Hypertrophic cardiomyopathy in adults: An overview

ConclusionsAlthough HCM is the most prevalent genetic disorder affecting the heart, it often goes undiagnosed until midlife after patients show symptoms of myocardial remodeling. Adults with cardiomyopathy suffer SCD or adverse events such as stroke and heart failure from HCM. Early diagnosis will prevent SCD, improve quality of life, and slow patient's progression to heart failure. Implications for practiceEarly recognition of HCM in adults by their primary care providers will improve patients’ quality of life and reduce incidence of SCD, heart failure, and stroke.
Source: Journal of the American Academy of Nurse Practitioners - Category: Nursing Authors: Tags: CE ARTICLE Source Type: research

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Publication date: June 2019Source: Archives of Cardiovascular Diseases Supplements, Volume 11, Issue 3Author(s): C. Heuze, L. Legrand, A. Diallo, M.L. Monin, C. Ewenczyk, R. Isnard, E. Vicaut, A. Durr, F. PoussetIntroductionFriedreich ataxia (FRDA) is a rare genetic ataxia. The causal mutation is an expanded trinucleotide repeat (GAA) in the frataxin gene. Hypertrophic cardiomyopathy in FRDA is the major cause of early death. Patients with progressive decline of the left ventricular ejection fraction (LVEF) have the worst prognosis. The aim of the study was to evaluate the prognostic value of 2D global longitudinal strain ...
Source: Archives of Cardiovascular Diseases Supplements - Category: Cardiology Source Type: research
Conclusion: These findings limit the possible roles of gene transcriptional changes in previously reported age-dependent pro-arrhythmic electrophysiologial changes observed in Pgc-1β-/- atria to an altered Ca2+-ATPase (Atp2a2) expression. This directly parallels previously reported arrhythmic mechanism associated with p21-activated kinase type 1 deficiency. This could add to contributions from the direct physiological outcomes of mitochondrial dysfunction, whether through reactive oxygen species (ROS) production or altered Ca2+ homeostasis. Introduction Atrial arrhythmias constitute a major public health probl...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
ConclusionsThis study, using two independent sources to verify vital status and cause of mortality, confirms that survival following septal myectomy is excellent. Hypertrophic cardiomyopathy was identified as the primary cause of death in less than 20%, with mortality in most patients unrelated to hypertrophic cardiomyopathy.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
In this study, we describe early outcomes of patients with apical HCM following transapical myectomy and compare survival to that of patients with hypertrophic cardiomyopathy listed for heart transplantation.
Source: The Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Source Type: research
Left ventricular ejection fraction (LVEF) is a commonly used index of systolic function. However, the LVEF often remains normal in patients with hypertrophic cardiomyopathy (HCM) despite disease progression until the end-stage of the disease. This is because myocardial hypertrophy results in progressive declines in ventricular capacitance and end-diastolic volume, which are accompanied by reduction in stroke volume.1-5 Accordingly, the LVEF does not correlate with functional capacity in HCM6 and is not predictive of adverse events including worsening heart failure, arrhythmias, sudden death, or appropriate implantable card...
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: Clinical Investigation Source Type: research
In conclusion, CA may be associated with a favorable long-term clinical course in HCM patients with AF.
Source: Heart and Vessels - Category: Cardiology Source Type: research
ConclusionsThe cardiovascular death and cardiovascular events are significantly increased in patients with HOCM and CAD who underwent CABG at the time of septal myectomy.
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
Abstract Some patients with hypertrophic cardiomyopathy (HCM) develop systolic dysfunction, called the dilated phase of HCM (d-HCM), which is associated with increased morbidity and mortality. We conducted a retrospective study using an HCM database to clarify the incidence, clinical characteristics, and long-term outcomes of d-HCM. We analyzed an HCM cohort consisting of 434 patients (273 with apical HCM and 161 with non-apical HCM; 18 had obstructive HCM, 16 had dilated HCM, and 127 had other HCM) diagnosed by echocardiography in our hospital between 1991 and 2010. The follow-up period was 8.4 ± 6.7 years...
Source: The Keio Journal of Medicine - Category: Universities & Medical Training Authors: Tags: Keio J Med Source Type: research
ConclusionsHere we report the case of a patient with hereditary cardiac amyloidosis associated with a Pro24Ser mutation in transthyretin, which is the first case reported in Japan. Technetium pyrophosphate scintigraphy was extremely useful for definitive diagnosis. Thus, we propose that the nuclear imaging technique should be taken into account even for an exploratory diagnosis of transthyretin cardiac amyloidosis.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Publication date: October 2018Source: Journal of the Saudi Heart Association, Volume 30, Issue 4Author(s): Sara Saleh Aldwailah, Dikra Al-Dobai, Deema Alduaiji, Shatha Naser Almutari, Azra Mahmud, Mohammad AlGhamdiIntroductionHypertrophic Cardiomyopathy (HCM), a relatively uncommon entity, is the most common form of genetic cardiovascular disorders. It is the most common cause of sudden death in the young and also heart failure which can occur at any age. While HCM is a well-described global disorder, there is paucity of data on this important clinical entity in Saudi Arabia. Therefore, the aim of this study was to explore...
Source: Journal of the Saudi Heart Association - Category: Cardiology Source Type: research
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