Anesthetic Management for EC-IC Bypass Procedure in a Patient with Fontan Physiology: Navigating a Vascular Multimorbidity

Abstract

The Fontan procedure is a palliative procedure that reduces mortality associated with complex congenital cardiac anomalies. With advanced surgical techniques and improved perioperative care, an increase in the survival rate of palliated complex heart disease patients is rightfully expected. These patients may be encountered in noncardiac surgical suites and require special considerations. We present an interesting case of a 34-year-old male with a rare combination of vascular anomalies in the form of Fontan physiology and moyamoya disease with secondary polycythemia, posted for extracranial-intracranial bypass. We describe the perioperative management of this patient, maintaining stable hemodynamic on dual fronts, avoiding compromise in pulmonary circulation under positive pressure ventilation (a consideration in Fontan physiology), as well as cerebral ischemic complications (known risk with moyamoya disease). Only one case has been reported so far with a similar combination of vascular multimorbidity, in a patient, who underwent pial synangiosis.

Keywords

moyamoya disease - Fontan physiology - combined vascular morbidities

Publikationsverlauf

Artikel online veröffentlicht:
15. September 2025

• References

• 1 Fruitman DS. Hypoplastic left heart syndrome: prognosis and management options. Paediatr Child Health 2000; 5 (04) 219-225
• 2 Plappert L, Edwards S, Senatore A, De Martini A. The epidemiology of persons living with Fontan in 2020 and projections for 2030: development of an epidemiology model providing multinational estimates. Adv Ther 2022; 39 (02) 1004-1015
• 3 Bang OY, Fujimura M, Kim SK. The pathophysiology of moyamoya disease: an update. J Stroke 2016; 18 (01) 12-20
• 4 Mazza GA, Gribaudo E, Agnoletti G. The pathophysiology and complications of Fontan circulation. Acta Biomed 2021; 92 (05) e2021260
• 5 Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart 2016; 102 (14) 1081-1086
• 6 Laubham M, Blais B, Kamp AN. Atrial arrhythmias in adults with Fontan palliation. Cardiol Ther 2023; 12 (03) 473-487
• 7 Yang KJ, Mistry P, Ayrian E. Update on the anesthesia management in adult patients with moyamoya disease. Curr Opin Anaesthesiol 2024; 37 (05) 439-445
• 8 Subramaniam B, Soriano SG, Michael Scott R, Kussman BD. Anesthetic management of pial synangiosis and intracranial hemorrhage with a Fontan circulation. Paediatr Anaesth 2006; 16 (01) 72-76