Background
Methods
Participants
Echocardiography
Clinical outcomes
Statistical analysis
Results
Demographic and clinical characteristics
Variables | n = 161 |
---|---|
Age (year) | 66 ± 16 |
Age stratified by decade (years) [n (%)] | |
– < 20 – 20-29 – 30-39 – 40-49 – 50-59 – 60-69 – 70-79 – 80-89 – ≥90 | 0 (0%) 4 (3%) 5 (3%) 19 (12%) 21 (13%) 36 (22%) 41 (25%) 32 (20%) 3 (2%) |
Age at presentation (year) | 58 ± 17 |
Female [n (%)] | 93 (58%) |
Family history of HCM/ SCD [n (%)] | 17 (12%)/13 (9%) |
History of syncope [n (%)] | 8 (5%) |
NYHA Class III-IV [n (%)] | 21 (13%) |
Systolic blood pressure (mmHg) | 130 ± 18 |
Diastolic blood pressure (mmHg) | 75 ± 12 |
Atrial fibrillation at presentation [n (%)] | 12 (8%) |
- Paroxysmal - Persistent/permanent | 4 (3%) 8 (5%) |
LVH on ECG [n (%)] | 92 (57%) |
Clinical presentation [n (%)] | |
Asymptomatic with abnormal murmur | 20 (12%) |
Asymptomatic with abnormal EKG | 27 (17%) |
Dyspnea on exertion | 40 (25%) |
Palpitation | 11 (7%) |
Chest pain | 33 (21%) |
Syncope | 8 (5%) |
Heart failure | 15 (9%) |
Stroke | 7 (4%) |
Functional capacity in Metabolic Equivalents (METS) | 7.9 ± 1.7 |
Hypotensive response to exercise [n (%)] | 2 (1%) |
Non-sustained ventricular tachycardia [n (%)] | 2 (1%) |
5-year ESC-SCD risk score | 2.1 ± 0.6% |
Type [n (%)] | |
– Asymmetrical septal hypertrophy – Pure apical – Apical with mid LV involvement – Concentric – Localized – Pure Mid | 81 (50%) 27 (17%) 24 (14%) 26 (16%) 2 (2%) 1 (1%) |
Interventricular septal thickness (mm) | 18 ± 6 |
Interventricular septal thickness ≥ 25 mm [n (%)] | 24 (15%) |
Interventricular septal thickness ≥ 30 mm [n (%)] | 9 (5%) |
Abnormal papillary muscle [n (%)] | 36 (22%) |
Resting systolic anterior motion [n (%)] | 77 (48%) |
Chordal Mild Moderate Severe | 36 23 12 6 |
Significant LVOT gradient (> 30 mmHg) [n (%)] | 38 (24%) |
LVEDD (mm) | 43 ± 8 |
LA diameter (mm) | 40 ± 9 |
LAVI (ml/m2) | 37 ± 18 |
LVEF (%) | 74 ± 10 |
Dilated HCM (LVEF< 50%) [n (%)] | 6 (4%) |
Beta blocker [n (%)] | 119 (74%) |
Calcium channel blocker [n (%)] | 19 (12%) |
Amiodarone [n (%)] | 14 (9%) |
Anticoagulant [n (%)] | 40 (25%) |
Cardiac implantable electronic device [n (%)] | |
– ICD – CRT-D – Pacemaker | 5 (3%) 0 (0%) 8 (5%) |
HCM –related deaths
Age distribution of the HCM-related deaths
Relationships between the HCM-related deaths and clinical characteristics
Major adverse cardiovascular events (MACEs)
SCD and nonfatal ventricular arrhythmia
Fatal and nonfatal heart failure
Fatal and nonfatal embolic stroke
Variables | HCM-Related Death | MACEs | ||||
---|---|---|---|---|---|---|
Univariate Analysis | Univariate Analysis | Multivariate Analysis | ||||
Hazard Ratio | p Value | Hazard Ratio | p Value | Hazard Ratio | p Value | |
Age ≥ 65 years | 0.78 | 0.66 | 2.01 | 0.01* | 1.66 | 0.09 |
Female | 1.24 | 0.65 | 2.33 | < 0.01* | 1.66 | 0.11 |
BSA ≤ 1.5 m2 | 2.03 | 0.11 | 1.97 | 0.01* | 1.70 | 0.08 |
NYHA III-IV | 1.36 | 0.58 | 3.00 | < 0.01* | 1.27 | 0.55 |
Heart rate > 90/min | 2.56 | 0.07 | 3.05 | < 0.01* | 2.18 | 0.04** |
Family History of SCD | 0.88 | 0.73 | 1.03 | 0.94 | ||
AF on ECG | 1.83 | 0.28 | 2.05 | 0.05 | 2.81 | 0.01** |
Heart failure at presentation | 1.32 | 0.59 | 3.44 | < 0.01* | 2.93 | < 0.01** |
Maximal septal thickness ≥ 25 mm | 1.15 | 0.80 | 0.97 | 0.94 | ||
LVEF < 60% | 0.60 | 0.36 | 1.75 | 0.12 | ||
Septal reductive therapy | 0.58 | 0.59 | 1.12 | 0.65 |