Exercise in hypertrophic cardiomyopathy: towards a personalised approach

Wilhelm, Matthias

doi:10.1177/2047487320957792

Cited by 2 publications

(4 citation statements)

References 24 publications

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“…In such a context, a maximal CPET might be useful to grade objectively the exercise impairment and, possibly, to follow-up their clinical course and to guide therapeutic options. Furthermore, throughout this safe and noninvasive approach, it could be possible to establish safe exercise training programs, the latter being an emerging issue since that the most of the HCM patients have important reservation about physical activity [ 18 , 19 , 32 , 33 ]. Last, a CPET assessment possibly combined with a contextual echocardiographic Doppler analysis might improve the physicians’ comprehension of the complex mechanisms underlying an impaired pVO 2 in HCM patients [ 17 ].…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, growing evidence suggests that a full CPET assessment, in combination with other clinical and instrumental variables, is able to stratify both the SCD and the HF risk in adult cohort of HCM patients [ 10 , 11 , 12 , 13 , 14 , 15 ]. Furthermore, regardless its potential prognostic value in pediatric HCM patients, it is likely that a systematic CPET assessment might be helpful in this setting to disclose an unsuspected functional limitation [ 16 , 17 ], to develop individualized exercise training programs to prevent deconditioning [ 18 , 19 ], and, last but not least, to instill confidence in young HCM patients and their relatives through a maximal exercise test performed without side effects [ 20 , 21 ].…”

Section: Introductionmentioning

confidence: 99%

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Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy

et al. 2021

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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, p = 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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy

et al. 2021

View full text Add to dashboard Cite

show abstract

“…In such a context, a maximal CPET might be useful to grade objectively the exercise impairment and, possibly, to follow-up their clinical course and to guide therapeutic options. Furthermore, throughout this safe and noninvasive approach, it could be possible to establish safe exercise training programs, the latter being an emerging issue since that the most of the HCM patients have important reservation about physical activity [18,19,32,33]. Last, a CPET assessment possibly combined with a contextual echocardiographic Doppler analysis might improve the physicians' comprehension of the complex mechanisms underlying an impaired pVO 2 in HCM patients [17].…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, growing evidence suggests that a full CPET assessment, in combination with other clinical and instrumental variables, is able to stratify both the SCD and the HF risk in adult cohort of HCM patients [10][11][12][13][14][15]. Furthermore, regardless its potential prognostic value in pediatric HCM patients, it is likely that a systematic CPET assessment might be helpful in this setting to disclose an unsuspected functional limitation [16,17], to develop individualized exercise training programs to prevent deconditioning [18,19], and, last but not least, to instill confidence in young HCM patients and their relatives through a maximal exercise test performed without side effects [20,21].…”

Section: Introductionmentioning

confidence: 99%

Insights from cardiopulmonary exercise testing in pediatric patients with hypertrophic cardiomyopathy

Gallo

Mastromarino

Limongelli

et al. 2021

European Heart Journal

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Background Hypertrophic cardiomyopathy (HCM) is characterized by extremely varied phenotypic expression ranging from asymptomatic to heart failure (HF) to sudden cardiac death (SCD). Although children with HCM are considered in the highest risk spectrum, the most common recommendations on pharmacological and non-pharmacological treatment (i.e. drugs, ICD, septal reduction procedures, inclusion in cardiac transplantation list) are often disregarded or too much postponed in this setting and strong evidence-based risk prediction models are missing. A systematic cardiopulmonary exercise test (CPET) assessment might be helpful to disclose an unsuspected functional limitation. Purpose The aim of our multicenter retrospective study was to investigate possible clinical insights, in terms of functional and prognostic assessment, coming from a full CPET assessment in a cohort of pediatric HCM outpatients aged less than 18 years old. Methods 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. An unique composite end-point of HF-related and SCD or SCD-equivalent events was also explored. During a median follow-up of 53 months, a total of 13 HF- and 7 SCD-related first events were collected. Results An impaired exercise capacity, consisting on peak VO2 values <80% of the predicted, has been found in the 78% of the study sample (n. 47 patients). Despite most of the HCM patients were classified in NYHA I functional class, most of them (n. 33, 73%) showed a reduced exercise capacity, the percentage of impaired exercise capacity raising in the NYHA II group (n. 14 patients, 93%). Respect to the control Group, the HCM patients showed a significantly poorer functional status in terms of maximum workload achieved, peak VO2 (regardless the adopted correction), circulatory power and VE/VCO2 slope values (Figure 1, panel A). HCM patients who experience adverse events during the follow-up (Event Group) showed the worst CPET profile (Figure 1, panel B). The composite end-point occurred more frequently in patients with the worst CPETs' profiles. At the univariate analysis, peak VO2% was the variable with the strongest association with adverse events at follow-up (C-index=0.72, p=0.025) and a cut-off value equal to 60% was the most accurate in identifying those patients at the highest risk (Figure 2). Conclusions Our findings support the role of CPET analysis as an insightful approach in the young HCM clinical management. In a group of young asymptomatic or slightly symptomatic HCM patients, the CPET allowed us to estimate accurately their functional capacity and to disclose a portion of un-recognized exercise impairment. Our data argue in favor of a possible role of some CPET-derived variables in the early identification of those young HCM patients at highest risk of HCM related events. FUNDunding Acknowledgement Type of funding sources: None. Figure 1 Figure 2

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Exercise in hypertrophic cardiomyopathy: towards a personalised approach

Cited by 2 publications

References 24 publications

Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy

Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy

Insights from cardiopulmonary exercise testing in pediatric patients with hypertrophic cardiomyopathy

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