Safety of Maximal Cardiopulmonary Exercise Testing in Pediatric Patients With Pulmonary Hypertension

Smith, Greg T.; Reyes, Janette T.; Russell, Jennifer; Humpl, Tilman

doi:10.1378/chest.08-1658

Cited by 30 publications

(29 citation statements)

References 13 publications

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“…Additionally, cooperation and motivation for the 6MW test may vary in children, challenging consistency, reproducibility, and interpretability. 20,21 Because CPET is a maximal test in adults and children, and in adult PAH patients PV O 2 correlates with 6MW distance, 22 PV O 2 was chosen for this study.…”

Section: Discussionmentioning

confidence: 99%

A Randomized, Double-Blind, Placebo-Controlled, Dose-Ranging Study of Oral Sildenafil Citrate in Treatment-Naive Children With Pulmonary Arterial Hypertension

et al. 2012

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Background-Safe, effective therapy is needed for pediatric pulmonary arterial hypertension. Methods and Results-Children (nϭ235; weight Ն8 kg) were randomized to low-, medium-, or high-dose sildenafil or placebo orally 3 times daily for 16 weeks in the Sildenafil in Treatment-Naive Children, Aged 1-17 Years, With Pulmonary Arterial Hypertension (STARTS-1) study. The primary comparison was percent change from baseline in peak oxygen consumption (PV O 2 ) for the 3 sildenafil doses combined versus placebo. Exercise testing was performed in 115 children able to exercise reliably; the study was powered for this population. Secondary end points (assessed in all patients) included hemodynamics and functional class. The estimated meanϮSE percent change in PV O 2 for the 3 doses combined versus placebo was 7.7Ϯ4.0% (95% confidence interval, Ϫ0.2% to 15.6%; Pϭ0.056). PV O 2 , functional class, and hemodynamics improved with medium and high doses versus placebo; low-dose sildenafil was ineffective. Most adverse events were mild to moderate in severity. STARTS-1 completers could enter the STARTS-2 extension study; patients who received sildenafil in STARTS-1 continued the same dose, whereas placebo-treated patients were randomized to low-, medium-, or high-dose sildenafil. In STARTS-2 (ongoing), increased mortality was observed with higher doses. Conclusions-Sixteen-week sildenafil monotherapy is well tolerated in pediatric pulmonary arterial hypertension. Percent change in PV O 2 for the 3 sildenafil doses combined was only marginally significant; however, PV O 2 , functional class, and hemodynamic improvements with medium and high doses suggest efficacy with these doses. Combined with STARTS-2 data, the overall profile favors the medium dose. Further investigation is warranted to determine optimal dosing based on age and weight. Clinical Trial Registration-http://www.clinicaltrials.gov. Unique identifier: NCT00159913. (Circulation. 2012;125:324-334.)

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Section: Discussionmentioning

confidence: 99%

A Randomized, Double-Blind, Placebo-Controlled, Dose-Ranging Study of Oral Sildenafil Citrate in Treatment-Naive Children With Pulmonary Arterial Hypertension

et al. 2012

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“…In the largest paediatric PH trial, CPET was used as a primary endpoint, but could only be performed ,50% of the recruited patients because of young age or lack of developmental ability to exercise reliably [31]. Taking this into account, it is important to note that both tests require specific expertise in the paediatric population, both for performing the tests and in analysing the data and can be very helpful in assessing prognosis and evaluating response to therapy [32,33].…”

Section: Exercise Capacity Assessmentsmentioning

confidence: 99%

Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice

Beghetti¹,

Berger

Schulze‐Neick

et al. 2013

Eur Respir J

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Current paediatric pulmonary hypertension (PH) diagnostic algorithms include some testing specifically for paediatrics, but it is unclear if this is used in clinical practice. We describe the current diagnostic workup of the TOPP (Tracking Outcomes and Practice in Paediatric Pulmonary hypertension) registry for suspected PH.We investigated 456 patients enrolled until February 2010. The majority had ECGs (94%), echocardiograms (96%) and/or chest radiographs (89%) performed and these were the noninvasive tests most frequently used for evaluation of suspected PH. No patient had all three tests considered normal, suggesting the potential for the combined use to rule out PH.For evaluation of complications associated with heart catheterisation (HC) we analysed a total of 908 HCs reported until February 2012. Of these, 554 were at diagnosis and 354 in follow-up. Complications were reported in 5.9% with five deaths considered related to HC, suggesting a higher rate of HC complications compared to adult studies. However, current recommendations support HC in paediatric PH. A proper application of the risk/benefit ratio for HC requires further data. Most children did not undergo the diagnostic workup currently recommended for adults, which highlights either incomplete awareness of current guidelines and/or challenges their appropriateness for children. @ERSpublications Most PH children don't undergo full diagnostic evaluations; despite complications, catheterisation is used for diagnosis

