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.


  • Berry TE, Bharati S, Muster AJ, et al. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery,
    intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome.
    Am J Cardiol 1982;49:108–16.
  • 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
  • Chiu I, Wang J, Wang M, Wang C. One-stage repair of aortopulmonary septal defect and interrupted aortic arch.
    Ann Thorac Surg 1994;58:1529–32.
  • Pietro A. Abbruzzese, MD, Maurizio Merlo, MD, Enrico Chiappa, MD, Renzo Bianco, MD, Federica Ferrero,
    MD, and Carlo M. Cappone, MDBerry Syndrome, a Complex Aortopulmonary Malformation: One-Stage Repair in a Neonate
    Ann Thorac Surg 1997;64:1167–9
  • Sano S, Mee RBB. Isolated myocardial perfusion during arch repair. Ann Thorac Surg 1990;49:970–2.
  • Burke RP, Rosenfeld HM. Primary repair of aortopulmonary septal defect, interrupted aortic arch and anomalous
    origin of the right pulmonary artery. Ann Thorac Surg 1994; 58:543–5.
  • Boonstra PW, Talsma M, Ebels T. Interruption of the aortic arch, distal aortopulmonary window, arterial duct and aortic origin of the right pulmonary artery in a neonate: report of a case successfully repaired in a one-stage operation. Int J Cardiol 1992;34:108–10.
  • Kitagawa T, Katoh I, Taki H, et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg 1991;51:680–2.
  • Alborino D, Guccione P, Di Donato R, Marino B. Aortopulmonary window coexisting with
    tetralogy of Fallot. J Cardiovasc Surg 2001;42:197-199.
  • Kuehn A, Oberhoffer R, Vogt M, Lange R, Hess J. Aortopulmonary window with
    ventricular septal defect and pulmonary atresia: prenatal diagnosis and successful early
    surgical correction. Ultrasound Obstet Gynecol 2004;24:793-796.
  • Park SY, Joo HC, Youn YN, Park YH, Park HK. Berry syndrome: two cases of successful
    surgical repair. Circ J 2008;72:492-495.

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