Outcome of mildly symptomatic or asymptomatic obstructive hypertrophic cardiomyopathy: a long-term follow-up study.

Published on Jul 14, 2009in Journal of the American College of Cardiology20.589
· DOI :10.1016/J.JACC.2009.01.079
Paul Sorajja39
Estimated H-index: 39
(Mayo Clinic),
Rick A. Nishimura128
Estimated H-index: 128
(Mayo Clinic)
+ 4 AuthorsSteve R. Ommen78
Estimated H-index: 78
(Mayo Clinic)
Sources
Abstract
Objectives The purpose of this study was to characterize the prognosis of minimally symptomatic patients with obstructive hypertrophic cardiomyopathy (HCM). Background Recent data have suggested that obstruction may be present in the majority of HCM patients, irrespective of cardiac symptoms. The prognosis of minimally symptomatic obstructive HCM remains poorly defined. Methods We examined 544 consecutive adult patients (age 59 ± 16 years; 55% men) with obstructive HCM documented by Doppler echocardiography who were free of severe cardiac symptoms, and we performed clinical follow-up (median 9.3 years). Results There was only a slight excess mortality of the cohort in comparison to the expected survival of a similar U.S. general population (10-year observed vs. expected survival, 69.3% vs. 71.9%; p = 0.04) and 46% of the deaths were attributable to noncardiac causes. However, there was a clear relation between increasing severity of the left ventricular outflow tract (LVOT) gradient and outcome. For patients with high resting gradients (Doppler peak velocity >4 m/s), survival was significantly impaired (53% at 10 years; p = 0.001 vs. expected), and death or severe symptoms occurred in 68% of these patients within 10 years after the initial evaluation. Conversely, there was no impairment of long-term survival for patients with less-severe resting obstruction. Independent predictors of mortality in the entire cohort were age, prior stroke, and LVOT gradient severity. Conclusions Patients with obstructive HCM and mild or no symptoms have only slight excess mortality. However, patients with markedly elevated resting LVOT gradients are at a high risk of heart failure and death. These findings may have important implications for therapy, including the timing of septal reduction therapy.
📖 Papers frequently viewed together
1,272 Citations
410 Citations
201123.60Circulation
14 Authors (Bernard J. Gersh, ..., Clyde W. Yancy)
651 Citations
References34
Newest
#1Joseph C. Vaglio (VUMC: Vanderbilt University Medical Center)H-Index: 2
#2Steve R. Ommen (Mayo Clinic)H-Index: 78
Last. Bernard J. Gersh (Mayo Clinic)H-Index: 163
view all 5 authors...
Background The purpose of this article is to describe the demographic and clinical features of patients with hypertrophic cardiomyopathy (HCM) and latent obstruction, with an emphasis on identifying factors associated with disease progression and survival. The presence of a resting left ventricular outflow obstruction in patients with HCM has been well described and is associated with increased symptoms and adverse long-term outcomes. However, less is known about patients with latent obstruction...
68 CitationsSource
#1Ahmad A. Elesber (Mayo Clinic)H-Index: 16
#2Rick A. Nishimura (Mayo Clinic)H-Index: 128
Last. David R. Holmes (Mayo Clinic)H-Index: 192
view all 6 authors...
It is important to identify patients with hypertrophic cardiomyopathy (HC) who have labile left ventricular outflow tract (LVOT) obstruction for consideration of ventricular septal reduction therapy. Although Doppler echocardiography has become the diagnostic modality to assess LVOT obstruction, it may not identify all patients with labile obstruction. This study assessed the diagnostic value of cardiac catheterization using isoproterenol challenge in 25 patients with HC in whom an LVOT obstruct...
62 CitationsSource
#1Iacopo OlivottoH-Index: 64
#2Steve R. Ommen (Mayo Clinic)H-Index: 78
Last. Barry J. MaronH-Index: 169
view all 5 authors...
Dynamic left ventricular outflow tract obstruction is an important pathophysiologic feature of hypertrophic cardiomyopathy (HCM) and a predictor of clinical deterioration and cardiovascular mortality. Patients with marked obstruction and severe limiting symptoms refractory to maximum medical management are considered candidates for invasive septal reduction therapy, which includes surgical myectomy and alcohol septal ablation (ASA). Availability of both surgical myectomy and ASA has polarized th...
160 CitationsSource
#1Rick A. Nishimura (Mayo Clinic)H-Index: 128
#2Steve R. Ommen (Mayo Clinic)H-Index: 78
Hypertrophic cardiomyopathy is a fascinating disease entity in which hypertrophy of the myocardium occurs, primarily as a result of mutations of genes encoding the cardiac sarcomere.1–3 Our knowledge of patients with this unique disease of the heart muscle has advanced significantly. Once thought to be a rare malignant disease with a high risk of sudden death, population studies have revealed hypertrophic cardiomyopathy to be a more common disease with a benign outlook in the majority of affecte...
62 CitationsSource
#1Martin S. Maron (Tufts Medical Center)H-Index: 70
#2Iacopo OlivottoH-Index: 64
Last. Barry J. MaronH-Index: 169
view all 10 authors...
Background— Nonobstructive hypertrophic cardiomyopathy (HCM) has been regarded as the predominant hemodynamic form of the disease on the basis of assessment of outflow gradient under resting conditions. We sought to prospectively define the prevalence, clinical profile, and significance of left ventricular (LV) outflow tract obstruction under resting conditions and with physiological exercise in a large HCM cohort. Methods and Results— We prospectively analyzed 320 consecutive HCM patients (age,...
679 CitationsSource
2,318 CitationsSource
#1Robert O. BonowH-Index: 166
#2Blase A. CarabelloH-Index: 74
Last. Barbara RiegelH-Index: 107
view all 14 authors...
