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 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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Insights from cardiopulmonary exercise testing in pediatric patients with hypertrophic cardiomyopathy

European Heart Journal ◽

10.1093/eurheartj/ehab724.1768 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

G Gallo ◽

V Mastromarino ◽

G Limongelli ◽

G Calcagni ◽

L Ragno ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Adverse Events ◽

Exercise Capacity ◽

Univariate Analysis ◽

Control Group ◽

Cardiopulmonary Exercise ◽

End Point ◽

Peak Vo2

Abstract 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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Risk Stratification in Hypertrophic Cardiomyopathy. Insights from Genetic Analysis and Cardiopulmonary Exercise Testing

Journal of Clinical Medicine ◽

10.3390/jcm9061636 ◽

2020 ◽

Vol 9 (6) ◽

pp. 1636

Author(s):

Damiano Magrì ◽

Vittoria Mastromarino ◽

Giovanna Gallo ◽

Elisabetta Zachara ◽

Federica Re ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Risk Stratification ◽

Cardiac Troponin ◽

Troponin I ◽

Troponin T ◽

Cardiopulmonary Exercise Testing ◽

Peak Oxygen Uptake ◽

Variant Of Uncertain Significance ◽

Cardiopulmonary Exercise

The role of genetic testing over the clinical and functional variables, including data from the cardiopulmonary exercise test (CPET), in the hypertrophic cardiomyopathy (HCM) risk stratification remains unclear. A retrospective genotype–phenotype correlation was performed to analyze possible differences between patients with and without likely pathogenic/pathogenic (LP/P) variants. A total of 371 HCM patients were screened at least for the main sarcomeric genes MYBPC3 (myosin binding protein C), MYH7 (β-myosin heavy chain), TNNI3 (cardiac troponin I) and TNNT2 (cardiac troponin T): 203 patients had at least an LP/P variant, 23 patients had a unique variant of uncertain significance (VUS) and 145 did not show any LP/P variant or VUS. During a median 5.4 years follow-up, 51 and 14 patients developed heart failure (HF) and sudden cardiac death (SCD) or SCD-equivalents events, respectively. The LP/P variant was associated with a more aggressive HCM phenotype. However, left atrial diameter (LAd), circulatory power (peak oxygen uptake*peak systolic blood pressure, CP%) and ventilatory efficiency (C-index = 0.839) were the only independent predictors of HF whereas only LAd and CP% were predictors of the SCD end-point (C-index = 0.738). The present study reaffirms the pivotal role of the clinical variables and, particularly of those CPET-derived, in the HCM risk stratification.

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Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study

European Respiratory Journal ◽

10.1183/13993003.01708-2016 ◽

2017 ◽

Vol 50 (1) ◽

pp. 1601708 ◽

Cited By ~ 17

Author(s):

Gabor Kovacs ◽

Alexander Avian ◽

Nora Wutte ◽

Franz Hafner ◽

Florentine Moazedi-Fürst ◽

...

Keyword(s):

Cardiopulmonary Exercise Testing ◽

Peak Oxygen Uptake ◽

Pulmonary Vascular Disease ◽

Significant Deterioration ◽

End Point ◽

Complete Dataset ◽

Systolic Pulmonary Arterial Pressure ◽

Right Heart Catheter ◽

Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.

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1411The effect of eplerenone on fibrosis in hypertrophic cardiomyopathy. A randomised controlled trial assessed using cardiopulmonary exercise testing and cardiovascular magnetic resonance

European Heart Journal ◽

10.1093/eurheartj/ehz748.0059 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

Y J Bhatti ◽

R O'hanlon ◽

T F Ismail ◽

D Pennell ◽

V Vassiliou ◽

...

