The incidence of congenital heart disease (CHD) was examined in relation to the consanguinity of the parents. The study was performed in five Arab villages in the Western Galilee, Israel, where consanguineous matings are known to be very high. All children up to the age of 7 years were included in this study; there were 1,546 children, 32% were the offspring of consanguineous marriages including first- and second-degree cousins. A higher percentage of isolated CHD was found in the offspring of consanguineous marriages: 2.81% out of 498 children compared to 1.24% in 1,048 offspring of unrelated parents. Among 373 children whose parents were first cousins the percentage of CHD rose to 3.22. The differences in CHD frequencies were found to be statistically significant. CHD is believed to have a multifactorial background. This study shows that the genetic influence is an important factor in the etiology of such malformations.

Three patients are described who had situs ambiguus and left isomerism (polysplenia syndrome) and advanced atrioventricular block. One presented with a complex bradyarrhythmia with Wenckebach block. The other two had congenital atrioventricular block with a narrow QRS at a ventricular rate of 80 per minute, an atrial rate of 150' per minute, and both had a P wave axis directed superiorly and to the right in one, and superiorly to the left in the other. This ECG pattern was not observed in more than 400 adult patients with complete A-V block treated in our service. It is our opinion that in infants and children with heart disease the presence of complete A-V block with narrow QRS and an unusual P waves axis directed superiorly is strongly suggestive of left isomerism. The incidence rate of complete A-V block in left isomerism is nearly twenty percent of the cases described.

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