Successful Fontan Completion in a Patient With Ellis-van Creveld Syndrome and a Hypoplastic Left Heart Syndrome Variant

Ellis–van Creveld (EVC) syndrome is a rare genetic disorder often associated with skeletal abnormalities and congenital heart defects. Hypoplastic left heart syndrome (HLHS) in EVC is extremely rare and typically has a poor prognosis. We report a case of a 2-year and 7-month-old girl with EVC and HLHS who successfully underwent staged single-ventricle palliation, including the Norwood, Glenn, and Fontan procedures. Pre-Fontan evaluation showed acceptable pulmonary vascular resistance, and a fenestrated extracardiac Fontan procedure was performed without complications. This case underscores the importance of early pulmonary vascular protection and careful perioperative planning in achieving favorable outcomes in complex EVC-related congenital heart disease.