Impact of Effective Management Strategies on Patients With the Most Extreme Phenotypic Expression of Hypertrophic Cardiomyopathy

Rowin, Ethan J.; Maron, Barry J.; Romashko, Mikhail; Wang, Wendy; Rastegar, Hassan; Link, Mark S.; Maron, Martin S.

doi:10.1016/j.amjcard.2019.04.002

Cited by 18 publications

(10 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…1,4-6,16,S-13-S-16 Established risk markers for patients with phenotypically expressed HCM are noninvasive and utilize personal and family history, contemporary imaging (echocardiography, contrast CMR), and ambulatory ECG monitoring (Table 1 and Figure 1). Individual risk factors most predictive of SD events in adults [4][5][6][7][8][9][10][11][12][13][14][15][16][19][20][21][22]44,52,[57][58][59][60][61][62][63][64][65][66] are recent unexplained syncope unlikely to be neurocardiogenic; HCM-related SD in first-degree or other close relatives; repetitive and/or prolonged NSVT episodes on Holter or extended ambulatory monitoring 59 ; massive left ventricular hypertrophy (LVH) (wall thickness 30 mm); extensive/diffuse LGE; endstage heart failure usually with systolic dysfunction; and thin-walled akinetic LV apical aneurysm with regional scarring (Table 1, and Figures 1, 2, and 4).…”

Section: Current Risk Factorsmentioning

confidence: 99%

“…The efficacy of ICDs is largely independent of age, including high-risk children and young adults 11,14,20,66 as well as patients with extreme phenotypic expression. 61 ICD performance in HCM is notable given the complex arrhythmogenic substrate characterized by greatly increased LV mass, outflow obstruction, diastolic dysfunction, and microvascular ischemia. 1 A novel 17-year study of prospective ICD decisionmaking for primary prevention in .2000 consecutive patients from the Tufts HCM Institute adds considerable support to the retrospective registry data (Table 1, and Figures 2 and 3).…”

Section: Strategies For Sd Prevention: the Icdmentioning

confidence: 99%

“…This is 3-fold lower than achieved with ACC-AHA risk markers, a strategy that is associated with 95% sensitivity (Table 3). 16,61,66,[88][89][90]102,103 Only one-third of patients who experienced appropriate therapy had high scores (6%/5 years) sufficient to recommend an ICD (Figure 3), and about 40% had low risk scores (,4%/5 years) that would have excluded an ICD recommendation, theoretically leaving a subpopulation of high-risk HCM patients unprotected by ICDs and vulnerable to arrhythmic SD (Figure 3). A similarly constructed risk score model has been proposed for children (HCM Risk-Kids) 69 but with uncertain sensitivity.…”

Section: Risk Scoresmentioning

confidence: 99%

See 2 more Smart Citations

Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: Twenty years with the implantable cardioverter-defibrillator

2021

Self Cite

View full text Add to dashboard Cite

Hypertrophic cardiomyopathy (HCM) is a relatively common inherited myocardial disorder, once regarded as largely untreatable with ominous prognosis and most visibly as a common cause of sudden death (SD) in the young. Over the last several years, HCM has been transformed into a contemporary treatable disease with management options that significantly alter clinical course. With the use of implantable cardioverter-defibrillators (ICDs) in the HCM patient population, a new paradigm has emerged, with primary prevention device therapy reliably terminating potentially lethal ventricular tachyarrhythmias (3%-4% per year) and being largely responsible for a .10-fold decrease in disease-related mortality (to 0.5% per year), independent of age.An evidenced-based and guideline directed clinical risk stratification algorithm has evolved, including variables identified with cardiac magnetic resonance. One or more risk markers judged major and relevant within a patient's clinical profile can be considered sufficient to recommend a primary prevention implant (associated with a measure of physician judgment and shared decision-making). ICD decisions using the prospective individual risk marker strategy have been associated with 95% sensitivity for identifying patients who subsequently experienced appropriate ICD therapy, (albeit often delayed substantially for .5 or .10 years after implant), but without heart failure deterioration or HCM death following device intervention. A rigid mathematically derived statistical risk model proposed by the European Society of Cardiology is associated with low sensitivity (ie, 33%) for predicting SD events.Introduction of prophylactically inserted ICDs to HCM 20 years ago has significantly altered the clinical course and landscape of this disease. SD prevention has reduced HCM mortality significantly, making preservation of life and the potential for normal longevity a reality for most patients.

show abstract

Section: Current Risk Factorsmentioning

confidence: 99%

Section: Strategies For Sd Prevention: the Icdmentioning

confidence: 99%

Section: Risk Scoresmentioning

confidence: 99%

See 1 more Smart Citation

Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: Twenty years with the implantable cardioverter-defibrillator

