Pediatric Cardiology

, Volume 33, Issue 1, pp 192–200 | Cite as

Various Modalities for Evaluation of a Fused Heart in Conjoined Twins

  • Hao-Chuan Liu
  • Chiao-Wei Lo
  • Zen-Chung Weng
  • Betau Hwang
  • Pi-Chang Lee
Case Report

Abstract

Conjoined twins are a rare congenital anomaly with an estimated incidence of 1/50,000 to 1/100,000. Among thoracopagus conjoined twins, 75% have a fused heart. We compare the usefulness of various modalities for evaluating cardiovascular structure in fused-heart conjoined twins. We report a series of 20 sets of thoracopagus conjoined twins as well as the results of a PubMed database literature review literature from 1982 to 2009. Twenty sets of fused-heart thoracopagus conjoined twins were evaluated by echocardiography, cardiac catheterization, magnetic resonance image (MRI), and three-dimensional computed tomography angiography (3D-CTA). Imaging results were compared to findings at surgery or autopsy. All sets of conjoined twins underwent postnatal echocardiography; 11 sets (55%) underwent cardiac catheterization; 4 sets (20%) underwent MRI; and 1 set (5%) underwent 3D-CTA. All intracardiac anatomy (ICA) was identified by echocardiography. Cardiac catheterization, MRI, and 3D-CTA were able to identify extracardiac vascular structures as well as the ICA. 3D-CTA, which can be performed as early as the first week of life, is a noninvasive, less expensive, and the safe examination with minimal risk due to its short procedural time. Three-dimensional CTA is an effective and safe modality for evaluating the cardiovascular anatomy of fused-heart conjoined twins before surgery.

Keywords

Anatomy Computed tomography Fused-heart conjoined twins Imaging Thoracopagus 

Notes

Acknowledgments

The authors sincerely thank Dr. Ying-Ju Kuo for providing the anatomic pathology pictures. We also thank Dr. Shu-Jen Chen for the conjoined twins’ neonatal intensive care consultation.

