Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

Objectives: Noninvasive ventilation is increasingly used in neonatal and pediatric patients, but the intensive care transport setting is lagging in terms of availability of noninvasive ventilation for children. The objective of this systematic review of the literature was to answer the question: In children 0 days to 18 years old, who are hospitalized with acute respiratory distress and require critical care transport, is noninvasive ventilation effective and safe during transport? Data Sources: MEDLINE via PubMed, EMBASE (via Scopus), Cochrane Central Register of Controlled Trials, African Index Medicus, Web of Science Citation Index, and the World Health Organization Trials Registry. Study Selection: Two reviewers independently reviewed all identified studies for eligibility and quality. Data Extraction: Data were extracted independently by two reviewers using piloted data extraction forms. Data Synthesis: Data were not synthesized due to nature of studies included. Results: A total of 1,287 records were identified; no randomized or quasi-randomized controlled trials were found. Eight observational studies, enrolling 858 patients were included, of these 60.4% were neonatal ICU and 39.6% were nonneonatal ICU patient transports. The mode of noninvasive ventilation during transport was continuous positive airway pressure in seven studies (708 patients) and high-flow nasal cannula in one study (150 patients). During transport on noninvasive ventilation, three of 858 patients (0.4%) required either intubation or escalation of noninvasive ventilation. In the 24 hours following transfer, 63 of 650 children (10%) transferred on noninvasive ventilation were intubated. The odds of intubation within 24 hours were significantly higher for continuous positive airway pressure transfer 60 of 500 (12%) compared with high-flow nasal cannula three of 150 (2%): odds ratio (95% CI) 6.68 (2.40–18.63), p value equals to 0.00003. Minor adverse events occurred in 1–4% of noninvasive ventilation transports. Conclusions: This review found observational study evidence of a rate of intubation or escalation of 0.4% during noninvasive ventilation transport of children 0–18 years old, with an in-transport adverse event rate of 1–4%. Further studies are required. If randomized trials are not possible, it is suggested that well-conducted observational studies are reported in a more standardized manner.

Low FO ≥ 10% prevalence with 28 day mortality 10%. Higher FO% with admission weight associated with mortality (p = 0.04). We advocate further investigation of FO% as a simple bedside tool.