Excerpt
Methods: A cross sectional survey of physicians providing care to children with acute PH crisis was distributed to academic and research institutions beginning in June 2015. Questions focused on pulmonary vasodilator and other vasoactive therapies, sedation, ventilation, and healthcare team composition.
Results: Ninety nine institutions were identified. Response collection is ongoing with a 31% to date. The majority of respondents are pediatric intensivists (79%), with 43% practicing in the Northeast. Care of acute PH crises occurs in large units with 75% having >20 beds. Forty six percent have formal consulting teams of cardiologists (92%), pulmonologists (46%), and intensivists (38%). Peri-operative care, new diagnosis, and infection are the top reported precipitating causes of acute PH crises. Cardiac catheterization is performed most often for new diagnosis and medication titration. All respondents use inhaled nitric oxide to treat PH crises; 72% use phosphodiesterase inhibitors, 68% prostanoids, and 60% endothelin receptor antagonists. Sedation is primarily achieved with fentanyl (96%) and dexmedetomidine (83%) infusions. Milrinone is the most frequently administered circulotrope (96% of respondents). No preferred ventilation strategy exists and steroids and a combination of anticoagulation and antiplatelet therapies are used in one third of responding institutions. Respondents cited pharmacological therapy as the next innovation in patient care.
Conclusions: Heterogeneity exists in the management of critically ill children with acute PH crisis with the largest variations in the composition of teams and details of directed pulmonary vasodilator therapies. Focused discussions are needed to develop best practices for children with acute PH crises.