Keyword(s):

Cardiovascular Magnetic Resonance ◽

Hypertrophic Cardiomyopathy ◽

Controlled Trial ◽

Cardiopulmonary Exercise Testing ◽

Left Ventricular ◽

Exclusion Criteria ◽

Cardiopulmonary Exercise ◽

Effect Of Treatment ◽

Randomised Controlled

Abstract Background Recent studies have implicated aldosterone, a hormone of the renin-angiotensin-aldosterone system to cardiac hypertrophy, and cardiac and vascular fibrosis in cardiomyopathy. High levels of circulating aldosterone have been shown to cause myocardial and aortic fibrosis in animal models. In humans, high aldosterone concentrations have been associated with endothelial dysfunction, myocardial infarction, left ventricular hypertrophy and death. Aldosterone antagonism with spironolactone or eplerenone reverses the process of fibrosis in animal models. Purpose Left ventricular dysfunction due to hypertrophic cardiomyopathy (HCM) is a disorder associated with an increased risk of morbidity and premature mortality. An important pathological feature seen in cardiomyopathy is the development of fibrosis and its association with functional impairment and arrhythmia, including predisposition to sudden cardiac death. This trial observes the effect of eplerenone on myocardial fibrosis and exercise tolerance in patients with HCM. Methods 42 patients were recruited into a double-blinded, placebo-controlled randomised study. Each patient had an established diagnosis of HCM without exclusion criteria (figure 1). 2 patients withdrew before enrolment. Enrolled patients were established on maximally tolerated doses of standard drugs used in the treatment of HCM and the doses of these drugs remained unchanged in the 2 months preceding enrolment to the trial. Patients were randomised to receive a maximal dose of 50mg eplerenone (n=21) or placebo (n=19). The patients underwent cardiopulmonary exercise testing and cardiovascular magnetic resonance (CMR) scan at baseline and 52 week follow up. Results At 52 weeks follow up there was no difference between placebo and treatment groups in peak oxygen consumption (−1.05 vs 0.63, p=0.32), anaerobic threshold (0.4 vs 1.45, p=0.31) or ventilatory efficiency slope (−0.77 vs 1.76, p=0.065). In addition, there was no significant difference observed in LV mass or fibrosis as assessed by CMR. Inclusion and Exclusion Criteria Conclusion In the first randomised controlled trial using eplerenone in humans with HCM, we report no effect of treatment after 52 weeks on exercise capacity compared with placebo. In addition, we report no effect of treatment on the presence of fibrosis detected by CMR. Interpretation of these results are limited by small sample size and further studies will require a multi-centre recruitment process. The data presented here support the feasibility of eplerenone treatment in patients with HCM and provide statistical power calculation for future trials.

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Cardiopulmonary Exercise Test in Patients with Hypertrophic Cardiomyopathy: A Systematic Review and Meta-Analysis

Journal of Clinical Medicine ◽

10.3390/jcm10112312 ◽

2021 ◽

Vol 10 (11) ◽

pp. 2312

Author(s):

Adrián Bayonas-Ruiz ◽

Francisca Muñoz-Franco ◽

Vicente Ferrer ◽

Carlos Pérez-Caballero ◽

María Sabater-Molina ◽

...

Keyword(s):

