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Article information. Full Text. Asymmetrical hypertrophy of the heart in young adults.. Br Heart J, 20 , pp. The logic of discovery: a case study of hypertrophic cardiomyopathy.. Acta Biotheoretica, 26 , pp.
Int J Cardiol, 90 , pp. Hypertrophic cardiomyopathy: lessons from history.. Heart, 94 , pp. Gazette de Medicine Paris , 24 , pp. Beitrag zur Kenntniss der idiopathischen Hertzmuskelerkrankungen..
Arch Klin Med, 48 , pp. Paul Wood Lecture. The master's legacy: the first Paul Wood Lecture.. Heart, 80 , pp. Wither the cardiac physical examination? J Am Coll Cardiol, 48 , pp. Aortic subvalvar stenosis; a report of 5 cases diagnosed during life.. Guys Hosp Rep, , pp. Functional obstruction of the left ventricle; acquired aortic subvalvar stenosis.. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort..
JAMA, , pp. Historical trends in reported survival rates in patients with hypertrophic cardiomyopathy.. Heart, 92 , pp. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy..
Circulation, , pp. A family with obstructive cardiomyopathy asymmetrical hypertrophy.. Br Heart J, 22 , pp. Obstructive cardiomyopathy simulating aortic stenosis.. Pseudoaortic stenosis produced by ventricular hypertrophy.. Am J Med, 25 , pp. Idiopathic ventricular septal hypertrophy causing muscular subaortic stenosis..
Circulation, 26 , pp. Hereditary cardiovascular dysplasia. A form of familial cardiomyopathy.. Am J Med, 31 , pp. A description of the disease based upon an analysis of 64 patients. Operative treatment in idiopathic hypertrophic subaortic stenosis. Techniques, and the results of preoperative and postoperative clinical and hemodynamic assessments..
Circulation, 37 , pp. Muscular subvalvular aortic stenosis; abnormal anterior mitral leaflet possibly the primary factor..
Acta Radiol Diagn Stockh , 2 , pp. Role of echocardiography in diagnostic and hemodynamic assessment of hypertrophic subaortic stenosis.. Circulation, 44 , pp. Mitral regurgitation in idiopathic hypertrophic subaortic stenosis..
N Engl J Med, , pp. Ultrasound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy.. Circulation, 40 , pp. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy.. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy..
Am J Cardiol, 48 , pp. Distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy: a two-dimensional echocardiographic study.. J Am Coll Cardiol, 2 , pp. Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy. Clinicopathologic features of hypertrophic cardiomyopathy managed by cardiac transplantation.
Maron BJ. Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration. Sudden deaths in young competitive athletes: analysis of deaths in the United States, Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from the American Heart Association and American College of Cardiology.
Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.
Termination of malignant ventricular arrhythmias with an implanted automatic defibrillator in human beings. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.
Circadian rhythm of appropriate implantable cardioverter defibrillator discharges in patients with hypertrophic cardiomyopathy. Pacing Clin Electrophysiol. Determinants of implantable defibrillator discharges in high-risk patients with hypertrophic cardiomyopathy. Long-term follow-up of implantable cardioverter defibrillator therapy for hypertrophic cardiomyopathy.
Implantable cardioverter-defibrillators in hypertrophic cardiomyopathy: patient outcomes, rate of appropriate and inappropriate interventions, and complications. Am Heart J. Outcome and complications after implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: systematic review and meta-analysis. Circ Heart Fail. Implantable cardioverter-defibrillator in patients with hypertrophic cardiomyopathy: efficacy and complications of the therapy in long-term follow-up.
J Cardiovasc Electrophysiol. Incidence of adequate ICD interventions in patients with hypertrophic cardiomyopathy supposed to be at high risk for sudden cardiac death. Acta Cardiol. Implantable cardioverter-defibrillators in patients with hypertrophic cardiomyopathy—dilemmas and difficulties. Kardiol Pol.
The implantable cardioverter-defibrillator and hypertrophic cardiomyopathy: experience at three centers [in Spanish]. Rev Esp Cardiol. Long-term follow-up of hypertrophic cardiomyopathy patients with implantable cardioverter-defibrillators [in Spanish]. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. Hypertrophic cardiomyopathy in children, adolescents, and young adults associated with low cardiovascular mortality with contemporary management strategies.
Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy.
Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors. Evolving treatment for patients with hypertrophic obstructive cardiomyopathy. Transaortic chordal cutting: mitral valve repair for obstructive hypertrophic cardiomyopathy with mild septal hypertrophy. Low operative mortality achieved with surgical septal myectomy: role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction.
Symptom assessment and exercise impairment in surgical decision making in hypertrophic obstructive cardiomyopathy: relationship to outcomes.
J Thorac Cardiovasc Surg. Clinical and echocardiographic determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy. Short and medium term outcomes of surgery for patients with hypertrophic obstructive cardiomyopathy. Ann Thorac Surg. Surgical septal myectomy versus alcohol septal ablation: assessing the status of the controversy in Long-term outcomes after medical and invasive treatment in patients with hypertrophic cardiomyopathy.
A systematic review and meta-analysis of long-term outcomes after septal reduction therapy in patients with hypertrophic cardiomyopathy. Survival after alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Implantable cardioverter-defibrillator therapy for primary prevention of sudden death after alcohol septal ablation of hypertrophic cardiomyopathy.
