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
