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Spectrum of Current Management of Pediatric Pulmonary Hypertensive Crisis
Meghan L Bernier1, Lewis H Romer, Melania M Bembea1
1Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
Insights
Pediatric pulmonary hypertensive crisis management varies widely in North American PICUs. This survey highlights diverse approaches to care, indicating a need for standardized guidelines for better outcomes.
Area of Science:
- Pediatric Critical Care Medicine
- Cardiology
- Pulmonology
Background:
- Pulmonary hypertension is an increasing concern in pediatric populations.
- Children can experience pulmonary hypertensive crisis, a critical condition requiring immediate medical intervention.
Purpose of the Study:
- To investigate and describe the range of treatments administered in North American Pediatric Intensive Care Units (PICUs) for children experiencing pulmonary hypertensive crisis.
Main Methods:
- A cross-sectional electronic survey was distributed to faculty members in PICUs across the United States and Canada.
- The survey collected data on institutional demographics, patient populations, diagnostic tools, pharmacotherapies, supportive care strategies, and multidisciplinary team structures.
Main Results:
- The survey achieved a 50% response rate from 99 identified institutions, with most respondents being experienced pediatric intensivists.
- Significant variability was observed in diagnostic protocols (e.g., echocardiography, cardiac catheterization indications) and therapeutic interventions, including mechanical ventilation, sedation, and vasoactive agents.
- Inhaled nitric oxide was universally used, with phosphodiesterase type 5 inhibitors being the most common additional vasodilator. Milrinone and epinephrine were frequent vasoactive infusions, while fentanyl and dexmedetomidine were preferred sedatives.
Conclusions:
- Current management of acute pulmonary hypertensive crisis in critically ill children is highly variable across North American PICUs.
- The findings underscore the absence of established North American guidelines and emphasize the need for further research to define best practices, standardize care, and improve patient outcomes.
Abstract:
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.
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