Abstract 4876: LAMP2 Cardiomyopathy is a Particularly Adverse Phenocopy of Sarcomeric Hypertrophic Cardiomyopathy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Barry Maron ◽  
William C Roberts ◽  
Michael Arad ◽  
Carolyn Y Ho ◽  
Tammy S Haas ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Heart Transplantation ◽  
Systolic Dysfunction ◽  
Ventricular Tachyarrhythmia ◽  
Young Patients ◽  
Disease Process ◽  
Left Ventricular ◽  
Asymptomatic Patients

Mutations in the X-linked lysosome-associated membrane protein gene (LAMP2; Danon disease) produce a morphologic phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM) in young patients, characterized by extreme left ventricular (LV) hypertrophy and pre-excitation. However, the natural history of this newly recognized cardiomyopathy is incompletely resolved. Seven young asymptomatic patients with LAMP2 cardiomyopathy were identified at ages 8 to 15 years; 6 were male. LV hypertrophy was particularly marked (septal thickness 25– 65 mm; mean 42±17) in the presence of nondilated LV cavity. On each ECG, Wolff-Parkinson-White pre-excitation pattern was associated with markedly increased voltages (74±38mm for R- or S-wave). Over the 7±3 year follow-up from initial cardiac diagnosis, all 7 patients experienced particularly adverse disease consequences associated with progressive LV wall thinning and cavity dilatation and systolic dysfunction (ejection fraction, 29±7%) by the ages of 12 – 24 years (mean 20). Of the 7 patients, 5 either died of progressive heart failure, had heart transplantation or were considered for a donor heart; 2 others had sudden death events, including one fatal ventricular tachyarrhythmia refractory to defibrillator therapy and one appropriate defibrillator shock in an asymptomatic female survivor. Pathologic examination of hearts at autopsy showed histopathologic findings compatible with both HCM due to sarcomere protein mutations (i.e., extensive myocyte disarray, intramural small vessel disease, myocardial replacement scarring), and also evidence of a storage disease process (i.e., clusters of myocytes with vacuolated sarcoplasm within fibrotic areas). Heart weights, 1266 and 1425 grams, are the most substantial recorded for hypertrophic cardiomyopathies. LAMP2 cardiomyopathy is a uniformly profound, and particularly deleterious disease entity, causing refractory heart failure with systolic dysfunction as well as sudden death in young patients < 25 years of age. This novel phenocopy of sarcomeric HCM underscores the power of molecular diagnosis for predicting prognosis, and should also raise consideration for intervention with early heart transplantation.

Abstract 19346: Peak Oxygen Consumption Predicts Outcome in Hypertrophic Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Carla Contaldi ◽  
Raffaella Lombardi ◽  
Alessandra Giamundo ◽  
Sandro Betocchi
Keyword(s):  
Heart Failure ◽  
Oxygen Consumption ◽  
Hypertrophic Cardiomyopathy ◽  
Nyha Class ◽  
Left Ventricular ◽  
Exercise Intolerance ◽  
Peak Oxygen Consumption ◽  
Class Iii ◽  
Asymptomatic Patients

