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Hypoplastic right-sided heart complex: a cluster of cases with associated congenital birth defects. A new syndrome?
Insights
Congenital heart defects in infants may be linked to viral infections during spring and summer conception. This study found a cluster of hypoplastic right-sided heart complex with other birth defects, possibly associated with enteroviruses.
Area of Science:
- Pediatric Cardiology
- Infectious Diseases
- Teratology
Background:
- A cluster of 14 infants born in the same region over two years presented with hypoplastic right-sided heart complex and other congenital anomalies.
- Previous studies have not identified common teratogenic exposures or genetic links for such clusters.
Purpose of the Study:
- To investigate potential environmental or infectious causes for a specific cluster of congenital heart defects in infants.
- To characterize the spectrum of cardiac and extracardiac anomalies in affected infants.
Main Methods:
- Retrospective review of medical records for 14 infants with hypoplastic right-sided heart complex.
- Analysis of infant birth dates to identify seasonal conception patterns.
- Review of serological data from the general referral population for evidence of viral infections during relevant periods.
Main Results:
- Twelve of the 14 infants were conceived during spring or summer months.
- Elevated serum antibody titers against coxsackie group B virus and echoviruses were observed in the general population during these seasons.
- All infants had hypoplastic right ventricle, with varying degrees of valvular and intracardiac defects. Extracardiac anomalies, including microcephaly, were present in 79% of cases.
Conclusions:
- The findings suggest a potential association between enteroviral infections during spring/summer and the development of hypoplastic right-sided heart complex with extracardiac anomalies.
- The high incidence of extracardiac defects distinguishes this syndrome from other congenital heart conditions.
Abstract:
Fourteen infants, all born within the same geographic area in a two-year period, had hypoplastic right-sided heart complex associated with a high incidence of other congenital defects. No common teratogenic exposure or genetic relationships were discovered. Twelve of the 14 infants were conceived in the spring or summer. Significant elevations in serum antibody titers against coxsackie group B virus and echoviruses were noted in the general referral population during those spring and summer quarters. Cardiac defects included a hypoplastic right ventricle in every case; three types were noted. Varying degrees of right-sided valvular lesions and other intracardiac defects accompanied the hypoplastic ventricle. Eleven infants had extracardiac congenital anomalies: 10 had microcephaly, two had micrognathia with posterior cleft palate, four had low-set ears, and one had arrhinencephaly. The high incidence of extracardiac defects in the syndrome (79%) is in distinct contrast to the incidence reported in the New England Regional Infant Cardiac Program in infants who had pulmonic atresia with intact ventricular septum.