Berry Syndrome, a Complex Aortopulmonary Malformation 1


Berry Syndrome, a Complex Aortopulmonary Malformation
Prenatal diagnosis by fetal echocardiography

a spotlight by s. gerboni

Berry syndrome is a rare association of distal aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, patent ductus arteriosus, and interrupted or hypoplastic aortic arch. Since the original description in 1982 was considered a specific syndrome rather than a random coincidence.
The constellation of aortic origin of the right aortopulmonary window has been attributed to the pulmonary artery, aortopulmonary septal defect, diminished blood flow into the right pulmonary artery hypoplasia or interruption of the aortic isthmus, and during fetal life. Being surgically correctable, patent ductus arteriosus was described by Berry in preoperative recognition of all these cardiovascular syndrome is mandatory Only a few neonatal cases have been reported and scattered reports in the literature have confirmed the possibility of correcting surgically this complex malformation Recently Matsubara et all report the first case of a successful prenatal diagnosis of Berry syndrome. Given its complexity and rarity, the disease has not been previously diagnosed by prenatal echocardiography. In the case of Matsubara, found that a characteristic feature of the APW was that the origins of the left and right pulmonary arteries were separated in the three-vessel view on echocardiography.
In Berry syndrome, postnatal pulmonary hypertension or ductus arteriosus closure can lead to high mortality. A few cases have been reported with favorable outcomes due to improved postnatal diagnosis and surgical techniques but we can believe that the chance of achieving a good outcome is improved by a correct preoperative as well an accurate prenatal diagnosis.This is the key to improving the outcome, including avoiding pulmonary hypertension and heart failure.

References

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  • Matsubara Y, Ota M, Bito A, Katayama T, Matsubara K, Ito M.: Prenatal diagnosis of Berry syndrome by fetal echocardiography Ultrasound Obstet Gynecol. 2010 Jan 26
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