Congenital Heart Disease
Comparison of outcome when hypoplastic left heart syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of these two conditions is made only postnatally

https://doi.org/10.1016/S0002-9149(99)00172-1Get rights and content

Abstract

We sought to determine the impact of prenatal diagnosis on the perioperative outcome of newborns with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). All neonates with HLHS or TGA encountered at Children’s Hospital, Boston, Massachusetts, from January 1988 to May 1996 were identified and outcomes documented. Birth characteristics, preoperative, operative, and postoperative variables of term newborns with a prenatal diagnosis of HLHS or TGA who underwent a Norwood operation (n = 27) or arterial switch operation (n = 14), respectively, were compared with newborns with a postnatal diagnosis of HLHS (n = 47) or TGA (n = 28) who had undergone surgery. Of 217 neonates with HLHS and 422 with TGA, 39 and 16, respectively, had a prenatal diagnosis. The preoperative mortality among neonates aggressively managed did not differ between the prenatal and postnatal diagnosis groups for either HLHS or TGA (p >0.05). Neonates with a prenatal diagnosis who underwent surgery had objective indicators of lower severity of illness preoperatively, including a higher lowest recorded pH (p = 0.03), lower maximum blood urea nitrogen (p = 0.002), and creatinine (p = 0.03) among newborns with HLHS, and a tendency toward higher minimum of partial pressure of arterial oxygen in the TGA group (p = 0.06). Prenatal diagnosis was not associated with an improved postoperative course or operative mortality (p <0.05) within a diagnostic group. Thus, a prenatal diagnosis improves the preoperative condition of neonates with HLHS and TGA, but may not significantly improve preoperative mortality or early postoperative outcome among neonates managed at a tertiary care center.

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Study design and patient selection

We retrospectively identified all neonates with the prenatal or postnatal diagnosis of HLHS or TGA encountered at the Children’s Hospital, Boston, Massachusetts, from January 1988 to May 1996. To assess the impact of the prenatal diagnosis on preoperative mortality associated with HLHS and TGA, we compared the number of preoperative deaths among neonates with a prenatal diagnosis of HLHS or TGA with those in whom the diagnosis was made after birth. Medical records were reviewed to document the

Results

From January 1988 to May 1996, 217 neonates with the diagnosis of HLHS were encountered at Children’s Hospital. Of these, 39 (18%) were diagnosed prenatally. Thirty (77%) of the prenatally diagnosed neonates, including 2 premature neonates and 1 with Turner syndrome, and 156 (88%) of those with a postnatal diagnosis underwent the Norwood operation (stage I palliation).

During the study period, 422 neonates with the diagnosis of TGA were managed at our institution. Sixteen (4%) were prenatally

Discussion

Postnatal survival in newborns with critical congenital heart disease largely depends on the timely initiation of specific treatment.4, 5, 6 Prenatal diagnosis followed by delivery at a tertiary care institution with an expertise in management of newborns with heart disease allows timely institution of specific treatment. It is not clear, however, whether this early resuscitation translates into reduced mortality and morbidity, at least for infants referred to a tertiary care center.

In the

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This study was supported in part by the Kobren Fund, New Delhi, India; AN American Heart Association, Massachusetts Affiliate Physician-Investigator Fellowship Award, and by Grants HL 41786, RR02172, and P30-HD18655 from the National Institutes of Health, Bethesda, Maryland.

Current address: Amrita Institute of Medical Sciences, Elamakkara PO, Kochi 682026, Kerala, India.

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