Perioperative management of Fontan operation for the child with panhypopituitarism: a case report

Area of Science:

• Pediatric Cardiology
• Pediatric Endocrinology
• Congenital Heart Surgery

Background:

• The Fontan operation is a palliative procedure for complex single-ventricle congenital heart disease.
• Panhypopituitarism, a condition of deficient pituitary hormone production, can arise from various causes, including craniopharyngioma removal.
• Managing patients with both congenital heart disease and endocrine disorders presents unique clinical challenges.

Observation:

• A 5-year-old boy with a history of complex congenital heart disease (single ventricle, double-outlet right ventricle, pulmonary atresia) and panhypopituitarism underwent a Fontan operation.
• The patient was on hormone replacement therapy including hydrocortisone and 1-desamino-8-D-arginine vasopressin (DDAVP).
• Intraoperative management of hormone levels was achieved, but significant postoperative pleural effusions and ascites developed.

Findings:

• Postoperative fluid management was complicated by the combined effects of Fontan physiology and panhypopituitarism.
• Careful adjustment of oral DDAVP dosage was necessary to control effusions, ascites, and normalize serum sodium levels.
• While intraoperative hormone management was feasible, postoperative fluid balance required meticulous attention.

Implications:

• This case highlights the importance of individualized perioperative hormone management in pediatric patients with complex congenital heart disease and panhypopituitarism.
• Effective management strategies are crucial for optimizing outcomes in this high-risk patient population.
• Further research into the specific fluid management nuances for Fontan patients with panhypopituitarism is warranted.