Suspected Malignant Hyperthermia During Biventricular Assist Device Implantation in a Patient With Left Ventricular Noncompaction Cardiomyopathy.

MALIGNANT HYPERTHERMIA (MH) is an inherited, pharmacogenetic disorder of skeletal muscle that predisposes susceptible individuals to a life-threatening hypermetabolic reaction after exposure to volatile anesthetics and depolarizing muscle relaxants.(1-3) The key clinical features of MH are hemodynamic instability, hypercapnia, metabolic acidosis, hyperthermia, and rhabdomyolysis.(1-3) In cardiac anesthesia, classic MH symptoms can be obscured because patients often undergo periods of hemodynamic instability with metabolic derangements, especially after separation from cardiopulmonary bypass (CPB).(2) For patients with cardiovascular diseases and accompanying neuromuscular comorbidities, the widespread use of volatile anesthetics in cardiac surgery requires special vigilance for MH susceptibility (MHS). Left ventricular (LV) noncompaction cardiomyopathy (LVNC) is characterized by a distinctive morphologic appearance of the left ventricular myocardium,(4,5) albeit of uncertain incidence and prevalence.(5,6) Data from a single center showed an LVNC prevalence of 3% in patients with heart failure.(7) LVNC is a genetically and clinically heterogenous disorder that can occur isolated or in association with genetic syndromes, congenital heart defects, and neuromuscular disorders.(5,6,8) The authors report the perioperative course of an LVNC heart failure patient who developed MH during implantation of a biventricular assist device (BiVAD) (EXCOR Adult; Berlin Heart GmbH, Berlin, Germany) and discuss potential implications for risk assessment regarding MHS in patients with LVNC.