Table 1.
Feature | (%) Nc or mean ± SD |
---|---|
Gender | |
Male | 68 % (130/192) |
Female | 32 % (62/192) |
Age at diagnosis (in years) | 36.8 ± 16.80 |
Median (range) | 38 (2–70) |
≤40 years | 53.1 % (102/192) |
>40 years | 45.8 % (88/192) |
Family history | 40.6 % (78/192) |
Of diagnosed HCM | 31.2 % (60/192) |
Of sudden death | 9.4 % (18/192) |
+vea for sarcomeric mutation/+ve family history | 62.8 % (49/78) |
Clinical presentation | |
Shortness of breath (NYHA) | |
I | 22.6 % (51/192) |
II | 44.3 % (85/192) |
III | 22.9 % (44/192) |
IV | 6.2 % (12/192) |
Chest pain | 65.6 % (126/192) |
Palpitation | 66.1 % (127/192) |
Syncope/presyncope | 52.6 % (101/192) |
Baseline echocardiographic parameters | |
Left atrium (mm) | 44.7 ± 9.3 |
LV outflow gradient ≥ 30 mmHg | 56.7 % (103/185) |
Max. LV thickness (mm) | 25.3 ± 19.7 |
<30 mm | 80.9 % (149/184) |
≥30 mm | 19.0 % (35/184) |
Symmetrical hypertrophy | 1.1 % (2/185) |
Asymmetrical hypertrophy | 98.9 % (183/185) |
Site of maximum thickness: | |
Isolated septal hypertrophy | 56.2 % (104/185) |
Septal and posterior wall hypertrophy | 28.7 % (53/185) |
Apical hypertrophy | 2.7 % (5/185) |
LV end-diastolic dimension (mm) | 44.2 ± 8.3 |
LV end-systolic dimension (mm) | 26.6 ± 6.5 |
Mitral regurgitation (MR): | 68 % (125/185) |
Trivial/mild MR | 47 % (87/185) |
Moderate MR | 14 % (26/185) |
Severe MR | 7 % (12/185) |
SAM | 62 % (114/185) |
Major cardiac eventsb | |
Sudden cardiac death | 2 % (2/100) |
Previous cardiac arrest | 2 % (2/100) |
Heart failure | 3 % (3/100) |
Stroke | 1 % (2/100) |
Interventions for obstruction/symptoms | |
Alcohol septal ablation | 1 % (2/192) |
Surgical septal myectomy | 27 % (52/192) |
ICD | 1 % (2/192) |
Pacemaker | 3 % (5/192) |
ICD implantable cardioverter-defibrillator, LV left ventricular, NYHA New York Heart Association, SAM systolic anterior motion
a+ve: positive
bTotal number includes cases with available follow-up data
cTotal number varies according to available clinical data of parameters assessed