Hypertrophic cardiomyopathy: Translating cellular cross talk into therapeutics

Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease with serious adverse outcomes, including heart failure, arrhythmias, and sudden cardiac death. The discovery that mutations in sarcomere protein genes cause HCM has enabled the development of mouse models that recapitulate clinical manifestations of disease. Studies in these models have provided unexpected insights into the biophysical and biochemical properties of mutated contractile proteins and may help to improve clinical diagnosis and management of patients with HCM.

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Dealing with Sudden Cardiac Death: Who Deserves Device Implantation

ACI (Acta Cardiologia Indonesiana) ◽

10.22146/aci.47675 ◽

2019 ◽

Vol 5 (1 (P)) ◽

pp. 12

Author(s):

Dicky Armein Hanafy

Keyword(s):

Heart Failure ◽

Coronary Heart Disease ◽

Heart Disease ◽

Sudden Cardiac Death ◽

Cardiac Arrhythmias ◽

Cardiac Death ◽

Cardiac Insufficiency ◽

Resynchronization Therapy ◽

Icd Implantation ◽

Life Threatening

Sudden cardiac death is one of the leading causes of death in the western industrial nations. Most people are affected by coronary heart disease (coronary heart disease, CHD) or heart muscle (cardiomyopathy). These can lead to life-threatening cardiac arrhythmias. If the heartbeat is too slow due to impulse or conduction disturbances, cardiac pacemakers will be implanted. High-frequency and life-threatening arrhythmias of the ventricles (ventricular tachycardia, flutter or fibrillation) cannot be treated with a pacemaker. In such cases, an implantable cardioverter-defibrillator (ICD) is used, which additionally also provides all functions of a pacemaker. The implantation of a defibrillator is appropriate if a high risk of malignant arrhythmias has been established (primary prevention). If these life-threatening cardiac arrhythmias have occurred before and are not caused by a treatable (reversible) cause, ICD implantation will be used for secondary prevention. The device can stop these life-threatening cardiac arrhythmias by delivering a shock or rapid impulse delivery (antitachycardic pacing) to prevent sudden cardiac death. Another area of application for ICD therapy is advanced heart failure (heart failure), in which both main chambers and / or different wall sections of the left ventricle no longer work synchronously. This form of cardiac insufficiency can be treated by electrical stimulation (cardiac resynchronization therapy, CRT). Since the affected patients are also at increased risk for sudden cardiac death, combination devices are usually implanted, which combine heart failure treatment by resynchronization therapy and the prevention of sudden cardiac death by life-threatening arrhythmia of the heart chambers (CRT-D device). An ICD is implanted subcutaneously or under the pectoral muscle in the area of the left collarbone. Like pacemaker implantation, ICD implantation is a routine, low-complication procedure today.

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Secondary prevention of sudden cardiac death in ischaemic cardiomyopathy

ESC CardioMed ◽

10.1093/med/9780198784906.003.0553_update_001 ◽

2018 ◽

pp. 2337-2341

Author(s):

Jens Cosedis Nielsen ◽

Jens Kristensen

Keyword(s):

Heart Failure ◽

Cardiac Arrest ◽

Heart Disease ◽

High Risk ◽

Sudden Cardiac Death ◽

Secondary Prevention ◽

Cardiac Death ◽

Cardioverter Defibrillator ◽

Ischaemic Cardiomyopathy ◽

Life Threatening

The most common reason for sudden cardiac death is ischaemic heart disease. Patients who survive cardiac arrest are at particularly high risk of recurrent ventricular arrhythmia and sudden cardiac death, and are candidates for secondary prevention defined as ‘therapies to reduce the risk of sudden cardiac death in patients who have already experienced an aborted cardiac arrest or life-threatening arrhythmias’. The mainstay therapy for secondary prevention of sudden cardiac death is implantation of an implantable cardioverter defibrillator. Furthermore, revascularization and optimal medical therapy for heart failure and concurrent cardiovascular diseases should be ensured.

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Exercise hemodynamics in hypertrophic cardiomyopathy identify risk of incident heart failure but not ventricular arrhythmias or sudden cardiac death

International Journal of Cardiology ◽

10.1016/j.ijcard.2018.07.110 ◽

2019 ◽

Vol 274 ◽

pp. 226-231 ◽

Cited By ~ 2

Author(s):

Eric D. Smith ◽

June Tome ◽

Ryan Mcgrath ◽

Suwen Kumar ◽

Maryann Concannon ◽

...

Keyword(s):

Heart Failure ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Ventricular Arrhythmias ◽

Exercise Hemodynamics ◽

Incident Heart Failure

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Coexistence of sudden cardiac death and end-stage heart failure in familial hypertrophic cardiomyopathy

Journal of the American College of Cardiology ◽

10.1016/0735-1097(93)90054-5 ◽

1993 ◽

Vol 22 (2) ◽

pp. 489-497 ◽

Cited By ~ 59

Author(s):

Gabriela M. Hecht ◽

Heinrich G. Klues ◽

William C. Roberts ◽

Barry J. Maron

Keyword(s):

Heart Failure ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Familial Hypertrophic Cardiomyopathy ◽

End Stage Heart Failure ◽

End Stage

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Sudden Cardiac Death in Three Generations of the Same Family: A case report

Cardiovascular Journal ◽

10.3329/cardio.v8i1.24779 ◽

2015 ◽

Vol 8 (1) ◽

pp. 73-77

Author(s):

MM Zahurul Alam Khan ◽

Abdul Wadud Chowdhury ◽

Tunaggina Afrin Khan ◽

Md Gaffar Amin ◽

Md Nur Alam ◽

...

