Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension: an international survey on current practice

Abstract

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6–12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients.

Practice patterns of pulmonary hypertension secondary to left heart disease among pediatric pulmonary hypertension providers

“…In the study by Trammell et al, 77% of adult PH providers consider the use of pulmonary vasodilators in PH‐LHD, strikingly similar to the 79% in this study 10 . The results of this survey and similar ones addressing the use of pulmonary vasodilators in WHO non‐group I PH patients, reflect the lack of evidence based practice within the PH community 21 – 25 . They also reflect the need for larger well designed studies to address these pressing questions.…”

“… 10 The results of this survey and similar ones addressing the use of pulmonary vasodilators in WHO non-group I PH patients, reflect the lack of evidence based practice within the PH community. 21 – 25 They also reflect the need for larger well designed studies to address these pressing questions.…”

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

“…In the group of IPAH, 16% of patients had a positive acute vasoreactivity test at diagnosis, but at the time of enrolment, only half of them were still considered vasoreactive. Other pediatric registries reported positive results of acute vasoreactivity testing ranging from 6% to 34%, but the comparison of different studies is difficult due to the various protocols of the test [4,6,7,12,26,27]. Still, it is important to check the acute pulmonary vasculature response to vasodilators, since as many as half of the patients with a positive test can be successfully treated with calcium channel blockers in the long-term.…”

Comparison of intravenous sildenafil with inhaled nitric oxide for acute vasodilator testing in pulmonary arterial hypertension

“… 24 Though iNO remains the standard of care agent for AVT, there are vast regional variations in the agent of choice even in current era depending on availability, the experience of use and resource limitations and each agent may elicit a different pattern of hemodynamic response. 10 , 25 The ideal agent for AVT should be short‐acting, easy to administer, safe with predictable vasodilator responses, and inexpensive. Sildenafil is the most common pulmonary vasodilator used for the treatment of PAH with a proven safety profile across all age groups.…”