Table 3.
Age at diagnosis of HCM | Arrhythmia | Age at first arrhythmia | Cardiac phenotype at presentation | Clinical information | Outcome |
9 | SVT and AF | 14 | Concentric LVH. No LVOTO. No systolic impairment. | Palpitations with SVT on ambulatory ECG. Treated with bisoprolol and diltiazem. At age 18 AF requiring DC cardioversion and therapy change (amiodarone). | Alive. |
15 | AF | 18 | Concentric LVH. No LVOTO. MWT 10 mm. | Fast AF with decompensated heart failure. Treated with diuretics, amiodarone and digoxin increased. At age 19 fast AF requiring DC cardioversion. | Died secondary to heart failure at age 19. |
10 | AET | 11 | Concentric LVH. No LVOTO. | Palpitations with AET on ambulatory ECG. Treated with verapamil. | Alive. |
10 | SVT | 13 | Concentric LVH. No systolic impairment. | Asymptomatic. Ambulatory monitoring showed SVT. Treated with B-blockers. | Alive. |
7 | Atrial flutter | 28 | Concentric LVH. MWT 14 mm. | Unknown clinical presentation. | Transitioned to adult care. |
10 | AF | 19 | Concentric LVH. MWT 12. No LVOTO. No systolic impairment. | Presented with chest pain and palpitations. | Transitioned to adult care. |
11 | Atrial flutter | 13 | Echo showed reduced EF and FS. Concentric LVH. No LVOTO. MWT 11 mm. | Palpitations with atrial flutter in the context of viral illness. Treated with digoxin and DC cardioversion. Maintenance therapy of flecainide, lisinopril and aspirin. | Transitioned to adult care. |
10 | AF with fast ventricular conduction | 17 | Mild concentric LVH. No LVOTO. MWT 13 mm. | Fast AF postoperatively with lactic acidosis. | Died secondary to decompensated heart failure at age 17. |
5 | NSVT | 14 | Presented at age 5 with symptoms suggestive of dilated cardiomyopathy, listed for transplant. Concentric LVH. MWT 12 mm. Systolic impairment. No LVOTO. | Asymptomatic. Treated with amiodarone. | Alive. |
14 | NSVT | 16 | Concentric LVH. MWT 11 mm. No LVOTO. No systolic impairment. | Detected during episode of pancreatitis. | Alive. |
11 | NSVT | 12 | Concentric LVH. No systolic impairment. No LVOTO. MWT 15 mm. | Palpitations with NSVT on ambulatory ECG. Treated with amiodarone. | Died at age 32, unknown cause. |
9 | NSVT | 17 | Concentric LVH. MWT 15 mm. | Asymptomatic. Treated with nifedipine. | Alive. |
AET, atrial ectopic tachycardia; AF, atrial fibrillation; DC, direct current; EF, ejection fraction; FS, fractional shortening; HCM, hypertrophic cardiomyopathy; LVH, left ventricular hypertrophy; LVOTO, left ventricular outflow tract obstruction; MWT, maximal wall thickness; NSVT, non-sustained ventricular tachycardia; SVT, supraventricular tachycardia.