Table 2.

Comparison of published guidelines for screening and management of BPD-PH from the American Heart Association/American Thoracic Society (AHA/ATS), European Pediatric Pulmonary Vascular Disease Network (EPPVDN), and Pediatric Pulmonary Hypertension Network (PPHNet)^7,32,39.

	AHA/ATS	EPPVDN	PPHNet
Screening for PH by echocardiogram	Recommended in infants with established BPD	If severe respiratory compromise is present in any preterm infant <28 weeks gestation In any infant with established BPD at corrected age of 36 weeks and before discharge In any infant with prolonged oxygen requirement, poor growth, or unsatisfactory clinical improvement	Severe hypoxemic respiratory failure shortly after birth attributed primarily to persistent pulmonary hypertension of the newborn (PPHN) physiology despite optimal management of underlying lung disease Continued need for ventilator support at postnatal day 7, as echocardiogram evidence of PH at day 7 suggests high risk for BPD and may alter therapy With sustained need for significant respiratory support at any age, especially with recurrent episodes of hypoxemia. At the time of formal BPD diagnosis per current practice (36 weeks PMA)
Cardiac catheterization	For infants who have persistent signs of severe cardiorespiratory disease or clinical deterioration not directly related to airways disease Infants who are suspected of having significant PH despite optimal management of their lung disease and associated morbidities Infants who are candidates for long-term PH-specific drug therapy Infants who have unexplained, recurrent pulmonary edema	Should be considered before introduction of a second PH-specific medication Advised if PH worsens under dual PH-specific therapy or if an unsatisfactory response to PH-specific therapy is seen in any infant with PH at a corrected postnatal age of >3 months Additional indications include concern for vascular abnormalities (e.g., pulmonary vein stenosis), other features of an atypical response to therapy, and/or failure to thrive	To confirm the echocardiographic diagnosis of PH Determine disease severity Evaluate the potential contributions of shunt lesions, PVS, LV diastolic dysfunction, aortopulmonary collaterals, and close shunts if appropriate To define the need for the addition of combination drug therapy, especially systemic prostanoid therapy In the setting of clinical deterioration and echocardiographic evidence of increasing PH or decreasing ventricular function
Evaluation of comorbidities	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 BPD-PH before initiation of PH-specific therapy	Maximizing non-invasive respiratory support as much as possible in order to avoid mechanical ventilation and therefore aggravation of lung injury is advisable Treatment with diuretics may be considered in infants with severe BPD Infants with severe BPD have higher caloric requirements, which should be accounted for in their nutritional plans	Evaluation and treatment of comorbidities that impact the severity of lung disease should be undertaken with the diagnosis of BPD-PH infants before the initiation of PH-specific therapy Studies should include evaluation for intermittent or sustained hypoxemia, aspiration, gastroesophageal reflux disease, structural airways disease, pulmonary artery and vein stenosis, left ventricular diastolic dysfunction, and aortopulmonary collaterals
Management	Supplemental oxygen therapy is reasonable to avoid episodic or sustained hypoxemia and with the goal of maintaining O2 saturations between 92%−95% PH-specific therapy can be useful for infants with BPD-PH on optimal treatment of underlying respiratory and cardiac disease Treatment with iNO can be effective for infants with established BPD and symptomatic PH	Supplemental oxygen should be supplied when target oxygen saturations are >93% for infants with suspected and >95% for infants with proven PH PH-specific therapy should be considered if PH persists after optimizing ventilation strategies and oxygenation Infants with BPD-PH are prone to a sudden elevation of PVR when undergoing stressful procedures (“PH crisis”) and often require sufficient sedation or even analgesia/relaxation before such procedures are performed	Supplemental oxygen therapy should be used to avoid episodic or sustained hypoxemia and with the goal of maintaining O2 saturations between 92%−95% PH-specific therapy should be considered for infants with BPD-PH after optimal treatment of underlying respiratory and cardiac disease Pharmacologic therapy should be initiated in patients with evidence of significantly elevated pulmonary vascular resistance and right ventricular impairment not related to left heart disease or pulmonary vein stenosis iNO should be used for acute PH crises and weaned after stabilization
Follow-up	Infants should be followed up by serial echocardiograms, which should be obtained at least every 2 to 4 weeks with the initiation of therapy and at 4- to 6-month intervals with stable disease	Infants should be followed by clinical evaluation, echocardiogram, and repeat NT-proBNP every 1–4 weeks while initiating therapy.	Infants should have outpatient follow-up with a multidisciplinary PH team at intervals of 3–4 months with use of echocardiograms, biomarkers, hemodynamic studies, and sleep studies when indicated, depending on disease severity and clinical progress