Hypoplastic right-sided heart complex: A cluster of cases with associated congenital birth defects. A new syndrome?

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.