The common causes of subaortic left ventricular outflow tract obstruction (LVOTO) are hypertrophic cardiomyopathy (HCM) and membranous/tunnel subaortic stenosis (SAS). Reoperation after corrective surgery may be due to recurrent disease, associated congenital defects, or complications of the initial procedure. This study compares the late outcomes of young patients with HCM and SAS. We studied clinical, echocardiographic, and operative data of patients ≤21 years of age at the time of surgery for LVOTO between August 1963 and August 2018. We stratified patients into HCM (n = 152) and congenital SAS (n = 63) groups and compared survival and cumulative incidence of reoperation. At initial repair, patients with HCM were older than patients with SAS (median [interquartile range] age, 15 [10-19] years vs 8 [5-13] years; P < .001), and patients with HCM were more symptomatic with dyspnea (P < .001), chest pain (P = .002), and presyncope/syncope (P = .005). Thirty-day mortality was 1.3% vs 0% for HCM and SAS groups. During a median follow-up of 13.1 years, survival was similar through the first 10 years; but during the second decade, patients with HCM had poorer survival (survival at 20 years, 80% vs 91% for patients with SAS; P = .007). Ten years after repair, reoperation for recurrent LVOTO was performed in 5% of patients with HCM vs 31% in those with SAS (P < .001). In this surgical cohort, patients with HCM were more symptomatic preoperatively than those with SAS. Late survival of patients with SAS was superior to that of patients with HCM despite a greater need for reoperation.

Left ventricular outflow tract (LVOT) obstruction is a component of many forms of congenital heart disease, including hypertrophic cardiomyopathy, membranous subaortic stenosis, tunnel subaortic stenosis, and outflow tract obstruction related to atrioventricular septal defects. We have gained a particularly extensive experience with the diagnosis and treatment of hypertrophic cardiomyopathy, having performed septal myectomy in over 3,800 patients. In the setting of this review of LVOT obstruction, we use hypertrophic cardiomyopathy as a template by which other pathologies causing LVOT obstruction can be understood. We review important surgical issues in patient selection, diagnostic evaluation, interpretation of imaging, and operative management. To this end, the review focuses on obstructive hypertrophic cardiomyopathy and then broadens to discuss other pathologies causing LVOT obstruction, with important similarities and differences in their management. These other pathologies share some similar presentations and operative techniques, and at times can be confused with hypertrophic cardiomyopathy, but also have important distinctions of which the surgeon should be aware.