Pediatric Pulmonary Hypertension Guidelines

Abman SH, Hansmann G, Archer SL, et al.
Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation 2015;Nov 3:[Epub ahead of print].

The following are 10 key points to remember from the American Heart Association/American Thoracic Society guidelines on pediatric pulmonary hypertension (PH):

  1. For the purposes of this document, PH was defined as mean pulmonary artery pressure (PAP) >25 mm Hg in children >3 months of age at sea level.
  2. Pediatric PH is a distinct entity from adult PH. Pediatric PH is linked to issues of lung growth and development, and influenced by prenatal and early postnatal factors.
  3. Catheterization is recommended before initiation of pulmonary artery hypertension (PAH) targeted therapy. Acute vasoreactivity testing (AVT) should be performed unless there is a specific contraindication. A positive response to AVT is defined as a >20% decrease in PAP and pulmonary vascular resistance/systemic vascular resistance without a decrease in cardiac output.
  4. B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP) should be measured at diagnosis and during follow-up to supplement clinical decision making.
  5. The 6-minute walk distance (6MWD) test should be used to follow exercise tolerance in pediatric PH patients of appropriate age.
  6. Calcium channel blockers should be given only to those patients who are reactive, as assessed by AVT and >1 year of age.
  7. High-risk features in pediatric PAH include clinical evidence of right ventricular failure, World Health Organization Class III and IV symptoms, recurrent syncope, significant right ventricular enlargement/dysfunction, or pericardial effusion on echocardiography. Hemodynamic parameters predicting risk include indexed pulmonary vascular resistance >20 WU•m2, cardiac index <2 L/min/m2, and pulmonary vascular resistance/systemic vascular resistance >1.0. Additional predictors of risk include significantly elevated BNP or NT-proBNP, 6MWD <300 m, and peak VO2 <15 ml/kg/min.
  8. Oral PAH-targeted therapy in children with lower-risk PAH is recommended and should include either a phosphodiesterase type 5 inhibitor or an endothelin receptor antagonist.
  9. Intravenous and subcutaneous prostacyclin analogs should be initiated without delay for patients with higher-risk PAH. The effective dose of epoprostenol may be higher in children than in adults with a range of optimal dosing from 40 to >150 nanograms•kg-1•min-1 with an average dose of approximately 80 nanograms•kg-1•min-1.
  10. There is controversy regarding the use and dosing of sildenafil in patients between 1 and 17 years of age over concerns of higher risk of mortality with higher sildenafil dosing. Current recommendations suggest treatment with low doses and close follow-up to determine the need for changes in therapy.

Keywords: Calcium Channel Blockers, Cardiac Catheterization, Echocardiography, Endothelin Receptor Antagonists, Epoprostenol, Exercise Tolerance, Heart Defects, Congenital, Hypertension, Hypertension, Pulmonary, Natriuretic Peptide, Brain, Pediatrics, Peptide Fragments, Pericardial Effusion, Phosphodiesterase 5 Inhibitors, Syncope, Vascular Resistance, Ventricular Dysfunction, Right

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