Prolonged prostaglandin E1 therapy in a neonate with pulmonary atresia and ventricular septal defect and the development of antral foveolar hyperplasia and hypertrophic pyloric stenosis

Abstract

Prostaglandin E1 (alprostadil) is widely used for maintaining the patency of ductus arteriosus in ductus-dependent congenital heart defects in neonates to improve oxygenation. Among more common side effects are fever, rash, apnoea, diarrhoea, jitteriness, and flushing. More severe side effects are brown fat necrosis, cortical hyperostosis, and gastric outlet obstruction, most commonly the result of antral foveolar hyperplasia or hypertrophic pyloric stenosis. We report on an infant with a ductus-dependent congenital heart defect who developed symptoms and sonographic evidence of focal foveolar hyperplasia and hypertrophic pyloric stenosis after prolonged treatment with prostaglandin E1. Gastrointestinal symptoms persisted after corrective cardiac surgery, and pyloromyotomy was required. Study of the case and of available literature showed an association between the total dose of prostaglandin E1 administered and duration of treatment and the development of gastric outlet obstruction. We conclude that if patients are treated with a prostaglandin E1 infusion, careful monitoring for symptoms and signs of gastric outlet obstruction is required.

Figure 1.

Longitudinal ultrasound images (left and right) of the pylorus on day 44 (2 days after cardiac operation and discontinuation of alprostadil). There is an elongation of the pyloric channel with moderately thickened pyloric musculature.

Figure 2.

A: Longitudinal ultrasound image of the pylorus on day 47 (5 days after cardiac surgery when the patient vomited a large amount of non-bilious gastric content) showing elongated pyloric channel and thickened pyloric musculature. B: Transverse ultrasound image of the antropyloric region showing marked antral mucosal hypertrophy.

Figure 3.

Transverse ultrasound image of the pylorus after pyloromyotomy, with antral mucosal thickening still present.

Figure 4.

Longitudinal ultrasound image of pylorus and antrum performed on day 58 showing diminished pylorus wall thickness.

Figure 5.

Prostaglandin E1 infusion rate and cumulative doses. Day 7, extubation; day 8, cardiac catheterization; day 12, infusion stopped and then restarted after 2 days; day 38, bolus dose of 105 μg of prostaglandin E1; day 42, surgery.