Related Experiment Video
Updated: May 29, 2025
Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Clinical burden of obstructive hypertrophic cardiomyopathy in France
Philippe Charron1, Carla Zema2, François-Emery Cotté3
1Departments of Genetics and Cardiology, Sorbonne Université, AP-HP, Referral Center for Cardiac Hereditary Diseases, Pitié-Salpêtrière University Hospital, IHU-ICAN, INSERM UMRS_1166, Paris, France.
Insights
Obstructive hypertrophic cardiomyopathy (HCM) patients face significant clinical burden and cardiovascular risks, particularly those in advanced New York Heart Association (NYHA) classes. Current treatments show limited impact, highlighting a need for novel therapies.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart disease with obstructive and non-obstructive subtypes.
- The clinical burden of obstructive HCM, especially by New York Heart Association (NYHA) class, is not well-characterized nationally.
- Understanding this burden is crucial for patient management and therapeutic development.
Purpose of the Study:
- To describe the clinical burden of patients hospitalized with obstructive HCM in France.
- To analyze treatment patterns and clinical outcomes stratified by NYHA class.
- To identify unmet needs in managing symptomatic obstructive HCM.
Main Methods:
- Retrospective, longitudinal, observational study using the French National Health Data System (2012-2018).
- Included adult patients with obstructive HCM (ICD-10 I42.1) or related codes and septal reduction therapy.
- NYHA class assigned via algorithm; outcomes assessed over a minimum 1-year follow-up.
Main Results:
- 6,823 obstructive HCM patients included; 60% were NYHA class III at baseline.
- High incidence of cardiovascular-related hospitalizations (117,229/100,000 patient-years), increasing with NYHA class.
- 73% remained in their baseline NYHA class, with 14% worsening and 13% improving over ~4.4 years.
Conclusions:
- Obstructive HCM patients face high risks of death and cardiovascular events, particularly in higher NYHA classes.
- Despite available treatments like beta-blockers and calcium-channel blockers, the clinical burden remains substantial.
- The study underscores the urgent need for innovative therapeutic strategies for symptomatic obstructive HCM.
Background:
Hypertrophic cardiomyopathy (HCM) can be genetic and occurs as obstructive and non-obstructive 21 subtypes. Little is known about the clinical burden of the obstructive subtype of HCM at a national 22 level, and how it may differ by New York Heart Association (NYHA) class. Therefore, this study 23 aimed to describe the clinical burden of patients hospitalized with obstructive HCM from a 24 nationwide study in France.
Methods:
This retrospective, longitudinal, observational study was performed using data from the French National Health Data System. All adult patients (≥ 18 years old) with a hospitalization related to obstructive HCM [International Classification of Diseases, Tenth Revision (ICD-10) code I42.1], or at least one hospitalization with ICD-10 code I42.2 or I42.9 and at least one code for septal reduction therapy between 2012 and 2018 were included. Patients were followed up for a minimum of 1 year. NYHA class was assigned using an algorithm based on treatment and symptom codes. Treatment patterns and clinical outcomes by NYHA class over time were examined.
Results:
Overall, 6,823 patients with obstructive HCM were included (54.7% male, mean [standard deviation (SD)] age 66.2 [16.7] years). At inclusion, the proportion of patients in NYHA classes I, II, III, and IV were 4%, 32%, 60%, and 4%, respectively. Over the follow-up [mean (SD) follow-up: 4.4 (2.5) years; cumulative patient follow-up: 30,021 patient-years], 73% of patients remained in the same NYHA class, 14% of patients worsened, and 13% improved. At inclusion, 22% of patients had no HCM-related treatment, 56% were receiving β-blockers, 12% calcium-channel blockers, and 11% a combination of both. The incidence of cardiovascular-related hospitalization was high (35,436 hospitalizations; 117,229 per 100,000 patient-years) and this risk increased with NYHA class (from 81,247 per 100,000 patient-years for NYHA class I/II patients to 140,790 per 100,000 patient-years for NYHA class III/IV patients, p < 0.0001).
Conclusions:
Patients with obstructive HCM are at high risk of death and cardiovascular outcomes, especially those in higher NYHA classes. Despite current therapeutics, the clinical burden of symptomatic obstructive HCM remains high, supporting the need for additional therapies.
Related Concept Videos
01:17Pathophysiology of Heart Failure
01:26Imbalances in Cardiac Output
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...