Alcohol septal ablation for the treatment of hypertrophic obstructive cardiomyopathy: a multicenter North American registry. Hypertrophic obstructive cardiomyopathy-alcohol septal ablation vs.
Long-term outcome of alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution. Ventricular arrhythmia following alcohol septal ablation for obstructive hypertrophic cardiomyopathy.
Updated meta-analysis of septal alcohol ablation versus myectomy for hypertrophic cardiomyopathy. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy.
Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy. Clinical spectrum, therapeutic options, and outcome of advanced heart failure in hypertrophic cardiomyopathy. Survival after cardiac transplantation in patients with hypertrophic cardiomyopathy. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. Successful therapeutic hypothermia in patients with hypertrophic cardiomyopathy. Independent assessment of the European Society of Cardiology sudden death risk model for hypertrophic cardiomyopathy.
Research priorities in hypertrophic cardiomyopathy: report of a working group of the National Heart, Lung, and Blood Institute. Identifying sarcomere gene mutations in hypertrophic cardiomyopathy: a personal history. Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. Prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and troponin T genes in hypertrophic cardiomyopathy: a comprehensive outpatient perspective.
Hypertrophic cardiomyopathy due to sarcomeric gene mutations is characterized by impaired energy metabolism irrespective of the degree of hypertrophy. Exacerbation of cardiac energetic impairment during exercise in hypertrophic cardiomyopathy: a potential mechanism for diastolic dysfunction.
Results of clinical genetic testing of 2, probands with hypertrophic cardiomyopathy: expanded panels offer limited additional sensitivity.
Genet Med. Cardiac myosin binding protein C regulates postnatal myocyte cytokinesis. Spirito P. The dawn of a better day for patients with hypertrophic cardiomyopathy. Maron BJ, Kalra A. The guideline, she notes, runs to 74 pages and recommendations, but comes with "Top 10 Take-Home Messages" to help clinicians absorb the most important details available in the ACC. Provided here are a few of the take-home messages particularly relevant to the general cardiologist.
General cardiologists should feel comfortable making the diagnosis of HCM looking at symptoms, the physical exam, initial EKG, echocardiogram, and family history, according to the guideline. They can also make initial treatment recommendations and do a rapid assessment for changes in the disease course. Michelle M. While there were no explicit recommendations in the guideline for when to refer, the new guideline says a referral should be made for Class 2b ICD decision-making, septal reduction therapies, advanced HF management, and decision-making surrounding high-intensity or competitive sports, pregnancy and pediatric HCM.
Kittleson, known for her kittlesonrules on Twitter, shared some management pearls for Cardiology readers. But the story is very different from what it was even a few years ago. This is a remarkable statement to make and light years different from a time not that long ago when you gave a patient a beta-blocker and hoped you didn't get a phone call that they had died," says Maron. Now, studies have shown this is not an independent risk factor for SCD, nor is the degree of LV outflow tract obstruction and the presence of genetic mutations, she says.
Another important change in the new guideline pertains to sports and activity. In the old guideline, low-intensity aerobic exercise was 'reasonable,' and "that was a generally proscriptive and narrow Class IIa recommendation with a Level of Evidence of C," says Kittleson. The new guideline has incorporated research from the past decade showing that in patients with HCM, sudden death is generally rare in exercise and that a moderate-intensity recreational exercise program is not only safe, but encouraged for most patients with HCM, just like for the general population.
But they should feel comfortable telling patients with HCM to play some doubles tennis or go on a hike. Patients should limit themselves based on their symptoms and go at a pace that feels comfortable," she says.
In terms of participation in competitive sports, that should "absolutely" be deferred to a comprehensive HCM center and an HCM specialist "because there is a lot of gray area and a lot of shared decision-making around this," Kittleson adds. There's a lot of expertise and new data since the last guideline about what works and what doesn't.
Mavacamten is big news in the HCM world. It's a first-in-class, cardiac-specific myosin inhibitor that reduces the number of available actin-myosin cross-bridges and thus decreases excessive myocardial contractility, improving symptoms and QOL in patients with obstructive HCM. Mavacamten-treated patients showed greater reductions in post-exercise left ventricular outflow tract LVOT gradient vs. Daniel Jacoby, MD. The trial was conducted in patients enrolled at 68 clinical centers in 13 countries.
Randomization was to mavacamten or placebo for 30 weeks. In his virtual presentation of the main results at ESC Congress , principal investigator Iacopo Olivotto, MD , called the findings a breakthrough that the community has been working towards for 60 years.
HCM is usually asymptomatic, but when symptoms appear — exercise intolerance, fatigue, angina, syncope — the effect on QOL can be profound and the primary goal of treatment is to alleviate symptoms. In a small subgroup with available data, these gains disappeared after treatment cessation. He expects the drug will likely be approved. But with that, it's also important to be discerning about what the trial did and did not show. Neither was this a trial that directly compared medical therapy and substrate reduction therapy,so we really should be careful about comparing these randomized data from EXPLORER-HCM with retrospective, observational data that we have on medical therapies and septal reduction," says Jacoby.
For his part, Maron is less bullish. We also need longer-term data on the drug, he feels, to understand how residual gradients impact longer-term efficacy.
Cost is also an important consideration, says Maron. Kittleson is taking a cautious but hopeful approach. Probably not.
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