Skip to main content
SpringerLink
Log in

Berry syndrome—a rare congenital cardiac anomaly

  • Review Article
  • Published:
Indian Journal of Thoracic and Cardiovascular Surgery Aims and scope Submit manuscript

Abstract

Berry syndrome is a rare congenital cardiac anomaly, characterized by distal aortopulmonary window, hypoplasia or interruption of the aortic arch, intact ventricular septum, and aortic origin of the right pulmonary artery and patent ductus arteriosus. Anatomic depiction of each component is important for the diagnosis. Single-stage surgical repair is challenging but feasible with good survival outcomes. The available literature on this anomaly is limited. Hence, this paper aims at reviewing the literature on Berry syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. 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.

    Article  CAS  Google Scholar 

  2. Hu R, Zhang W, Liu X, Dong W, Zhu H, Zhang H. Current outcomes of one-stage surgical correction for Berry syndrome. J Thorac Cardiovasc Surg. 2017;153:1139–47.

    Article  Google Scholar 

  3. Murin P, Sinzobahamvya N, Ch Blaschczok H, et al. Aortopulmonary window associated with interrupted aortic arch: report of surgical repair of eight cases and review of literature. Thorac Cardiovasc Surg.2012; 60:215–220.

  4. Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol. 1987;59:443–7.

    Article  CAS  Google Scholar 

  5. Jayaram N, Knowlton J, Shah S, Gelatt M, Lofland G, Raghuveer G. Berry syndrome: a possible genetic link. Pediatr Cardiol. 2013;34:1511–3.

    Article  Google Scholar 

  6. Yang YL, Wang XF, Xie MX, et al. Echocardiographic diagnosis of Berry syndrome. Chin J Ultrasonogr. 2008;17:926–9.

    Google Scholar 

  7. Zhang X, Liu X-W, Gu X-Y, et al. Prenatal diagnosis of Berry syndrome by fetal echocardiography: A report of four cases. Echocardiography. 2018;35:563–565.

  8. Li J, Yang Y, Duan X, et al. Berry syndrome: a rare cardiac malformation with extra-cardiac findings. Sci China Life Sci. 2017;60:772–4.

    Article  Google Scholar 

  9. Mannelli L, Mosca R, Henry G, Srichai-Parsia MB. A case of Berry syndrome type 2B. Congenit Heart Dis. 2011;6:389–92.

    Article  Google Scholar 

  10. Ding W-X, Su Z-K, Cao D-F, Jonas RA. One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch. Ann Thorac Surg.1990;49:664–666.

  11. Dietl CA, Torres AR. Repair of interrupted aortic arch with an augmented aortic anastomosis. Ann Thorac Surg. 1993;56:142–8.

    Article  CAS  Google Scholar 

  12. Jonas RA. Modified arch anastomosis for interrupted aortic arch. Ann Thorac Surg. 1993;56:5–6.

  13. Yoo SJ, Choi HY, Park IS, Hong CY, Song MG, Kim SH. Distal aortopulmonary window with aortic origin of the right pulmonary artery and interruption of the aortic arch (Berry syndrome): diagnosis by MR imaging. AJR Am J Roentgenol. 1991;157:835–6.

  14. Alva-Espinosa C, Jiménez-Arteaga S, Díaz-Díaz E, et al. Diagnosis of berry syndrome in an infant by two-dimensional and color Doppler echocardiography. Pediatr Cardiol. 1995;16: 42–44.

  15. Ghelani SJ, Quinonez LG, Rathod RH. Prenatal diagnosis and management of Berry syndrome, a rare conotruncal anatomy. Circulation. 2015;132:1593–4.

    Article  Google Scholar 

  16. Chang YH, Sung SC, Kim H, Lee HD. Anterior translocation of the right pulmonary artery for relief of airway compression in the repair of distal aortopulmonary window and interrupted aortic arch. Ann Thorac Surg. 2012;93:e159–e61.

    Article  Google Scholar 

  17. 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;58:543-545.

