Case 23-2023: A 21-Year-Old Man with Progressive Dyspnea

A 21-year-old college student presented with progressive dyspnea, initially misdiagnosed as exercise-induced asthma. Diagnostic imaging and cardiac catheterization revealed precapillary pulmonary hypertension due to a congenital extrahepatic portosystemic shunt (Abernethy malformation), with absent main portal vein and liver abnormalities. Treatment involved pulmonary vasodilators and ultimately a successful liver transplantation, which improved pulmonary pressures and led to long-term stability.

A 21-year-old college student presented with progressive dyspnea, initially misdiagnosed as exercise-induced asthma. Diagnostic imaging and cardiac catheterization revealed precapillary pulmonary hypertension due to a congenital extrahepatic portosystemic shunt (Abernethy malformation), with absent main portal vein and liver abnormalities. Treatment involved pulmonary vasodilators and ultimately a successful liver transplantation, which improved pulmonary pressures and led to long-term stability.