Although rare, hypertrophic cardiomyopathy (HCM) in children, as well as in adults, is associated with the risk of sudden cardiac death (SCD) and heart failure (HF) developing.
While in adults HCM patients major advances have been made in the SCD primary prevention strategies as well in approaches directed to contain/minimize the HF-related consequences, to date no information regarding the risk estimation of SCD nor of HF progression in children are available. Thus, new simple clinical variables, easy-to-obtain and non-invasive, in order to improve the risk evaluation in these patients are highly desirable.
In the last years, several evidences derived from studies performed in adults supports the use of cardiopulmonary exercise testing (CPET) as a useful tool to predict the risk of progression to HF and even of SCD in HCM patients. Although nowadays CPET serves an important role in assessing aerobic exercise capacity in children with different cardiac, pulmonary, metabolic and also oncological disorders, where exercise capacity is closely linked to prognosis, information regarding the practical clinical value of CPET in paediatric HCM is limited.
Our study aims to investigate if a comprehensive functional assessment throughout a CPET analysis, when technically feasible, could supply further clinical insights in a paediatric HCM setting and predict prognosis.
Major advances have been made in the sudden cardiac death (SCD) primary prevention strategies for adult diagnosed with hypertrophic cardiomyopathy (HCM) as well in approaches directed to contain/minimize the heart failure (HF) related consequences [3]. However, to date, we have no information available regarding the risk estimation of the SCD nor of the HF related events in children with HCM. In this subset of patients, an improved risk stratification able to identify patients at risk of worse outcome is highly needed. Thus, new simple clinical variables, easy-to-obtain and non-invasive, are desirable.
In the last years, several evidences from adult studies supports the use of cardiopulmonary exercise testing (CPET) as a useful tool to predict the risk of progression to HF and even of SCD in HCM patients. [1-2] Indeed, most of the HCM pathophysiological features, such as left ventricular (LV) hypertrophy, LV outflow tract obstruction, diastolic dysfunction, myocardial fiber disarray, microvascular ischemia and interstitial fibrosis, are closely implied in reducing the exercise capacity [3], and also in determining the HF development and progression, as well as the arrhythmias propensity.
Although nowadays CPET serves an important role in assessing aerobic exercise capacity in children with different cardiac, pulmonary, metabolic and also oncological disorders [4-6], where exercise capacity is closely linked to prognosis, information regarding the practical clinical value of CPET in paediatric HCM is limited.
Our study aims to investigate if a comprehensive functional assessment throughout a CPET analysis, when technically feasible, could supply further clinical insights in a paediatric HCM setting and predict prognosis.
1. Magrì D, et al. Circ J 2016;80: 2204¿2211.
2. Magrì D, et al. Heart 2016;102:602¿609
3. Patel V, et al. Curr Pharm Des 2015;21(4):466-72.
4. Takken T, et al. Ann Am Thorac Soc. 2017 Jul;14(Supplement_1):S123-S128.
5. Nixon PA, et al. N Engl J Med 1992;327:1785¿1788.
6. van Brussel M, et al. Pediatr Phys Ther. 2011;23(1):2-14.