DOI: 10.5176/2382-5669_CCMR13.36

Authors: Cristine Catindig – Santelices MD and Michael Joseph T. Reyes MD


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 with good functional capacity and 6 minute walk test done was normal at 300 meters. Pertinent physical findings were a right ventricular heave, a grade 3/6 systolic murmur over the 2nd-3rd 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 outpatient clinic and there are no planned immediate surgical interventions at present.

Keywords: Univentricular Heart, Ventricular Septal Defect, Pulmonic Valve Stenosis, Malposition of the Great Arteries, Major Aorto Pulmonary Collaterals

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