[Pulmonary arterial hypertension and porto-caval fistula in a 17 year old patient].
The authors report the case of a 17 year old patient with severe pulmonary arterial hypertension accompanied with a congenital porto-caval shunt. This patient had been admitted with dyspnoea on exertion with signs of pulmonary arterial hypertension on clinical examination. Echocardiography revealed a systolic pulmonary arterial pressure of 80 mmHg with no left heart anomaly or intracardiac shunt. Pulmonary investigations, as well as immunology, clotting, liver function and retroviral serology were all negative. A routine abdominal ultrasound showed a 10 mm proximal porto-caval fistula with no sign of portal hypertension. This case shows that anomalies of the portal system can cause pulmonary arterial hypertension even in the absence of any symptoms suggestive of hepatic disorder.
Drighil
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Service de cardiologie, CHU Ibn Rochd, Casablanca, Maroc. a.drighil@caramail.com
Chraibi
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Tahiri
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D000293:Adolescent; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D016157:Vascular Fistula / Q000150:complications / Q000175:diagnosis*; D014682:Vena Cava, Inferior / Q000002:abnormalities*