Intraoperative management of liver transplantation in patients with hypertrophic cardiomyopathy: a review.

Hypertrophic cardiomyopathy (HCM) is a genetic disorder defined by the presence of a hypertrophied nondilated left ventricle in the absence of other known causes. Anatomic variants exist, and dynamic features of this disease process may include left ventricular outflow tract obstruction during systole, systolic anterior motion of the mitral valve, and mitral regurgitation. Patients with HCM are at higher risk for sudden cardiac death, stroke, atrial fibrillation, atrial reentrant tachycardia, syncope, and congestive heart failure (CHF). Few studies have evaluated the perioperative risk of noncardiac surgery in this patient population. However, there appears to be a relatively high incidence of perioperative adverse cardiac events, such as CHF, myocardial ischemia, stable and life-threatening arrhythmias, and transient hypotension. Interoperative challenges of patients with HCM are exacerbated in the setting of end-stage liver disease (ESLD) and liver transplantation. ESLD physiology includes relative hypovolemia, decreased systemic vascular resistance and arterial pressure, and hyperdynamic circulation characterized by increased cardiac output. General anesthesia, release of ascites, temporary occlusion of the inferior vena cava, and reperfusion of the donor liver can result in cardiovascular instability. Liver transplantation is associated with blood loss, hypovolemia, vasodilation, tachycardia, and hypotension. Anesthetic goals to limit the dynamic features of HCM include avoiding tachycardia and increased contractility, as well as maintaining preload and afterload. Transesophageal echocardiography (TEE) is an ideal monitoring technique for patients with HCM undergoing liver transplantation. Benefits of TEE include real-time visualization of cardiac function and structure, better indication of intravascular volume, and immediate evaluation of pharmacologic interventions.