@article{Topriceanu:2026:10.1016/j.jacc.2026.03.176,
author = {Topriceanu, C-C and Balakrishnan, ID and Vissing, CR and Raja, AA and Parikh, VN and Zwetsloot, P-P and Michels, M and Argiro, A and Maurizi, N and Stendahl, JC and Lampert, R and Marine, JE and Ware, JS and Rossano, JW and Owens, A and Lin, KY and Ryan, TD and Abrams, DJ and Ingles, J and Gray, B and Moon, JC and Captur, G and Crotti, L and Saberi, S and Helms, AS and Day, SM and Olivotto, I and Bundgaard, H and Lakdawala, NK and Ho, CY},
doi = {10.1016/j.jacc.2026.03.176},
journal = {J Am Coll Cardiol},
pages = {1850--1870},
title = {Natural History of Asymptomatic Phenotypically Mild HCM: Insights From the SHaRe Registry.},
url = {http://dx.doi.org/10.1016/j.jacc.2026.03.176},
volume = {88},
year = {2026}
}

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TY  - JOUR
AB  - BACKGROUND: Patients with phenotypically mild hypertrophic cardiomyopathy (HCM) do not require symptom management, but may be at an earlier stage in the disease course, with potential to benefit from disease-modifying therapies. However, little is known about the natural history and predictors of major adverse cardiovascular events (MACE). OBJECTIVES: Using the Sarcomeric Human Cardiomyopathy Registry, we identified predictors of incident MACE and characterized disease progression in phenotypically mild HCM. METHODS: Phenotypically mild HCM was defined as: having shorter disease duration (<10 years since diagnosis or age ≤30 years), no previous MACE, being NYHA functional class I, and having a left ventricular (LV) maximal wall thickness (MWT) <25 mm. These individuals were followed prospectively for the development of symptoms or MACE: atrial fibrillation (AF), malignant ventricular arrhythmia (MVA) (sudden cardiac death, resuscitated arrest, or appropriate defibrillator therapy), heart failure (HF) (cardiac transplantation, LV assist device implantation, LV ejection fraction <35%, or NYHA functional class III or IV symptoms), stroke, or all-cause mortality. Cox regression identified MACE predictors. Linear and latent class mixed models characterized LV remodeling trajectories and risk clusters. RESULTS: Of 2,500 participants with phenotypically mild HCM (mean age 43 years, 31% women) followed for a mean duration of 7 ± 6 years, 534 (21%) developed MACE, including 289 with AF, 69 with MVA, and 193 with HF. Individuals who progressed from NYHA functional class I to ≥ II symptoms during follow-up (n = 585, 23%) were 2.79 times (95% CI: 2.30-3.39 times) more likely to experience MACE. Age at baseline (HR: 1.24; 95% CI: 1.17-1.32 per 10-year increase), body mass index (HR: 1.10; 95% CI: 1.01-1.21 per 5-kg/m2 increase), left atrial (LA) diameter (HR: 1.16; 95% CI: 1.09-1.25 per 5-mm increase), LV MWT (HR: 1.27; 95% CI: 1.10-1.46 per 5-mm increase)
AU  - Topriceanu,C-C
AU  - Balakrishnan,ID
AU  - Vissing,CR
AU  - Raja,AA
AU  - Parikh,VN
AU  - Zwetsloot,P-P
AU  - Michels,M
AU  - Argiro,A
AU  - Maurizi,N
AU  - Stendahl,JC
AU  - Lampert,R
AU  - Marine,JE
AU  - Ware,JS
AU  - Rossano,JW
AU  - Owens,A
AU  - Lin,KY
AU  - Ryan,TD
AU  - Abrams,DJ
AU  - Ingles,J
AU  - Gray,B
AU  - Moon,JC
AU  - Captur,G
AU  - Crotti,L
AU  - Saberi,S
AU  - Helms,AS
AU  - Day,SM
AU  - Olivotto,I
AU  - Bundgaard,H
AU  - Lakdawala,NK
AU  - Ho,CY
DO  - 10.1016/j.jacc.2026.03.176
EP  - 1870
PY  - 2026///
SP  - 1850
TI  - Natural History of Asymptomatic Phenotypically Mild HCM: Insights From the SHaRe Registry.
T2  - J Am Coll Cardiol
UR  - http://dx.doi.org/10.1016/j.jacc.2026.03.176
UR  - https://www.ncbi.nlm.nih.gov/pubmed/42417692
VL  - 88
ER  - 

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