Systematic Review ◽

Oxygen Consumption ◽

Hypertrophic Cardiomyopathy ◽

Functional Capacity ◽

Exercise Test ◽

Cardiopulmonary Exercise Test ◽

Cycle Ergometer ◽

Left Ventricular ◽

Exercise Intolerance ◽

Cardiopulmonary Exercise

Background: Patients with chronic diseases frequently adapt their lifestyles to their functional limitations. Functional capacity in Hypertrophic Cardiomyopathy (HCM) can be assessed by stress testing. We aim to review and analyze the available data from the literature on the value of Cardiopulmonary Exercise Test (CPET) in HCM. Objective measurements from CPET are used for evaluation of patient response to traditional and new developing therapeutic measurements. Methods: A systematic review of the literature was conducted in PubMed, Web of Science and Cochrane in Mar-20. The original search yielded 2628 results. One hundred and two full texts were read after the first screening, of which, 69 were included for qualitative synthesis. Relevant variables to be included in the review were set and 17 were selected, including comorbidities, body mass index (BMI), cardiac-related symptoms, echocardiographic variables, medications and outcomes. Results: Study sample consisted of 69 research articles, including 11,672 patients (48 ± 14 years old, 65.9%/34.1% men/women). Treadmill was the most common instrument employed (n = 37 studies), followed by upright cycle-ergometer (n = 16 studies). Mean maximal oxygen consumption (VO2max) was 22.3 ± 3.8 mL·kg−1·min−1. The highest average values were observed in supine and upright cycle-ergometer (25.3 ± 6.5 and 24.8 ± 9.1 mL·kg−1·min−1; respectively). Oxygen consumption in the anaerobic threshold (ATVO2) was reported in 18 publications. Left ventricular outflow tract gradient (LVOT) > 30 mmHg was present at baseline in 31.4% of cases. It increased to 49% during exercise. Proportion of abnormal blood pressure response (ABPRE) was higher in severe (>20 mm) vs. mild hypertrophy groups (17.9% vs. 13.6%, p < 0.001). Mean VO2max was not significantly different between severe vs. milder hypertrophy, or for obstructive vs. non-obstructive groups. Occurrence of arrhythmias during functional assessment was higher among younger adults (5.42% vs. 1.69% in older adults, p < 0.001). Twenty-three publications (9145 patients) evaluated the prognostic value of exercise capacity. There were 8.5% total deaths, 6.7% cardiovascular deaths, 3.0% sudden cardiac deaths (SCD), 1.2% heart failure death, 0.6% resuscitated cardiac arrests, 1.1% transplants, 2.6% implantable cardioverter defibrillator (ICD) therapies and 1.2 strokes (mean follow-up: 3.81 ± 2.77 years). VO2max, ATVO2, METs, % of age-gender predicted VO2max, % of age-gender predicted METs, ABPRE and ventricular arrhythmias were significantly associated with major outcomes individually. Mean VO2max was reduced in patients who reached the combined cardiovascular death outcome compared to those who survived (−6.20 mL·kg−1·min−1; CI 95%: −7.95, −4.46; p < 0.01). Conclusions: CPET is a valuable tool and can safely perform for assessment of physical functional capacity in patients with HCM. VO2max is the most common performance measurement evaluated in functional studies, showing higher values in those based on cycle-ergometer compared to treadmill. Subgroup analysis shows that exercise intolerance seems to be more related to age, medication and comorbidities than HCM phenotype itself. Lower VO2max is consistently seen in HCM patients at major cardiovascular risk.

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Could we suspect pulmonary veno-oclussive disease with a cardiopulmonary exercise test?

European Heart Journal ◽

10.1093/ehjci/ehaa946.2251 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

C Perez Olivares Delgado ◽

T Segura De La Cal ◽

A Flox ◽

V Prudencio ◽

M.J Cristo ◽

...

Keyword(s):