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“…12 Within each category, the severity of the responses was graded on a scale of 0 to 3: 0, non-significant; 1, mild; 2, moderate; and 3, severe.…”

Section: Categorization Of Exercise Testsmentioning

confidence: 99%

Incremental Shuttle Walk Test as a Valuable Assessment of Exercise Performance in Patients With Pulmonary Arterial Hypertension

Irisawa

Takeuchi

Inui

et al. 2014

Circ J

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Circulation Journal Official Journal of the Japanese Circulation Society http://www. j-circ.or.jp chievement of reliable results in clinical trials requires an accurate assessment technique. Many clinical trials investigating pulmonary arterial hypertension (PAH) have used the 6-min walk test (6MWT) as a clinical endpoint. Measurement of peak oxygen consumption (pV O2) and oxygen consumption at anaerobic threshold (V O2 at AT) via indirect calorimetry provides an objective, reliable and non-invasive technique to assess exercise tolerance in patients with various cardiac diseases. Furthermore, V O2 at AT describes the highest V O2 that the patient can sustain without developing lactic acidosis, and appears to be an independent marker of PAH severity. 1 In the Sitaxsentan Therapy for Pulmonary Arterial Hypertension (STRIDE-1) study, pV O2 was adopted as the clinical endpoint. 2 Measurement of pV O2, however, requires sophisticated equipment, laboratory facilities, and highly trained personnel. As a result, this test is not widely used and is often confined to large hospitals or research centers. These tests are also expensive and time-consuming, and may not be practical for frequent assessments or large groups of patients. Because of these limitations, field exercise tests such as the submaximal 6MWT have been developed as inexpensive and simple alternative measures of exercise tolerance. 3 The 6MWT measures the distance a patient can voluntarily traverse in a 6-min period. The test protocol, however, is selfpaced, difficult to standardize, and potentially influenced by motivation and encouragement. In addition, the 6MWT has only a moderate correlation with pV O2, 4 and is not a reliable predictor of mortality in patients with congestive heart failure. 5 The development of a simple objective field test that is more highly predictive of pV O2 and easily standardized and used is therefore desirable for patients with PAH.An incremental, externally paced 10-m shuttle walk test (SWT) has been developed for patients with chronic airway limitation. This test is highly reliable and reproducible after only 1 practice walk. 6 In contrast, the 6MWT requires at least 2 practice trials to obtain reliable results. 3 Most importantly, SWT performance relates strongly to pV O2 (r=0.88) in patients with chronic airway limitation, 7 and a strong relationship Background: Nearly all clinical trials investigating patients with pulmonary arterial hypertension (PAH) have used the 6-min walk test (6MWT) to evaluate exercise tolerance. The incremental shuttle walk test (SWT), however, has been proposed as a more valid and reproducible alternative to the 6MWT in the evaluation of exercise tolerance in patients with chronic obstructive pulmonary disease. The efficacy of SWT in clinical practice to evaluate the exercise capacity of patients with PAH was investigated.

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Safety of Maximal Cardiopulmonary Exercise Testing in Pediatric Patients With Pulmonary Hypertension

Cited by 30 publications

References 13 publications

A Randomized, Double-Blind, Placebo-Controlled, Dose-Ranging Study of Oral Sildenafil Citrate in Treatment-Naive Children With Pulmonary Arterial Hypertension

A Randomized, Double-Blind, Placebo-Controlled, Dose-Ranging Study of Oral Sildenafil Citrate in Treatment-Naive Children With Pulmonary Arterial Hypertension

Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice

Incremental Shuttle Walk Test as a Valuable Assessment of Exercise Performance in Patients With Pulmonary Arterial Hypertension

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