Heart failure & cardiomyopathiesCohort study
The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis
- Retrospective cohort study
- multiregistry (SHaRe and IPHCC)
Authors' conclusion
In pediatric HCM, massive LVH disproportionately affects those diagnosed in early childhood with sarcomeric disease, with increased risk for adverse events. Significant MLVWT regression is seen in nearly a quarter of patients.
Study context
- Population
- Patients with pediatric-onset hypertrophic cardiomyopathy (HCM) from 2 registries (SHaRe, IPHCC), encounters 1960/1970 through March 202454 female [30%]
- Participants
- 587 patients
- Exposure
- Massive left ventricular hypertrophy (LVH) vs no massive LVH
- Massive LVHn = 186
Main finding
As reported in the abstract
Composite outcomes, massive vs no massive LVH
Massive LVH vs No massive LVHEstimateP value
HCM-related mortality
unadjusted HR 3.3(95% CI 1.2–9.7)
P = 0.026major adverse cardiac events
HR 2.6(CI 1.7–3.9)
P < 0.001major ventricular arrhythmia
HR 3.1(CI 1.8–5.2)
P < 0.001HF
HR 1.9(CI 1.1–3.1)
P = 0.013Observational data: associations, not effects.
Further findings
Sarcomeric genetic variants
P = 0.034
| Outcome | Massive LVH | No massive LVH | P value |
|---|---|---|---|
| Age at diagnosis | median 9.2 (IQR 2.1–13.1) years | 13.6 (IQR 9.7–15.5) years | P < 0.001 |
Serial maximal left ventricular wall thickness (MLVWT) in massive LVH (n=115 with serial data, 62%), first vs last measurement
- Absolute MLVWT: median 26 mm [IQR 18-32 mm] vs 31 mm [26-35 mm]
- P < 0.001
- MLVWT z score: median +22 [IQR +18 to +26] vs +23 [+20 to +28]
- P = 0.25
- Last absolute MLVWT >5 mm less than largest recorded MLVWT
- 25 patients (22%)
Original publication
The Natural History of Massive Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: A Multiregistry Analysis. Circulation. Published 17 April 2026. doi:10.1161/circulationaha.126.078843
Summarised from the published abstract; every number is checked against it