Targeted Therapy for Pulmonary Hypertension in Premature Infants
Pulmonary hypertension (PH) is common in premature infants with bronchopulmonary dysplasia (BPD) and is associated with significant mortality. Despite expert consensus suggesting the use of targeted therapies such as phosphodiesterase inhibitors, endothelin receptor antagonists, and prostanoids, the...
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2020-08-01
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doaj-330d8391df244766a79513521ba3d02f2021-04-02T13:16:53ZengMDPI AGChildren2227-90672020-08-017979710.3390/children7080097Targeted Therapy for Pulmonary Hypertension in Premature InfantsShannon N Nees0Erika B Rosenzweig1Jennifer L Cohen2Gerson A Valencia Villeda3Usha S Krishnan4Division of Pediatric Cardiology, Columbia University Irving Medical Center, New York, NY 10032, USADivision of Pediatric Cardiology, Columbia University Irving Medical Center, New York, NY 10032, USADivision of Pediatric Cardiology, Mount Sinai Medical Center, New York, NY 10029, USADivision of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, FL 32806, USADivision of Pediatric Cardiology, Columbia University Irving Medical Center, New York, NY 10032, USAPulmonary hypertension (PH) is common in premature infants with bronchopulmonary dysplasia (BPD) and is associated with significant mortality. Despite expert consensus suggesting the use of targeted therapies such as phosphodiesterase inhibitors, endothelin receptor antagonists, and prostanoids, there is little data on safety and outcomes in infants with BPD-associated PH (BPD-PH) treated with these medications. We sought to describe the pharmacologic management of BPD-PH and to report outcomes at our institution. Premature infants with BPD-PH born between 2005 and 2016 were included. Follow-up data were obtained through January 2020. A total of 101 patients (61 male, 40 female) were included. Of these, 99 (98.0%) patients were treated with sildenafil, 13 (12.9%) with bosentan, 35 (34.7%) with inhaled iloprost, 12 (11.9%) with intravenous epoprostenol, and nine (8.9%) with subcutaneous treprostinil. A total of 33 (32.7%) patients died during the study period and 10 (9.9%) were secondary to severe to pulmonary hypertension. Of the surviving patients, 57 (83.8%) had follow-up data at a median of 5.1 (range 0.38–12.65) years and 44 (77.2%) were weaned off PH medications at a median 2.0 (range 0–8) years. Mortality for BPD-PH remains high mostly due to co-morbid conditions. However, for those patients that survive to discharge, PH therapies can frequently be discontinued in the first few years of life.https://www.mdpi.com/2227-9067/7/8/97pulmonary hypertensionprematuritybronchopulmonary dysplasia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Shannon N Nees Erika B Rosenzweig Jennifer L Cohen Gerson A Valencia Villeda Usha S Krishnan |
spellingShingle |
Shannon N Nees Erika B Rosenzweig Jennifer L Cohen Gerson A Valencia Villeda Usha S Krishnan Targeted Therapy for Pulmonary Hypertension in Premature Infants Children pulmonary hypertension prematurity bronchopulmonary dysplasia |
author_facet |
Shannon N Nees Erika B Rosenzweig Jennifer L Cohen Gerson A Valencia Villeda Usha S Krishnan |
author_sort |
Shannon N Nees |
title |
Targeted Therapy for Pulmonary Hypertension in Premature Infants |
title_short |
Targeted Therapy for Pulmonary Hypertension in Premature Infants |
title_full |
Targeted Therapy for Pulmonary Hypertension in Premature Infants |
title_fullStr |
Targeted Therapy for Pulmonary Hypertension in Premature Infants |
title_full_unstemmed |
Targeted Therapy for Pulmonary Hypertension in Premature Infants |
title_sort |
targeted therapy for pulmonary hypertension in premature infants |
publisher |
MDPI AG |
series |
Children |
issn |
2227-9067 |
publishDate |
2020-08-01 |
description |
Pulmonary hypertension (PH) is common in premature infants with bronchopulmonary dysplasia (BPD) and is associated with significant mortality. Despite expert consensus suggesting the use of targeted therapies such as phosphodiesterase inhibitors, endothelin receptor antagonists, and prostanoids, there is little data on safety and outcomes in infants with BPD-associated PH (BPD-PH) treated with these medications. We sought to describe the pharmacologic management of BPD-PH and to report outcomes at our institution. Premature infants with BPD-PH born between 2005 and 2016 were included. Follow-up data were obtained through January 2020. A total of 101 patients (61 male, 40 female) were included. Of these, 99 (98.0%) patients were treated with sildenafil, 13 (12.9%) with bosentan, 35 (34.7%) with inhaled iloprost, 12 (11.9%) with intravenous epoprostenol, and nine (8.9%) with subcutaneous treprostinil. A total of 33 (32.7%) patients died during the study period and 10 (9.9%) were secondary to severe to pulmonary hypertension. Of the surviving patients, 57 (83.8%) had follow-up data at a median of 5.1 (range 0.38–12.65) years and 44 (77.2%) were weaned off PH medications at a median 2.0 (range 0–8) years. Mortality for BPD-PH remains high mostly due to co-morbid conditions. However, for those patients that survive to discharge, PH therapies can frequently be discontinued in the first few years of life. |
topic |
pulmonary hypertension prematurity bronchopulmonary dysplasia |
url |
https://www.mdpi.com/2227-9067/7/8/97 |
work_keys_str_mv |
AT shannonnnees targetedtherapyforpulmonaryhypertensioninprematureinfants AT erikabrosenzweig targetedtherapyforpulmonaryhypertensioninprematureinfants AT jenniferlcohen targetedtherapyforpulmonaryhypertensioninprematureinfants AT gersonavalenciavilleda targetedtherapyforpulmonaryhypertensioninprematureinfants AT ushaskrishnan targetedtherapyforpulmonaryhypertensioninprematureinfants |
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