Cirrhosis of the Liver Simulating Congenital Cyanotic Heart Disease

Pediatric Cardiology and Cardiovascular Surgery: 1950-2000

“…The similarities to pulmonary arteriovenous shunting in severe liver disease are interesting, and their reversal by liver transplantation is provocative. 141,142 Indeed, this complication has been well documented after the Kawashima operation and reversed in most after hepatic vein inclusion, 143,144 a successful maneuver also reported by others after inclusion of the hepatic veins in the pulmonary circulation after a superior cavopulmonary connection. Historically, the development of pulmonary arteriovenous malformations after the classic Glenn anastomosis has been very well documented, but in none of the substantial reviews of this complication emanating from either Yale or Toronto was hepatic venous exclusion considered to be causal.…”

“…This is not the forum to explore all these issues, but a few words about pulmonary arteriovenous malformations are germane. [131][132][133][134][135][136][137][138][139][140][141][142] The etiology for the development of pulmonary arteriovenous malformations after the Fontan operation remains uncertain. Clinical practice currently suggests that exclusion of the hepatic veins and hepatic venous effluent from the pulmonary circuit may be causal to the development of pulmonary arteriovenous malformations and that inclusion of the hepatic venous blood into the pulmonary circuit may reverse their formation.…”

The aetiology of clubbing and hypertrophic osteoarthropathy

“…The cyanosis is not always fully correctable by inhaling oxygen [26]-a recognized test for macroscopic rightto-left shunts of blood. In some cases of cirrhosis multiple small arteriovenous connections in the lungs have been shown radiographically, and even by open lung biopsy [77]. Tests of the pulmonary vasculature with manufactured particulate matter (technetiumlabelled macroaggregates of albumin) indicate that in severe liver disease there is intrapulmonary right-toleft shunting through abnormally distended channels which can allow particles of 20-50 p diameter to pass through [26,74].…”

A Case Report of Uncommon Cause of Cyanosis in a Child

“…Hansoti, and Sharma 1989 [16] also reported cases of similar severity in India. They reported 20 cases over a 20-year period with arterial desaturation due to liver cirrhosis that was so severe so as to simulate congenital cyanotic heart disease.…”

“…This systolic murmur is almost certainly due to the hyperkinetic circulation. Hepatic and splenic enlargement is usual and point to the liver pathology [16].…”