Survey Finds Wide Variation in Management of PH in Premature Infants with Bronchopulmonary Dysplasia

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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Diagnosing and managing pulmonary hypertension in premature American infants with bronchopulmonary dysplasia vary a lot from doctor to doctor, a survey of 306 neonatologists indicates.

The study, “Practices surrounding pulmonary hypertension and bronchopulmonary dysplasia amongst neonatologists caring for premature infants,” was published in the Journal of Perinatology.

More than 55,000 U.S. births a year occur before the fetus is 28 weeks old. This premature infant population can suffer from pulmonary complications. Up to 68 percent are diagnosed with bronchopulmonary dysplasia, a condition characterized by damage to the lungs’ airway passages, or bronchi. One of the most severe consequences of bronchopulmonary dysplasia is the development of pulmonary hypertension.

In 2015, the American Heart Association and the American Thoracic Society published guidelines that call for “echocardiography (ECHO)-based screening for pulmonary hypertension (PH) in premature newborns with established moderate to severe BPD [bronchopulmonary dysplasia].”

A group of doctors decided to survey the bronchopulmonary dysplasia practices of neonatologists caring for premature infants with and without PH, including their screening strategies and treatment. The survey asked about doctors’ practices after the 2015 guidelines were published.

The electronic survey, composed of 29 questions, was sent to neonatologists practicing at a nursery where babies were born after being in the womb 28 weeks or less. In total, 306 neonatologists responded to the survey.

The guidelines recommend that children with a bronchopulmonary dysplasia diagnosis have echocardiography, or ECHO, screening for PH. Despite this, only 46 percent of the survey respondents confirmed a formal screening protocol at their institution. But 83 percent said they would do an ECHO at 36 weeks post-menstrual age — a combination of a baby’s time in the womb and after birth — for premature infants with moderate to severe bronchopulmonary dysplasia.

Another provision in the guidelines is that neonatologists should evaluate airway obstruction and reflux in premature babies with bronchopulmonary dysplasia and PH. But “only 29 percent of [the survey] respondents mentioned ordering such evaluation for airway obstruction,” the study reported.

Ninety percent of the surveyed neonatologists prescribed PH medications — phosphodiesterase 5 inhibitors, endothelin antagonists, or prostacyclines — during hospitalization. But a very small percentage used cardiac catheterization to confirm a PH diagnosis. One percent said they always used it, while 7 percent said they often used it.

Among neonatologists practicing more than five years, 54 percent reported an increase in the number of premature infants they have diagnosed with PH.

As for a need for home oxygen therapy, only 65 percent advised it for PH.

Given the range in survey answers, the study team concluded that “the evaluation, the treatment, and the follow-up of these patients was highly variable across centers in the U.S.,” despite the guidelines that came out in 2015.



A Conversation With Rare Disease Advocates