Kevin C. Ong

Atrial Fibrillation in Hypertrophic Cardiomyopathy: Prevalence, Clinical Correlations, and Mortality in a Large High‐Risk Population

Background Atrial fibrillation (AF) is a common sequela of hypertrophic cardiomyopathy (HCM), but evidence on its prevalence, risk factors, and effect on mortality is sparse. We sought to evaluate the prevalence of AF, identify clinical and echocardiographic correlates, and assess its effect on mortality in a large high‐risk HCM population. Methods and Results We identified HCM patients who underwent evaluation at our institution from 1975 to 2012. AF was defined by known history (either chronic or paroxysmal), electrocardiogram, or Holter monitoring at index visit. We examined clinical and echocardiographic variables in association with AF. The effect of AF on overall and cause‐specific mortality was evaluated with multivariate Cox proportional hazards models. Of 3673 patients with HCM, 650 (18%) had AF. Patients with AF were older and more symptomatic (P<0.001). AF was less common among patients with obstructive HCM phenotype and was associated with larger left atria, higher E/e’ ratios, and worse cardiopulmonary exercise tolerance (all P values<0.001). During median (interquartile range) follow‐up of 4.1 (0.2 to 10) years, 1069 (29%) patients died. Patients with AF had worse survival compared to those without AF (P<0.001). In multivariate analysis adjusted for established risk factors of mortality in HCM, the hazard ratio (95% confidence interval) for the effect of AF on overall mortality was 1.48 (1.27 to 1.71). AF did not have an effect on sudden or nonsudden cardiac death. Conclusions In this large referral HCM population, approximately 1 in 5 patients had AF. AF was a strong predictor of mortality, even after adjustment for established risk factors.

Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy.

AIMS Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM. METHODS AND RESULTS The study cohort consisted of 1570 (54 ± 15 years; 53% male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) >36 mmHg] was identified in 38% of patients who were older (57 ± 15 vs. 52 ± 15 years, P < 0.0001), more likely to be female (59 vs. 40%, P < 0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45%, P < 0.0001) and atrial fibrillation (29 vs. 15%, P < 0.0001) vs. those without PH. Only 12% had moderate or severe PH (PASP >50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95% CI 1.28-1.96, P < 0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95% CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT. CONCLUSIONS Over one-third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.

Transapical myectomy for severe mid-ventricular obstructive hypertrophic cardiomyopathy

An 83-year-old woman with hypertrophic cardiomyopathy was referred with progressive exertional dyspnoea. She had marked apical and mid-ventricular hypertrophy with mid-ventricular obstruction and a small apical aneurysm ( Panels A1–3 , Supplementary material online, Video S1 ). Doppler echocardiography through the left ventricle ( Panel B1 ) identified an early peak systolic gradient of 36 mmHg ( Panel B1 …

A notch in time: midventricular hypertrophic cardiomyopathy

Imaging of a 62-year-old woman with hypertrophic cardiomyopathy revealed mid-septal hypertrophy (22 mm), an apical aneurysm ( Panel A ), and midventricular obstruction (MVO) with an unusual continuous-wave Doppler (CWD) profile ( Panel B ). Initially, CWD showed an increase in midventricular systolic velocities to 2.7 m/s ( Panels B 1 and C 1). In midsystole, MVO became sufficiently severe to cause …

I can hear it, but where is it coming from? A case of iatrogenic arteriovenous fistula after pacemaker lead extraction

A 54-year-old woman was referred to our institution for the assessment of a loud continuous murmur detected 3 years after pacemaker lead extraction. She previously had a dual-chamber pacemaker implanted for symptomatic sinus node dysfunction. Owing to recurrent and severe symptomatic phrenic nerve pacing, she underwent laser lead extraction. The procedure was complicated by a tear at junction of the superior vena …

Three-Dimensional Echocardiographic Assessment of Mitral Annular Physiology in Patients With Degenerative Mitral Valve Regurgitation Undergoing Surgical Repair: Comparison between Early- and Late-Stage Severe Mitral Regurgitation

Background: Ventricular‐annular decoupling is thought to exist in all degenerative myxomatous mitral valve (MV) diseases. However, the annular physiology of degenerative MV disease may differ when severe mitral regurgitation (MR) presents at different stages. The aim of this study was to assess differences in mitral annular physiology and surgical effects between early‐ and late‐stage severe MR. Methods: Three‐dimensional (3D) transesophageal echocardiography was performed before and after MV surgery in 74 patients with degenerative MV disease, including 57 with early‐stage severe MR (without left ventricular remodeling) and 17 with late‐stage MR (with left ventricular remodeling). A control group comprised 46 patients without MV disease. Novel 3D MV software was used to evaluate mitral annular dynamics. The degree of annular saddle shape was calculated as the ratio of annular height (AH) to lateromedial diameter (LM). Ventricular‐annular decoupling was defined as insufficient systolic AH/LM compared with the control group. Results: Prebypass 3D measurements demonstrated that systolic AH/LM in the early‐stage group (0.19 ± 0.04) was similar to that in the control group (0.21 ± 0.05; P = .101), while systolic AH/LM in the late‐stage group (0.17 ± 0.04) was lower than that in the control group (P = .011). Postbypass comparison showed saddle shape accentuation in the early‐stage group (0.20 ± 0.04), similar to that in the control group (P = .3127); the mitral annulus remained flat in the late‐stage group (0.17 ± 0.03; P = .004). Conclusions: Ventricular‐annular decoupling, present in the late‐stage group, was absent in the early‐stage group. MV repair surgery did not disrupt mitral annular saddle shape in the early‐stage group; however, it failed to correct annular dysfunction in the late‐stage group. Sequential 3D transesophageal echocardiographic analysis provides comprehensive mitral annular evaluation beyond conventional two‐dimensional parameters for determining stages of severe MR. HIGHLIGHTSVentricular‐annular decoupling is thought to exist in all degenerative severe MR.Mitral annular saddle shape was compared between early‐ and late‐stage severe MR.Ventricular‐annular decoupling seen in late‐severe MR is absent in early‐stage MR.Surgery in early‐stage severe MR preserves mitral annular function.3D TEE provides full mitral annular evaluation which is beyond 2D parameters.