General information

Alprostadil is PGE 1 available for exogenous administration. Alprostadil is widely used in neonates with cyanotic congenital heart disease to maintain the patency of the ductus arteriosus. Reported adverse effects include fever, apnea, flushing, bradycardia, and hyperostosis. Continuous chronic infusion of alprostadil via a portable pump and neuromuscular electrical stimulation help to improve the quality of life in patients with severe chronic heart failure waiting for a donor heart, as both treatments can be performed at home.

Of 15 neonates with hypoplastic left heart syndrome (nine boys and six girls; median weight 3123 g) included in a cardiac transplant program between January 1993 and August 1996, who received continuous perfusion of alprostadil from the time of diagnosis of the cardiomyopathy, 13 received transplants and six died in the operating room [ ]. All had short-term adverse effects from the continuous perfusion of alprostadil, including slight fever and irritability. However, none had apneic pauses. Cortical hyperostosis occurred in 13 and antral hyperplasia in 12, but in all transplanted cases regression of the antral hyperplasia was seen after 6 months and regression of the cortical hyperostosis was seen after 12 months.

Drug studies

Observational studies

Prostaglandin E 1 (PGE 1 ) is a routine palliative therapy for maintaining ductal patency in ductus arteriosus. It is usually given as an infusion of short duration. At the start of the treatment, several adverse effects, usually reversible, can occur. However, in some neonates, therapy longer than 2 weeks may be needed. There has been a retrospective analysis of nine patients who received alprostadil for more than 14 days [ ]. The leukocyte count remained high throughout the treatment period, and the proportion of neutrophils was over 50%. There were transient feeding difficulties, abdominal distension, and possible signs of gastric-outlet obstruction in two cases. In three patients, cortical hyperostosis developed after different cumulative doses (1584, 3384, and 4320 micrograms/kg). There were significant correlations between the doses of alprostadil and serum potassium concentrations and bicarbonate concentrations. The three patients who received the largest cumulative doses developed pseudo-Bartter’s syndrome.

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