Introduction: Peak oxygen consumption (VO 2 ) has a strong and independent prognostic value in systolic heart failure; in contrast no data support its prognostic role in hypertrophic cardiomyopathy (HCM). Hypothesis: We assess if peak VO 2 is a long-term predictor of outcome in HCM. Methods: We studied 92 HCM patients (40±15 years). Peak VO 2 was expressed as percentage (%) of the predicted value. Follow up was 76±57 months. The primary composite endpoint (CE) was atrial fibrillation, progression to NYHA class III or IV, myotomy-myectomy (MM), heart transplantation (HT) and cardiac death. An ancillary endpoint (HFE) included markers of heart failure (progression to NYHA class III or IV, MM and HT). Results: At baseline, 62% of patients were asymptomatic, 35% NYHA class II and 3% NYHA class III; 26% had left ventricular outflow tract obstruction. During follow up, 30 patients met CE with 43 events. By multivariate Cox survival analysis, we analyzed 2 models, using the CE, and in turn HFE. For CE, maximal left atrial diameter (LAD) (HR: 1.12; 95% CI: 1.04 to 1.22), maximal wall thickness (MWT) (HR: 0.14; 95% CI: 1.04 to 1.23) and % predicted peak VO 2 (HR: -0.03; 95% CI: 0.95 to 0.99) independently predicted outcome (overall, p<0.0001). For HFE, maximal LAD (HR:0.31; 95% CI: 1.09 to 1.70), MWT (HR: 0.35; 95% CI: 1.08 to 1.84) and % predicted peak VO 2 (HR: -0.06; 95% CI: 0.89 to 0.98) independently predicted outcome (overall, p<0.0001). Only 19% of mildly symptomatic or asymptomatic patients with % predicted peak VO 2 >80% had events, as opposed to 53% of them with % predicted peak VO 2 < 55% (p= 0.04). Event-free survival for both endpoints was significantly lower in patients with % predicted peak VO 2 < 55% as compared to those with it between 55 and 80 and >80% , Figure. Conclusion: In mildly or asymptomatic patients severe exercise intolerance may precede clinical deterioration. In HCM, peak VO 2 provides excellent risk stratification with a high event rate in patients with % predicted value <55%.

Abstract 13943: Hypertrophic Cardiomyopathy with Left Ventricular Apical Aneurysm Represents a High-Risk Subgroup Necessitating Aggressive Preventive Therapy: A Long-term Follow-Up Study

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Death ◽  
Left Ventricular ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Apical Aneurysm

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.

MIOCARDIOPATIA NÃO COMPACTADA - UMA REVISÃO DA LITERATURA

2018 ◽  
Vol 5 (2) ◽  
pp. 74-78
Author(s):  
Danilo Silva Sousa ◽  
Eduardo Akio Pereira I ◽  
Carlos Roberto de Oliveira Júnior ◽  
Ricardo Mendonça de Paula ◽  
Genildo Ferreira Nunes
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Heart Association ◽  
Clinical Findings ◽  
Left Ventricular ◽  
World Health ◽  
Main Diagnosis ◽  
Insuficiencia Cardiaca ◽  
Health Organization

RESUMO Introdução - A miocardiopatia não compactada (MNC) é uma patologia de ocorrência familiar com histórico relevante de morte súbita e insuficiência cardíaca; é considerada como uma miocardiopatia não classificada pela Organização Mundial da Saúde (OMS). Segundo a Associação Americana de Cardiologia, é uma cardiomiopatia primária. Desenvolvimento - A MNC resulta de uma alteração genética que leva a uma parada do processo de compactação miocárdica, caracterizada pela persistência de trabeculações e recessos profundos que se comunicam com a cavidade ventricular. A apresentação clínica inicial varia de assintomático a apresentação de sintomas relacionados à dor torácica, insuficiência cardíaca e arritmias. Os principais métodos de diagnóstico para MNC são estudos ecocardiográficos e ressonância magnética cardíaca, tendo como principais diagnósticos diferenciais a forma apical de cardiomiopatia hipertrófica, a combinação de cardiomiopatia hipertrófica apical e MNC, cardiomiopatia hipertensiva, fibroelastose endocárdica, trombo apical ou tumores entre outros. Considerações finais - A terapêutica disponível inclui tratamento para insuficiência cardíaca, arritmias e eventos tromboembólicos, mas é necessário particularizar a terapêutica relacionada à anticoagulação e prevenção primária de morte súbita cardíaca.   Palavras-chave: Cardiomiopatias; insuficiência cardíaca; arritmias cardíacas. ABSTRACT The left ventricular noncompaction (LVNC) is a patology with a familiar occurrence and relevant historic of sudden death and heart failure; it's considered an unclassified cardiomyopathy by World Health Organization (WHO). According to American Heart Association (AHA), it's a primary cardiomyopathy. Development - The LVNC results in a genetic alteration which leads to stop miocardial compaction process, characterized by persistence of trabeculation and deep intertrabecular recesses communicating with the ventricular cavity. The initial clinical findings may vary from asymptomatic to chest pain, heart failure and arrhythmias. The main diagnosis methods for LVNC are echocardiography and cardiac magnetic resonance imaging, having as main differential diagnosis the apical form of hypertrophic cardiomyopathy, a combination of both apical hypertrophic cardiomyopathy and LVNC, hypertensive cardiomyopathy, endocardial fibroelastosis, apical thrombus, or tumours among others. Final considerations - The menagement available includes the treatment for heart failure, arrhythmias and thromboembolic events, but it's neceassary particularize the therapeutics related to anticoagulation and primary prevention of sudden death. Keywords: Cardiomyopathies; heart failure; cardiac arrhythmias.

