Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: An autopsy study

Abstract

At autopsy, hepatic fibrosis was commonly observed in patients who had undergone the Fontan operation. Portal fibrosis has been previously unrecognized in this population. Significant portal fibrosis occurred in most who died soon after the Fontan procedure and was associated with pre-Fontan morbidity. Hepatic disease in the single-ventricle population is multifactorial and may begin before the Fontan operation.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

“…120 In addition to being subjected to the physiological alterations characteristic of Fontan physiology (increased central venous pressure and low cardiac output), the liver in the patient with a single ventricle is vulnerable to a number of insults to the liver that begin well before Fontan completion (hypoxia, low output state, and perioperative insult). 350,351 Neuroendocrine tumors such as pheochromocytoma and paraganglioma have been documented in the setting of cyanotic CHD going back to the 1960s. [352][353][354][355] Chronic hypoxia was hypothesized to be the trigger on the basis of observations of a higher incidence in high-altitude dwellers.…”

Human Heterotaxy Syndrome

“…87 Significant portal fibrosis had occurred in most of those who died soon after the Fontan procedure, suggesting that hepatic fibrosis begins before the Fontan operation. 109 Recently, cases of hepatocellular carcinoma after the Fontan operation have been reported. 110 Careful observation is necessary to detect the hepatic changes long after the Fontan operation.…”

Chronic Heart Failure in Congenital Heart Disease

“…Over time, increased CVP and low cardiac output may lead to chronic congestive hepatopathy with hepatic dysfunction, cirrhosis, ascites, varices, hepatorenal syndrome, and risk of hepatocellular carcinoma. 16,[193][194][195][196][197][198][199][200][201][202][203][204][205][206][207][208][209] Sluggish flow through an atriopulmonary connection increases the risk of thrombosis and embolism, which can further increase the pulmonary vascular resistance and thus CVP. Atrial arrhythmias and sinus node dysfunction are very common, both as a cause and as a consequence of abnormal Fontan hemodynamics, and are important contributors to HF symptoms.…”