Abstract
BACKGROUND: Pheochromocytoma is a rare tumour producing catecholamines and has been more frequently reported than expected in patients with a Fontan physiology. CASE SUMMARY: The patient was a 39-year-old woman born with a univentricular atrioventricular connection and pulmonary valve stenosis. A Fontan operation was performed when she was 22 years old. At the age of 38, she was diagnosed with pheochromocytoma. The most serious problem was the increased occurrence of supraventricular arrhythmias, which easily caused heart failure. We decided to perform a laparoscopic resection. Postoperative recovery was good. There were no findings of malignancy and all postoperative catecholamine levels normalized. She was discharged home on the 14th day after the surgery, walking unaided. DISCUSSION: Diagnosis and treatment by tumour resection in the early phase are crucial in patients who undergo the Fontan procedure. However, patients after Fontan surgery have a very low cardiac reserve. Thus, the decision to perform a surgical treatment is a very difficult one to take. In this case, fenestration had been naturally closed. As fenestration provides a consistent source of systemic ventricular preload, we performed transcatheter fenestration dilatation to improve heart failure management before surgical pheochromocytoma removal. Although laparoscopic surgery is generally considered to be less invasive, pneumoperitoneum may interfere with venous return in Fontan physiology patients. There is also a risk of thrombosis via fenestration. Here, we reported the case of a successful laparoscopic pheochromocytoma resection in an adult Fontan patient.