Heart failure & cardiomyopathies
Hypertrophic Cardiomyopathy and Ventricular Preexcitation in the Young: Cause and Accessory Pathway Characteristics
Authors' conclusion
Young patients with HCM and preexcitation have a high likelihood of underlying storage disease or metabolic disease. Nonisolated HCM should be suspected in young patients with large QRS and delta wave amplitudes. Surface ECG is not adequate to discriminate preexcitation from a benign FVF from that secondary to potentially life-threatening AP.
Study context
- Population
- Patients <21 years with hypertrophic cardiomyopathy (HCM) and ventricular preexcitation, 2000–202228 with preexcitation
- Participants
- 345 patients
- Exposure
- Isolated HCM vs Storage disorder vs Metabolic disease vs Genetic syndrome
- Isolated HCMn = 220
- Storage disordern = 17
- Metabolic diseasen = 19
- Genetic syndromen = 89
- Outcome
- Preexcitation by HCM cause
Main finding
As reported in the abstract
Isolated HCM
10
events
Storage disorder
8
events
Metabolic disease
5
events
Genetic syndrome
5
events
- Preexcitation, overall
- 28 (8%)
Observational data: associations, not effects.
Further findings
Clinical AF (preexcitation patients)
- Clinical AF
- 6 (21%)
- Shortest preexcited RR interval <250 ms
- 1
Electrophysiology study findings
- Patients undergoing EP study
- 22
- True accessory pathways (AP) identified
- 23
- Fasciculoventricular fibers (FVF) identified
- 16
- Preexcitation exclusively FVF mediated
- 8 (36%)
- AP with high-risk conduction properties (≥1 patient in each etiologic group)
- 5 (23%)
- Multiple AP
- 8 (36%)
- AP plus FVF
- 10 (45%)
Ablation
- Acutely successful
- 13 of 14
- Recurrence
- 3
Surface ECG features
- Significant differences in QRS amplitude and delta wave amplitude between groups
- No surface ECG features differentiated AP from FVF
Safety
Ablation complication
- Complete heart block after ablation of a high-risk midseptal AP
- 1
Original publication
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