Anaesthesia for acute subarachnoid hemorrhage in a patient with Eisenmenger Syndrome: A case report

Alka Makovsek, Natasa Kovac, Ivona Hanzek, Matija Majic
Background: Eisenmenger syndrome is classified as a cyanotic congenital heart disease that develops in patients with left to right (L-R) intracardiac shunts. These shunts progressively damage pulmonary vascular tissue, resulting in increased pulmonary vascular resistance (PVR) and pulmonary hypertension. When PVR exceeds systemic vascular resistance (SVR), the shunt flow reverses into right to left (R-L). This paper discusses the anaesthesia of an Eisenmerger syndrome patient with severe pulmonary hypertension and transposition of the great arteries, who presented with acute subarachnoid haemorrhage (SAH) due to cerebral arteriovenous fistula.
Case report: This case study describes a 27-year-old female who presented with headaches and a grand mal seizure. She was diagnosed with SAH and an emergency embolization of the AVf was performed. Her medical history revealed that she was born with dextro-transposition of the great arteries. She underwent a Rashkind septostomy after birth. Later, the patient developed pulmonary hypertension and Eisenmenger syndrome, which resulted in chronic respiratory insufficiency, polyglobulinemia, and osteoarthropathy, as well as asthma. 
Discussion: Patients with Eisenmenger syndrome have a high perioperative mortality rate due to their complex pathology. The principal challenge is to keep SVR from decreasing and PVR from increasing. Decreased SVR results in greater shunt flow, which results in exaggerated hypoxia, which is resistant to increasing FiO2. In fact, increasing FiO2 would be detrimental as it could also lead to an increase in shunt flow. In our case, we had to consider both the patient’s primary pathology and her acute brain trauma. Balanced anesthesia was induced using fentanyl, etomidate, and rocuronium. Sevoflurane was chosen to maintain anaesthesia. These drugs were chosen to maximize hemodynamic stability.
The procedure passed uneventfully, and the patient was extubated soon afterward. Neurological examination revealed that her hemiparesis had regressed. 

Learning point: 
Eisenmenger patients are challenging because of their complex pathophysiology. The main objective is to avoid disrupting the patient’s unusual physiological balance between PVR and SVR, because any changes could result in increased intracardiac shunt volume, thus leading to hypoxia and associated pathology.

Anaesthesia for acute subarachnoid hemorrhage in a patient with Eisenmenger syndrome: a case report