scholarly journals Graves’ Thyrotoxicosis-Induced Reversible Cardiomyopathy: A Case Report

2013 ◽  
Vol 6 ◽  
pp. CCRep.S10534 ◽  
Author(s):  
Ahmad S. Al-Ghamdi ◽  
Naji Aljohani
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Case Report ◽  
Ejection Fraction ◽  
Systolic Dysfunction ◽  
Clinical Status ◽  
Young Patients ◽  
Left Ventricular ◽  
Graves Disease ◽  
Rapid Improvement

The objective of this report is to present a case of Graves’ thyrotoxicosis-induced cardiomyopathy. This is a case of a 26 year old woman that presented with severe symptomatic congestive heart failure and was subsequently diagnosed with dilated cardiomyopathy secondary to Graves’ disease. Despite an initial left ventricular systolic ejection fraction of 20% on echocardiography, treatment with anti-thyroid agents led to rapid improvement of her clinical status and normalization of her ejection fraction. The proposed mechanisms underlying the development of systolic dysfunction in thyrotoxicosis are discussed and the literature on similar cases previously reported is highlighted. Cardiomyopathy should be considered even in young patients with Graves’ thyrotoxicosis.

scholarly journals Heart transplantation in patients with hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Marta Farrero Torres ◽  
Felix Perez-Villa
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Life Expectancy ◽  
Sudden Death ◽  
Systolic Dysfunction ◽  
Treatment Options ◽  
Advanced Heart Failure ◽  
Clinical Presentations ◽  
End Stage ◽  
Ventricular Filling

[first paragraph of article]Hypertrophic cardiomyopathy has a broad spectrum of clinical presentations, from asymptomatic to patients with advanced heart failure and sudden death. Treatment options are limited, especially in non-obstructive forms. A minority of patients (around 3.5%) can progress to an end-stage state, characterized by systolic dysfunction and restrictive ventricular filling, related to extensive fibrotic replacement and chamber remodeling. In these cases, life expectancy is significantly reduced: a mean 3-year survival time has been reported.

P1243Comparison of long-term clinical course and outcome of MYBPC3 - versus MYH7 - related hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Fumagalli ◽  
E Fedele ◽  
M Beltrami ◽  
N Maurizi ◽  
S Passantino ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Diastolic Dysfunction ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Septal Myectomy ◽  
Lv Dysfunction ◽  
Instrumental Records

