Sudden cardiac death (SCD) and heart failure (HF) progression represent devastating manifestation and, respectively, an emerging concern in hypertrophic cardiomyopathy (HCM). However, few evidence are available in paediatric HCM patients, likely because of the small sample tested and the questions innate in a paediatric patient evaluation. Thus, studies on techniques, possibly non-invasive, to be used in clinical management of this group are challenging.
In paediatric HCM patients, followed at the HCM Center of Sant'Andrea Hospital and at the Department of Pediatric Cardiology of Bambino Gesù Childrens Hospital, we plan to analyze: a) the autonomic nervous system balance (heart rate variability, time and frequency domain); b) the myocardial repolarization dispersion (spatial and temporal domain); c) the functional capacity (cardiopulmonary exercise test).
Data will be challenged with the known HCM risk factors and their ability in improving the actual risk stratification strategies will be tested.
Each of the above-mentioned techniques (HRV power spectral analysis, spatial/temporal QT variability analysis, CPET assessment) represents an easy-to-obtain and noninvasive approach able to improve the daily clinical management and the risk evaluation in a specific HCM setting where, up to now, no specific risk score tool for estimation of the SCD nor of the HF risk are recognizable.
Both the myocardial repolarization dispersion as well as of the autonomic nervous system balance will be obtained by analyzing ECG data recorded by means of newly developed external devices (Miocardio Srl) which could bypass a number of technical questions innate in the paediatric evaluation.
In conclusion, the present study aims to improve, throughout an easy and noninvasive approach, the daily clinical management and the risk evaluation in a specific HCM setting, namely the paediatric HCM patients, where no specific risk score tool for
estimation of the SCD nor of the HF risk are recognizable. If a novel non-invasive and inexpensive high-yield diagnostic approach will be generated, cost-effectiveness can be achieved resulting in a beneficial economic impact on the SSN.