Heart failure & cardiomyopathiesCohort study
Phenotype, genotype and prognosis of apical hypertrophic cardiomyopathies: a French multicentric cohort
- French multicentric cohort
- consecutive patients
- 5 French centres
Authors' conclusion
ApHCM presents with less frequent mutations and better prognosis than non-ApHCM. However, rhythmic complications are frequent in ApHCM but are not predicted by the SCD-risk score. Apical aneurysms are more frequent in apical HCM. Fabry’s disease may mimic an apical HCM phenotype and should be ruled out when facing an apical HCM pattern.
Study context
- Population
- Patients with a phenotype of apical hypertrophic cardiomyopathy (apical hypertrophic cardiomyopathies (ApHCM)), compared with patients with non-apical HCMconsecutive ApHCM patients, 7 excluded (Fabry's disease n = 6, amyloidosis n = 1), leaving 201 ApHCM for comparative analyses, compared with 419 non-apical HCM · ApHCM 208 · non-apical HCM 419
- Participants
- 208 patients
- Exposure
- ApHCM vs non-apical HCM
- ApHCMn = 201
- non-apical HCMn = 419
Main finding
As reported in the abstract
ApHCM
22.6%
- including GLA mutations
- 6
- including TTR mutations
- 1
Observational data: associations, not effects.
Further findings
LV obstruction · less with ApHCM
P < 0.001
LV aneurysm · more frequent with ApHCM
P < 0.001
Mutations · less frequent with ApHCM
P < 0.001
Left atrial volume · smaller with ApHCM
P < 0.001
| Outcome | ApHCM | non-apical HCM | P value |
|---|---|---|---|
| GLS · more impaired with ApHCM | −14.4 ± 4 % | −15.3 ± 4 % | P = 0.019 |
| Long-term survival · better in ApHCM | P = 0.026 |
- Rhythmic events in ApHCM · 5-year follow-up
- 6% · 12 of 201
LVEF
- Higher LVEF in ApHCM
Rhythmic events in ApHCM · 5-year follow-up
- All of them had a SCD-risk score <4%
Original publication
Summarised from the published abstract; every number is checked against it