Carbon Dioxide ◽

Functional Capacity ◽

Exercise Test ◽

Peak Oxygen Uptake ◽

Cardiopulmonary Exercise Test ◽

Carbon Dioxide Tension ◽

Channel Blockers ◽

Exercise Limitation ◽

Cardiopulmonary Exercise ◽

End Tidal

Abstract Background Cardiopulmonary exercise test (CPET) provides information about the pathophysiology of the exercise limitation. CPET parameters in pulmonary arterial hypertension (PAH) exhibit distinctive features, such as low peak oxygen uptake (pVO2), low end tidal carbon dioxide tension (PetCO2) and high ventilation-carbon dioxide slope (VE/VCO2).Pulmonary veno-oclussive disease (PVOD) is a PAH subgroup with particular underlying physiology, difficult diagnosis and poor prognosis. Purpose We hypothesized that different CPET patterns could be identified in PAH subgroups, and this may contribute to their diagnosis workup. Methods CPETs performed on the following PAH subgroups between September 2019 and January 2020 were prospectively included: Idiopathic PAH (IPAH), Heritable PAH (HPAH), and PAH responders to calcium channel blockers (CCB-R). Due to its low prevalence and its clinical relevance, CPET data on PVOD patients was retrospectively included. Results 57 CPET were included: 20 IPAH (35%); 13 HPAH (22.8%); 9 CCB-R (15.8%),15 PVOD (26.3%). All patients presented impaired functional capacity with reduced peakVO2 and increased VE/VCO2). Remarkably, PVOD patients,in spite of a younger age, exhibit the worst functional capacity and ventilatory efficiency even showing downward PetCO2 kinetics. CCB-R group achieved the highest peakVO2 and had better ventilatory parameters.(Table 1) Conclusion CPET is a useful tool to classify patients by PAH subgroups. Very low PetCO2 at rest and a downward PetCO2 kinetic strongly suggest PVOD, which has significant implications on treatment. Funding Acknowledgement Type of funding source: None

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Assessment of functional capacity with cardiopulmonary exercise testing in non-severe COVID-19 patients at three months follow-up

10.1101/2020.11.15.20231985 ◽

2020 ◽

Author(s):

Piero Clavario ◽

Vincenzo De Marzo ◽

Roberta Lotti ◽

Cristina Barbara ◽

Annalisa Porcile ◽

...

Keyword(s):

Oxygen Uptake ◽

Exercise Testing ◽

Functional Capacity ◽

Cardiopulmonary Exercise Testing ◽

Peak Oxygen Uptake ◽

Maximal Strength ◽

Long Term Effects ◽

Capacity Limitation ◽

Cardiopulmonary Exercise

ABSTRACTIntroductionLong-term effects of Coronavirus Disease of 2019 (COVID-19) and their sustainability in a large number of patients are of the utmost relevance. We aimed to determine: 1)functional capacity of non-severe COVID-19 survivors by cardiopulmonary exercise testing (CPET); 2)those characteristics associated with worse CPET performance.MethodsWe prospectively enrolled the first 150 consecutive subjects with laboratory-confirmed COVID-19 infection discharged alive from March to April 2020 at Azienda Sanitaria Locale (ASL)3, Genoa, Italy. At 3-month from hospital discharge, complete clinical evaluation, trans-thoracic echocardiography, cardiopulmonary exercise testing (CPET), pulmonary function test (PFT), and dominant leg extension (DLE) maximal strength evaluation were performed.ResultsExcluding severe and incomplete/missing cases, 110 patients were analyzed. Median percent predicted peak oxygen uptake (%pVO2) was 90.9(79.2-109.0)%. Thirty-eight(34.5%) patients had %pVO2 below, whereas 72(65.5%) above the 85% predicted value (indicating normality). Median PFT parameters were within normal limits.Eight(21.1%) patients had a mainly respiratory, 9(23.7%) a mainly cardiac, 3(7.9%) a mixed-cardiopulmonary, and 18(47.4%) a non-cardiopulmonary limitation of exercise. Eighty-one(73.6%) patients experimented at least one symptom, without relationship with %pVO2 (p>0.05).Multivariate linear regression analysis showed age (β=0.46, p=0.020), percent weight loss (β=-0.77, p=0.029), active smoke status (β=-7.07, p=0.019), length of hospital stay (β=-0.20, p=0.042), and DLE maximal strength (β=1.65, p=0.039) independently associated with %pVO2.ConclusionsHalf of non-severe COVID-19 survivors show functional capacity limitation mainly explained by muscular impairment, albeit cardiopulmonary causes are possible. These findings call for future research to identify patients at higher risk of long-term effects, that may benefit from careful surveillance and targeted rehabilitation.Take-home messagesat 3-month cardiopulmonary exercise testing 38/110(34.5%) non-severe COVID-19 survivors had percent predicted peak oxygen uptake (%pVO2) < 85% (indicating normality). Half of them had functional capacity limitation mainly explained by muscular impairment.

