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.

Anesthetic Challenges in the Management of Intracranial Aneurysm Clipping in a Patient with Hypertrophic Cardiomyopathy

“…Choice of drugs to mitigate this stress response in HCM is limited. 3,4 Dexmedetomidine has emerged as an excellent anesthetic adjuvant for aneurysmal surgeries due to its ability to attenuate stress response and maintain stable hemodynamics perioperatively. 10 Its opioid sparing and antiemetic action ensures rapid yet smooth emergence from anesthesia allowing early assessment after neurosurgery.…”

“…Hypotension and bradycardia can be prevented by skipping the loading dose. 4 Single case series documents use of dexmedetomidine in two pediatric patients with HCM undergoing noncardiac surgery. 4 Its use in adults with HCM undergoing aneurysmal surgery has not been reported yet, making our case first of a kind.…”

“…2 Anesthetic management of such highrisk comorbidities is troublesome and limited literature is available in this context. 3,4 We describe the perioperative management of a patient with HCM undergoing emergency anterior communicating artery (ACOM) aneurysm clipping and discuss the pertinent anesthetic concerns.…”

“…2 Anesthetic management of such high-risk comorbidities is troublesome and limited literature is available in this context. 3 4…”

Use of peripheral nerve blocks in perioperative management of cases with hypertrophic cardiomyopathy undergoing lower limb orthopedic surgeries

“…[ 3 ] Most important parameters are preload, afterload and ventricular contractility that are prone to fluctuations in HOCM patients in the perioperative period due to the surgical procedure, anesthetic agents and changes in intravascular volume. [ 4 ] These circulatory changes may increase the pressure gradient across LVOT. [ 3 5 ] These lead to increased chances of arrhythmias and myocardial ischemia and can pose significant morbidity and mortality in HCM patients perioperatively.…”