Table 3. Clinical, echocardiographic, and demographic features in 224 children and adolescents with HCM who underwent ICD implantation for primary or secondary prevention.
Primary Prevention | Secondary Prevention | ||||
---|---|---|---|---|---|
Characteristic | All Study Patients | Overall | ≥1 Appropriate Intervention | Overall | ≥1 Appropriate Intervention |
Number of patients | 224 | 188 | 26 | 36§ | 17 |
Age at implantation (yrs) | 14.5 ± 3.6 | 14.7 ± 3.5* | 14.0 ± 2.7† | 12.9 ± 4.4 | 12.9 ± 4.5 |
Age at first ICD intervention (yrs) | 16.4 ± 4.8 | – | 17.0 ± 3.4 | – | 15.6 ± 6.4 |
Male | 151 (67%) | 124 (66%) | 16 (62%)† | 27 (75%) | 12 (71%) |
Follow-Up duration (yrs) | 4.3 ± 3.3 | 4.5 ± 3.3* | 3.0 ± 2.7 | 3.5 ± 3.4 | 2.7 ± 2.7 |
NYHA class at implantation | |||||
I | 159 (71%) | 126 (67%) | 20 (77%)† | 33 (92%) | 16 (94%) |
II | 49 (22%) | 46 (24%)* | 3 (12%)† | 3 (8%) | 1 (6%) |
III/IV | 16 (7%) | 16 (9%)* | 3 (12%)† | 0 | 0 |
NYHA class at last evaluation | |||||
I | 159 (71%) | 133 (71%) | 18 (69%) | 26 (72%) | 9 (53%) |
II | 47 (21%) | 39 (21%) | 6 (23%) | 8 (19%) | 6 (35%) |
III/IV | 18 (8%) | 16 (9%) | 2 (8%) | 2 (6%) | 2 (12%) |
Maximal LV wall thickness (mm) | 25.5 ± 9.8 | 26.0 ± 9.3 | 29.8 ± 11.4‡ | 22.7 ± 11.8 | 21.5 ± 9.4 |
LV end-diastolic dimension (mm) | 40.2 ± 8.8 | 40.5 ± 8.6 | 41.8 ± 15.2† | 38.7 ± 9.7 | 39.5 ± 10.5 |
Left atrial dimension (mm) | 37.8 ± 9.2 | 37.9 ± 9.1 | 37.5±9.8† | 37.6 ± 9.8 | 34.5 ± 9.8 |
LV outflow gradient at rest | |||||
>30 mm Hg | 54 (24%) | 48 (26%) | 10 (38%)† | 6 (17%) | 3 (18%) |
<30 mm Hg | 170 (76%) | 140 (74%) | 16 (62%) | 30 (83%) | 14 (82%) |
Cardioactive medications at implantation | 158 (71%) | 135 (72%) | 21 (81%) | 23 (64%) | 11 (65%) |
Cardioactive medications at first appropriate intervention | 36 (84%) | – | 21 (81%) | – | 15 (88%) |
Ejection fraction <50% | 9 (4%) | 5 (3%) | 1 (4%) | 4 (11%) | 3 (8%) |
Notes.
Values are mean ± SD or n (%).
Significant difference versus secondary prevention (p < 0.01).
Variables unassociated with the likelihood of a primary prevention ICD appropriate intervention (p = 0.054 to 0.83).
Significant difference versus secondary prevention appropriate interventions (p = 0.017).
Includes 10 patients with extreme LV hypertrophy with wall thickness ≥30 mm, of whom 4 had appropriate ICD shocks.
- HCM
- hypertrophic cardiomyopathy
- ICD
- implantable cardioverter-defibrillator
- LV
- left ventricular
- NYHA
- New York Heart Association