Spectrum of Current Management of Pediatric Pulmonary Hypertensive Crisis

Pulmonary hypertension is a growing pediatric problem and children may present with pulmonary hypertensive crisis—a life-threatening emergency requiring acute interventions. The aim of this study was to characterize the broad spectrum of care provided in North American PICUs for children who present with pulmonary hypertensive crisis. DESIGN: Electronic cross-sectional survey. Survey questions covered the following: demographics of the respondents, institution, and patient population; pulmonary hypertension diagnostic modalities; pulmonary hypertension-specific pharmacotherapies; supportive therapies, including sedation, ventilation, and inotropic support; and components of multidisciplinary teams. SETTING: PICUs in the United States and Canada. SUBJECTS: Faculty members from surveyed institutions. INTERVENTIONS: None. MEASUREMENT AND MAIN RESULTS: The response rate was 50% of 99 identified institutions. Of the respondents, 82.2% were pediatric intensivists from large units, and 73.9% had over a decade of experience beyond training. Respondents provided care for a median of 10 patients/yr with acute pulmonary hypertensive crisis. Formal echocardiography protocols existed at 61.1% of institutions with varying components reported. There were no consistent indications for cardiac catheterization during a pulmonary hypertensive crisis admission. All institutions used inhaled nitric oxide, and enteral phosphodiesterase type 5 inhibitor was the most frequently used additional targeted vasodilator therapy. Milrinone and epinephrine were the most frequently used vasoactive infusions. Results showed no preferred approach to mechanical ventilation. Fentanyl and dexmedetomidine were the preferred sedative infusions. A formal pulmonary hypertension consulting team was reported at 51.1% of institutions, and the three most common personnel were pediatric cardiologist, pediatric pulmonologist, and advanced practice nurse. CONCLUSIONS: The management of critically ill children with acute pulmonary hypertensive crisis is diverse. Findings from this survey may inform formal recommendations - particularly with regard to care team composition and pulmonary vasodilator therapies - as North American guidelines are currently lacking. Additional work is needed to determine best practice, standardization of practice, and resulting impact on outcomes.