Abstract Introduction The presence of sarcomere mutations is a powerful predictor of heart failure-related outcomes in Hypertrophic Cardiomyopathy (HCM). However, whether the prevalence of left ventricular (LV) dysfunction differs in patients with mutations in the two most prevalent HCM-associated genes (i.e. MYBPC3 and MYH7) is unclear. Purpose To ascertain lifetime trends in prevalence of LV dysfunction in HCM associated with pathogenic or likely-pathogenic MYBPC3 versus MYH7 mutations. Methods Clinical and instrumental records of 402 HCM patients with MYBPC3 (N=251) or MYH7 (N=151) mutations were retrospectively reviewed. Presence of systolic dysfunction (ejection fraction [EF] <50%) and diastolic dysfunction (Grade II and III) were assessed for each patient. In vitro analysis of septal myectomy samples was performed to further compare electro-mechanic properties of MYBC3 and MYH7 patients. Results Patients were diagnosed at a mean age of 39±17 years and 63% were men. At first evaluation MYBPC3-HCM patients were less frequently obstructive (15% vs 26% in MYH7; p=0.005) and had lower LVEF (61±11% vs 64±9%; p=0.01). Prevalence of diastolic dysfunction increased with age and was lowest in MYBPC3 patients <40 years at diagnosis (19.5% vs 35.4% in MYH7, p=0.043). At a mean follow-up (FU) of 13±11 years, patients developed comparable left atrium enlargement (MYBPC3 52±29 ml/m2 vs 41±18 at baseline, p<0.001; MYH7 54±25ml/m2 vs 45±22, p=0.003). Prevalence of diastolic dysfunction was also similar. MYBPC3 patients had lower LVEF at final evaluation (61±11% vs 64±9% in MYH7, p=0.01) with greater prevalence of overt systolic dysfunction (EF<50%, MYBPC3 vs MYH7: 15% vs 5%, OR: 2.3 95% CI: 1.2–5.8, p=0.013). No significant differences were observed in terms of NYHA class change, atrial fibrillation, stroke, heart failure, appropriate ICD intervention or cardiovascular death. However, prevalence of NSVT was higher for MYBPC3 (39% vs 14% in MYH7, p<0.0001). At Cox multivariable analysis independent predictors of systolic dysfunction at follow-up were MYBPC3 positive status (HR 2.53 95% CI: 1.09–5.82, p=0.029) and age at initial evaluation (HR 1.03 95% CI 1.00–1.06, p=0.027). In vitro cross-sectional evaluation of myocardial samples taken during septal myectomy at different ages showed a decline in contraction-relaxation properties after age 40 in MYPBC3 carriers, but preserved function in MYH7 patients (Figure). Kinetic of myosin cross-bridges Conclusions In HCM patients, mutations in the MYBPC3 gene and early diagnosis are associated with slowly progressing systolic impairment leading to overt dysfunction in 15% compared to 5% in MYH7-HCM. However, outcome was similar in the two subsets. These differences in lifetime myocardial performance between the two most common HCM-associated genes suggest diverse pathways of disease progression, potentially amenable to requiring different molecular approaches.

Heart failure due to chronic experimental aortic regurgitation

1994 ◽  
Vol 267 (2) ◽  
pp. H556-H562 ◽  
Author(s):  
N. M. Magid ◽  
G. Opio ◽  
D. C. Wallerson ◽  
M. S. Young ◽  
J. S. Borer
Keyword(s):  
Heart Failure ◽  
Aortic Regurgitation ◽  
Systolic Dysfunction ◽  
Disease Process ◽  
Experimental Models ◽  
Left Ventricular ◽  
Sham Operation ◽  
Systolic Performance ◽  
Baseline Values ◽  
Chronic Aortic Regurgitation

Previously reported experimental models of aortic regurgitation generally have manifested normal systolic performance and have not developed heart failure [Magid et al. Am. J. Physiol. 263 (Heart Circ. Physiol. 32): H226–H233, 1992]. To determine whether more severe chronic experimental aortic regurgitation would generate systolic malperformance, heart failure, and emulate the human disease process, 11 New Zealand White rabbits underwent surgical induction of aortic regurgitation and 5 control animals underwent sham operation. Doppler echocardiography was performed serially for up to 3 yr, and pathological studies were performed at necropsy. Left ventricular internal dimension at end diastole increased 80% (P < 0.00002) and left ventricular weight increased 250% (P < 0.0002) in aortic regurgitant rabbits (regurgitant fraction 52 +/- 13%) compared with baseline values. Six of 11 aortic regurgitant animals died with pathological evidence of congestive heart failure at 1.5 +/- 0.8 yr postoperatively; 2 of these developed severe systolic malperformance, manifest as fractional shortenings of 15 and 19% at 1.6 and 1.7 yr, respectively. Five of 11 aortic regurgitant animals survived until killed at 2.9 +/- 0.1 yr. Thus moderate-to-severe chronic aortic regurgitation in rabbits frequently results in heart failure and systolic dysfunction and may usefully model chronic aortic regurgitation and heart failure in humans.

