What are the American Heart Association (AHA) and American Thoracic Society (ATS) treatment guidelines for persistent pulmonary hypertension of the newborn (PPHN)?

Updated: Sep 03, 2019
  • Author: Kate A Tauber, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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In November 2015, the American Heart Association (AHA) and American Thoracic Society (ATS) released updated guidelines on the diagnosis, evaluation, and management of pediatric pulmonary hypertension. Among their recommendations are the following [32] :

  • Inhaled nitric oxide should be used to reduce the need for extracorporeal membrane oxygenation (ECMO) in term and near-term infants with PPHN or hypoxemic respiratory failure who have an oxygenation index that exceeds 25.

  • Cardiac catheterization beyond the neonatal period should include acute vasoreactivity testing unless there is a specific contraindication.

  • Genetic testing with counseling can be useful for children with idiopathic pulmonary artery hypertension (PAH) or in families with heritable PAH to define the pathogenesis, to determine family members at risk, and for family planning.

  • Longitudinal care in an interdisciplinary pediatric pulmonary hypertension program is recommended for infants following congenital diaphragmatic hernia repair who have pulmonary hypertension or are at risk of developing late pulmonary hypertension. Extracorporeal membrane oxygenation (ECMO) is recommended for patients with congenital diaphragmatic hernia with severe pulmonary hypertension who do not respond to medical therapy.

  • Screening for pulmonary hypertension by echocardiogram is recommended in infants with bronchopulmonary dysplasia. Evaluation and treatment of lung disease, including assessments for hypoxemia, aspiration, structural airway disease, and the need for changes in respiratory support, are recommended in infants with bronchopulmonary dysplasia and pulmonary hypertension before initiation of PAH-targeted therapy.

  • Anticoagulation should not be used in young children with PAH because of concerns about harm from hemorrhagic complications.

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