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.
Similar content being viewed by others
References
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
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
Antonelli D, Shmilovitz L, Dharan M (1986) Conjoined hearts. Br Heart J 56:486–488
Baldwin VJ (1993) Pathology of multiple pregnancy. Springers, New York
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
Cywes S, Millar AJW, Rode H, Brown RA (1997) Conjoined twins: the Cape Town experience. Pediatr Surg Internat 12:234–248
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
Edmonds LD, Layde PM (1982) Conjoined twins in the United States, 1970–1977. Teratology 25:301–308
Edwards WD, Hagel DR, Thompson J, Whorton CM, Edwards JE (1977) Conjoined thoracopagus twins. Circulation 56:491–497
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
Hoyle RM (1990) Surgical separation of conjoined twins. Surg Gynecol Obstet 170:549–562
Jenkins EW, Watson TR, Mosenthal WT (1958) Surgery in conjoined twins. Arch Surg 76:35–40
Li SR, Zhou DY, Lu MM, Morrisey EE (2004) Advanced cardiac morphogenesis does not require heart tube fusion. Science 305:1619–1622
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
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
Marin-Padilla M, Chin AJ, Marinpadilla TM (1981) Cardiovascular abnormalities in thoracopagus twins. Teratology 23:101–113
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
McMahon CJ, Spencer R (2006) Congenital heart defects in conjoined twins: outcome after surgical separation of thoracopagus. Pediatr Cardiol 27:1–12
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
Noonan JA (1978) Twins, conjoined twins, and cardiac defects. Am J Dis Child 132:17–18
Reamon-Buettner SM, Borlak J (2006) Hey2 mutations in malformed hearts. Hum Mutat 27:118
Riker W, Traisman H (1964) Conjoined twins: report of three cases. Ill Med J 126:450–454
Seo JW, Shin SS, Chi JG (1985) Cardiovascular system in conjoined twins: an analysis of 14 Korean cases. Teratology 32:151–161
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
Spencer R (2000) Theoretical and analytical embryology of conjoined twins: Part I: Embryogenesis. Clin Anat 13:36–53
Spencer R (2000) Theoretical and analytical embryology of conjoined twins: Part II: Adjustments to union. Clin Anat 13:97–120
Spitz L (2005) Conjoined twins. Prenat Diag 25:814–819
Spitz L, Kiely EM (2002) Experience in the management of conjoined twins. Br J Surg 89:1188–1192
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
Acknowledgments
The authors thank Alvin J. Chin, MD, for his insight in the preparation of this article.
Author information
Authors and Affiliations
Corresponding author
Rights 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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-011-0125-3