Secondary effects of prostaglandin E1 on the management of hypoplastic left heart syndrome while waiting for heart transplantation

OBJECTIVE: Newborns with hypoplastic left heart syndrome (HLHS) included in the heart transplant program depend on continuous prostaglandin E1 (PGE1) perfusion for a prolonged period of time, which can result in various side effects. The objective of this study was to analyze the side effects derived from the use of PGE1 in newborns with HLHS who are awaiting heart transplantation. PATIENTS AND METHODS: Fifteen newborns with HLHS included in a cardiac transplant program between January 1993 and August 1996 were studied. All received continuous endovenous perfusion of PGE1 from the time of diagnosis of the cardiopathy. RESULTS: Thirteen patients were transplanted, with 6 dying in the operating room. Seven survived. Two patients died while waiting for a transplant. All presented short term side effects derived from the continuous perfusion of PGE1, including a slight fever and irritability. However, none of them presented apnea pauses. Cortical hyperostosis and anthral hyperplasia were observed in 13 (83.3%) and 12 (80%) cases, respectively, but in all transplanted cases regression of the anthral hyperplasia was seen after 6 months and regression of the cortical hyperostosis was seen after 12 months. CONCLUSIONS: PGE1 was effective in maintaining the permeability of the ductus arteriosus. The side effects derived from the treatment were well tolerated. The appearance of cortical hyperostosis and anthral hyperplasia is related to the duration of the perfusion and these disappear in the weeks following the suspension of PGE1 treatment.