Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy

• Main Authors: Giovanna Gallo, Vittoria Mastromarino, Giuseppe Limongelli, Giulio Calcagni, Antonello Maruotti, Luca Ragni, Fabio Valente, Maria Beatrice Musumeci, Rachele Adorisio, Marta Rubino, Camillo Autore, Damiano Magrì
• Format: Article
• Language: English
• Published: MDPI AG 2021-03-01
• Series: Biomolecules
• Subjects: hypertrophic cardiomyopathy; pediatric; clinical assessment; cardiopulmonary exercise test
• Online Access: https://www.mdpi.com/2218-273X/11/3/376

The usefulness of cardiopulmonary exercise test (CPET) in adult hypertrophic cardiomyopathy (HCM) patients is well-known, whereas its role in pediatric HCM patients has not yet been explored. The present study investigates possible insights from a CPET assessment in a cohort of pediatric HCM outpatients in terms of functional and prognostic assessment. Sixty consecutive pediatric HCM outpatients aged <18 years old were enrolled, each of them undergoing a full clinical assessment including a CPET; a group of 60 healthy subjects served as controls. A unique composite end-point of heart failure (HF) related and sudden cardiac death (SCD) or SCD-equivalent events was also explored. During a median follow-up of 53 months (25th–75th: 13–84 months), a total of 13 HF- and 7 SCD-related first events were collected. Compared to controls, HCM patients showed an impaired functional capacity with most of them showing peak oxygen uptake (pVO₂) values of <80% of the predicted, clearly discrepant with functional New York Heart Association class assessment. The composite end-point occurred more frequently in patients with the worst CPETs’ profiles. At the univariate analysis, pVO₂% was the variable with the strongest association with adverse events at follow-up (C-index = 0.72, <i>p </i>= 0.025) and a cut-off value equal to 60% was the most accurate in identifying those patients at the highest risk. In a pediatric HCM subset, the CPET assessment allows a true functional capacity estimation and it might be helpful in identifying early those patients at high risk of events.