No specific directions have been published regarding the use of inotropes and vasopressors, a fact that was reflected in our study by a preference for epinephrine, but also wide variability in the use of all agents. 61.1% of institutions with varying components reported. There were no consistent indications SB 258585 HCl 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. strong class=”kwd-title” Keywords: cardiology, intensive care unit, pediatric, pediatrics, pulmonary hypertension, pulmonary medicine, vasodilator agent Despite recent advances in the targeted treatment of pulmonary vascular disease, pulmonary hypertension (PH) remains a progressive and often fatal disease (1, 2). In pediatrics, the burden of disease continues to grow. PH is associated with substantial morbidity and mortality, and the financial cost is significantly higher than that for other chronic illnesses (3C5). The etiologies and pathophysiology of pediatric PH vary from those most often encountered in adult PH patients and are often related to developmental disorders and diseases of the lung (2, 3). Chronic PH may lead to right ventricular (RV) failure as a result of maladaptive mechanisms and ultimately death (6). Some PH patients may present in extremis with pulmonary hypertensive crisisan abrupt and sustained increase in pulmonary vascular resistance with often suprasystemic elevations in pulmonary arterial pressure. These changes result in fulminant RV failure and low cardiac output syndrome with immediate need for emergency intervention (6, 7). Management of acute alterations in pulmonary hemodynamics includes optimization of preload, afterload, and THBS5 contractility with well-integrated adjustments of fluid status, pulmonary vasomotor tone, and circulotropic support of the right ventricle. These priorities are most commonly managed by multidisciplinary teams in ICUs (7, 8). Additional vital adjunctive therapies for the critically ill patient in the ICU with pulmonary hypertensive crisis may include management of sedation, airway, and ventilation with strategies that optimize systemic and pulmonary vascular resistance and cardiopulmonary interactions (7). Historically, in children who developed acute pulmonary hypertensive crisis after congenital heart surgery, the mortality has SB 258585 HCl been found to be as high as 22% and 55% (9, 10). Improvements in overall care and pulmonary vasodilator therapy have reduced the mortality risk, and contemporary single-center studies of patients with PH undergoing noncardiac surgical procedures have reported a mortality rate of ~1% when pulmonary hypertensive crisis occurred postoperatively (11, 12). Treatment of children with PH is directed toward controlling the underlying condition, if identifiable and if possible, and involves therapies that augment pulmonary vasodilatation and SB 258585 HCl reduce vascular remodeling. However, for patients who acutely present with hemodynamic compromise with pulmonary hypertensive crisis, it is paramount to control and stabilize the pulmonary vasculature while maintaining function of SB 258585 HCl other vital organs. Therapeutic options for children are mainly extrapolated from adult trials, as evidence in the pediatric population is limited and largely based on expert opinion (13). Algorithms have been published for the management.