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Published on: May 11, 2015
Pulmonary hypertension specific treatment in infants with bronchopulmonary dysplasia
Gili Kadmon1, Ofer Schiller2, Tamir Dagan3
1Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Insights
Pulmonary hypertension (PH) in infants with bronchopulmonary dysplasia (BPD) improved with PH medications like sildenafil. This treatment led to better clinical and hemodynamic status and a high survival rate in a 6-year study.
Area of Science:
- Pediatric Pulmonology
- Cardiology
- Neonatology
Background:
- Bronchopulmonary dysplasia (BPD) complicated by pulmonary hypertension (PH) significantly increases morbidity and mortality in infants.
- Current guidelines do not include BPD-associated PH for PH-specific medication indications.
- Limited data suggest potential benefits of PH-specific treatments for BPD-associated PH.
Purpose of the Study:
- To evaluate the 6-year experience of treating BPD-associated PH with PH medications, primarily sildenafil.
- To assess the clinical and hemodynamic outcomes in infants with BPD and PH treated with specific medications.
Main Methods:
- Retrospective review of medical records for 20 infants diagnosed with BPD-associated PH between 2008-2014.
- Defined clinical improvement as a decrease in Ross functional class and hemodynamic improvement as a decrease in PH severity by echocardiography.
- Patients received PH medications including sildenafil and bosentan.
Main Results:
- Ninety-five percent survival rate was observed in the study cohort.
- Significant decrease in mean functional class from 3.2 to 1.7.
- Seventy-eight percent of patients showed improvement in PH class by echocardiography, with 72% initially diagnosed with moderate or severe PH.
Conclusions:
- Treatment of BPD with complicated PH using PH-specific medications, particularly sildenafil, is associated with positive clinical and hemodynamic improvements.
- The study demonstrates a low mortality rate in infants with BPD-associated PH treated with these medications.
- Findings support the use of PH-specific medications in managing BPD-associated PH.
Objective:
When bronchopulmonary dysplasia (BPD) is complicated by pulmonary hypertension (PH), morbidity and mortality are significantly increased. BPD-associated PH is not included in the current indications for PH medications. However, limited data demonstrate hemodynamic improvement and decreased mortality with PH-specific treatment. This report describes our 6-year experience treating BPD-associated PH with PH medications, mainly sildenafil.
Study Design:
The medical records of 20 infants diagnosed with BPD-associated PH at a tertiary pediatric pulmonary hypertension clinic in 2008-2014 were reviewed. Clinical improvement was defined as a decrease in Ross functional class by at least one degree. PH severity was classified by echocardiography as mild, moderate, or severe. Hemodynamic improvement was defined as a decrease in PH severity by at least one level.
Results:
Eighteen out of 20 patients were treated with PH medications: 12 sildenafil, 5 sildenafil and bosentan, and 1 bosentan. Median follow-up time was 2 years. Mean functional class significantly decreased from 3.2 ± 0.9 at diagnosis to 1.7 ± 0.9 at the last follow-up. Improvement in functional class was observed in 15/16 children (94%). Moderate or severe PH was found in 13/18 children (72%) at diagnosis, and in three (17%, all moderate PH) at the last follow-up. Improvement in PH class by echocardiography was demonstrated in 14/18 children (78%). The survival rate was 95%.
Conclusion:
Treatment of BPD complicated by PH with PH-specific medications, mainly sildenafil, is associated with improvement in both clinical and hemodynamic parameters and a low mortality rate. Pediatr Pulmonol. 2017;52:77-83. © 2016 Wiley Periodicals, Inc.
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