Abstract 15863: Macrophage Foxp1 is a Regulator of Pathologic Cardiac Hypertrophy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Shuichi Yoneda ◽  
Saptarsi M Haldar ◽  
Jessica Jenkins ◽  
Yunmei Wang ◽  
Teruo Inoue ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Hypertrophy ◽  
Molecular Mechanisms ◽  
Interstitial Fibrosis ◽  
Systolic Dysfunction ◽  
Disease Process ◽  
Left Ventricular ◽  
Negative Regulator ◽  
Control Male ◽  
Pathologic Cardiac Hypertrophy

Introduction: Pathologic cardiac hypertrophy is a maladaptive response to neurohormonal and hemodynamic stress that is a hallmark of human heart failure. While inflammation has been implicated in pathologic hypertrophy, the molecular mechanisms underlying innate immune dysregulation in this disease process are incompletely defined. We have previously demonstrated that the forkhead transcription factor Foxp1 controls monocyte differentiation and suppresses inflammatory activation of macrophages. In this study, we hypothesized that monocyte/macrophage Foxp1 regulates pathologic cardiac hypertrophy. Methods: Macrophage-specific Foxp1 over-expressing (macFoxp1tg=anti-inflammatory) vs. non-tg controls, as well as macrophage-specific Foxp1 knockdown (macFoxp1ko=pro-inflammatory) vs. Cre-control male mice were subject to chronic angiotensin II (AII) infusion (1.8 mcg/kg/min via subcutaneous osmotic mini-pump) for 4 weeks. Results: AII-mediated cardiac hypertrophy (heart mass and cardiomyocyte cross-sectional area), left ventricular (LV) systolic dysfunction, LV dilation, interstitial fibrosis and macrophage (Mac-3+ cells) accumulation were significantly attenuated in macFoxp1tg mice compared to non-tg controls. In contrast, AII-mediated cardiac hypertrophy, LV systolic dysfunction and cavity dilation were significantly exacerbated in macFoxp1ko mice compared to Cre controls. There were no differences in systemic blood pressure between these groups, corroborating a load-independent role for macrophage Foxp1 in cardiac hypertrophy. Conclusion: These studies identify macrophage Foxp1 as a novel negative regulator of pathologic cardiac hypertrophy in vivo. Modulation of Foxp1 signaling may provide a novel strategy for prevention and treatment of heart failure.

scholarly journals Kematian Mendadak Akibat Kardiomiopati Hipertrofi Pada Dewasa Muda

2020 ◽  
Vol 7 (2) ◽  
pp. 470-475
Author(s):  
Raja Al Fath Widya Iswara ◽  
Arif Rahman Sadad ◽  
Intarniati Nur Rohmah ◽  
Sigid Kirana Lintang Bhima
Keyword(s):  
Heart Failure ◽  
Young Adults ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Left Ventricular Hypertrophy ◽  
Myocardial Fibrosis ◽  
Ventricular Hypertrophy ◽  
Histopathological Examination ◽  
Interventricular Septum ◽  
Left Ventricular

