2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy

Ommen, Steve R.; Mital, Seema; Burke, Michael A.; Day, Sharlene M.; Deswal, Anita; Elliott, Perry; Evanovich, Lauren L.; Hung, Judy; Joglar, José A.; Kantor, Paul F.; Kimmelstiel, Carey; Kittleson, M.; Link, Mark S.; Maron, Martin S.; Martinez, Matthew W.; Miyake, Christina Y.; Schaff, Hartzell V.; Semsarian, Christopher; Sorajja, Paul; O’Gara, Patrick T.; Beckman, Joshua A.; Levine, Glenn N.; Al‐Khatib, Sana M.; Armbruster, Anastasia L.; Birtcher, Kim K.; Ciggaroa, Joaquin; Dixon, Dave L.; Fuentes, Lisa de las; Fleisher, Lee A.; Gentile, Federico; Goldberger, Zachary D.; Görenek, Bülent; Haynes, Norrisa; Hernandez, Adrian F.; Mark, Daniel B.; Jones, W. Schuyler; Marine, Joseph E.; Mark, Daniel B.; Palaniappan, Latha; Piano, Mariann R.; Tamis-Holland, Jacqueline; Wijeysundera, Duminda N.; Woo, Y. Joseph

doi:10.1016/j.jtcvs.2021.04.001

Cited by 78 publications

(103 citation statements)

References 477 publications

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“…Molecular biology has had limited relevance to contemporary HCM patient management (28,29). Of the multitude of sarcomere mutations now reported in HCM patients, none have evidencebased prognostic power (1,4,5,25). Basic science in this disease continues to be focused on myosin structure-function in genetically engineered murine models (26,27).…”

Section: Prevalence and Etiologymentioning

confidence: 99%

“…The power of the ICD for changing the clinical course of HCM with preservation of life is due to an evolving algorithm for the selection of patients. The major guideline-directed noninvasive sudden death risk markers are 95% sensitive for predicting which patients will experience appropriate device therapy (5,10,40). Patient age plays an important role in ICD decision making; for example, recognition of low sudden death risk in stable patients at least 60 years old has suppressed prophylactic implants in this age group (41).…”

Section: Prevention Of Sudden Deathmentioning

confidence: 99%

“…Hypertrophic cardiomyopathy (HCM) is an inheritable, relatively common, and increasingly recognized cardiac disorder with global occurrence, characterized by left ventricular (LV) hypertrophy. It is an important cause of arrhythmic sudden death, heart failure, and atrial fibrillation (AF) (1)(2)(3)(4)(5)(6)(7)(8). Indeed, for much of its 60-year history, HCM has been perceived as a grim and unyielding rare condition with uncertain prognosis and limited treatment options.…”

Section: Introductionmentioning

confidence: 99%

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Hypertrophic Cardiomyopathy: New Concepts and Therapies

2022

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Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited myocardial disorder, transformed over the last several years into a treatable condition with the emergence of effective management options that alter natural history at all ages. Now available are a matured risk stratification algorithm selecting patients for prophylactic implantable defibrillators that prevent arrhythmic sudden death; low-risk, high-benefit surgical myectomy to reverse progressive heart failure symptoms due to left ventricular outflow obstruction; anticoagulation prophylaxis to prevent atrial fibrillation–mediated embolic stroke; and heart transplant for refractory end-stage disease in the absence of obstruction. Those strategies have resulted in reduction of HCM-related morbidity and reduction of mortality to 0.5% per year.

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Section: Prevalence and Etiologymentioning

confidence: 99%

Section: Prevention Of Sudden Deathmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Hypertrophic Cardiomyopathy: New Concepts and Therapies

2022

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“…The recently updated 2020 American Heart Association and American College of Cardiology Guidelines now recommend a multi-disciplinary HCM center as the preferred model of care. 1 Not all programs have the same access to advanced care, and in those cases, referral arrangements can be identified to ensure the best outcomes. AHA/ACC established minimum standards for invasive therapies in the original Guidelines in 2011, and the HCMA believes high volume experience is critical to ensure the best outcomes for patients.…”

Section: Lessons Learnedmentioning

confidence: 99%

The role of patient advocacy in hypertrophic cardiomyopathy: The 25-year journey of the HCMA

Salberg¹

2022

Asian Cardiovasc Thorac Ann

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“…Our findings are consistent with the desired consensus guidelines outcomes that advocate SM at dedicated centres. 22…”

Section: Operative Outcomesmentioning

confidence: 99%

The first 200 septal myectomies: Ensuring gold standard outcomes

Bajona

2021

Asian Cardiovasc Thorac Ann

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Objectives Septal myectomy remains the gold standard treatment for symptomatic left ventricular outflow tract obstruction refractory to medical treatment. It is recommended that this operation be performed in dedicated hypertrophic obstructive cardiomyopathy centres by experienced surgeons. The septal myectomy option remains unavailable to many patients based solely on geography, including those who would clearly benefit more substantially from surgery than other therapeutic options. Here, we share our experience in starting new hypertrophic cardiomyopathy programmes. Methods We retrospectively reviewed initial septal myectomy experiences at two hypertrophic cardiomyopathy programmes starting in 2014. Results Two-hundred septal myectomies were performed. Mean age was 58.8 years and 51% were females. Advanced heart failure symptoms were present in 95.5% of patients and 23.5% had experienced syncope. Mean maximal intraventricular gradient was 89 mmHg and 48.5% underwent concomitant procedures at the time of septal myectomy. There was no perioperative (in-hospital or 30 days) mortality. Ninety-two per cent had provoked left ventricular gradients of ≤ 15 mmHg and 97% had none/mild mitral regurgitation at post-operative assessment. In our contemporary cohort, there were 2 (1%) intraoperative ventricular septal defects and 5% required a permanent pacemaker. Conclusions Our early septal myectomy experience targeted a complex population, frequently in need of concomitant procedures. Abolition of left ventricular obstruction and resolution of systolic anterior motion mediated mitral regurgitation can be expected. The safety and efficacy of septal myectomy carried at hypertrophic cardiomyopathy centres by properly trained surgeons achieved the desired outcomes established by recent hypertrophic cardiomyopathy guidelines.

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2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy

Cited by 78 publications

References 477 publications

Hypertrophic Cardiomyopathy: New Concepts and Therapies

Hypertrophic Cardiomyopathy: New Concepts and Therapies

The role of patient advocacy in hypertrophic cardiomyopathy: The 25-year journey of the HCMA

The first 200 septal myectomies: Ensuring gold standard outcomes

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