Skip to main content
Log in

Conjoined Hearts in Thoracopagus Twins

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children’s Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports. The study group included nine sets of conjoined twins with a mean gestational age at birth of 33.8 ± 5.5 weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22 days (range, 0–345 days). Major congenital heart disease was present in 94.4% (17/18) of the hearts, and 72.2% (13/18) of the hearts had single-ventricle physiology. Total anomalous pulmonary venous return occurred in 39% (7/18) of the cases. The clinical outcome for thoracopagus twins with conjoined hearts remains poor because of inability to separate conjoined and single ventricles. Surgical nonintervention and palliative care should be strongly considered for these patients.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Aeba R, Katogi T, Takeuchi S, Kawada S (1997) Long-term follow-up of surgical patients with single-ventricle physiology: prognostic anatomical determinants. Cardiovasc Surg 5:526–532

    Article  PubMed  CAS  Google Scholar 

  2. Andrews RE, McMahon CJ, Cullen S, Yates RW, de Leval MR, Kiely EM, Spitz L, Sullivan ID (2004) Echocardiographic assessment of conjoined twins. Circulation 110:690

    Google Scholar 

  3. Antonelli D, Shmilovitz L, Dharan M (1986) Conjoined hearts. Br Heart J 56:486–488

    Article  PubMed  CAS  Google Scholar 

  4. Baldwin VJ (1993) Pathology of multiple pregnancy. Springers, New York

    Google Scholar 

  5. Benjamin LC, Nahar J, Sable C, Midgley FM, Di Russo GB (2005) Separation of thoracopagus-cardiopagus twins joined by a myocardial bridge. J Thorac Cardiovasc Surg 130:1212–1213

    Article  PubMed  Google Scholar 

  6. Cywes S, Millar AJW, Rode H, Brown RA (1997) Conjoined twins: the Cape Town experience. Pediatr Surg Internat 12:234–248

    CAS  Google Scholar 

  7. Donovan J, Kordylewska A, Jan YN, Utset MF (2002) Tetralogy of Fallot and other congenital heart defects in Hey2 mutant mice. Curr Biol 12:1605–1610

    Article  PubMed  CAS  Google Scholar 

  8. Edmonds LD, Layde PM (1982) Conjoined twins in the United States, 1970–1977. Teratology 25:301–308

    Article  PubMed  CAS  Google Scholar 

  9. Edwards WD, Hagel DR, Thompson J, Whorton CM, Edwards JE (1977) Conjoined thoracopagus twins. Circulation 56:491–497

    PubMed  CAS  Google Scholar 

  10. Geroulanos S, Jaggi F, Wydler J, Lachat M, Cakmakci M (1993) Thoracopagus symmetricus: on the separation of Siamese twins in the 10th century A.D. by Byzantine physicians. Gesnerus 50(Pt 3–4):179–200

    PubMed  Google Scholar 

  11. Hoyle RM (1990) Surgical separation of conjoined twins. Surg Gynecol Obstet 170:549–562

    PubMed  CAS  Google Scholar 

  12. Jenkins EW, Watson TR, Mosenthal WT (1958) Surgery in conjoined twins. Arch Surg 76:35–40

    Article  CAS  Google Scholar 

  13. Li SR, Zhou DY, Lu MM, Morrisey EE (2004) Advanced cardiac morphogenesis does not require heart tube fusion. Science 305:1619–1622

    Article  PubMed  CAS  Google Scholar 

  14. Lin CR, Kioussi C, O’Connell S, Briata P, Szeto D, Liu F, Izpisua-Belmonte JC, Rosenfeld MG (1999) Pitx2 regulates lung asymmetry, cardiac positioning, and pituitary and tooth morphogenesis. Nature 401:279–282

    Article  PubMed  CAS  Google Scholar 

  15. Lodge AJ, Rychik J, Nicolson SC, Ittenbach RF, Spray TL, Gaynor JW (2004) Improving outcomes in functional single-ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg 78:1688–1695

    Article  PubMed  Google Scholar 

  16. Marin-Padilla M, Chin AJ, Marinpadilla TM (1981) Cardiovascular abnormalities in thoracopagus twins. Teratology 23:101–113

    Article  PubMed  CAS  Google Scholar 

  17. Mathewson JW, Waldman JD, George L, Kirkpatrick SE, Turner SW, Pappelbaum SJ (1984) Shared coronary arteries and coronary venous drainage in thoracopagus twins. J Am Coll Cardiol 3:1019–1025

    Article  PubMed  CAS  Google Scholar 

  18. McMahon CJ, Spencer R (2006) Congenital heart defects in conjoined twins: outcome after surgical separation of thoracopagus. Pediatr Cardiol 27:1–12

    Article  PubMed  CAS  Google Scholar 

  19. Nichols B, Blattner R, Rudolph A (1967) General clinical management of thoracopagus twins. In: Bergsma D, Blattner R, Nichols B, Rudolph A (eds) Conjoined twins. The National Foundation, New York, pp 38–51

    Google Scholar 

  20. Noonan JA (1978) Twins, conjoined twins, and cardiac defects. Am J Dis Child 132:17–18

    PubMed  CAS  Google Scholar 

  21. Reamon-Buettner SM, Borlak J (2006) Hey2 mutations in malformed hearts. Hum Mutat 27:118

    Article  PubMed  Google Scholar 

  22. Riker W, Traisman H (1964) Conjoined twins: report of three cases. Ill Med J 126:450–454

    Google Scholar 

  23. Seo JW, Shin SS, Chi JG (1985) Cardiovascular system in conjoined twins: an analysis of 14 Korean cases. Teratology 32:151–161

    Article  PubMed  CAS  Google Scholar 

  24. Sinzobahamvya N, Arenz C, Reckers J, Photiadis J, Murin P, Schindler E, Hraska V, Asfour B (2009) Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle. Cardiol Young 19:594–600

    Article  PubMed  Google Scholar 

  25. Spencer R (2000) Theoretical and analytical embryology of conjoined twins: Part I: Embryogenesis. Clin Anat 13:36–53

    Article  PubMed  CAS  Google Scholar 

  26. Spencer R (2000) Theoretical and analytical embryology of conjoined twins: Part II: Adjustments to union. Clin Anat 13:97–120

    Article  PubMed  CAS  Google Scholar 

  27. Spitz L (2005) Conjoined twins. Prenat Diag 25:814–819

    Article  Google Scholar 

  28. Spitz L, Kiely EM (2002) Experience in the management of conjoined twins. Br J Surg 89:1188–1192

    Article  PubMed  CAS  Google Scholar 

  29. Synhorst D, Matlak M, Roan Y, Johnson D, Byrne J, Mcgough E (1979) Separation of conjoined thoracopagous twins joined at the right atria. Am J Cardiol 43:662–665

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

The authors thank Alvin J. Chin, MD, for his insight in the preparation of this article.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to R. Thomas Collins II.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Thomas Collins, R., Weinberg, P.M., Gruber, P.J. et al. Conjoined Hearts in Thoracopagus Twins. Pediatr Cardiol 33, 252–257 (2012). https://doi.org/10.1007/s00246-011-0125-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-011-0125-3

Keywords

Navigation