References

  1. 1.
    Andrews RE, McMahon CJ, Yates RW, Cullen S, de Leval MR, Kiely EM et al (2006) Echocardiographic assessment of conjoined twins. Heart 92:382–387PubMedCrossRefGoogle Scholar
  2. 2.
    Barth RA, Filly RA, Goldberg JD, Moore P, Silverman NH (1990) Conjoined twins: prenatal diagnosis and assessment of associated malformations. Radiology 177:201–207PubMedGoogle Scholar
  3. 3.
    Bean MJ, Pannu H, Fishman EK (2005) Three-dimensional computed tomographic imaging of complex congenital cardiovascular abnormalities. J Comput Assist Tomogr 29:721–724PubMedCrossRefGoogle Scholar
  4. 4.
    Becker C, Soppa C, Fink U, Haubner M, Muller-Lisse U, Englmeier KH et al (1997) Spiral CT angiography and 3D reconstruction in patients with aortic coarctation. Eur Radiol 7:1473–1477PubMedCrossRefGoogle Scholar
  5. 5.
    Benavidez OJ, Gauvreau K, Jenkins KJ, Geva T (2008) Diagnostic errors in pediatric echocardiography: development of taxonomy and identification of risk factors. Circulation 117:2995–3001PubMedCrossRefGoogle Scholar
  6. 6.
    Bennett D, Marcus R, Stokes M (2005) Incidents and complications during pediatric cardiac catheterization. Paediatr Anaesth 15:1083–1088PubMedGoogle Scholar
  7. 7.
    Carnevale FC, Borges MV, Affonso BB, Pinto RA, Tannuri U, Maksoud JG (2006) Importance of angiographic study in preoperative planning of conjoined twins: case report. Clinics 61:167–170PubMedCrossRefGoogle Scholar
  8. 8.
    Casele HL, Meyer JR (2000) Ultrafast magnetic resonance imaging of cephalopagus conjoined twins. Obstet Gynecol 95:1015–1017PubMedCrossRefGoogle Scholar
  9. 9.
    Chun E, Vade A, Rizvi MD, Elsayed H (1999) Imaging of an unusual case of parasitic twinning. Comput Med Imaging Graph 23:219–222PubMedCrossRefGoogle Scholar
  10. 10.
    Dillman JR, Hernandez RJ (2009) Role of CT in the evaluation of congenital cardiovascular disease in children. AJR Am J Roentgenol 192:1219–1231PubMedCrossRefGoogle Scholar
  11. 11.
    Dorfman AL, Levine JC, Colan SD, Geva T (2005) Accuracy of echocardiography in low birth weight infants with congenital heart disease. Pediatrics 115:102–107PubMedGoogle Scholar
  12. 12.
    Gaca AM, Jaggers JJ, Dudley LT, Bisset GS 3rd (2008) Repair of congenital heart disease: a primer-part 1. Radiology 247:617–631PubMedCrossRefGoogle Scholar
  13. 13.
    Gilbert-Barness E, Debich-Spicer D, Opitz JM (2003) Conjoined twins: morphogenesis of the heart and a review. Am J Med Genet A 120A:568–582PubMedCrossRefGoogle Scholar
  14. 14.
    Hollingsworth CL, Yoshizumi TT, Frush DP, Chan FP, Toncheva G, Nguyen G et al (2007) Pediatric cardiac-gated CT angiography: assessment of radiation dose. AJR Am J Roentgenol 189:12–18PubMedCrossRefGoogle Scholar
  15. 15.
    Hopkins KL, Patrick LE, Simoneaux SF, Bank ER, Parks WJ, Smith SS (1996) Pediatric great vessel anomalies: initial clinical experience with spiral CT angiography. Radiology 200:811–815PubMedGoogle Scholar
  16. 16.
    Katz M, Konen E, Rozenman J, Szeinberg A, Itzchak Y (1995) Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies. J Comput Assist Tomogr 19:564–568PubMedCrossRefGoogle Scholar
  17. 17.
    Kelekis L, Kelekis D, Mameletzis K, Kelemouridis B, Artopoulos J (1980) Thoracopagus twins studied with computed tomography. J Comput Assist Tomogr 4:405–406PubMedCrossRefGoogle Scholar
  18. 18.
    Lardo AC (2000) Real-time magnetic resonance imaging: diagnostic and interventional applications. Pediatr Cardiol 21:80–98PubMedCrossRefGoogle Scholar
  19. 19.
    Mann MD, Coutts JP, Kaschula RO, Fraser CB, Fisher RM, Cywes S (1983) The use of radionuclides in the investigation of conjoined twins. J Nucl Med 24:479–484PubMedGoogle Scholar
  20. 20.
    Marin-Padilla M, Chin AJ, Marin-Padilla TM (1981) Cardiovascular abnormalities in thoracopagus twins. Teratology 23:101–113PubMedCrossRefGoogle Scholar
