Amelioration of High Cardiac Output and Pulmonary Hypertension by Occlusion of Congenital Porto-Systemic Shunt

Abstract

A 69-year-old woman was evaluated for progressive dyspnea and pulmonary hypertension. She had World Health Organization class III symptoms, was obese (41 kg/m2 body mass index), and had chronic atrial fibrillation, obstructive sleep apnea, and mild chronic obstructive pulmonary disease. Positive findings on physical examination included an elevated jugular venous pressure (14 cm), irregular rhythm, accentuated pulmonic second sound, and lower extremity edema. Blood pressure was 122/60 mm Hg, and heart rate was 68 beats per minute. The distance covered during a 6-minute walk test was 266 m. Transthoracic echocardiogram documented preserved left ventricular ejection fraction, moderate right ventricular enlargement, mild decrease in right ventricular function, and estimated right ventricular systolic pressure of 102 mm Hg, assuming a right atrial pressure of 10 mm Hg (Table 1 and online-only Data Supplement Movie I). Severe tricuspid regurgitation and trivial mitral regurgitation were present without other valvular abnormalities. Aortic flow was elevated, and the calculated cardiac index was 4.2 L/min/m2. …