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A case of pentalogy of Cantrell with double outlet right ventricle
* For correspondence: a.m.idhrees@gmail.com
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A full term female child of 2.3 kg, presented to the department of Paediatric Surgery, Government Rajaji Hospital, Madurai, Tamil Nadu, India, in December 2013, one hour after birth with external evident beating heart, difficulty in breathing and cyanosis (SpO2 =87%). The child was delivered in a primary health centre via lower section caesarean to a primigravida of age 22 yr. Anterior thoraco-abdominal defect was extending from the angle of Louis to the umbilicus, widest at the diaphragmatic level (15 cm) (Figs 1, 2). The beating heart was visible in the lower part of the defective thoracic cavity extending well below the diaphragm into the abdominal cavity. The protruding part of the liver was covered by a serous membrane. The umbilicus seemed to be shifted more cranially. Echocardiogram revealed a double outlet right ventricle with two normal atrioventricular valves. Since the cyanosis was worsening, she was placed on mechanical ventilation. The child expired after six hours of the extra-uterine life.
- A case of pentalogy of Cantrell with anterior thoroco-abdominal defect extending from angle of Louis to umbilicus. The Right atrium (††) and the Left atrium (†) with a common ventricle (#) can be seen protruding out of the defect. Liver (^^) can be seen bulging through the abdominal defect with the umblicus (++) being shifted more cranially. Maximum width of defect is 15 cm.
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(a) Heart is seen propping about 5 cm above the skin level. (b) Blood vessel attaching to the posterior aspect of the heart (arrow).
