C. Fernandez*a (Dr), S. Sahaa (Dr)

a University of Buffalo: School of Medicine, Buffalo, UNITED STATES

* chaneljf@buffalo.edu


The Fontan procedure was first performed in 1971, and has been used to treat congenital heart defects involving a single ventricle. Performed by Frances Fontan and Eugene Baudet, an anastomosis is created directly to the pulmonary artery from the right atrium, bypassing the ventricle, allowing for systemic venous blood flow into the lungs. This rare procedure is performed for less than 0.02% of live births per year. Known pulmonary risks associated with this procedure include the development of pulmonary AVMS and plastic bronchitis. Fontan associated liver damage (FALD) includes hepatic fibrosis and hepatocellular carcinoma. However, there are only 6 Fontan cases that suffered from hepatopulmonary syndrome (HPS) as a complication (Choi et al 2009). We present the seventh case thus documented.

Case report

26-year-old female history of coarctation of the aorta, hypoplastic left heart status post Fontan procedure at Day 2 of life; was admitted for acute hypoxic respiratory failure and decompensated liver cirrhosis. Symptoms included chronic clubbing of her digits, LE edema, abdominal pain, ascites, central and peripheral cyanosis. Labs revealed elevated ALP, bilirubin and secondary polycythemia. CT imaging showed a right pleural effusion, portosystemic venous shunt, esophageal and inferior epigastric varices, dilated sub-pleural pulmonary vessels concerning for AVMs. Echocardiogram revealed endocardial cushion defect and dilated right ventricle. Peritoneal fluid was positive for SBP, and pleural fluid was positive for spontaneous bacterial pleuritis. Patient was treated with antibiotics and evaluated for possible transplant.


There is limited data in the development of HPS status post-Fontan surgery. Rodriguez et al (2018) suggests an increased risk of mortality in those with HPS, and a correlation between mortality and disease severity of HP. Given there is an overlap of HPS, PPHTN (portopulmonary hypertension), and FALD; this case serves to highlight the complexity of managing similar patients.

Disclosure of funding source(s): none