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268 ACUTE ONSET RIGHT-SIDED HEART FAILURE AND CRYPTOGENIC CIRRHOSIS: A CASE OF RAPIDLY PROGRESSIVE PULMONARY HYPERTENSION K Novak, Z Gao, D Helmerson, R Leigh, KW Burak CASE REPORT: A 45-year-old man awaiting liver transplant, presented to hospital in December, 2005 with progressive dyspnea. He was a healthy, non-drinker with no concomitant conditions other than cryptogenic cirrhosis. Complications of portal hypertension were previously documented including esophageal varicies, splenomegaly and ascites. Medications included spironolactone, furosemide, norfloxacin, and ursodiol. Family history was non-contributory with no history of thrombosis. Physical exam at admission was unremarkable, with no evidence of pulmonary hypertension. Diagnostic imaging revealed a negative CT study for pulmonary embolism (PE). Echocardiogram including exam for shunts was normal. Pulmonary function tests, room air arterial blood gas, and shunt fractions were also normal.
University of Calgary, Calgary, Alberta
Nine days later he presented to hospital with severe dyspnea. Repeat echocardiogram showed normal left ventricular (LV) structure and function, but significant right atrial and RV dilation. Repeat CT for PE was again, negative. He then underwent a right heart catheterization, confirming mean pulmonary arterial pressure of 47mmHg. After deteriorating with refractory hypotension and severe abdominal pain, he was transferred to ICU for intubation, ventilation and inotropic support. Abdominal ultrasound at the time showed no portal blood flow and he was anticoagulated. Despite maximal physiologic support including prostacyclin for the treatment of severe pulmonary hypertension, he developed multisystem organ failure and died in the ICU (20 days after normal echo). Autopsy revealed chronic Budd-Chiari syndrome and severe pulmonary hypertension, with characteristic bilateral plexogenic arterial lesions of the pulmonary arterioles. To date, there are no reports of acute onset portopulmonary hypertension, secondary to chronic Budd-Chiari syndrome leading to cirrhosis.