity, his 6 minute walk test was normal at 300 meters. Pertinent physical findings were a right ventricular heave, a grade 3/6 systolic murmur over the 2 nd -3 rd intercostal spaces left parasternal border, a continuous murmur over the interscapular area and cyanosis of the nailbeds. Laboratory exams showed mild erythrocytosis and hypoxemia. Transthoracic and transesophageal echocardiographic findings showed a large ventricular septal defect (apical intraventricular septum remnant), atrioventricular discordance, malposition of the great arteries and severe pulmonary stenosis. Major aorto-pulmonary collaterals were demonstrated on hemodynamics study. Patient is stable and living a normal life but advised to avoid moderate to heavy strenuous activities. He is on a regular follow – up at our out patient clinic and there are no planned immediate surgical interventions at present.