Latar Belakang : Kematian mendadak merupakan kasus yang paling sering terjadi dan dapat ditemukan dalam berbagai macam kondisi. Penyebab kematian mendadak terbanyak adalah sistem kardiovaskular dan salah satu kelainan yang jarang terjadi adalah kardiomiopati hipertrofi. Kardiomiopati hipertrofi merupakan kelainan jantung yang ditandai dengan hipertrofi miokardial akibat mutasi sarkomer dengan angka kejadian 1 dari 500 orang dewasa. Temuan utama pada kardiomiopati hipertrofi antara lain adanya hipertofi ventrikel dan atau septum interventrikel, kerusakan miosit dan peningkatan fibrosis miokardium. Terdapat variasi manifestasi klinis pada Kardiomiopati hipertrofi, dari asimptomatik hingga mengakibatkan kematian mendadak akibat gagal jantung. Tujuan laporan kasus ini adalah mengetahui diagnosis kematian akibat kardiomiopati hipertrofi pada dewasa muda. Kasus : Seorang laki-laki usia 18 tahun ditemukan meninggal di kamar kostannya dibawa ke kamar jenazah RSUP dr. Kariadi Semarang untuk diotopsi. Pemeriksaan luar tidak ditemukan tanda-tanda kekerasan. Pemeriksaan dalam didapatkan adanya jendalan darah dalam ventrikel, hipertrofi ventrikel kiri, penebalan pada katub jantung, pengerasan pada otot jantung dan penggantung katub serta tanda asfiksia. Pemeriksaan histopatologi menunjukkan kardiomiopati hipertrofi. Pembahasan : Patogenesis kardiomiopati hipertrofi dapat menyebabkan asfiksia yaitu terjadinya mutasi intrasarkomer yang meningkatkan peningkatan sensitivitas dan produksi Calsium yang mengakibatkan peningkatan kontraksi miokardium sehingga menyebabkan hipertrofi ventrikel kiri. Selain itu juga terjadi peningkatan sintesis kolagen yang mengakibatkan terjadinya fibrosis miokard yang menyebabkan hipertrofi ventrikel kiri. Terjadinya hipertrofi ventrikel kiri jangka panjang akan menyebabkan kondisi gagal jantung yang dapat mengakibatkan asfiksia. Simpulan : Kematian mendadak akibat kardiomiopati hipertrofi merupakan hal yang jarang. Oleh karena itu dibutuhkan otopsi yang teliti dan pemeriksaan histopatologi untuk mendiagnosis dengan pasti. Kata Kunci : Kematian mendadak, kardiomiopati hipertrofi, dewasa muda, sarkomer   Background : Sudden death is the most common case and can be found in a variety of conditions. The most common cause of death is the cardiovascular system and a rare one disorders is hypertrophic cardiomyopathy. Hypertrophy cardiomyopathy is a heart disorder characterized by myocardial hypertrophy due to sarcomere mutations with an incidence of 1 in 500 adults. The main findings in hypertrophic cardiomyopathy include the presence of ventricular hypertrophy and / or interventricular septum, myocyte damage and increased myocardial fibrosis. There are variations in clinical manifestations in hypertrophic cardiomyopathy, from asymptomatic to sudden death due to heart failure. The purpose of this case report is to know the diagnosis of sudden death due to hypertrophic cardiomyopathy in young adults Case : A 18-year-old man was found dead in his boarding room. On the external examination there were no signs of violence. On the internal examination in the presence of blood in the ventricles, left ventricular hypertrophy, thickening of the entire heart valve, hardening of the heart muscle and hanging valves and signs of asphyxia. Histopathological examination showed hypertrophic cardiomyopathy. Discussion : The pathogenesis of hypertrophic cardiomyopathy can cause asphyxia is the occurrence of intrasarcomere mutations that increase the sensitivity and production of calcium which results in increased contraction of the myocardium causing left ventricular hypertrophy. In addition there is also an increase in collagen synthesis which results in the occurrence of myocardial fibrosis which causes left ventricular hypertrophy. The occurrence of long-term left ventricular hypertrophy will cause a condition of heart failure which can lead to asphyxia. Conclusion : Sudden death due to hypertrophic cardiomyopathy is rare one. Therefore a careful autopsy is needed and histopathological examination is needed to get definitive diagnose. Keywords : Sudden death, hypertrophic cardiomyopathy, young adults, sarcomere

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