Significant biventricular obstruction in non-syndromic hypertrophic cardiomyopathy

Abstract

DESCRIPTIONAlthough hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle (LV), we present a rare case of biventricular HCM presenting in middle age.A 57-year-old man without a family history of HCM and sudden death presented with presyncope and New York Heart Association (NYHA) class II breathlessness. Clinical examination was normal except for ejection systolic murmur. ECG showed biventricular hypertrophy with deep T-wave inversions. Echocardiography demonstrated pronounced asymmetric LV hypertrophy and thickened right ventricle (RV) muscular components (figure 1A-C; videos 1-3). Doppler revealed a peak LV mid-cavity gradient of 109 mm Hg and subvalvar obstruction of the RV outflow tract with a peak gradient of 138 mm Hg ( figure 2A-C). The patient underwent successful surgical myectomy and has been stable during the course of follow-up. The histopathological specimen revealed myofibril disarray as is expected in a case of HCM (figure 3). Genetic testing for Noonan's syndrome and three of the most common mutations were performed which were negative.

Mid-term outcomes of biventricular obstruction and left ventricular outflow tract obstruction after surgery correction in child and adolescent patients with hypertrophic cardiomyopathy

“…The previous works indicated that biventricular resection was ineffective and associated with a high risk of death. However, biventricular resection was successfully performed in a 54-year-old woman (LVIPG = 150 mmHG, RVIPG = 130 mmHG) [ 25 ] and a 57-year-old man (LVIPG = 109 mmHg, RVIPG = 138 mmHg) [ 26 ] with lasting outcomes. Quintana, Johnson [ 19 ] recently indicated that relief of BVOTO could be achieved with low mortality and good long-term outcomes via biventricular resection in children and young adults.…”

Right Ventricle Outflow Obstruction in Biventricular Hypertrophic Cardiomyopathy in Amyloidosis

“…In this report, we describe the very rare condition of biventricular hypertrophy with right ventricle obstruction. This condition is extremely uncommon and results from excessive muscular hypertrophy [4] . Analysis of speckle tracking of the myocardium showed typical normal longitudinal strain values at the apex of the left ventricle, and reduced strain at all basal segments, suggesting amyloidosis.…”