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Determinants of oxygen uptake and prognostic factors in cardiopulmonary exercise test in patients with Fontan surgery

Cardiology in the Young ◽

10.1017/s1047951121004054 ◽

2021 ◽

pp. 1-4

Author(s):

Maria Martin Talavera ◽

Begoña Manso ◽

Pilar Cejudo Ramos ◽

Maria Jose Rodriguez Puras ◽

Amadeo J Wals Rodriguez ◽

...

Keyword(s):

Oxygen Consumption ◽

Anaerobic Threshold ◽

Functional Capacity ◽

Exercise Test ◽

Univariate Analysis ◽

Clinical Situation ◽

Cardiopulmonary Exercise Test ◽

Vo2 Max ◽

Cardiopulmonary Exercise

Abstract Introduction: Cardiopulmonary exercise test (CPET) allows quantification of functional capacity of patients with Fontan. The objective of this study was to determine the role of CPET parameters in predicting a higher maximum oxygen consumption (VO2 max) and to analyse the role of CPET parameters in predicting an unfavourable outcome. Methods: A retrospective, cross-sectional, descriptive study was carried out on 57 patients with Fontan, who had undergone incremental CPET with cycloergometer between 2010 and 2020. Determinants of VO2 max and determinants of clinical deterioration were analysed. Results: In the univariate analysis, the variables significantly related to VO2 max were: age, sex, body mass index (BMI), years of Fontan evolution, intracardiac Fontan, oxygen consumption at anaerobic threshold (VO2AT), CO2 equivalents at anaerobic threshold (VE/VCO2) and chronotropic insufficiency. The multiple linear regression model that best fitted the relationship between VO2 max and independent variables (correlation coefficient 0.73) included sex (correlation index 3.35; p = 0.02), BMI (−0.27; p = 0.02), chronotropic failure (−2.79; p = 0.01) and VO2AT (0.92; p < 0.0001). In the univariate analysis of the prognostic CPET variables related to an unfavourable clinical situation, significance was only obtained with chronotropic insufficiency (p = 0.003). In multivariate analysis, chronotropic insufficiency maintains its association [p= 0.017, OR = 4.65 (1.3–16.5)]. Conclusions: In conclusion, together with the anthropometric parameters universally related to VO2 max, chronotropic insufficiency and VO2AT are the main determinants of functional capacity in patients with Fontan. Moreover, chronotropic insufficiency is closely related to unfavourable clinical evolution. Our data would support the intensive treatment of chronotropic insufficiency in order to improve the quality of life and the clinical situation of patients with Fontan.

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Do rebreathing manoeuvres for non-invasive measurement of cardiac output during maximum exercise test alter the main cardiopulmonary parameters?

European Journal of Preventive Cardiology ◽

10.1177/2047487319845967 ◽

2019 ◽

Vol 26 (15) ◽

pp. 1616-1622 ◽

Cited By ~ 3

Author(s):

Carlo Vignati ◽

Marco Morosin ◽

Laura Fusini ◽

Beatrice Pezzuto ◽

Emanuele Spadafora ◽

...

Keyword(s):

