Introduction
Material
Study Patients
Data Collection
Events Data
Statistics
Results
Baseline Characteristics
Clinical parameters | Study group n = 112 patients |
---|---|
Age at clinical evaluation | |
Cohort median, years (IQR) | 14.1 (7.8–16.6) |
Age ≤ 1 | 3 (2.7) |
Age > 1 and ≤5 | 8 (7.1) |
Age > 5 and ≤10 | 25 (22.3) |
Age > 10 and <18 | 76 (67.9) |
Age at diagnosis HCM | |
Cohort median, years (IQR) | 4.83 (0.54–11) |
Age ≤ 1 | 38 (33.9) |
Age > 1 and ≤5 | 21 (18.8) |
Age > 5 and ≤10 | 21 (18.8) |
Age > 10 and <18 | 32 (28.5) |
Male | 67 (60) |
Family history | |
HCM | 48 (42.9) |
SCD | 25 (22.3) |
Presenting sign/symptom | |
Murmur | 107 (95.5) |
Chest pain | 34 (30.4) |
Syncope | 17 (15.2) |
Dyspnoea on exertion | 24 (21.4) |
Resuscitated sudden death | 3 (2.7) |
Palpitations | 22 (19.6) |
Heart failure (NYHA III–IV) | 20 (17.9) |
Follow-up, years | |
Median (IQR) | 6.5 (2.9–9.6) |
Death | 13 (11.6) |
Heart failure death | 7 (6.2) |
SCD | 6 (5.4) |
ICD appropriate discharge | 5 (4.5) |
Resuscitated cardiac arrest | 2 (1.8) |
NSVT | 17 (15.2) |
Heart transplant | 3 (2.7) |
Echocardiographic data at last follow-up | |
Left ventricular morphology | |
Asymmetric septal hypertrophy | 78 (69.6) |
Concentric LV hypertrophy | 31 (27.7) |
Apical LV hypertrophy | 3 (2.7) |
Septal wall, mm | 15.3 (11–21.2) |
Septal wall, z-score | 7.8 (4.9–15.1) |
Posterior wall, mm | 8.7 (7.3–11.5) |
Posterior wall, z-score | 1.6 (0.07–3.5) |
LA size, mm | 34.3 (26.4–45) |
LA size, z-score | 1.7 (0.6–5.1) |
LVOTO ≥ 30 mmHg | 28 (25) |
Drug treatment | |
Beta-blockers | 101 (89.3) |
Calcium-blockers | 11 (9.8) |
Antiarrhythmics (sotalol or amiodarone) | 9 (8.0) |
ACE inhibitors | 9 (8.0) |
Diuretics (furosemide or spironolactone) | 34 (30.3) |
Myectomy | 8 (7.1) |
ICD implantation | 21 (18.8) |
Primary prevention | 15 (13.4) |
Secondary prevention | 6 (5.4) |
Outcomes
Risk Factors for Primary and Secondary Outcomes
Risk factor (overall % of patients) | Number of observations | Patients without primary end point (n = 89) | Patients with primary end point (n = 23) |
p value | Cutoff point value | RR (95 % CI) |
---|---|---|---|---|---|---|
Family history of SCD (22 %) | 25/112 | 20 | 5 | 0.84 | – | 0.967 (0.399–2.343) |
Prior cardiac arrest (3 %) | 3/112 | 0 | 3 |
0.004
| – |
5.450 (3.668–8.098) |
Syncope (15 %) | 17/112 | 14 | 3 | 0.99 | – | 0.838 (0.280–2.514) |
NSVT (15 %) | 17/112 | 11 | 6 | 0.19 | – | 1.972 (0.909–4.279) |
Extreme LVH ≥ 30 mm (8.9 %) | 10/112 | 8 | 2 | 0.71 | – | 0.971 (0.266–3.553) |
ABPR to exercise (31 %) | 22/70 | 17/62 | 5/8 | 0.11 | No cutoff | 0.439 (0.224–0.859) |
LVOTO ≥ 30 mmHg (25 %) | 28/112 | 20 | 8 | 0.34 | No cutoff | 1.600 (0.760–3.367) |
LVOT gradient, mmHg | 112 | 6.8 (5–19) | 10.9 (7–31.4) |
0.04
| No cutoff | – |
Age at diagnosis, years | 112 | 5 (0.6–12) | 4 (0.2–10) | 0.48 | No cutoff | – |
SWT, mm | 112 | 14.9 (11.1–21.2) | 16.5 (10.3–20) | 0.94 | 30 | 1.922 (0.292–12.657) |
SWT, z-score | 112 | 7.6 (4.7–14.9) | 11.3 (5.6–16.8) | 0.37 | 7 | 0.856 (0.410–1.788) |
LVPWT, mm | 112 | 8.6 (7.2–11.3) | 9.6 (7.4–11.8) | 0.39 | 10 | 1.315 (0.636–2.719 |
LVPWT, z-score | 1.4 (0.1–3.2) | 3 (0.1–6.3) | 0.068 |
4
| 2.488 (1.234–5.013) | |
SWT of >20, mm (26.8 %) | 30/112 | 25 | 5 | 0.54 | – | 0.759 (0.309–1.864) |
SWT expressed as percent of 95th centile value for age | 112 | 146.9 (116.7–219.1) | 181.6 (125.8–222.2) | 0.72 | 195 | 0.680 (0.330–1.402) |
SWT > 190 % of upper normal limit for age (38.4 %) | 43/112 | 32 | 11 | 0.3 | – | 1.471 (0.713–3.034) |
QTc dispersion | 112 | 0.04 (0.04–0.06) | 0.06 (0.04–0.06) |
0.0042
|
0.055
|
3.153 (1.506–6.603) |
QTc | 112 | 0.41 (0.38–0.43) | 0.42 (0.39–0.45) | 0.27 | 0.415 | 1.214 (0.586–2.518) |
Heart failure at presentation (17.9 %) | 20/112 | 10 | 10 |
0.0003
| – |
3.538 (1.815–6.899) |
LA size, mm | 112 | 34 (25.7–44.4) | 39 (33–47) | 0.1 | 38 | 2.496 (1.184–5.262) |
LA size, z-score | 1.57 (0.34–3.82) | 4.8 (2.2–8.2) |
0.001
|
3
|
3.125 (1.450–6.736) | |
LVPWT-to-cavity ratio | 112 | 0.22 (0.18–0.29) | 0.26 (0.18–0.37) | 0.15 | 0.6 | 2.650 (1.086–6.468) |
LVPWT-to-cavity ratio >0.3 (26 %) | 29/112 | 19 | 10 |
0.03
| – |
2.202 (1.085–4.468) |
E/A, cm/s | 101/112 | 1.56 (1.25–1.9) | 1.39 (1.03–1.55) | 0.24 | 3 | 1.516 (0.262–8.770) |
E/A, z-score | −0.72 (−1.19) to (−0.27) | −1 (−1.57) to (−0.78) | 0.16 | 1 | 1.753 (0.596–5.155) | |
E vel, cm/s | 101/112 | 85.2 (73.8–97) | 63 (46–91) |
0.04
| 70 |
0.220 (0.090–0.538) |
E vel, z-score | −0.5 (−1) to (0.31) | −1.94 (−2.4) to (−0.25) |
0.027
| −1
|
0.265 (0.107–0.653) |