2021

Self Cite

View full text Add to dashboard Cite

show abstract

“…Over the last decade, HCM has been highlighted as a major cause of sudden cardiac death in young adults 4 , 5 , and most research on HCM has focused on early diagnosis and risk stratification to prevent sudden cardiac death 6 – 8 . Thanks to recent progress in the management of HCM, however, the life expectancy of patients with HCM has significantly increased, leading to changes in the main causes of morbidity and mortality in HCM population 9 .…”

Section: Introductionmentioning

confidence: 99%

Emergency department utilization in patients with hypertrophic cardiomyopathy: a nationwide population-based study

Choi

Kim

Lee

et al. 2022

Sci Rep

View full text Add to dashboard Cite

Despite the increasing burden of hypertrophic cardiomyopathy (HCM) on healthcare resources, data on emergency department (ED) utilization in HCM are lacking. This nationwide population-based study extracted 14,542 HCM patients from the National Health Insurance Service database between 2015–2016, and investigated their ED utilization during a one-year period. The reason for ED utilization was defined as the primary diagnosis upon discharge from EDs. The clinical outcome was defined as hospitalization or all-cause mortality within 90 days after the ED visits. A total of 3209 (22.1%) HCM patients visited EDs within a one-year period (mean age, 66.8 ± 13.8 years; male, 57.4%). The majority (71.1%) of HCM patients who visited the EDs were aged ≥ 60 years. The ED utilization rate was higher in women than in men (26.3% versus 19.7%, P < 0.001). Cardiovascular diseases were the most common reason for ED visits (n = 1333, 41.5%). Among HCM patients who visited EDs, 1195 (37.2%) were hospitalized, and 231 (7.2%) died within 90 days. ED visits for cardiovascular disease was associated with a higher 90-day all-cause mortality (adjusted odds ratio, 2.72; 95% confidence interval 1.79–4.12). These findings would serve as a basis for future research to establish medical policies on ED utilization in HCM.

show abstract

“…HCM patients with extreme interventricular septal thickness (IVST) may represent a special cohort. However, most of these studies are all based on HCM patients, both Original Article Surgical outcome for hypertrophic cardiomyopathy patients with extreme interventricular septal thickness: a propensity score matched study obstructed and non-obstructed (6)(7)(8). Few studies have reported the surgical outcomes of patients with extreme IVST who underwent septal myectomy.…”

Section: Introductionmentioning

confidence: 99%

Surgical outcome for hypertrophic cardiomyopathy patients with extreme interventricular septal thickness: a propensity score matched study

Ren

Wang

et al. 2020

J Thorac Dis

View full text Add to dashboard Cite

Background: Hypertrophic cardiomyopathy (HCM) with extreme interventricular septal thickness (IVST) is associated with a higher incidence of adverse cardiovascular events. However, the results of these patients who underwent septal myectomy are unclear. Methods: We studied 47 HCM patients with IVST ≥30 mm who underwent septal myectomy between 2011 and 2018 in Anzhen Hospital. After a 2:1 propensity score matching, the study cohort included 141 patients and 94 patients with IVST <30 mm. Results: Patients with IVST ≥30 mm had a longer clinical course of disease, high incidence of syncope, palpitation, and moderate or severe mitral regurgitation. After a mean follow-up of 34.0±21.3 months, 6 patients died, including 5 with IVST ≥30 mm and 1 with IVST <30 mm. The 5-year survival free of allcause mortality was significantly higher in patients with IVST ≥30 mm than in those with IVST <30 mm (98.9% vs. 85.7%, P=0.03). Multivariable Cox analysis revealed that IVST ≥30 mm (HR: 1.12, 95% CI:1.01-1.25, P=0.028) was an independent risk factor for all-cause mortality. Meanwhile, left ventricular end diastole diameter (HR: 0.72, 95% CI: 0.54-0.97, P=0.031) and age (HR: 0.91, 95% CI: 0.83-0.99, P=0.025) were also independent risk factors for all-cause mortality in this special cohort. Furthermore, the incidence of NYHA class III or IV was significantly higher in patients with IVST ≥30 mm. Conclusions:The surgical outcome was poor in a matched cohort of HCM patients with IVST ≥30 mm, which was mainly reflected by mortality and the incidence of NYHA class III or IV.

show abstract

Impact of Effective Management Strategies on Patients With the Most Extreme Phenotypic Expression of Hypertrophic Cardiomyopathy

Cited by 18 publications

References 28 publications

Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: Twenty years with the implantable cardioverter-defibrillator

Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: Twenty years with the implantable cardioverter-defibrillator

Emergency department utilization in patients with hypertrophic cardiomyopathy: a nationwide population-based study

Surgical outcome for hypertrophic cardiomyopathy patients with extreme interventricular septal thickness: a propensity score matched study

Contact Info

Product

Resources

About