Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy
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 outpati...
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doaj-82c25419df584fcdb5cd5ffb2b76f8b92021-03-03T00:06:03ZengMDPI AGBiomolecules2218-273X2021-03-011137637610.3390/biom11030376Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic CardiomyopathyGiovanna Gallo0Vittoria Mastromarino1Giuseppe Limongelli2Giulio Calcagni3Antonello Maruotti4Luca Ragni5Fabio Valente6Maria Beatrice Musumeci7Rachele Adorisio8Marta Rubino9Camillo Autore10Damiano Magrì11Department of Clinical and Molecular Medicine, Sapienza University, 00189 Rome, ItalyPaediatric Cardiology and ACHD Unit, S. Orsola, Malpighi Hospital, 40138 Bologna, ItalyCardiologia SUN, Monaldi Hospital, II University of Naples, 80100 Naples, ItalyDepartment of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital, IRCCS, 00050 Rome, ItalyDepartment of Scienze Economiche, Politiche e delle Lingue Moderne, Libera Università SS Maria Assunta, 00193 Rome, ItalyPaediatric Cardiology and ACHD Unit, S. Orsola, Malpighi Hospital, 40138 Bologna, ItalyCardiologia SUN, Monaldi Hospital, II University of Naples, 80100 Naples, ItalyDepartment of Clinical and Molecular Medicine, Sapienza University, 00189 Rome, ItalyDepartment of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital, IRCCS, 00050 Rome, ItalyCardiologia SUN, Monaldi Hospital, II University of Naples, 80100 Naples, ItalyDepartment of Clinical and Molecular Medicine, Sapienza University, 00189 Rome, ItalyDepartment of Clinical and Molecular Medicine, Sapienza University, 00189 Rome, ItalyThe 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 (pVO<sub>2</sub>) 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, pVO<sub>2</sub>% 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.https://www.mdpi.com/2218-273X/11/3/376hypertrophic cardiomyopathypediatricclinical assessmentcardiopulmonary exercise test |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
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 |
spellingShingle |
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 Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy Biomolecules hypertrophic cardiomyopathy pediatric clinical assessment cardiopulmonary exercise test |
author_facet |
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 |
author_sort |
Giovanna Gallo |
title |
Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy |
title_short |
Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy |
title_full |
Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy |
title_fullStr |
Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy |
title_full_unstemmed |
Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy |
title_sort |
insights from cardiopulmonary exercise testing in pediatric patients with hypertrophic cardiomyopathy |
publisher |
MDPI AG |
series |
Biomolecules |
issn |
2218-273X |
publishDate |
2021-03-01 |
description |
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 (pVO<sub>2</sub>) 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, pVO<sub>2</sub>% 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. |
topic |
hypertrophic cardiomyopathy pediatric clinical assessment cardiopulmonary exercise test |
url |
https://www.mdpi.com/2218-273X/11/3/376 |
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