Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction

TLDR

Nonobstructive hypertrophic cardiomyopathy has traditionally been viewed as the predominant form of the disease based on resting outflow gradient assessments. The study aimed to prospectively determine the prevalence, clinical profile, and significance of left‑ventricular outflow tract obstruction at rest and during exercise in a large cohort of HCM patients. A prospective analysis of 320 consecutive HCM patients measured LV outflow gradients at rest, with Valsalva, and during exercise echocardiography. Seventy percent of patients exhibited LV outflow obstruction at rest or during exercise, with 119 having resting gradients ≥50 mm Hg and an additional 106 developing exercise‑induced obstruction (including 76 with gradients ≥50 mm Hg and 46 symptomatic), while Valsalva underestimated obstruction and exercise echocardiography should be routinely used to identify patients who may benefit from septal reduction therapy.

Abstract

Nonobstructive hypertrophic cardiomyopathy (HCM) has been regarded as the predominant hemodynamic form of the disease on the basis of assessment of outflow gradient under resting conditions. We sought to prospectively define the prevalence, clinical profile, and significance of left ventricular (LV) outflow tract obstruction under resting conditions and with physiological exercise in a large HCM cohort.We prospectively analyzed 320 consecutive HCM patients (age, 47+/-17 years), measuring LV outflow gradient at rest, with Valsalva maneuver, and with exercise echocardiography. LV outflow obstruction was present at rest and/or with exercise in 225 patients (70%); 119 had rest gradients > or = 50 mm Hg and were not exercised. Of the other 201 patients with gradients < 50 mm Hg at rest (average, 4+/-9 mm Hg), 106 developed mechanical obstruction to LV outflow resulting from mitral valve-septal contact after exercise (80+/-43 mm Hg), including 76 with marked gradients > or = 50 mm Hg and 46 with heart failure symptoms. The remaining 95 patients (30%) had no or small gradients (< 30 mm Hg) both at rest and with exercise. Valsalva maneuver underestimated the presence and magnitude of exercise-induced obstruction.Among those patients who come to clinical evaluation, HCM is a predominantly obstructive disease in which LV outflow gradients, frequently associated with heart failure symptoms and often identified only with exercise, are evident in most patients (ie, 70%). Identification of LV outflow obstruction with exercise echocardiography may broaden management options in HCM by identifying symptomatic patients not otherwise regarded as potential candidates for septal reduction therapy. Assessment of subaortic gradients with exercise should be a routine component of the evaluation of HCM patients without outflow obstruction under resting conditions.