Background The clinical needs of infants with severe bronchopulmonary dysplasia (BPD) that remain ventilator-dependent are complex, and management strategies that optimize survival and long-term outcomes controversial. We hypothesized that an interdisciplinary ventilator care program (VCP), committed to the care of this population will improve survival through standardized approaches to cardiopulmonary care and related comorbidities, enhanced communication, and continuity of care. Methods Retrospective chart reviews were performed on patients at Children’s Hospital Colorado’s neonatal intensive care unit, who underwent tracheostomy placement between 2000 and 2013. Data were collected for two time periods: 2000 to 2005 and 2006 to 2013, before and after initiation of the VCP. Collected data on infants with ventilator-dependent BPD included: gestational age (GA), age at tracheostomy, respiratory severity score (RSS; mean airway pressure [MAP] × fraction of inspired oxygen [FiO2]), comorbidities, medication use, and the age of death. Tracheostomy patients without severe BPD were excluded. Results Despite no difference, pre and post VCP regarding GA, birth weight or morbidities and survival to discharge increased from 50% to 85% after implementation of the VCP (p < 0.05). Between period 1 and 2, there were differences in systemic and inhaled steroid use and mucolytic use. Conclusion These findings suggest an interdisciplinary approach to the care of infants with ventilator-dependent BPD can improve survival.

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Pulmonary vascular disease and pulmonary hypertension (PH) contributes significantly to the pathogenesis, pathophysiology, and clinical course of infants with bronchopulmonary dysplasia (BPD). This article briefly reviews the impact of premature birth on the developing lung circulation, mechanisms that contribute to the development of PH in premature newborns, and the diagnostic evaluation and management of severe PH in infants with BPD.

IntroductionRiociguat, an oral soluble guanylate cyclase stimulator, has been approved for use in adults with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension. However, there is limited data on its therapeutic use in children.Case PresentationWe report the case of two infants with severe suprasystemic pulmonary hypertension who were successfully treated with riociguat after failure to wean off inhaled nitric oxide (iNO) despite combination PAH therapy. Case 1 is a 6-month-old term male with TBX4 deletion who presented with severe hypoxemic respiratory failure and severe PAH immediately after birth. Initial cardiac catheterization showed PVRi 15.5 WU*m2. Marked hypoxemia and PAH persisted despite aggressive therapy with sildenafil, bosentan, intravenous treprostinil, and milrinone. The infant required high doses of inhaled nitric oxide (60 ppm) and manifested significant post-ductal hypoxemia and hemodynamic instability with any attempt at weaning. After discontinuation of sildenafil, initiation, and very slow uptitration of riociguat, the patient was able to maintain hemodynamic stability and wean from nitric oxide over 6 weeks with persistently severe but not worsened pulmonary hypertension. Case 2 is a 4-month-old term male with compound heterozygous SLC25A26 mutation and severe pulmonary hypertension. Initial cardiac catheterization showed PVRi 28.2 WU*m2. After uptitration of sildenafil, bosentan, and IV treprostinil, serial echocardiograms continued to demonstrate near-systemic pulmonary hypertension. He failed multiple attempts to wean off typical doses of iNO (10–20 ppm) over the following weeks with tachypnea, hypoxemia, and worsening pulmonary hypertension on echocardiogram despite continued aggressive combination targeted therapy. After a 24-h sildenafil washout, he was initiated and uptitrated on riociguat with concomitant, successful wean of nitric oxide over one week that was well tolerated. No serious adverse effects in the titration period were observed.ConclusionRiociguat may be considered as an adjuvant therapeutic agent in selected children with severe PAH who are poorly responsive to sildenafil therapy and unable to wean from iNO.

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