scholarly journals Hypertrophic Cardiomyopathy Mimicking Acute Anterior Myocardial Infarction Associated with Sudden Cardiac Death

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Y. Daralammouri ◽  
M. El Garhy ◽  
K. Same ◽  
B. Lauer

Hypertrophic cardiomyopathy is the most common genetic disease of the heart. We report a rare case of hypertrophic obstructive cardiomyopathy mimicking an acute anterior myocardial infarction associated with sudden cardiac death. The patient presented with acute ST elevation myocardial infarction and significant elevation of cardiac enzymes. Cardiac catheterization showed some atherosclerotic coronary artery disease, without significant stenosis. Echocardiography showed left ventricular hypertrophy with a left ventricular outflow tract obstruction; the pressure gradient at rest was 20 mmHg and became severe with the Valsalva maneuver (100 mmHg). There was no family history of sudden cardiac death. Six days later, the patient suffered a syncope on his way to magnetic resonance imaging. He was successfully resuscitated by ventricular fibrillation.

2009 ◽  
Vol 104 (5) ◽  
pp. 695-699 ◽  
Author(s):  
Georgios K. Efthimiadis ◽  
Despina G. Parcharidou ◽  
Georgios Giannakoulas ◽  
Efstathios D. Pagourelias ◽  
Panagiotis Charalampidis ◽  
...  

2015 ◽  
Vol 139 (3) ◽  
pp. 413-416 ◽  
Author(s):  
Linda Kocovski ◽  
John Fernandes

Hypertrophic cardiomyopathy is one of the most common causes of sudden cardiac death among young adults and adolescents. Unfortunately, the first manifestation of the condition may be sudden death during exertion, such as sporting activities. Other clinical symptoms include exertional dyspnea, angina, and syncope. Postmortem examination often reveals asymmetrical septal thickening and mural plaque formation in the left ventricular outflow tract. Histologic analysis shows cardiac myocyte hypertrophy, myofiber disarray, and interstitial and replacement fibrosis. Molecular analysis for known genetic abnormalities is essential to genetic counseling of living relatives of decedents to assess and reduce the risk of sudden cardiac death from hypertrophic cardiomyopathy.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Norrish ◽  
T Ding ◽  
E Field ◽  
C O'Mahony ◽  
P.M Elliott ◽  
...  

Abstract Background The most common mode of death in childhood hypertrophic cardiomyopathy (HCM) is sudden cardiac death (SCD). Left ventricular outflow tract obstruction (LVOTO) is an established risk factor for SCD in adults with the disease. In contrast, the prognostic implications of LVOTO in childhood disease is unclear, with recent studies suggesting that it may have an inverse relationship with the risk of SCD. Purpose The aim of this study was to explore the role of LVOTO and the risk of SCD in childhood HCM. Methods A multi-centre, retrospective, longitudinal cohort of 871 children (diagnosed with HCM <16 years of age) was used to explore the relationship between SCD and LVOTO (LVOT gradient ≥30mmHg). Results 189 patients (23%) had LVOTO, which was mild (30–50mmHg), moderate (50–100mmHg) or severe (>100mmHg) in 58 (6.7%), 98 (11.3%) and 33 (3.8%), respectively. The risk of SCD showed an inverse relation to LVOT gradient severity compared to those with no obstruction: mild HR 1.75 (95% CI 0.89–3.44), moderate HR 1.04 (95% 0.55–1.98), and severe HR 0.7 (0.36–1.35) [figure].On univariable analysis [table] LVOTO was associated with heart failure symptoms (NYHA>1) [p <0.001], maximal wall thickness (MWT) [p <0.001], left atrial (LA) diameter [p <0.001], and future myectomy occurring during follow up [p <0.001]. The inverse relationship observed was not altered by the presence or absence of other traditional risk factors. Conclusions LVOTO appears to have a complex relationship with the risk of SCD in childhood HCM, with multiple contributing factors. The pathophysiological mechanisms behind this observation need further exploration, which may be limited by low patient numbers Cummulative incidence of SCD by LVOTO Funding Acknowledgement Type of funding source: Other. Main funding source(s): British Heart Foundation


Author(s):  
Albree Tower-Rader ◽  
Christopher M. Kramer ◽  
Stefan Neubauer ◽  
Sherif F. Nagueh ◽  
Milind Y. Desai

In hypertrophic cardiomyopathy, multimodality imaging is crucial to confirm diagnosis, assess for presence and mechanism of left ventricular outflow tract obstruction, and risk stratification for sudden cardiac death. This review will focus on the application of imaging to assess established and emerging factors to be considered in sudden cardiac death risk stratification.


Author(s):  
Constantinos O’Mahony

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.


2011 ◽  
Vol 9 (2) ◽  
pp. 108 ◽  
Author(s):  
Constantinos O’Mahony ◽  
Saidi A Mohiddin ◽  
Charles Knight ◽  
◽  
◽  
...  

Hypertrophic cardiomyopathy (HCM) is an inherited myocardial disorder characterised by left ventricular hypertrophy. A subgroup of patients develops limiting symptoms in association with left ventricular outflow tract obstruction (LVOTO). Current international guidelines recommend that symptomatic patients are initially treated by alleviating exacerbating factors and negatively inotropic medication. Drug-refractory symptoms require a comprehensive evaluation of the mechanism of LVOTO and review by a multidisciplinary team to consider the relative merits of myectomy, alcohol septal ablation (ASA) and pacing. This article provides a brief overview of HCM and the pathophysiology of LVOTO, and reviews the use of ASA in patients with drug-refractory symptoms secondary to LVOTO.


Author(s):  
B.M. Todurov ◽  
◽  
G.I. Kovtun ◽  
A.V. Khokhlov ◽  
O.V. Pantazi ◽  
...  

Hypertrophic obstructive cardiomyopathy іs a relatively common condition and one of the most common causes of sudden cardiac death in young age. One of the options for the surgical treatment of this pathology is septal myoectomy, which has been the gold standard for decades. However, despite this, surgical treatment is intended for young patients with a low risk of postoperative complications, while patients with concomitant diseases and a higher surgical risk require alternative treatment. Today, alcohol septal ablation is considered an effective, minimally invasive method for treating hypertrophic obstructive cardiomyopathy in patients with a left ventricular outflow tract gradient ≥ 50 mm Hg. The article presents the experience of using alcohol septal ablation in 57 patients with obstruction of the left ventricular outflow tract. Key words: alcoholic septal ablation, hypertrophic cardiomyopathy, left ventricular outflow tract obstruction.


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