beraprost and Persistent-Fetal-Circulation-Syndrome

The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237 th day of life as a result of worsening PH associated with infection.

Topics: Administration, Inhalation; Bosentan; Bronchodilator Agents; Dose-Response Relationship, Drug; Drug Therapy, Combination; Epoprostenol; Fatal Outcome; Female; Follow-Up Studies; Humans; Infant, Newborn; Nitric Oxide; Persistent Fetal Circulation Syndrome; Piperazines; Platelet Aggregation Inhibitors; Pulmonary Alveoli; Purines; Sildenafil Citrate; Sulfonamides; Sulfones; Time Factors; Vasodilator Agents

Persistent pulmonary hypertension of the newborn (PPHN) is one of the most serious conditions in neonates resulting in a high mortality and morbidity. New alternative therapies for PPHN have been sought to improve survival and reduce morbidity.. To report an initial experience of using beraprost sodium (BPS) to treat infants with PPHN and to assess its effect on oxygenation and hemodynamic stability over a 72-hour study period.. The clinical data of neonates who received BPS as an adjunctive therapy for PPHN in our hospital between July 2007 and June 2008 were retrospectively reviewed.. During the study period, 7 infants with PPHN were successfully treated with BPS. The mean gestational age and birth weight were 39.3 ± 1.5 weeks and 3,365.7 ± 569.8 g, respectively. BPS was initiated at a median age of 42.7 h after birth (range: 2.1-166.5 h) with a baseline mean oxygen index (OI) of 33.9 ± 15.7 and a baseline mean systolic blood pressure (SBP) of 79.4 ± 9.9 mm Hg. The mean difference of OI at 24, 48 and 72 h following the treatment was -15.7 ± 14.8 (p = 0.043), -18.2 ± 12.3 (p = 0.018) and -16.7 ± 17.5 (p = 0.042), respectively. The mean SBP was significantly reduced as early as 6 h after initiation of treatment (-11.1 ± 11.5 mm Hg, p = 0.034) without changes in heart rate. Three cases were complicated with chronic lung disease, and the remaining 4 cases were normal at hospital discharge. No neurodevelopmental and cardiopulmonary disorders were observed in all cases at 1 year of age.. BPS may be used as an alternative treatment for infants with PPHN giving a significant improvement in oxygenation.

Topics: Blood Pressure; Epoprostenol; Female; Gestational Age; Humans; Hypertension; Infant, Newborn; Male; Persistent Fetal Circulation Syndrome; Retrospective Studies; Treatment Outcome; Vasodilator Agents