Keyword(s):

Heart Failure ◽

Young Adults ◽

Case Report ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Premature Death ◽

Young Person ◽

Current Case ◽

History Of

Every one of us has heard about tragic and sudden death of a healthy young person and which is often stated as inexplicable. The current case report focuses on a 20 year old young man with hypertrophic cardiomyopathy facing premature death with history of similar sudden premature death of his grandmother, father and brother. Hypertrophic cardiomyopathy is the commonest cause of sudden cardiac death in young adults and is also an important substrate for heart failure disability at any age.Cardiovasc. j. 2015; 8(1): 73-77

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Cardiomyopathy Associated With Tertiary Adrenal Insufficiency Manifesting as Refractory Heart Failure, Shock, and Sudden Cardiac Death: A Case Report

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.720154 ◽

2021 ◽

Vol 8 ◽

Author(s):

Xuefeng Wang ◽

Yong Luo ◽

Jian Feng

Keyword(s):

Heart Failure ◽

Sudden Cardiac Death ◽

Dilated Cardiomyopathy ◽

Adrenal Insufficiency ◽

Cardiac Death ◽

Clinical Manifestations ◽

Underlying Disease ◽

Pituitary Hormones ◽

Idiopathic Dilated Cardiomyopathy ◽

Cardiac Resynchronization

Dilated cardiomyopathy is an etiologically heterogeneous disorder. Early diagnosis and prompt treatment of the underlying disease are of great significance. Primary and secondary adrenal insufficiency are considered quite rare causes of dilated cardiomyopathy. However, to the best of our knowledge, no case of cardiomyopathy associated with tertiary adrenal insufficiency has been reported. Herein, we described a 68-year-old woman with a 15-year history of seasonal dermatitis presented with frequent heart failure and shock. At first, she was diagnosed with idiopathic dilated cardiomyopathy, but standard heart failure and antishock treatment failed. Given her long-term use of dexamethasone for treating seasonal dermatitis, and clinical manifestations consistent with adrenal insufficiency, we tested her basal plasma cortisol, simultaneous corticotropin, and other pituitary hormones, confirming that she had tertiary adrenal insufficiency. Additionally, abdominal enhanced computed tomography revealed atrophic bilateral adrenal glands, indicating long-standing and severe adrenal insufficiency. Then hydrocortisone replacement therapy was initiated, and she recovered rapidly. During the next 2 years of follow-up, she never experienced any episodes of heart failure and shock. Unfortunately, she refused the implantation of defibrillator with cardiac resynchronization therapy (CRT-D) and died of sudden cardiac death 2 years later. Although we could not exclude the coincidence of idiopathic dilated cardiomyopathy with tertiary adrenal insufficiency with 100% certainty, her unique clinical course strongly indicated that her cardiomyopathy resulted from tertiary adrenal insufficiency. This case demonstrates that patients on corticosteroids are at risk for tertiary adrenal insufficiency, which may result in refractory cardiomyopathy and even sudden cardiac death.

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High-Goal ‘Lytes: Repletion Gone Awry?

Journal of Hospital Medicine ◽

10.12788/jhm.3274 ◽

2019 ◽

Vol 14 (12) ◽

pp. 785-786

Author(s):

Ramzi Dudum ◽

Steven J Lahti ◽

Michael J Blaha

Keyword(s):

Myocardial Infarction ◽

Heart Failure ◽

Heart Disease ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Acute Decompensated Heart Failure ◽

Decompensated Heart Failure ◽

Ventricular Ectopy ◽

Serum Electrolytes ◽

Per Se

Electrolyte imbalances, per se, predispose to ventricular ectopy and, in extreme cases, sudden cardiac death.1 As these outcomes are more common in the presence of intrinsic heart disease, serum electrolytes—particularly potassium and magnesium—are routinely monitored and made replete in patients with myocardial infarction (MI) or acute decompensated heart failure (ADHF).

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Hypertrophic Cardiomyopathy: A Review

Clinical Medicine Insights Cardiology ◽

10.4137/cmc.s15717 ◽

2014 ◽

Vol 8s1 ◽

pp. CMC.S15717 ◽

Cited By ~ 10

Author(s):

Brian A. Houston ◽

Gerin R. Stevens

Keyword(s):

Heart Failure ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Ventricular Arrhythmias ◽

Clinical Syndrome ◽

Monogenic Disease ◽

Sarcomeric Genes ◽

Ethnic Origins

Hypertrophic cardiomyopathy (HCM) is a global disease with cases reported in all continents, affecting people of both genders and of various racial and ethnic origins. Widely accepted as a monogenic disease caused by a mutation in 1 of 13 or more sarcomeric genes, HCM can present catastrophically with sudden cardiac death (SCD) or ventricular arrhythmias or insidiously with symptoms of heart failure. Given the velocity of progress in both the fields of heart failure and HCM, we present a review of the approach to patients with HCM, with particular attention to those with HCM and the clinical syndrome of heart failure.

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