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Results of surgical septal myectomy for obstructive hypertrophic cardiomyopathy: the Tufts experience.

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TLDR
This experience demonstrates that, with the appropriate support, new HCM surgical programs can provide patients successful relief of outflow obstruction, extended longevity and restored of quality of life.
Abstract
Background: For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM). Over this time in the United States, the majority of myectomy operations have been performed in a small number of select referral centers. Methods: We have taken the opportunity to report results from the relatively new Tufts HCM Center and surgical program, incorporated 13 years ago, during which 507 myectomies (52±14 years of age; 56% male) were performed by one cardiothoracic surgeon, Dr. Hassan Rastegar. Results: Resting left ventricular (LV) outflow gradients were reduced from 56±42 mmHg preoperatively to 1.2±6.8 mmHg on most recent echocardiogram 2.0±2.5 years after surgery, and 94% of patients showed clinical improvement to NYHA functional class I or II. The first 200 myectomies were performed without mortality or major complications. Among all patients, 30-day mortality rate was 0.8%. Over follow-up of 3.2±2.8 years, 11 patients died (four due to HCM causes) with long-term survival after myectomy of 94% at 5 years (95% CI: 89–96%) and 91% at 10 years (95% CI: 84–95%), which did not differ from the age- and gender-matched general U.S. population (log-rank P=0.9). Conclusions: This experience demonstrates that, with the appropriate support, new HCM surgical programs can provide patients successful relief of outflow obstruction, extended longevity and restored of quality of life.

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Journal ArticleDOI

Clinical Course and Management of Hypertrophic Cardiomyopathy

TL;DR: The most common cause of sudden death frompertrophic Cardiomyopathy is sudden cardiac arrest, and the most common type of heart attack is sudden death by atypical causes.
Journal ArticleDOI

Management of hypertrophic cardiomyopathy.

TL;DR: Hypertrophic cardiomyopathy (HCM) patients with disease-related complications benefit from: matured risk stratification in which major markers reliably select patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy (with percutaneous alcohol ablation a selective alternative) that reverses progressive heart failure caused by outflow obstruction; anticoagulation pro-phylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients as discussed by the authors .
Journal ArticleDOI

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines

TL;DR: This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy.
Journal ArticleDOI

Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

Petar M. Seferovic, +49 more
TL;DR: E epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies are focused on.
References
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Journal ArticleDOI

Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction

TL;DR: Identification of LV outflow obstruction with exercise echocardiography may broaden management options in HCM by identifying symptomatic patients not otherwise regarded as potential candidates for septal reduction therapy.
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