Myocardial adaptation after surgical therapy differs for aortic valve stenosis and hypertrophic obstructive cardiomyopathy

Parbhudayal, Rahana Y.; Güçlü, Ahmet; Zweerink, Alwin; Biesbroek, P. Stefan; Croisille, Pierre; Clarysse, Patrick; Michels, Michelle; Stooker, Wim; Vonk, Alexander B.A.; Ven, Peter M. van der; Rossum, Albert C. van; Velden, Jolanda van der; Nijveldt, Robin

doi:10.1007/s10554-019-01563-3

Cited by 6 publications

(6 citation statements)

References 36 publications

(39 reference statements)

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“…Thus, they would not have captured the development of systolic dysfunction or death-equivalent outcomes of cardiac transplantation or LVAD implantation (30 patients in our cohort). Nonetheless, 2 smaller studies also identified an increased risk of developing impaired LV function after SRT 20 and emphasized that the duration of follow-up needs to be relatively long, at least 8 to 10 years, to capture this adverse remodeling. 21 This time frame is supported by our current findings.…”

Section: Incident Development Of Systolic Dysfunctionmentioning

confidence: 99%

Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction

et al. 2020

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Background: The term “end stage” has been used to describe hypertrophic cardiomyopathy (HCM) with left ventricular systolic dysfunction (LVSD), defined as occurring when left ventricular ejection fraction is <50%. The prognosis of HCM-LVSD has reportedly been poor, but because of its relative rarity, the natural history remains incompletely characterized. Methods: Data from 11 high-volume HCM specialty centers making up the international SHaRe Registry (Sarcomeric Human Cardiomyopathy Registry) were used to describe the natural history of patients with HCM-LVSD. Cox proportional hazards models were used to identify predictors of prognosis and incident development. Results: From a cohort of 6793 patients with HCM, 553 (8%) met the criteria for HCM-LVSD. Overall, 75% of patients with HCM-LVSD experienced clinically relevant events, and 35% met the composite outcome (all-cause death [n=128], cardiac transplantation [n=55], or left ventricular assist device implantation [n=9]). After recognition of HCM-LVSD, the median time to composite outcome was 8.4 years. However, there was substantial individual variation in natural history. Significant predictors of the composite outcome included the presence of multiple pathogenic/likely pathogenic sarcomeric variants (hazard ratio [HR], 5.6 [95% CI, 2.3–13.5]), atrial fibrillation (HR, 2.6 [95% CI, 1.7–3.5]), and left ventricular ejection fraction <35% (HR, 2.0 [95% CI, 1.3–2.8]). The incidence of new HCM-LVSD was ≈7.5% over 15 years. Significant predictors of developing incident HCM-LVSD included greater left ventricular cavity size (HR, 1.1 [95% CI, 1.0–1.3] and wall thickness (HR, 1.3 [95% CI, 1.1–1.4]), left ventricular ejection fraction of 50% to 60% (HR, 1.8 [95% CI, 1.2, 2.8]–2.8 [95% CI, 1.8–4.2]) at baseline evaluation, the presence of late gadolinium enhancement on cardiac magnetic resonance imaging (HR, 2.3 [95% CI, 1.0–4.9]), and the presence of a pathogenic/likely pathogenic sarcomeric variant, particularly in thin filament genes (HR, 1.5 [95% CI, 1.0–2.1] and 2.5 [95% CI, 1.2–5.1], respectively). Conclusions: HCM-LVSD affects ≈8% of patients with HCM. Although the natural history of HCM-LVSD was variable, 75% of patients experienced adverse events, including 35% experiencing a death equivalent an estimated median time of 8.4 years after developing systolic dysfunction. In addition to clinical features, genetic substrate appears to play a role in both prognosis (multiple sarcomeric variants) and the risk for incident development of HCM-LVSD (thin filament variants).

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Section: Incident Development Of Systolic Dysfunctionmentioning

confidence: 99%

Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction

et al. 2020

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“… 4 Another study demonstrated worsening of septal systolic strain rates and no improvements in global and regional longitudinal strain after septal myectomy despite improvement in exercise capacity in 10 patients with obstructive HCM. 32 In this study, the amount of late gadolinium enhancement showed no significant increase after septal myectomy. 32 The reduction of the hypertrophied LV and wall thinning after septal myectomy may also have contributed to the impaired LV longitudinal systolic function.…”

Section: Discussionmentioning

confidence: 45%

“… 32 In this study, the amount of late gadolinium enhancement showed no significant increase after septal myectomy. 32 The reduction of the hypertrophied LV and wall thinning after septal myectomy may also have contributed to the impaired LV longitudinal systolic function. However, there was no difference in the septal wall strain in our study.…”