New York ◽

Cardiac Output ◽

Oxygen Uptake ◽

Anaerobic Threshold ◽

Exercise Testing ◽

Exercise Test ◽

Cardiopulmonary Exercise Testing ◽

Heart Association ◽

Cardiopulmonary Exercise Test ◽

Cardiopulmonary Exercise

Background Inert gas rebreathing has been recently described as an emergent reliable non-invasive method for cardiac output determination during exercise, allowing a relevant improvement of cardiopulmonary exercise test clinical relevance. For cardiac output measurements by inert gas rebreathing, specific respiratory manoeuvres are needed which might affect pivotal cardiopulmonary exercise test parameters, such as exercise tolerance, oxygen uptake and ventilation vs carbon dioxide output (VE/VCO2) relationship slope. Method We retrospectively analysed cardiopulmonary exercise testing of 181 heart failure patients who underwent both cardiopulmonary exercise testing and cardiopulmonary exercise test+cardiac output within two months (average 16 ± 15 days). All patients were in stable clinical conditions (New York Heart Association I–III) and on optimal medical therapy. Results The majority of patients were in New York Heart Association Class I and II (78.8%), with a mean left ventricular ejection fraction of 31 ± 10%. No difference was found between the two tests in oxygen uptake at peak exercise (1101 (interquartile range 870–1418) ml/min at cardiopulmonary exercise test vs 1103 (844–1389) at cardiopulmonary exercise test-cardiac output) and at anaerobic threshold. However, anaerobic threshold and peak heart rate, peak workload (75 (58–101) watts and 64 (42–90), p < 0.01) and carbon dioxide output were significantly higher at cardiopulmonary exercise testing than at cardiopulmonary exercise test+cardiac output, whereas VE/VCO2 slope was higher at cardiopulmonary exercise test+cardiac output (30 (27–35) vs 33 (28–37), p < 0.01). Conclusion The similar anaerobic threshold and peak oxygen uptake in the two tests with a lower peak workload and higher VE/VCO2 slope at cardiopulmonary exercise test+cardiac output suggest a higher respiratory work and consequent demand for respiratory muscle blood flow secondary to the ventilatory manoeuvres. Accordingly, VE/VCO2 slope and peak workload must be evaluated with caution during cardiopulmonary exercise test+cardiac output.

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Feasibility and safety of cardiopulmonary exercise testing in children with pulmonary hypertension

Cardiology in the Young ◽

10.1017/s1047951115001961 ◽

2015 ◽

Vol 26 (6) ◽

pp. 1144-1150 ◽

Cited By ~ 6

Author(s):

Mohammad R. Abumehdi ◽

Andrew J. Wardle ◽

Rewa Nazzal ◽

Athanasios Charalampopoulos ◽

Ingram Schulze-Neick ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Exercise Testing ◽

Cardiopulmonary Exercise Testing ◽

Peak Oxygen Uptake ◽

Cardiopulmonary Exercise Test ◽

World Health ◽

Peak Exercise ◽

Exercise Tests ◽

Cardiopulmonary Exercise ◽

Patient Reported

AbstractBackgroundCardiopulmonary exercise testing helps prognosticate and guide treatment in adults with pulmonary hypertension. Concerns regarding its feasibility and safety limit its use in children with pulmonary hypertension. We aimed to assess the feasibility and safety of cardiopulmonary exercise testing in a large paediatric pulmonary hypertension cohort.MethodsWe reviewed all consecutive cardiopulmonary exercise tests performed between March, 2004 and November, 2013. The exclusion criteria were as follows: height <120 cm, World Health Organization class IV, history of exercise-induced syncope, or significant ischaemia/arrhythmias. Significant events recorded were as follows: patient-reported symptoms, arrhythmias, electrocardiogram abnormalities, and abnormal responses of arterial O2 saturation.ResultsA total of 98 children underwent 167 cardiopulmonary exercise tests. The median age was 14 years (inter-quartile range 10–15 years). Peak oxygen uptake was 20.4±7.3 ml/kg/minute, corresponding to 51.8±18.3% of the predicted value. Peak respiratory quotient was 1.08±0.16. All the tests except two were maximal, being terminated prematurely for clinical reasons. Baseline Oxygen saturation was 93.3±8.8% and was 81.2±19.5% at peak exercise. A drop in arterial O2 saturation >20% was observed in 23.5% of the patients. Moreover, five patients (3.0%) experienced dizziness, one requiring termination of cardiopulmonary exercise testing; five children (3.0%) experienced chest pain, with early cardiopulmonary exercise test termination in one patient. No significant arrhythmias or electrocardiogram changes were observed.ConclusionExercise testing in non-severely symptomatic children with pulmonary hypertension is safe and practical, and can be performed in a large number of children with pulmonary hypertension in a controlled environment with an experienced team. Side-effects were not serious and were resolved promptly with test termination.

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