Hereditary pulmonary arterial hypertension burden in pediatrics: A single referral center experience.

Introduction: Hereditary pulmonary arterial hypertension (HPAH) is a rare yet serious type of pulmonary arterial hypertension (PAH). The burden in the pediatric population remains high yet underreported. The objective of this study is to describe the distribution of mutations found on targeted PAH panel testing at a large pediatric referral center. Methods: Children with PAH panel administered by the John Welsh Cardiovascular Diagnostic Laboratory at Texas Children's Hospital and Baylor College of Medicine in Houston, Texas between October 2012 to August 2021 were included into this study. Medical records were retrospectively reviewed for clinical correlation. Results: Sixty-six children with PAH underwent PAH genetic testing. Among those, 9 (14%) children were found to have pathogenic mutations, 16 (24%) children with variant of unknown significance and 41 (62%) children with polymorphism (classified as likely benign and benign). BMPR2 mutation was the most common pathogenic mutation, seen in 6 of the 9 children with detected mutations. Hemodynamic studies showed higher pulmonary vascular resistance among those with pathogenic mutations than those without (17.4 vs. 4.6 Wood units). All children with pathogenic mutations had severe PAH requiring triple therapy. There were tendencies for higher lung transplantation rate but lower mortality among those with pathogenic mutations. Conclusions: Abnormalities on genetic testing are not uncommon among children with PAH, although majority are of unclear significance. However, children with pathogenic mutations tended to present with more severe PAH requiring aggressive medical and surgical therapies. Genetic testing should be routinely considered due to consequences for treatment and prognostic implications. Larger scale population studies and registries are warranted to characterize the burden of HPAH in the pediatric population specifically.

The Development of Tracheal Intubation Proficiency Outside the Operating Suite During Pediatric Critical Care Medicine Fellowship Training: A Retrospective Cohort Study Using Cumulative Sum Analysis.

OBJECTIVE: Tracheal intubation is a core technical skill for pediatric critical care medicine fellows. Limited data exist to describe current pediatric critical care medicine fellow tracheal intubation skill acquisition through the training. We hypothesized that both overall and first-attempt tracheal intubation success rates by pediatric critical care medicine fellows improve over the course of training. DESIGN: Retrospective cohort study at a single large academic children's hospital. MATERIALS AND METHODS: The National Emergency Airway Registry for Children database and local QI database were merged for all tracheal intubations outside the Operating Suite by pediatric critical care medicine fellows from July 2011 to January 2015. Primary outcomes were tracheal intubation overall success (regardless of number of attempts) and first attempt success. Patient-level covariates were adjusted in multivariate analysis. Learning curves for each fellow were constructed by cumulative sum analysis. RESULTS: A total of 730 tracheal intubation courses performed by 33 fellows were included in the analysis. The unadjusted overall and first attempt success rates were 87% and 80% during the first 3 months of fellowship, respectively, and 95% and 73%, respectively, during the past 3 months of fellowship. Overall success, but not first attempt success, improved during fellowship training (odds ratio for each 3 months, 1.08; 95% CI, 1.01-1.17; p = 0.037) after adjusting for patient-level covariates. Large variance in fellow's tracheal intubation proficiency outside the operating suite was demonstrated with a median number of tracheal intubation equal to 26 (range, 19-54) to achieve a 90% overall success rate. All fellows who completed 3 years of training during the study period achieved an acceptable 90% overall tracheal intubation success rate. CONCLUSIONS: Tracheal intubation overall success improved significantly during the course of fellowship; however, the tracheal intubation first attempt success rates did not. Large variance existed in individual tracheal intubation performance over time. Further investigations on a larger scale across different training programs are necessary to clarify intensity and duration of the training to achieve tracheal intubation procedural competency.

Hemodynamic Bedside Ultrasound Image Quality and Interpretation After Implementation of a Training Curriculum for Pediatric Critical Care Medicine Providers.

OBJECTIVE: Bedside ultrasound for hemodynamic evaluation in critically ill children is increasingly recognized as an important skill for pediatric critical care medicine providers. Our institution implemented a training curriculum leading to institutional credentialing for pediatric critical care providers in nonprocedural bedside ultrasound core applications. We hypothesized that hemodynamic studies performed or supervised by credentialed providers (credentialed providers group) have better image quality and greater accuracy in interpretation than studies performed by non-credentialed providers without supervision (non-credentialed providers group). DESIGN: Retrospective descriptive study. SETTING: Single-center tertiary non-cardiac 55-bed PICU in a children's hospital. PATIENTS: Patients from October 2013 to January 2015, with hemodynamic bedside ultrasound performed and interpreted by pediatric critical care providers exposed to bedside ultrasound training. INTERVENTIONS: A cardiologist blinded to performer scored hemodynamic bedside ultrasound image quality for five core cardiac views (excellent = 3, good = 2, fair = 1, unacceptable = 0; median = quality score) and interpretation within 5 hemodynamic domains (agreement = 3, minor disagreement = 2, major disagreement = 1; median = interpretation score), as well as a global assessment of interpretation. MEASUREMENTS AND MAIN RESULTS: Eighty-one studies (45 in the credentialed providers group and 36 in the non-credentialed providers group) were evaluated. There was no statistically significant difference in quality score between groups (median: 1.4 [interquartile range: 0.8-1.8] vs median: 1.2 [interquartile range: 0.75-1.6]; p = 0.14]. Studies in the credentialed providers group had higher interpretation score than those in the non-credentialed providers group (median: 3 [interquartile range: 2.5-3) vs median: 2.67 [interquartile range: 2.25-3]; p = 0.04). Major disagreement between critical care provider and cardiology review occurred in 25 of 283 hemodynamic domains assessed (8.8%), with no statistically significant difference between credentialed providers and non-credentialed providers groups (6.1% vs 11.9%; p = 0.12). CONCLUSION: Hemodynamic bedside ultrasound performed or supervised by credentialed pediatric critical care providers had more accurate interpretation than studies performed by unsupervised non-credentialed providers. A rigorous pediatric critical care medicine bedside ultrasound credentialing program can train intensivists to attain adequate images and interpret those images appropriately.