Perioperative Management of Two Pediatric Patients With Hypertrophic Cardiomyopathy Undergoing Minimally Invasive Surgical Procedures

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common genetic disease of the heart and remains an important etiology of sudden cardiac death in adolescents and young adults. Autosomal dominant inheritance of a mutation in one of the numerous genes encoding for proteins in the myocardial sarcomere leads to an abnormal myocyte growth pattern with hypertrophy. Perioperative management of patients with HCM for any type of surgical procedure carries an increased risk of morbidity and mortality related to the propensity for arrhythmias and myocardial ischemia. During the perioperative period, myocardial function may be impacted by alterations in preload, afterload, and myocardial contractility related to the surgical procedure, anesthetic agents, and changes in intravascular volume. We present two pediatric patients with a known diagnosis of HCM who presented for non-cardiac, minimally invasive surgical (MIS) procedures. The perioperative implications of HCM are reviewed with particular emphasis placed on the potential cardiovascular alterations induced by the MIS procedure with increases in intra-thoracic or intra-abdominal pressure. J Med Cases. 2016;7(4):115-119 doi: http://dx.doi.org/10.14740/jmc2410w