Amelioration of high cardiac output and pulmonary hypertension by occlusion of congenital porto-systemic shunt.

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/m 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/m. Right heart catheterization demonstrated a right atrial pressure of 11 mm Hg, mean pulmonary artery pressure of 46 mm Hg, mean pulmonary capillary wedge pressure of 19 mm Hg, and cardiac output (CO) of 13.4 L/min (6.8 L/min/m cardiac index, Fick method), consistent with pulmonary hypertension and high output heart failure. Oxygen saturation studies, performed as part of a shunt run, demonstrated the following: superior vena cava 74%, mid-right atrium 79%, and inferior vena cava 87%, suggestive of a systemic shunt. Thyroid function (thyroid stimulating hormone, 1.9 mIU/L) was normal, and hemoglobin was 12.7 g/dL. Computed tomography of the chest, abdomen, and pelvis did not demonstrate an arteriovenous fistula, but there was evidence of a large congenital venous malformation with a shunt between the inferior mesenteric vein and inferior vena cava (Figure). The porto-systemic shunt was confirmed on mesenteric angiogram/venogram with preferential blood flow from the splenic vein through the inferior mesenteric vein and then into the inferior vena cava via a tangle of collateral vessels in the retroperitoneum. The net effect of this pathway resulted in shunting of most of the splenic venous blood flow back to the systemic circulation, bypassing the liver. The porto-systemic shunt was embolized with coils and Amplatzer plugs (Figure, C and D) after plasma sampling at Table 1. Catheterization and Echocardiographic Hemodynamic Assessment Before and After Closure of the Congenital Anomalous Shunt