Double outlet right ventricle is that condition in which both arterial trunks emerge
almost completely or completely from the right ventricle. This diverse group of cardiac
malformations are characterized by the relationship of the great arteries to the
ventricular septal defect and the presence or absence of valve or subvalvular pulmonic
stenosis.
The terminal portion of the hindgut is called the cloaca. The cloaca is deviled by a
coronal sheet or wedge of mesenchyme, the urorectal septum, which develops in the
angle between the allantois and the hindgut at 4weeks. By the end of the sixth week,
the urorectal septum has fused with the cloacal membrane deciding the cloaca into
urogenital sinus anti the rectum. If the urorectal septum does not develop and the
cloacal membrane persist, an anomaly that the urogenital system arid the digestive
system are connected with each other is developed.
We report an autopsy case of double outlet right ventricle and imperforated cloacal
membrane with cystic dilatation. This case was accompanied by atrial septal defect,
patent ductus arteriosus, agenesis of the right ear, left lower met ear with hypogenesis,
lordosis, focal defect of the anterior abdominal wall defect just below umbilicus.
The aorta was dextroposed and it arouse from the right ventricle, right to the
pulmonary artery. The pulmonary artery was in normal position and the pulmonary
stenosis was not found. The atrial septal defect and patent ductus arteriosus were also
found. The baby had rudimentary phallus without opening. The abdomen wart markedly
dilate6 due to a cystic mass in the abdominal cavity. Both ureters and the large
intestine drained into the cystic structure and the distal part of the left ureter was
dilated. There were neither urethral opening nor anal opening connected to the outside.
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