Pediatric Cardiology

, Volume 35, Issue 2, pp 253–260 | Cite as

The Spectrum of Congenital Heart Disease and Outcomes After Surgical Repair Among Children With Turner Syndrome: A Single-Center Review

  • Jonathan W. CramerEmail author
  • Peter J. Bartz
  • Pippa M. Simpson
  • Steven D. Zangwill
Original Article


Turner syndrome (TS), a genetic abnormality affecting 1 in 2,500 people, is commonly associated with congenital heart disease (CHD). However, the surgical outcomes for TS patients have not been well described. This study reviewed the spectrum of CHD in TS at the authors’ center. The authors report outcomes after coarctation of the aorta (CoA) repair or staged palliation of hypoplastic left heart syndrome (HLHS) and then compare the surgical outcomes with those of non-TS patients undergoing like repair. This retrospective chart review was conducted at the Children’s Hospital of Wisconsin from 1999 to 2011. Of the 173 patients with TS, 77 (44.5 %) were found to have CHD. Left-sided obstructive lesions were the most common. However, the spectrum of CHD was wide and included systemic and pulmonary venous abnormalities as well as abnormalities of the coronary arteries. In the comparative analysis of CoA repair, the TS patients younger than 60 days had longer aortic cross-clamp times (24 vs. 16 min; p = 0.001) and longer hospital stays (12 vs. 6 days; p ≤ 0.0001) than the non-TS patients. At the follow-up assessment after 8.8 ± 9.1 years, 17 % of the TS patients had hypertension, but no patient had required reintervention, and no deaths had occurred. Finally, three of the four TS patients with HLHS died within the first year. The spectrum of CHD within TS is wide and not limited to bicuspid aortic valve or CoA. Additionally, patients with TS undergoing CoA repair may have a more challenging early postoperative course but experience outcomes similar to those of non-TS patients. Finally, patients who have TS combined with HLHS remain a challenging population with generally poor survival.


Coarctation Congenital heart disease Hypoplastic left heart Outcomes Turner syndrome 