PREAMBLE......e4 APPENDIX 1......e121 APPENDIX 2......e122 APPENDIX 3......e124 REFERENCES......e124 It is important that the medical profession play a significant role in critically evaluating the use of diagnostic procedures and therapies as they are introduced in the detection, management,
6,200 CitationsSource
#1Perry M. Elliott (UCL: University College London)H-Index: 112
#2Juan R. Gimeno (UCL: University College London)H-Index: 31
Last. William J. McKenna (UCL: University College London)H-Index: 1
view all 8 authors...
Aims Left ventricular outflow tract obstruction (LVOTO) is associated with reduced survival in patients with hypertrophic cardiomyopathy (HCM). The influence of LVOTO on survival from SD in relation to other recognized clinical risk markers is unknown. Methods and results A total of 917 patients with HCM (554 males, 43±15 years) were studied; 288 (31.4%) had LVOTO at rest (≥30 mmHg). During follow-up [median 61 (30;99) months], 54 (5.9%) patients died suddenly (SD), survived ventricular fibrilla...
410 CitationsSource
#1Paul Sorajja (Mayo Clinic)H-Index: 39
#2Rick A. Nishimura (Mayo Clinic)H-Index: 128
Last. Bernard J. Gersh (Mayo Clinic)H-Index: 163
view all 6 authors...
We examined the 10-year outcome of 107 patients with hypertrophic cardiomyopathy and massive myocardial hypertrophy as assessed by echocardiography. These patients demonstrated excess mortality in comparison with their expected survival with respect to a general population of individuals (10-year survival 77% vs 95%, P
91 CitationsSource
#1Steve R. Ommen (Mayo Clinic)H-Index: 78
#2Barry J. Maron (Mayo Clinic)H-Index: 169
Last. Rick A. Nishimura (Mayo Clinic)H-Index: 128
view all 14 authors...
Objectives This study sought to determine the impact of surgical myectomy on long-term survival in hypertrophic cardiomyopathy (HCM). Background Left ventricular (LV) outflow tract obstruction in HCM increases the likelihood of heart failure and cardiovascular death. Although surgical myectomy is the primary treatment for amelioration of outflow obstruction and advanced drug-refractory heart failure symptoms, its impact on long-term survival remains unresolved. Methods Total and HCM-related mort...
648 CitationsSource
Cited By89
Newest
#1William R. Miranda (Mayo Clinic)H-Index: 14
#2Alexander C. Egbe (Mayo Clinic)H-Index: 20
Last. Heidi M. Connolly (Mayo Clinic)H-Index: 75
view all 4 authors...
Abstract Background Data on the features of double-chambered right ventricular (DCRV) in adults are limited. It is also unclear how invasive and Doppler-derived assessments of right ventricular (RV) obstruction in DCRV correlate. Methods Retrospective review of 17 adults (age ≥18 years) diagnosed with DCRV who underwent right heart catheterization at Mayo Clinic, MN between 1990 and 2021. Clinical, echocardiographic, and invasive hemodynamics were abstracted from the medical charts. Results Mean...
Source
6 CitationsSource
Objectives The aim of this study is to evaluate the impact of percutaneous transluminal septal myocardial ablation (PTSMA) on remodeling in asymptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) and severe left ventricular outflow tract (LVOT) obstruction. Background Symptoms justify invasive treatment in HOCM patients with LVOT obstruction. Adverse structural and functional changes (remodeling) in the heart occur preceding heart failure and sudden cardiac death. Early invasiv...
Source
Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. Methods A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from P...
22 CitationsSource
#2Steve R. OmmenH-Index: 78
#3Seema MitalH-Index: 49
Last. Paul SorajjaH-Index: 24
view all 19 authors...
5 CitationsSource
#2Steve R. OmmenH-Index: 78
#3Seema MitalH-Index: 49
Last. Paul SorajjaH-Index: 24
view all 19 authors...
Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy i...
4 CitationsSource
#1Amine Mazine (Sunnybrook Health Sciences Centre)H-Index: 1
#2Derrick Y. TamH-Index: 19
Last. Stephen E. FremesH-Index: 71
view all 3 authors...
Source
#1Stephanie N. Nguyen (UCI: University of California, Irvine)
#2Yuichi J. Shimada (UCI: University of California, Irvine)
Last. Hiroo Takayama (UCI: University of California, Irvine)H-Index: 1
view all 4 authors...
Source
#1Hao Cui (Mayo Clinic)H-Index: 4
#2Hartzell V. Schaff (Mayo Clinic)H-Index: 131
Last. Steve R. Ommen (Mayo Clinic)H-Index: 78
view all 7 authors...
Abstract Objective This study was conducted to determine the influence of early septal reduction therapy (SRT) after referral on survival in patients with obstructive hypertrophic cardiomyopathy. Methods We reviewed the patients with obstructive hypertrophic cardiomyopathy (resting pressure gradient ≥30 mm Hg or provoked pressure gradient ≥50 mm Hg) who were evaluated at our clinic from 2000 to 2012. Early SRT was defined as undergoing septal myectomy or alcohol septal ablation during the 6 mont...
4 CitationsSource
1. Introducao Esta cientificamente comprovado, sendo algo incorporado ao senso comum, que ser fisicamente ativo contribui para preservar e recuperar a boa saude do corpo e da mente. Os efeitos favoraveis da reabilitacao cardiovascular (RCV) com enfase nos exercicios fisicos tem sido consistentemente documentados, inclusive em meta-analises de estudos clinicos randomizados, que demonstram significativas reducoes da morbimortalidade cardiovascular e global, bem como da taxa de hospitalizacao, , co...
6 CitationsSource