Risk Stratification for Sudden Death in Hypertrophic Cardiomyopathy: Extreme Left Ventricular Hyptertrophy as a New Indicator of Risk

Risk stratification and identification of those patients with hypertrophic cardiomyopathy (HCM) at the highest risk for sudden death is a major issue for patient management, given the effective option of the implantable defibrillator in this disease. For example, the prognostic significance of syncope has not been investigated systematically in large HCM cohorts, and therefore treatment strategies related to this symptom have been based largely on intuition and experience. We assessed the relationship between syncope and sudden death in 1511 consecutive HCM patients. Unexplained (n=153) or neurally-mediated (n=52) syncope occurred in 205 (14%) of patients. Over a 5.6±5.2 year follow-up, 74 patients died suddenly. Relative risk of sudden death was 1.78 (95% CI 0.88 –3.51; p=0.08) in patients with unexplained syncope, and 0.91 (95% CI 0.00 –3.83; p=1.0) in those with neurally-mediated syncope, compared to patients without syncope. However, the time interval between unexplained syncope and initial patient evaluation proved to have a strong impact on sudden death risk. Patients with recent syncope (≤6 months from initial evaluation) showed a 5-fold increase in risk compared to patients without syncope (adjusted HR=4.89; CI 2.19 –10.94; p=0.006), a relationship maintained throughout all age groups (<18, 18 –39 and ≥40 years). Conversely, adult patients (18 –39 and ≥40 years) with remote syncope (>6 months from evaluation) showed no increase in sudden death rate (4.8; 95% CI 0.1–26.7 and 3.2; 95% CI 0.1–17.8 per 1000 person-years, respectively), compared to patients of similar age without syncope (11.8; 95% CI 7.6 –17.6 and 5.7%; 95% CI 3.8 – 8.3 per 1000 person-years). Left ventricular outflow obstruction was not associated with sudden death risk (p=0.29). In this large HCM cohort, unexplained syncope proved to be a risk factor for sudden death. In addition, the temporal proximity of syncopal events to patient evaluation is of clinical relevance. Recent unexplained syncope is associated with an increased risk for sudden death in all age groups, while remote syncopal events do not convey increased risk to adult patients.

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Risk stratification and prevention of sudden death in hypertrophic cardiomyopathy

Current Treatment Options in Cardiovascular Medicine ◽

10.1007/s11936-007-0037-y ◽

2007 ◽

Vol 9 (6) ◽

pp. 431-435 ◽

Cited By ~ 5

Author(s):

Camillo Autore ◽

Giovanni Quarta ◽

Paolo Spirito

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Death ◽

Risk Stratification

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Magnitude of Left Ventricular Hypertrophy and Risk of Sudden Death in Hypertrophic Cardiomyopathy

New England Journal of Medicine ◽

10.1056/nejm200006153422403 ◽

2000 ◽

Vol 342 (24) ◽

pp. 1778-1785 ◽

Cited By ~ 682

Author(s):

Paolo Spirito ◽

Pietro Bellone ◽

Kevin M. Harris ◽

Paola Bernabò ◽

Paolo Bruzzi ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Death ◽

Left Ventricular Hypertrophy ◽

Ventricular Hypertrophy ◽

Left Ventricular

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

Circulation ◽

10.1161/circ.118.suppl_18.s_955 ◽

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.

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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 ◽

10.1161/circ.130.suppl_2.13943 ◽

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.

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MIOCARDIOPATIA NÃO COMPACTADA - UMA REVISÃO DA LITERATURA

Revista de Patologia do Tocantins ◽

10.20873/uft.2446-6492.2018v5n2p74 ◽

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.

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Abstract 17215: Differences in Anatomy, Physiology and Outcomes Between Blacks and Whites With Hypertrophic Cardiomyopathy

Circulation ◽

10.1161/circ.142.suppl_3.17215 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Milla E Arabadjian ◽

Gary Yu ◽

Mark Sherrid ◽

Victoria V Dickson

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Death ◽

Left Ventricular ◽

Similar Proportion ◽

Black African ◽

Lv Mass ◽

The Us ◽

Blacks And Whites ◽

Black Populations ◽

Prospective Longitudinal

Introduction: Hypertrophic cardiomyopathy (HCM) in Black populations remains underexplored even though this inherited disorder can affect more than 220,000 Blacks in the US alone. Existing data suggest that Blacks with HCM are less likely to have obstructive physiology, but uncommon phenotypes, such as mid-ventricular obstruction have not been reported. Mortality outcomes have been reported as similar between groups. Objective: To evaluate structural HCM features and outcomes in Blacks and Whites with HCM Methods: The study design was a retrospective analysis of a prospective longitudinal observational study of a referred patient population with confirmed HCM and self-identifying as Black/African American and White/Caucasian. Results: We studied 434 individuals, 57 (13.1%) were Black. Males were 52.6% of the Black cohort and 59% of the White cohort. Blacks were younger, mean (SD) 54.6 (13.4) vs 62.6 (14.8) years, p<0.001. Ejection fraction and left ventricular hypertrophy (LVH) magnitude were similar between groups but LVH distribution differed with Blacks having more sub-basal LVH, which includes apical, apical-mid and mid LVH, 22 (38.6%) vs 56 (14.9%), p<0.001, and diffuse LVH, 6 (10.5%) vs 15 (4.0%), p=0.017, than Whites. Blacks also had more mid-ventricular obstruction, 7 (12.3%) vs 21 (5.5%), p=0.025. Of those who had a cardiac MRI, Blacks had more significant fibrosis (≥15% of LV mass), 10 (30.3%) vs 20 (9.5%), p<0.001. Mortality was low overall and both groups had similar proportion of ICD insertion for primary prevention of sudden death. However, Blacks had more appropriate ICD interventions, 5 (38.5%) vs 5 (6.8%), p=0.001. Conclusions: Blacks with HCM have more sub-basal LVH and mid-ventricular obstruction than Whites with HCM, phenotypes associated with worse disease progression. They are more likely to have significant myocardial fibrosis which has a pro-arrhythmic effect. They also had more aborted sudden death events. Precise evaluation of HCM structural and physiologic characteristics can improve targeted treatment and outcomes in this population.

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