New Strategy Saving Those With Genetic Heart Disease

BOSTON (CBS) – We’ve all heard the tragic tales of teens and young adults collapsing while playing sports from a rare heart condition. Now, a father-son team in Boston has developed a strategy to save those patients most at risk. “It was devastating.”  Kevin Whelan recalled the moment he learned his oldest brother, Kyle, collapsed and died. He was only 26 years old. Kyle had hypertrophic cardiomyopathy, or HCM, a genetic disease of the heart and the most common cause of sudden death in otherwise healthy young people. But Kyle wasn’t the only one in the family with it.  His father and all three sons had HCM, including Kevin. “After Kyle died, I was wondering when was my event,” remembered Dr. Martin Maron checks Kyle Whelan. (WBZ-TV) Searching for answers, the Whelan family found doctors Barry and Martin Maron, a father-son team of cardiologists at Tufts Medical Center in Boston who had developed the “sudden cardiac death prevention strategy.” “The best way to attack this problem of sudden death is to pick out the patients who are most likely to have sudden death and use the defibrillator selectively in those patients,” explained Dr. Barry Maron. Using a patient’s history, physical exam and special imaging, they can now determine with near certainty who needs a defibrillator and who does not. “We really have been able to reduce sudden death in this disease to almost zero,” said Dr. Ma...
Source: WBZ-TV - Breaking News, Weather and Sports for Boston, Worcester and New Hampshire - Category: Consumer Health News Authors: Tags: Boston News Health Healthwatch Syndicated Local Dr. Mallika Marshall Hypertrophic Cardiomyopathy Source Type: news

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This article discusses diagnostic criteria, treatments, and nursing interventions, including patient teaching, for adults with HCM.
Source: Nursing - Category: Nursing Tags: Feature: CE Connection Source Type: research
ConclusionsIn patients with HCM undergoing AF ablation, QTc prolongation, specifically>448 ms, and presence of fQRS are independent risk factors for arrhythmia recurrence at follow ‐up. The combination of these two parameters has greater predictive value and would help to identify patients who are at the highest risk of procedural failure.This article is protected by copyright. All rights reserved.
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: ORIGINAL ‐ CLINICAL Source Type: research
ConclusionCatheter ablation of VF through „De‐Networking” of the Purkinje system in patients without overt arrhythmia substrate or trigger appears safe and effective and will require further study in a larger patient cohort.This article is protected by copyright. All rights reserved
Source: Pacing and Clinical Electrophysiology : PACE - Category: Cardiology Authors: Tags: ELECTROPHYSIOLOGY Source Type: research
ConclusionData from the present registry display a surprisingly high mortality in patients with electrical heart disease equivalent to the control group. A high proportion of patients who received an ICD for secondary prevention may be regarded as a major determinant for these results, while severe comorbidities such as diabetes, hypertension, and renal failure are major determinants for mortality in the control cohort.
Source: Clinical Research in Cardiology - Category: Cardiology Source Type: research
New research shows the role that a diseased extracellular matrix plays in the progression of the genetic condition hypertrophic cardiomyopathy.
Source: Yale Science and Health News - Category: Universities & Medical Training Source Type: news
CONCLUSIONS: Our results show the feasibility, efficacy, and safety of RNAi therapeutics directed at human restrictive cardiomyopathy. This is a promising step towards targeted therapy for a prevalent human disease. PMID: 31315475 [PubMed - as supplied by publisher]
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
Background: The prevalence of atrial fibrillation (AF) has not been well described in young patients with inherited heart diseases such as the inherited cardiomyopathies: hypertrophic cardiomyopathy (HCM), familial dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), left ventricular non-compaction (LVNC); or inherited arrhythmia syndromes including long QT syndrome (LQTS), Brugada syndrome (BrS) or catecholaminergic polymorphic ventricular tachycardia (CPVT).
Source: Heart, Lung and Circulation - Category: Cardiology Authors: Tags: 455 Source Type: research
M, Rosengren A Abstract BACKGROUND: Modifiable lifestyle factors in relation to risk for cardiomyopathy, a common and increasing cause of heart failure in the young, have not been widely studied. We sought to investigate a potential link between obesity, a recognized predictor of early heart failure, in adolescence and being diagnosed with cardiomyopathy in adulthood. METHODS: This was a nationwide register-based prospective cohort study of 1 668 893 adolescent men (mean age, 18.3 years; SD, 0.7 years) who enlisted for compulsory military service from 1969 to 2005. At baseline, body mass index (BMI), blood pr...
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
BOSTON (CBS) – We’ve all heard the tragic tales of teens and young adults collapsing while playing sports from a rare heart condition. Now, a father-son team in Boston has developed a strategy to save those patients most at risk. “It was devastating.”  Kevin Whelan recalled the moment he learned his oldest brother, Kyle, collapsed and died. He was only 26 years old. Kyle had hypertrophic cardiomyopathy, or HCM, a genetic disease of the heart and the most common cause of sudden death in otherwise healthy young people. But Kyle wasn’t the only one in the family with it.  His father and...
Source: WBZ-TV - Breaking News, Weather and Sports for Boston, Worcester and New Hampshire - Category: Consumer Health News Authors: Tags: Boston News Health Healthwatch Syndicated Local Dr. Mallika Marshall Hypertrophic Cardiomyopathy Source Type: news
Conclusions: 11C-PIB PET/MR may be valuable in the noninvasive diagnosis of CA. Further study with a larger cohort of patients would be needed to confirm it.
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Cardiovascular YIA Symposium Source Type: research
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