Impact of provocable left ventricular outflow tract obstruction on estimated sudden cardiac death risk in children with hypertrophic cardiomyopathy.

• Applicant: Dr. Anna Michaelis
• Subject Area: Pediatric and Adolescent Medicine
• Term: since 2025
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 556180622

Prediction of sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy is still challenging. In 2019 a novel risk prediction model “HCM Risk- Kids” was published providing the possibility to identify patients at high risk for SCD and thus justifies a prophylactic internal cardiac defibrillator (ICD) implantation. A recent validation multicenter study could show that HCM Risk- Kids could provide an individualized risk stratification for HCM in childhood (1-16 years) with an identification of about 70% of events in patients with a 5- year predicted risk > 6%. However, clinicians have to face the fact that ICD implantation in children causes a high complication rate including lead malfunction, inappropriate shocks, wound infections, endocarditis and psychological effects. Therefore, shared decision making with the families is of great importance but counceling could be very difficult, especially due to the fact of usually initially asymptomatic patients. The resting left ventricular outflow tract gradient seems to be inversely associated with the risk for SCD. Yet, the fact that a higher LVOT- gradient could be protective for SCD seems unlikely and further research on this subject is necessary. Recent studies could show that exercise stress echocardiography could unmask latent LVOT dynamic obstruction during exercise in patients with absence of a resting gradient. Therefor, the current pilot study aims to prove whether the provocative LVOT gradient using exercise stress- echocardiography with a supine bicycle ergometer would change the estimated risk of SCD or an equivalent event by HCM Risk- Kids model.

• DFG Programme: WBP Fellowship
• International Connection: United Kingdom
• Host: Professor Dr. Juan Pablo Kaski