Section: Discussionmentioning

confidence: 45%

“…The data indicated that septal myectomy can correct haemodynamic changes but cannot alter the genetic mutation of these patients and therefore cannot stop progressive myocardial fibrosis 4 . Another study demonstrated worsening of septal systolic strain rates and no improvements in global and regional longitudinal strain after septal myectomy despite improvement in exercise capacity in 10 patients with obstructive HCM 32 . In this study, the amount of late gadolinium enhancement showed no significant increase after septal myectomy 32 .…”

Section: Discussionmentioning

confidence: 54%

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Effects of septal myectomy on left atrial and left ventricular function in obstructive hypertrophic cardiomyopathy

Choi

Lee

et al. 2023

ESC Heart Failure

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AimsMechanical function of the left atrium (LA) and the left ventricle (LV) has been demonstrated to be a prognostic factor in patients with hypertrophic cardiomyopathy (HCM). We explore whether myocardial mechanical function can be improved by septal reduction therapy in symptomatic obstructive HCM.Methods and resultsAmong 65 patients who underwent septal myectomy for symptomatic obstructive HCM from 2006 to 2022, 44 were analysed after excluding those who underwent simultaneous valve repair or replacement or maze operation. LA and LV functional variables including LA strain and LV global longitudinal strain were evaluated by two‐dimensional and speckle‐tracking echocardiography and compared before and 1 year after surgery. After septal myectomy, LA volume index (58.1 ± 18.3 vs. 45.3 ± 14.6 mL/m2, P = 0.001) decreased significantly. As LV end‐systolic dimension increased after surgery, the LV ejection fraction decreased (73.8 ± 6.7 vs. 62.9 ± 8.3%, P < 0.001). LA strain (24.4 ± 9.3 vs. 30.5 ± 13.6%, P = 0.004) improved after septal myectomy, but LV global longitudinal strain deteriorated (−12.6 ± 3.6 vs. −11.6 ± 4.3%, P = 0.033), mainly related to worsening non‐septal longitudinal strain (−14.4 ± 4.3 vs. −10.9 ± 8.4%, P = 0.005).ConclusionsAs haemodynamic loads due to LV outflow tract obstruction was relieved through surgical septal reduction therapy in patients with symptomatic obstructive HCM, there was a significant reduction in LA volume and restoration of LA mechanical dysfunction. However, LV mechanical dysfunction deteriorated even after surgical septal reduction therapy.

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“…In order to understand the differences in remodeling after afterload removal in myocardium with intrinsic myocardial abnormality vs. that with presumably normal intact intracellular pathways, it is interesting to consider the structural and functional recovery after myectomy for oHCM and aortic valve replacement (AVR) for aortic valve stenosis, an extrinsic cause of hypertrophy ( 58 ). A small prospective study of 10 patients with oHCM and 10 patients with severe aortic stenosis (AS) were examined with echocardiography, CMR, and exercise testing.…”

Section: Myocardial Remodeling In Hcmmentioning

confidence: 99%

Prospects for remodeling the hypertrophic heart with myosin modulators

Sewanan

Shimada²

2022

Front. Cardiovasc. Med.

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Hypertrophic cardiomyopathy (HCM) is a complex but relatively common genetic disease that usually arises from pathogenic variants that disrupt sarcomere function and lead to variable structural, hypertrophic, and fibrotic remodeling of the heart which result in substantial adverse clinical outcomes including arrhythmias, heart failure, and sudden cardiac death. HCM has had few effective treatments with the potential to ameliorate disease progression until the recent advent of inhibitory myosin modulators like mavacamten. Preclinical investigations and clinical trials utilizing this treatment targeted to this specific pathophysiological mechanism of sarcomere hypercontractility in HCM have confirmed that myosin modulators can alter disease expression and attenuate hypertrophic remodeling. Here, we summarize the state of hypertrophic remodeling and consider the arguments for and against salutary HCM disease modification using targeted myosin modulators. Further, we consider critical unanswered questions for future investigative and therapeutic avenues in HCM disease modification. We are at the precipice of a new era in understanding and treating HCM, with the potential to target agents toward modifying disease expression and natural history of this most common inherited disease of the heart.

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Myocardial adaptation after surgical therapy differs for aortic valve stenosis and hypertrophic obstructive cardiomyopathy

Cited by 6 publications

References 36 publications

Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction

Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction

Effects of septal myectomy on left atrial and left ventricular function in obstructive hypertrophic cardiomyopathy

Prospects for remodeling the hypertrophic heart with myosin modulators

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