BACKGROUND Heterotaxy has been demonstrated to reduce survival. There are several different subgroups of patients, however, and no single study has had a large number of patients and analyzed survival across the different subgroups such as patients born in different eras, patients with right and left isomerism, and patients with biventricular or functionally univentricular hearts. This study po...

So-called "heterotaxy" is a laterality defect characterized by isomerism of the thoracic organs and random arrangement of the abdominal organs. These findings go beyond anatomic curiosity and have functional implications. It is, thus, of the utmost importance to be able to properly identify these findings. Radiologic studies can be invaluable in determining anomalies in the central nervous, pul...

The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as "heterotaxy." We have previously shown that, when assessed on the basis of the extent of the pectinate muscles relative to the atrioventri...

The atrial morphology and venous connections were assessed "blind" in 51 necropsy specimens from patients with visceral heterotaxy. This was compared with bronchial morphology as established by dissection. Six specimens were found to have both atria and bronchi in situs solitus or inversus, and were rejected. In the remainder, atrial isomerism was diagnosed, though this required minor revision ...

In a series of more than 3500 pregnancies referred for fetal echocardiography, 29 cases of atrioventricular septal defect were detected in the fetus. There was a chromosomal anomaly in 14 of these cases, left atrial isomerism in 12, and right atrial isomerism in two. Complete heart block was found in 11 of the cases with left atrial isomerism. Many associated cardiac abnormalities were found, p...

BACKGROUND The location of the sinus node is known to be at best abnormal, or at worst unknown, in patients with isomerism of the morphologically left atrial appendage. In contrast, the sinus node is known to be an excellent histological marker of the morphologically right appendage, being duplicated in those with right isomerism. The aim of the study was to investigate this condition further i...

We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, an...