The Prevalence and Long-Term Outcomes of Extreme Right versus Extreme Left Ventricular Hypertrophic Cardiomyopathy

Abstract

Objectives: Extreme left ventricular hypertrophy (LVH) is a known risk factor for sudden cardiac death in hypertrophic cardiomyopathy (HCM). Extreme right ventricular hypertrophy (RVH) is rare, and whether it is linked to a poor outcome is unknown. This study was designed to investigate differences between HCM patients with extreme RVH and those with extreme LVH. Methods: Among 2,413 HCM patients, 31 with extreme RVH (maximum right ventricular wall thickness ≥10 mm) and 194 with extreme LVH (maximum left ventricular wall thickness ≥30 mm) were investigated. The main clinical features and natural history were compared between the 2 groups. Results: The prevalence of extreme RVH and extreme LVH was 1.3 and 8.0%, respectively. Patients with extreme RVH tended to be younger and female (p < 0.01). Cardiovascular-related mortality and morbidity within 10 years were significantly greater in the extreme RVH group (p < 0.05). Multivariate analysis demonstrated 3 independent predictors for cardiovascular mortality - extreme RVH, left ventricular end-diastolic dimension ≥50 mm, and age ≤18 years at baseline - and 2 for morbidity - extreme RVH and presyncope. Conclusions: Compared with extreme LVH, extreme RVH was quite uncommon in HCM and had a worse prognosis. A right ventricle examination should be performed in routine HCM evaluation.

Comparison of Long-Term Outcome between Apical and Asymmetric Septal Hypertrophic Cardiomyopathy

“…Female gender was associated with a worse prognosis, and especially a higher rate of HF [12,23]. Patients who were diagnosed at a young age were inclined to experience cardiovascular death [11]. LVOTO could adversely affect the HCM prognosis [13,14].…”

“…LVOTO was defined as a peak pressure gradient of LV outflow tract ≥30 mm Hg at rest or after provocation [20]. The diagnostic criterion for extreme LV hypertrophy was a maximum LV wall thickness of ≥30 mm [11]. …”

“…Several studies have compared the long-term prognosis between ApHCM and asymmetric septal HCM (ASHCM) [6,8,9]. However, factors such as age at diagnosis [5,10,11], gender [5,12], and LV outflow tract obstruction (LVOTO) at rest [13] or provocation [14], which have been shown to interfere with prognosis, were not considered in the previous studies. We intended to compare the long-term outcome between ApHCM and ASHCM after eliminating confounding through matching.…”

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

“…The LVOT and RVOT pressure gradients were measured using continuous-wave Doppler echocardiography, at rest and after provocative maneuvers. Patients were considered to have biventricular obstruction if the LVOT pressure gradient was ≥30 mm Hg and RVOT pressure gradient was ≥25 mm Hg at the same time[ 14 , 15 ]. Patients with rheumatic valvular heart disease, double-chambered RV, pulmonary stenosis, or pulmonary arterial hypertension were excluded.…”

Echocardiographic Assessment of Right Ventricular Size and Function in Cats With Hypertrophic Cardiomyopathy

“…7 hypertrophy is associated with increased risk of hospitalization for congestive heart failure (CHF), thromboembolic complications, ventricular tachyarrhythmias, and sudden death; 9,10 and, severe RV hypertrophy, albeit rare, is associated with worse prognosis in humans with HCM. 11 The impact of RV performance in the clinical status and outcome of humans affected with HCM also has been studied. [12][13][14][15] However, in cats, studies focusing on the assessment of RV size and function are limited, and it is unknown whether RV functional abnormalities occur in cats with HCM.…”