New Technology Saves 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. Barry 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. Mar...
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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Abstract BACKGROUND: Chronic valvular heart disease leads to systolic dysfunction and left atrial enlargement that ultimately results in heart failure. PURPOSE: To investigate prognostic importance of Echocardiography and plasma natriuretic peptide levels that increase as a compensatory response and can be used as predictive markers for cardiac hypertrophy. MATERIAL AND METHODS: The patients were divided into three groups: 51 with left ventricle hypertrophy due to aortic valve disease; 126 with left atrial enlargement due to mitral valve dysfunction; and 76 with both conditions. Atrial natriuretic peptid...
Source: Acta Radiologica - Category: Radiology Authors: Tags: Acta Radiol Source Type: research
In this study we aimed to evaluate management strategies in our pediatric cardiac transplantation candidates with PH and high PVR prior to OHT. METHOD: Twenty-six cardiac transplantation candidates (age: 10.2 ± 4.6, 1-17 years) underwent cardiac catheterization for the determination of PVR and pulmonary arterial pressure. They were admitted to the hospital and received 1-3 days of intravenous (IV) vasodilator therapy; 0.5-3 μg/kg/min nitroglyserin and/or 0.5-3 μg/kg/min nitroprusside, 5-15 μg/kg/min dobutamin and/or dopamin to keep systolic blood pressure above 80 mmHg. RESULTS: Thirteen patients ...
Source: The Turkish Journal of Pediatrics - Category: Pediatrics Authors: Tags: Turk J Pediatr Source Type: research
AbstractSudden cardiac death (SCD) is a pivotal health problem worldwide. The identification of subjects at increased risk of SCD is crucial for the accurate selection of candidates for implantable cardioverter defibrillator (ICD) therapy. Current strategies for arrhythmic stratification largely rely on left ventricular (LV) ejection fraction (EF), mostly measured by echocardiography, and New York Heart Association functional status for heart failure with reduced EF. For specific diseases, such as hypertrophic and arrhythmogenic cardiomyopathy, some risk scores have been proposed; however, these scores take into account so...
Source: Heart Failure Reviews - Category: Cardiology Source Type: research
This study assesses clinical presentations and outcomes of patients with previous coronary revascularization undergoing septal myectomy.
Source: International Journal of Cardiology - Category: Cardiology Authors: Source Type: research
Conclusions: The systematic pictorial review of 18F-FDG PET/CT image features of benign non-neoplastic heart disease is helpful to recognize and differentiate cardiac metastasis in oncological patients.
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Cardiovascular Sciences (Poster Session) Source Type: research
AbstractBackgroundPatients with existing or anticipated indications for cardiac resynchronisation therapy (CRT), bradycardia or anti ‐tachy pacing should not be offered subcutaneous defibrillators (SQIDs) but it remains unclear how clinicians should predict future need for these therapies.MethodsWe applied three SQID selection policies to data collected retrospectively from TV ‐ICD implants:•Approach A: SQID used in inherited channelopathies and idiopathic ventricular fibrillation only;•Approach B: as above, plus all hypertrophic cardiomyopathy and grown‐up congenital heart disease patients;•Approach C...
Source: Pacing and Clinical Electrophysiology : PACE - Category: Cardiology Authors: Tags: DEVICES Source Type: research
Aims The diagnostic performance of the new Peguero–Lo Presti ECG criteria for left ventricular hypertrophy (LVH) has not been validated by cardiac magnetic resonance (CMR). The aim of this study was to evaluate and compare the diagnostic performance of Peguero–Lo Presti, Cornell and Sokolow--Lyon voltage criteria for LVH as defined by CMR in an all-comers European population. Methods A total of 240 consecutive patients referred for CMR who had a concomitant electrocardiogram for review were evaluated. LVH group patients were defined according to the reference values for sex and age of left ventricular mass...
Source: Journal of Cardiovascular Medicine - Category: Cardiology Tags: Research articles: Myocardial hypertrophy Source Type: research
Conclusions: Our findings provide compelling evidence that ferritin plays a major role in protecting against cardiac ferroptosis and subsequent heart failure, thereby providing a possible new therapeutic target for patients at risk of developing cardiomyopathy. PMID: 32349646 [PubMed - as supplied by publisher]
Source: Circulation Research - Category: Cardiology Authors: Tags: Circ Res Source Type: research
ConclusionsAdolescent RHR is associated with future risk of HF, regardless of associated aetiological condition. The association was strongest for HF associated with CM, driven by the association with dilated CM. These findings indicate a causal pathway between elevated RHR and myocardial dysfunction that warrants further investigation.
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Original Research Article Source Type: research
(Mayo Clinic) People with certain heart diseases may be more susceptible to worse outcomes with COVID-19, but the reason why has remained unknown. New research from Mayo Clinic indicates that in patients with one specific type of heart disease ? obstructive hypertrophic cardiomyopathy (HCM) ? the heart increases production of the ACE2 RNA transcript and the translated ACE2 protein.
Source: EurekAlert! - Infectious and Emerging Diseases - Category: Infectious Diseases Source Type: news
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