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):
- 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.
- 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.
- 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.
- 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.
- The 6-minute walk distance (6MWD) test should be used to follow exercise tolerance in pediatric PH patients of appropriate age.
- Calcium channel blockers should be given only to those patients who are reactive, as assessed by AVT and >1 year of age.
- 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.
- 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.
- 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.
- 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.
Clinical Topics: Anticoagulation Management, Congenital Heart Disease and Pediatric Cardiology, Dyslipidemia, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Pericardial Disease, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Congenital Heart Disease, CHD & Pediatrics and Imaging, CHD & Pediatrics and Prevention, Lipid Metabolism, Heart Failure and Cardiac Biomarkers, Pulmonary Hypertension, Echocardiography/Ultrasound, Hypertension
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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