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SCOPE

DISEASE/CONDITION(S)

Pulmonary hypertension, pulmonary artery hypertension, bronchopulmonary dysplasia, chronic lung disease, prematurity.

GUIDELINE OBJECTIVE(S)

Provide screening recommendations including population, mode of screening, timing, and frequency of screening. Provide recommendations for evaluation of infant with concern for bronchopulmonary dysplasia (BPD)-associated pulmonary hypertension (PH) based on screening test. Provide recommendations for treatment (general and PH-specific) of the infant with BPD-associated PH.

BRIEF BACKGROUND

Pulmonary hypertension is an increasingly recognized complication of BPD and chronic lung disease (CLD) of prematurity. Prevalence in retrospective studies of infants with moderate to severe BPD is reported as high as 37%; however, a prospective study reported a lower rate of 17.9%. Pulmonary hypertension in this population has been shown to be an independent risk factor for increased morbidity and mortality and mortality rates are impacted by PH severity. Two-year survival in those with systemic to suprasystemic pulmonary artery pressures was only 25% in one series.

There is significant variability in screening practices, evaluation of affected patients, and treatment of BPD-associated PH. Level of evidence is low for most recommendations, which are largely based on expert opinion.

RECOMMENDATIONS

Infants with moderate to severe BPD are at increased risk for PH. Intrauterine growth restriction is an important risk factor over and above prematurity and BPD. Other factors such as maternal pre-eclampsia, infection, and duration of ventilation may also play disease-modifying roles. These risk factors should be considered when deciding on frequency of screening assessment. The suggested guidelines below are based on a standard-risk infant with moderate to severe BPD. Signs that suggest that PH may be present include labile oxygen saturations, failure to thrive, and need for respiratory support out of proportion to the perceived degree of lung disease. Presence of any of these signs should prompt consideration of assessment for PH outside of a screening paradigm, as there is clinical suspicion for that disease based on more than risk factors.

Screening for BPD-associated PH is largely done by echocardiogram given its widespread availability and ability to noninvasively assess heart function and estimate pulmonary artery pressure as well as evaluate for patent ductus arteriosus (PDA), atrial septal defect (ASD), and other common congenital heart diseases that can mimic or contribute to PH and lung disease. Due to the complexity in interpreting infant echocardiograms in the setting of shunt lesions and other confounders, testing should be performed by sonographers experienced in pediatric cardiac ultrasound and interpreted by pediatric cardiologists.

There is a wide degree of variability in screening, diagnosis, and treatment of this condition across centers. Below are some options obtained from several centers and from published protocols. We have added an asterisk after the guidelines we recommend at our institution.

SCREENING

Whom to Screen

  • Based on ...

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