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Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly.

ABSTRACT: The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family history of HCM and/or sudden cardiac death and the presence of exertional symptoms usually support HCM, though they are less likely in older patients with HCM, and poorly investigated in individuals with VSB. A more severe hypertrophy and the presence of left ventricular outflow obstruction are considered diagnostic of HCM, though stress echocardiography has not been consistently used in VSB. Mitral annulus calcification is very prevalent in both conditions, whereas a restrictive filling pattern is found in a minority of older patients with HCM. Genetic testing has low applicability in this differential diagnosis at the current time, given that a causative mutation is found in less than 10% of elderly patients with suspected HCM. Emerging imaging modalities that allow non-invasive detection of myocardial fibrosis and disarray may help, but have not been fully investigated. Nonetheless, there remains a considerable morphological overlap between the two conditions. Comprehensive studies, particularly imaging based, are warranted to offer a more evidence-based approach to elderly patients with focal septal thickening.

SUBMITTER: Canepa M

PROVIDER: S-EPMC6127866 | biostudies-literature | 2016 Jul

REPOSITORIES: biostudies-literature

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Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly.

Canepa Marco M Pozios Iraklis I Vianello Pier Filippo PF Ameri Pietro P Brunelli Claudio C Ferrucci Luigi L Abraham Theodore P TP

Heart (British Cardiac Society) 20160427 14

The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family h ...[more]

PMID: 27122487

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Project description:BackgroundThe thoracoscopic trans-mitral approach can not only facilitate exposure of the ventricular septum, mitral valve, and subvalvular apparatus, it also enables the surgeons to perform concomitant mitral valve intervention. This study aimed to determine the safety and efficacy of thoracoscopic trans-mitral septal myectomy in elderly patients with hypertrophic obstructive cardiomyopathy (HOCM).MethodsWe reviewed the demographic to clinical characteristics and outcomes of patients who underwent thoracoscopic trans-mitral septal myectomy in our center between April 2019 and April 2021. The population was divided into a younger group (<60 years) and an elderly group (≥60 years).ResultsThere were 46 and 20 patients in the younger and elderly groups, respectively. The majority of patients in the elderly group were female (39.1 vs. 80.0%, P < 0.01). Patients in the elderly group were more likely to be in New York Heart Association Class IV (2.2 vs. 80.0%, P < 0.01). The European System for Cardiac Operation Risk Evaluation II predicted mortality rates were significantly higher (3.97 ± 1.81 vs. 1.62 ± 0.86%, P < 0.01) in the elderly group. In the elderly group, a patient converted to median sternotomy due to left ventricular posterior free wall rupture following septal myectomy and mitral bioprosthetic valve replacement. The patient then underwent double-patch sandwich repair for rupture and mitral mechanical valve replacement and was eventually discharged. All patients in the elderly group were discharged, while one in the younger group died. No patient in the elderly group required permanent pacemaker implantation vs. one in the younger group. Patients in the elderly group were more likely to spend more time in the intensive care unit than those in the younger group (5.44 ± 5.80 days vs. 3.07 ± 2.72, P < 0.05). However, there was no significant intergroup difference in in-hospital mortality or complications. Importantly, the left ventricular outflow tract pressure gradient was significantly decreased from 96.15 ± 32.89 mmHg to 8.2 ± 3.42 mmHg with no residual obstruction in the elderly group. The interventricular septal thickness was significantly decreased from 19.73 ± 3.14 mm to 11.30 ± 2.23 mm. Postoperative mitral regurgitation severity was significantly improved in the elderly group.ConclusionThis study demonstrated that thoracoscopic trans-mitral septal myectomy is a feasible option for selected elderly patients with satisfactory outcomes similar to those of young patients.

Project description:Background: Apical hypertrophic cardiomyopathy (aHCM) is thought to have a more benign clinical course compared to septal HCM (sHCM), but most data have been derived from Asian cohorts. Comparative data on clinical outcome in Caucasian aHCM cohorts are scarce, and the results are conflicting. The aim of this study was to estimate the prevalence and outcome of aHCM in French-Canadians of Caucasian descent. Methods and results: We conducted a retrospective, single-center cohort study. The primary endpoint was a composite of documented sustained ventricular arrhythmia (VA), appropriate ICD therapy, arrhythmogenic syncope, cardiac arrest, or all-cause mortality. A total of 301 HCM patients (65% males) were enrolled including 80/301 (27%) with aHCM and 221/301 (73%) with sHCM. Maximal wall thickness was similar in both groups. Left ventricular apical aneurysm was significantly more common in aHCM (10 vs. 0.5%; p < 0.001). The proportion of patients with myocardial fibrosis ≥ 15% of the left ventricular mass was similar between aHCM and sHCM (21 vs. 24%; p = 0.68). Secondary prevention ICDs were more often implanted in aHCM patients (16 vs. 7%; p = 0.02). The primary endpoint occurred in 26% of aHCM and 10.4% of sHCM patients (p = 0.001) and was driven by an increased incidence of sustained VA (10 vs. 2.3%; p = 0.01). Multivariate analysis identified apical aneurysm and a phenotype of aHCM as independent predictors of the primary endpoint and the occurrence of sustained ventricular tachycardia. Unexplained syncope and a family history of sudden cardiac death were additional predictors for sustained VA. Apical HCM was associated with an increased risk of ventricular arrhythmia even when excluding patients with apical aneurysm. Conclusions: The phenotype of apical HCM is much more common in French-Canadians (27%) of Caucasian descent compared to other Caucasian HCM populations. Apical HCM in French-Canadians is associated with an increased risk for ventricular arrhythmia.

Project description:Surgical septal myectomy is increasingly utilized for patients with hypertrophic obstructive cardiomyopathy who remain symptomatic despite maximum doses of medical therapy. Deep and extensive septal muscle resections may lead to iatrogenic ventricular septal defects that are detected on transesophageal echocardiography immediately after weaning from cardiopulmonary bypass and immediately corrected in the same surgery. However markedly thinned out ventricular septum after myectomy may be prone to late rupture from high left ventricular systolic pressures causing delayed detection of a ventricular septal defect when the patients present with new onset symptoms. Additionally, a surgical injury to the first septal perforator artery during the myocardial resection leading to septal infarction may contribute to delayed occurrence of ventricular septal defect. Such a predisposing deep septal resection or septal infarction may be associated with varying degrees of atrioventricular nodal block warranting a permanent pacing. A new onset interventricular shunt from such an iatrogenic ventricular septal defect often leads to heart failure as the filling pressures increase disproportionately in the thick hypertrophied left ventricle. Transcatheter closure is an alternative to a high-risk repeat surgery. This report of device closure of two delayed septal ruptures after myectomy discusses the reasons, presentation, catheter approaches, and procedural challenges. <Learning objective: Deep extended septal myectomy for medically refractory hypertrophic obstructive cardiomyopathy may lead to early or late iatrogenic ventricular septal defect. Such an extensive loss of septal muscle may be additionally indicated by co-occurrence of atrioventricular nodal block that needs permanent pacing. Heart failure from the new onset interventricular shunt is not clinically tolerated by the hypertrophied left ventricle due to elevated end diastolic pressures. Transcatheter closure of the ventricular septal defect is an attractive alternative to a morbid repeat surgery.>.

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Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly.

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Distinguishing ventricular septal bulge versus hypertrophic cardiomyopathy in the elderly.

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