Children with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension Treated with Pulmonary Vasodilators-The Pediatric Cardiologist Point of View.

Anna Migdał,Edyta Ryciak,Alicja Mirecka-Rola,Grażyna Brzezińska-Rajszys,Anna Sądel-Wieczorek,Małgorzata Żuk

doi:10.3390/children8050326

Anna Migdał, Edyta Ryciak + Show 4 more

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https://doi.org/10.3390/children8050326

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Abstract

Pulmonary hypertension in children with bronchopulmonary dysplasia (BPD-PH) significantly worsens the prognosis. Pulmonary vasodilators are often used in BPD-PH but the short-term outcome of treatment is not well described. The aim of this study was to evaluate BPD-PH children diagnosed beyond 36 weeks postmenstrual age treated with pulmonary vasodilators (sildenafil, bosentan, or both) and to assess the short and long-term effect of oral pulmonary vasodilators treatment. Twenty patients were included in the study. Cardiology evaluation (WHO-FC, NTproBNP, oxygen saturation, pulmonary to systemic pressure ratio PAP/SAP) was performed at diagnosis and after treatment initiation. In the majority of patients improvement in all evaluated factors was observed. No side effects of vasodilators were observed. PH resolved in 10 patients after a mean of 21.4 months of treatment. Six patients died. The number of poor prognostic factors commonly used to assess patients with pulmonary arterial hypertension (PAH) decreased significantly during BPD-PH treatment. The influence of BPD-PH perinatal risk factors on prognosis was considered but was not confirmed. In conclusion, the treatment of BPD-PH with pulmonary vasodilators was well tolerated and led to a clinical improvement with the possibility of discontinuation without recurrence of PH. Prognostic factors used in pediatric PAH risk stratification also seem to be useful in assessing treatment efficacy and prognosis in patients with BPD-PH.

Highlights

Pulmonary hypertension (PH) occurs in 17–43% of premature infants with bronchopulmonary dysplasia (BPD) and significantly worsens the prognosis
Despite previous reports on the long-term outcomes of patients with bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) treated with vasodilators [9,10,11,12,13,14], it is still unclear whether the prognosis is related to PH-specific therapy or spontaneous PH resolution with age
The aim of the study was to evaluate the clinical profile of patients diagnosed beyond 36 weeks postmenstrual age (PMA) with late BPD-PH referred to pulmonary hypertension specialist for PH-specific therapy and to assess the short-and long-term effects of oral pulmonary vasodilator treatment

Summary

Introduction

Pulmonary hypertension (PH) occurs in 17–43% of premature infants with bronchopulmonary dysplasia (BPD) and significantly worsens the prognosis. Guidelines for the early diagnosis and management of BPD-PH have been recently established [7,8], some infants may develop late BPD-PH—above 36 weeks postmenstrual age (PMA), despite even normal echocardiography at discharge. Despite previous reports on the long-term outcomes of patients with BPD-PH treated with vasodilators [9,10,11,12,13,14], it is still unclear whether the prognosis is related to PH-specific therapy or spontaneous PH resolution with age. The aim of the study was to evaluate the clinical profile of patients diagnosed beyond 36 weeks PMA with late BPD-PH referred to pulmonary hypertension specialist for PH-specific therapy and to assess the short-and long-term effects of oral pulmonary vasodilator treatment

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