A Case of Creation of a Transcatheter Fenestration in Fontan Failure with Chronic Pleural Effusion Associated with High Altitude

Failed fontan circulation in single ventricle presented with low cardiac output, plastic bronchitis, protein-losing enteropathy, and recurrent pleural effusions. High altitude was known as a risk factor for fontan failure. We report the case of creation of a transcatheter fenestration in 9-years-old child who had chronic recurrent both pleural effusion due to failing fontan circulation associated with high altitude. He was diagnosed double-outlet right ventricle with remote ventricular septal defect in Ulaanbaatar, Mongolia at an altitude of 1350 meters. He was initially palliated with bilateral bidirectional Glenn shunt at 4-years-old in India, and underwent extracardiac conduit Fontan operation (non-fenestration) in 7-years-old in Seoul St. Mary’s Hospital, Korea. After returning to Mongolia, he had exertional dyspnea, facial edema, and cyanosis with chronic recurrent both pleural effusion for 2 years. Successful Fontan fenestration with stent implantation was performed in our institution. At 1-year follow-up, his symptoms of pleural effusion improved significantly. This case may help clinicians stratify patients at risk of fontan failure in advance and improve outcomes in these critically-ill patients.