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      Pulmonary Vascular Underperfusion Score in Premature Infants with Bronchopulmonary Dysplasia and Pulmonary Hypertension

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          Abstract

          Pulmonary hypertension (PH) is a complication of bronchopulmonary dysplasia (BPD). The underlying pathophysiology of BPD-associated PH is complex and poorly understood. Echocardiogram may underestimate the severity of pulmonary hypertensive vascular disease in severe BPD. Digital subtraction pulmonary angiography (DSPA) is a potentially useful imaging modality for evaluating changes in the pulmonary vasculature of BPD-associated PH. In this study, we objectively quantified the pulmonary hypertensive vascular changes demonstrated by DSPA using a novel pulmonary vascular underperfusion score (PVUS) and correlated the scoring system with echocardiography parameters and cardiac hemodynamics by right heart catheterization.

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          Early pulmonary vascular disease in preterm infants at risk for bronchopulmonary dysplasia.

          Pulmonary hypertension (PH) is associated with poor outcomes among preterm infants with bronchopulmonary dysplasia (BPD), but whether early signs of pulmonary vascular disease are associated with the subsequent development of BPD or PH at 36 weeks post-menstrual age (PMA) is unknown.
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            Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia

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              Vasodilator therapy for primary pulmonary hypertension in children.

              This report presents 13 years of experience with vasodilator therapy for primary pulmonary hypertension (PPH) in children. Two eras were involved: between 1982 and 1987, oral calcium channel blockers were the only agents available for long-term therapy; after 1987, prostacyclin (PGI2) has been available for long-term intravenous use. Seventy-four children underwent short-term vasodilator testing with intravenous PGI2. Those who manifested pulmonary vasodilation ("acute responders") were treated with oral calcium channel blockers. Until 1987, "acute nonresponders" were treated in the same way as long as they had no serious side effects. When PGI2 became available for long-term administration, all nonresponders, as well as those who failed to improve clinically and hemodynamically on calcium channel blockers, were treated with long-term PGI2. In the 31 responders, calcium channel blockers improved survival compared with the 43 nonresponders (P=0.0002). Survival was also better in 24 PGI2-treated nonresponders compared with 22 nonresponders for whom PGI2 was unavailable (P=0.0005) as well as in all children who failed conventional therapy (n=31; P=0.002). Long-term vasodilator therapy improves survival in children with PPH. In acute responders, oral calcium channel blockers generally suffice. In both nonresponders to short-term testing and responders who fail to improve on calcium channel blockers, continuous intravenous infusion of PGI2 improves survival.
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                Author and article information

                Journal
                Medicina (Kaunas)
                medicina
                Medicina
                MDPI
                1010-660X
                1648-9144
                09 July 2019
                July 2019
                : 55
                : 7
                : 359
                Affiliations
                Joe DiMaggio Children’s Hospital Heart Institute, Memorial Healthcare, Hollywood, FL 33021, USA
                Author notes
                [* ]Correspondence: bdas99@ 123456hotmail.com
                Author information
                https://orcid.org/0000-0001-9530-8785
                Article
                medicina-55-00359
                10.3390/medicina55070359
                6681366
                31324065
                76d70fdc-b621-4a97-8ea7-cd22241ed1cb
                © 2019 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

                History
                : 01 June 2019
                : 04 July 2019
                Categories
                Concept Paper

                premature infant,bronchopulmonary dysplasia,pulmonary hypertension,digital subtraction pulmonary angiography

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