title: Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Pulmonary Hypertension.
abstract: OBJECTIVE
  To review the pharmacologic treatment options for pulmonary arterial hypertension in the cardiac intensive care setting and summarize the most-recent literature supporting these therapies.
  
  
  METHODS
  Literature search for prospective studies, retrospective analyses, and case reports evaluating the safety and efficacy of pulmonary arterial hypertension therapies.
  
  
  METHODS
  Mechanisms of action and pharmacokinetics, treatment recommendations, safety considerations, and outcomes for specific medical therapies.
  
  
  RESULTS
  Specific targeted therapies developed for the treatment of adult patients with pulmonary arterial hypertension have been applied for the benefit of children with pulmonary arterial hypertension. With the exception of inhaled nitric oxide, there are no pulmonary arterial hypertension medications approved for children in the United States by the Food and Drug Administration. Unfortunately, data on treatment strategies in children with pulmonary arterial hypertension are limited by the small number of randomized controlled clinical trials evaluating the safety and efficacy of specific treatments. The treatment options for pulmonary arterial hypertension in children focus on endothelial-based pathways. Calcium channel blockers are recommended for use in a very small, select group of children who are responsive to vasoreactivity testing at cardiac catheterization. Phosphodiesterase type 5 inhibitor therapy is the most-commonly recommended oral treatment option in children with pulmonary arterial hypertension. Prostacyclins provide adjunctive therapy for the treatment of pulmonary arterial hypertension as infusions (IV and subcutaneous) and inhalation agents. Inhaled nitric oxide is the first-line vasodilator therapy in persistent pulmonary hypertension of the newborn and is commonly used in the treatment of pulmonary arterial hypertension in the ICU. Endothelin receptor antagonists have been shown to improve exercise tolerance and survival in adult patients with pulmonary arterial hypertension. Soluble guanylate cyclase stimulators are the first drug class to be Food and Drug Administration approved for the treatment of chronic thromboembolic pulmonary hypertension.
  
  
  CONCLUSIONS
  Literature and data supporting the safe and effective use of pulmonary arterial hypertension therapies in children in the cardiac intensive care are limited. Extrapolation of adult data has afforded safe medical treatment of pulmonary hypertension in children. Large multicenter trials are needed in the search for safe and effective therapy of pulmonary hypertension in children.
authors.affiliation: 1Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO. 2Department of Cardiology, Harvard Medical School, Children's Hospital Boston, Boston, MA. 3Departments of Pharmacy and Pediatrics (Cardiology), Stanford University, Lucile Packard Children's Hospital, Palo Alto, CA.
authors.forename: John S
authors.initials: JS
authors.lastname: Kim
authors.forename: Julia
authors.initials: J
authors.lastname: McSweeney
authors.forename: Joanne
authors.initials: J
authors.lastname: Lee
authors.forename: Dunbar
authors.initials: D
authors.lastname: Ivy
mesh_terms: D000328:Adult; D002121:Calcium Channel Blockers / Q000008:administration & dosage / Q000009:adverse effects; D002317:Cardiovascular Agents / Q000627:therapeutic use*; D002648:Child; D003325:Coronary Care Units; D003422:Critical Care / Q000592:standards*; D065128:Endothelin Receptor Antagonists / Q000008:administration & dosage / Q000009:adverse effects; D006330:Heart Defects, Congenital / Q000150:complications; D006333:Heart Failure / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D015278:Intensive Care Units, Pediatric; D009609:Nitrous Oxide / Q000008:administration & dosage / Q000009:adverse effects; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000009:adverse effects
chemical_list: D002121:Calcium Channel Blockers; D002317:Cardiovascular Agents; D065128:Endothelin Receptor Antagonists; D014665:Vasodilator Agents; D009609:Nitrous Oxide