Cirrhosis of liver simulating congenital cyanotic heart disease

During the last 26 years, 21 patients with cirrhosis of the liver with severe cyanosis and gross clubbing simulating congenital cyanotic heart disease were subjected to cardiac catheterisation and angiography, splenography, liver function tests and liver biopsy. One patient had tetralogy of Fallot in addition, which was satisfactorily corrected. He continued to be intensely cyanosed and dyspnoeic despite adequate surgical correction of his cardiac defect as demonstrated on cardiac catheterisation and angiocardiography. Portupulmonary fistulas could not be demonstrated in any patient. The cyanosis and clubbing were secondary to right to left intrapulmonary shunting across multiple tiny pulmonary arteriovenous fistulas in all cases. In 16 cases selective pulmonary angiography revealed discrete arteriovenous fistulas. In 5 cases the angiogram did not reveal any convincing evidence of pulmonary arteriovenous fistulas. In 2 of these 5 cases peripheral vein contrast echocardiography, which seems to be a sensitive investigation, demonstrated right to left intrapulmonary shunting. Open lung biopsy in one case showed evidence of pulmonary arteriovenous fistulas.