Results: Twenty five studies (3394 patients) met inclusion criteria. We identified four conventional major risk factors which were evaluated in at least 4 studies and found to be statistically associated with an increased risk of death in at least 2 studies: previous adverse cardiac event (pooled hazard ratio 5.4 (95% CI 3.67-7.95), p <0.001 ); non-sustained ventricular tachycardia (pooled hazard ratio 2.13 (95% CI 1.21-3.74), p=0.009); unexplained syncope (pooled hazard ratio 1.89 (95% CI 0.69-5.16), p=0.22);and extreme left ventricular hypertrophy (pooled hazard ratio 1.80 (95% CI 0.75-4.32), p=0.19). Left atrial diameter did not meet the major risk factor criteria, however is likely to be an additional significant risk factor. 'Minor' risk factors included a family history of sudden cardiac death, gender, age, symptoms, ECG changes, abnormal blood pressure response to exercise and left ventricular outflow tract obstruction.
Conclusions:A lack of well-designed, large population based studies in childhood hypertrophic cardiomyopathy means the evidence-base for individual risk factors is not robust. We have identified four clinical parameters which are likely to be associated with increased risk of SCD, SCD-equivalent event or CVD. Multi-centre prospective studies are needed to further determine their relevance in predicting SCD in childhood HCM and to identify novel risk markers.
Condensed abstract:3 A systematic review and meta-analysis of clinical risk factors predicting sudden cardiac death (SCD) in childhood hypertrophic cardiomyopathy (HCM) was performed identifying four 'major' factors: previous adverse cardiac event; non-sustained ventricular tachycardia; syncope and extreme left ventricular hypertrophy. Well-designed multi-centre studies are required in the future to confirm these findings.4