Cirrhosis of Liver Simulating Congenital Cyanotic Heart Disease

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Abstract
Intense cyanosis gross clubbing and marked effort intolerance of 2-4 years duration simulating congenital cyanotic heart disease was observed in 7 young patients with histopathologically proved portal cirrhosis of liver. They also had hepatosplenomegaly and ejection systolic murmur at the pulmonary area. Only 2 patients had few engorged veins on their abdomen and 3 showed esophageal varices. Two other patients had arterial spiders and telengiectesis on their chest, back and arms. Their chest X-rays and electrocardiograms were almost normal. Liver function tests were abnormal in 6. Portal venous pressure was raised in all. Portal splenography did not demonstrate any portopulmonary shunt. The portal vein was not visualized but a large channel was seen to open into the inferior venacava. Right heart catheter and angiocardiography did not show any intracardiac abnormality but the pulmonary vasculature appeared blotchy. Femoral arterial and pulmonary venous blood samples were highly desaturated. Selective high speed pulmonary angiography in 2 patients demonstrated multiple small pulmonary arteriovenous aneurysms which were confirmed by open lung biopsy in one patient. These aneurysms account for the right to left shunts in these cases.