  1. 1.
    Beder SD, Driscoll DJ, McNamara DG, Cooley DA (1982) Coarctation repair in children with Turner’s syndrome. Tex Heart Inst J 9:49–52PubMedCentralPubMedGoogle Scholar
  2. 2.
    Bondy CA (2008) Congenital cardiovascular disease in Turner syndrome. Congenit Heart Dis 3:2–15CrossRefPubMedGoogle Scholar
  3. 3.
    Bondy CA (2008) Aortic dissection in Turner syndrome. Curr Opin Cardiol 23:519–526PubMedCentralCrossRefPubMedGoogle Scholar
  4. 4.
    Brandt B III, Heintz SE, Rose EF, Ehrenhaft JL, Clark EB (1984) Repair of coarctation of the aorta in children with Turner syndrome. Pediatr Cardiol 5:175–177CrossRefPubMedGoogle Scholar
  5. 5.
    Carlson M, Silberbach M (2007) Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature. J Med Genet 44:745–749PubMedCentralCrossRefPubMedGoogle Scholar
  6. 6.
    Forgosh LB, Conetta DA (1992) Cardiac assessment in Turner’s syndrome: a case for the inclusion of coronary angiography. J Am Soc Echocardiogr 5:281–284CrossRefPubMedGoogle Scholar
  7. 7.
    Ghanayem NS, Hoffman GM, Mussatto KA, Frommelt MA, Cava JR, Mitchell ME, Tweddell JS (2010) Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. J Thorac Cardiovasc Surg 140:857–863CrossRefPubMedGoogle Scholar
  8. 8.
    Gøtzsche CO, Krag-Olsen B, Nielsen J, Sørensen KE, Kristensen BO (1994) Prevalence of cardiovascular malformations and association with karyotypes in Turner’s syndrome. Arch Dis Child 71:433–436PubMedCentralCrossRefPubMedGoogle Scholar
  9. 9.
    Ho VB, Bakalov VK, Cooley M, Van PL, Hood MN, Burklow TR, Bondy CA (2004) Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. Circulation 110:1694–1700CrossRefPubMedGoogle Scholar
  10. 10.
    Høimyr H, Christensen TD, Emmertsen K, Johnsen SP, Riis A, Hansen OK, Hjortdal VE (2006) Surgical repair of coarctation of the aorta: up to 40 years of follow-up. Eur J Cardiothorac Surg 30:910–916CrossRefPubMedGoogle Scholar
  11. 11.
    Jacobs JP, O’Brien SM, Chai PJ, Morell VO, Lindberg HL, Quintessenza JA (2008) Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007. Ann Thorac Surg 85:1691–1696 (discussion 1697)CrossRefPubMedGoogle Scholar
  12. 12.
    Jenkins NP, Ward C (1999) Coarctation of the aorta: natural history and outcome after surgical treatment. QJM 92:365–371CrossRefPubMedGoogle Scholar
  13. 13.
    Kim HK, Gottliebson W, Hor K, Backeljauw P, Gutmark-Little I, Salisbury SR, Racadio JM, Helton-Skally K, Fleck R (2011) Cardiovascular anomalies in Turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population. Am J Roentgenol 196:454–460CrossRefGoogle Scholar
  14. 14.
    Luckraz H, Mohammed A, Youhana A (2006) A prominent collateral coronary artery. Ann Thorac Surg 81:1518CrossRefPubMedGoogle Scholar
  15. 15.
    Mazzanti L, Cacciari E (1998) Congenital heart disease in patients with Turner’s syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr 133:688–692CrossRefPubMedGoogle Scholar
  16. 16.
    Morgan T (2007) Turner syndrome: diagnosis and management. Am Fam Physician 76:405–410PubMedGoogle Scholar
  17. 17.
    Oohara K, Yamazaki T, Sakaguchi K, Nakayama M, Kobayashi A (1995) Acute aortic dissection, aortic insufficiency, and a single coronary artery in a patient with Turner’s syndrome. J Cardiovasc Surg Torino 36:273–275PubMedGoogle Scholar
  18. 18.
    Ravelo HR, Stephenson LW, Friedman S, Chatten J, Rashkind WJ, Vidas M, Edmunds LH Jr (1980) Coarctation resection in children with Turner’s syndrome: a note of caution. J Thorac Cardiovasc Surg 80:427–430PubMedGoogle Scholar
  19. 19.
    Reis PM, Punch MR, Bove EL, van de Ven CJ (1999) Outcome of infants with hypoplastic left heart and Turner syndromes. Obstet Gynecol 93:532–535CrossRefPubMedGoogle Scholar
  20. 20.
    Saenger P (1996) Turner’s syndrome. N Engl J Med 335:1749–1754CrossRefPubMedGoogle Scholar
  21. 21.
    Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt CH (2006) Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 91:3897–3902CrossRefPubMedGoogle Scholar
  22. 22.
    Sybert VP (1998) Cardiovascular malformations and complications in Turner syndrome. Pediatrics 101:E11CrossRefPubMedGoogle Scholar
  23. 23.
    Völkl TM, Degenhardt K, Koch A, Simm D, Dörr HG, Singer H (2005) Cardiovascular anomalies in children and young adults with Ullrich–Turner syndrome the Erlangen experience. Clin Cardiol 28:88–92CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Jonathan W. Cramer
    • 1
    • 2
    Email author
  • Peter J. Bartz
    • 1
    • 2
  • Pippa M. Simpson
    • 1
    • 3
  • Steven D. Zangwill
    • 1
    • 2
  1. 1.Medical College of WisconsinMilwaukeeUSA
  2. 2.Children’s Hospital of WisconsinMilwaukeeUSA
  3. 3.Children’s Research InstituteMilwaukeeUSA

Personalised recommendations