  18. Kitagawa T, Katoh I, Taki H, et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg. 1991;51:680–2.

    Article  CAS  Google Scholar 

  19. Codispoti M, Mankad PS. One-stage repair of interrupted aortic arch, aortopulmonary window, and anomalous origin of right pulmonary artery with autologous tissues. Ann Thorac Surg. 1998;66:264-267.

  20. Abbruzzese PA, Merlo M, Chiappa E, Bianco R, Ferrero F, Cappone CM. Berry syndrome, a complex aortopulmonary malformation: one-stage repair in a neonate. Ann Thorac Surg. 1997;64:1167–9.

    Article  CAS  Google Scholar 

  21. Alsoufi B, Schlosser B, McCracken C, et al. Current outcomes of surgical management of aortopulmonary window and associated cardiac lesions. Ann Thorac Surg. 2016;102:608–14.

    Article  Google Scholar 

  22. Konstantinov IE, Karamlou T, Williams WG, et al. Surgical management of aortopulmonary window associated with interrupted aortic arch: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg. 2006;131:1136–41.e2.

  23. Park SY, Joo H-C, Youn Y-N, Park Y-H, Park HK. Berry syndrome: two cases of successful surgical repair. Circ J. 2008;72:492–495.

  24. Sharma J, Saleh M, Das BB. Berry syndrome with trisomy 13. Pediatr Cardiol. 2002;23:205–9.

    Article  CAS  Google Scholar 

  25. Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40-year experience. Eur J Cardiothorac Surg. 2002;21:773–9.

    Article  CAS  Google Scholar 

  26. Senzaki H, Asano H, Masutani S, et al. Anomalous origin of the left coronary artery from the main pulmonary artery associated with Berry syndrome. J Thorac Cardiovasc Surg. 2003;126:1645–7.

    Article  Google Scholar 

  27. Fong N-c, Kong C-t, Mak W-y, et al. Early detection of Berry syndrome in a newborn with differential cyanosis. Chin Med J (Engl). 2006;119:1485–8.

    Article  Google Scholar 

  28. Konstantinov IE, Oka N, d’Udekem Y, Brizard CP. Surgical repair of aortopulmonary window associated with interrupted aortic arch: long-term outcomes. J Thorac Cardiovasc Surg. 2010;140:483–4.

    Article  Google Scholar 

  29. Morito H, Masuzawa A, Kobayashi J, Takeuchi K. One-stage surgical repair for Berry syndrome with preoperative diagnosis by 3-dimensional CT. World J Pediatr Congenit Heart Surg. 2011;2:491–494.

  30. Liu Y , Cheng L, Qian X, et al. Surgical correction of anomalous origin of one pulmonary artery without grafts in infants. J Card Surg. 2015;30:85–91.

  31. Remon JI, Briston DA, Stern KW. Berry syndrome: the importance of genetic evaluation before surgical intervention. Cardiol Young. 2016;26:188–90.

  32. Duyen MD, Haranal MY, Dillon J, Sivalingam S. A rare complication of myocardial ischaemia following single-stage repair in a case of Berry syndrome. Interact Cardiovasc Thorac Surg. 2020;31:576–7.

Download references

Funding

None.

Author information

Authors and Affiliations

  1. Department of Cardiac Surgery, National Heart Institute, 145, Jalan Tun Razak, 50400, Kuala Lumpur, Malaysia

    Maruti Haranal, Duyen Mai Dinh & Sivakumar Sivalingam

  2. Department of Pediatric Cardiac Surgery, Amrita Institute of Medical Sciences, Kerala, Kochi, India

    Balaji Srimurugan

Authors
  1. Maruti Haranal
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Balaji Srimurugan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Duyen Mai Dinh
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sivakumar Sivalingam
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maruti Haranal.

Ethics declarations

Ethics committee approval

Not required.

Informed consent

Not applicable.

Statement of human and animal rights

No animals involved.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Haranal, M., Srimurugan, B., Dinh, D.M. et al. Berry syndrome—a rare congenital cardiac anomaly. Indian J Thorac Cardiovasc Surg 37, 526–532 (2021). https://doi.org/10.1007/s12055-021-01206-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12055-021-01206-0

Keywords

Search

Navigation