  21. 21.
    McAdams RM, Milhoan KA, Hall BH, Richardson RG (2004) Prenatal and postnatal imaging of thoracopagus conjoined twins with a shared six-chamber heart. Pediatr Radiol 34:816–819PubMedCrossRefGoogle Scholar
  22. 22.
    McHugh K, Kiely EM, Spitz L (2006) Imaging of conjoined twins. Pediatr Radiol 36:899–910; quiz 1002–1003Google Scholar
  23. 23.
    McMahon CJ, Mullins CE, Vick GW 3rd, Caville CC, Bezold LI, Nihill MR (2000) Cardiac catheterization in diagnosis and management of congenital heart disease in thoracopagus conjoined twins. Catheter Cardiovasc Interv 51:159–167PubMedCrossRefGoogle Scholar
  24. 24.
    Mehta R, Lee KJ, Chaturvedi R, Benson L (2008) Complications of pediatric cardiac catheterization: a review in the current era. Catheter Cardiovasc Interv 72:278–285PubMedCrossRefGoogle Scholar
  25. 25.
    Rossi P, Cozzi F, Iannaccone G (1981) CT for assessing feasibility of separation of thoracopagus twins. J Comput Assist Tomogr 5:574–576PubMedCrossRefGoogle Scholar
  26. 26.
    Sanders SP, Chin AJ, Parness IA, Benacerraf B, Greene MF, Epstein MF et al (1985) Prenatal diagnosis of congenital heart defects in thoracoabdominally conjoined twins. N Engl J Med 313:304–370CrossRefGoogle Scholar
  27. 27.
    Sarikouch S, Schaeffler R, Korperich H, Dongas A, Haas NA, Beerbaum P (2009) Cardiovascular magnetic resonance imaging for intensive care infants: safe and effective? Pediatr Cardiol 30:146–152PubMedCrossRefGoogle Scholar
  28. 28.
    Spencer R (1996) Anatomic description of conjoined twins: a plea for standardized terminology. J Pediatr Surg 31:941–944PubMedCrossRefGoogle Scholar
  29. 29.
    Spitz L (1996) Conjoined twins. Br J Surg 83:1028–1030PubMedCrossRefGoogle Scholar
  30. 30.
    Spitz L, Kiely EM (2002) Experience in the management of conjoined twins. Br J Surg 89:1188–1192PubMedCrossRefGoogle Scholar
  31. 31.
    Spitz L, Kiely EM (2003) Conjoined twins. JAMA 289:1307–1310PubMedCrossRefGoogle Scholar
  32. 32.
    Szmuk P, Rabb MF, Curry B, Smith KJ, Lantin-Hermoso MR, Ezri T (2006) Anaesthetic management of thoracopagus twins with complex cyanotic heart disease for cardiac assessment: special considerations related to ventilation and cross-circulation. Br J Anaesth 96:341–345PubMedCrossRefGoogle Scholar
  33. 33.
    Tavli V, Kayhan B, Okur FF, Kirman M, Tekdogan M (2000) Complications of pediatric cardiac catheterization: 18-month study. Turk J Pediatr 42:294–297PubMedGoogle Scholar
  34. 34.
    Tsai IC, Chen MC, Jan SL, Wang CC, Fu YC, Lin PC et al (2008) Neonatal cardiac multidetector row CT: why and how we do it. Pediatr Radiol 38:438–451PubMedCrossRefGoogle Scholar
  35. 35.
    Vitiello R, McCrindle BW, Nykanen D, Freedom RM, Benson LN (1998) Complications associated with pediatric cardiac catheterization. J Am Coll Cardiol 32:1433–1440PubMedCrossRefGoogle Scholar
  36. 36.
    Zhang YQ, Wu LP, Wu LJ, Chen GZ, Sun K, Zhang ZF, Shen R (2009) Echocardiographic assessment of conjoined twins with congenital heart disease in Shanghai. Echocardiography 26:691–698PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Hao-Chuan Liu
    • 1
  • Chiao-Wei Lo
    • 2
  • Zen-Chung Weng
    • 4
  • Betau Hwang
    • 5
  • Pi-Chang Lee
    • 3
  1. 1.Department of PediatricsTaipei Veterans General Hospital Yuanshan BranchTaiwanRepublic of China
  2. 2.Department of PediatricsNational Yang-Ming University HospitalTaipei, TaiwanRepublic of China
  3. 3.Division of Pediatric Cardiology, Department of PediatricsTaipei Veterans General Hospital and National Yang-Ming UniversityTaipei, TaiwanRepublic of China
  4. 4.Department of SurgeryTaipei Veterans General Hospital and National Yang-Ming UniversityTaipei, TaiwanRepublic of China
  5. 5.Taipei City Hospital Zhongxiao BranchTaipei, TaiwanRepublic of China

Personalised recommendations