Target population for a selective cardiac myosin inhibitor in hypertrophic obstructive cardiomyopathy: Real-life estimation from the French register of hypertrophic cardiomyopathy (REMY) - 14/06/24
for the
REMY Working Group of the French Society of Cardiology
Graphical abstract |
Highlights |
• | We aimed to identify the real-life target population for a selective cMI in HCM. |
• | We studied 1059 adult patients with HCM enrolled in REMY (a French HCM registry) |
• | 325 (30.7%) of these patients were potential candidates for a cMI. |
• | 9% more became eligible for a cMI during follow-up. |
Abstract |
Background |
The efficacy of current pharmacological therapies in hypertrophic cardiomyopathy is limited. A cardiac myosin inhibitor, mavacamten, has recently been approved as a first-in-class treatment for symptomatic hypertrophic obstructive cardiomyopathy.
Aims |
To assess the profile and burden of cardiac myosin inhibitor candidates in the hypertrophic cardiomyopathy prospective Register of hypertrophic cardiomyopathy (REMY) held by the French Society of Cardiology.
Methods |
Data were collected at baseline and during follow-up from patients with hypertrophic cardiomyopathy enrolled in REMY by the three largest participating centres.
Results |
Among 1059 adults with hypertrophic cardiomyopathy, 461 (43.5%) had obstruction; 325 (30.7%) of these were also symptomatic, forming the “cardiac myosin inhibitor candidates” group. Baseline features of this group were: age 58±15years; male sex (n=196; 60.3%); diagnosis-to-inclusion delay 5 (1–12)years; maximum wall thickness 20±6mm; left ventricular ejection fraction 69±6%; family history of hypertrophic cardiomyopathy or sudden cardiac death (n=133; 40.9%); presence of a pathogenic sarcomere gene mutation (n=101; 31.1%); beta-blocker or verapamil treatment (n=304; 93.8%), combined with disopyramide (n=28; 8.7%); and eligibility for septal reduction therapy (n=96; 29%). At the end of a median follow-up of 66 (34–106) months, 319 (98.2%) were treated for obstruction (n=43 [13.2%] received disopyramide), 46 (14.2%) underwent septal reduction therapy and the all-cause mortality rate was 1.9/100 person-years (95% confidence interval 1.4–2.6) (46 deaths). Moreover, 41 (8.9%) patients from the initial hypertrophic obstructive cardiomyopathy group became eligible for a cardiac myosin inhibitor.
Conclusions |
In this cohort of patients with hypertrophic cardiomyopathy selected from the REMY registry, one third were eligible for a cardiac myosin inhibitor.
Le texte complet de cet article est disponible en PDF.Keywords : Hypertrophic cardiomyopathy, Obstruction, Registry, Cardiac myosin inhibitor
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Vol 117 - N° 6-7
P. 427-432 - juin 2024 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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