Craniotomy in a Case of Eisenmenger’s Syndrome with Pulmonary Artery Hypertension: A Neuroanesthetic Challenge

Background: The Eisenmenger syndrome is a form of cyanotic congenital heart disease not amenable to corrective surgery. It develops in patients with left-to-right shunts that results in concomitant pulmonary hypertension and right heart volume overload. The reported perioperative mortality of these critically ill patients is as high as 20 to 30%.

Case Description: An 18-year-old female patient presented to our institute for neurosurgical management of third ventricular colloid cyst. She was a diagnosed case of double-outlet right ventricle (DORV) with patent ductus arteriosus (PDA) with hypoplastic left aortic arch with pulmonary arterial hypertension (PAH) and Eisenmenger syndrome. She underwent right frontal craniotomy and tumor decompression under general anesthesia. She was managed with the hemodynamic goals to avoid any decrease in preload, decrease in systemic vascular resistance, increase in pulmonary vascular resistance, and decrease in myocardial contractility.

Conclusion: We would like to discuss the unique set of challenges encountered in perioperative management of these critically ill patients with reference to the present guidelines available and share how we managed this case.

Publication History

Article published online:
25 March 2020