Pediatric Cardiology

, Volume 36, Issue 5, pp 987–992 | Cite as

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

  • John P. Costello
  • Allison Weiderhold
  • Clauden Louis
  • Conner Shaughnessy
  • Syed M. Peer
  • David Zurakowski
  • Richard A. Jonas
  • Dilip S. NathEmail author
Original Article


The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution’s Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.


Trisomy 13 Patau syndrome Trisomy 18 Edwards syndrome Congenital heart disease 


Conflict of interest

The authors did not receive any financial support for the study and declare that they have no conflict of interest.


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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • John P. Costello
    • 1
    • 2
  • Allison Weiderhold
    • 1
  • Clauden Louis
    • 1
  • Conner Shaughnessy
    • 1
  • Syed M. Peer
    • 1
  • David Zurakowski
    • 3
  • Richard A. Jonas
    • 1
  • Dilip S. Nath
    • 1
    Email author
  1. 1.Division of Cardiovascular SurgeryChildren’s National Health SystemWashingtonUSA
  2. 2.The Sheikh Zayed Institute for Pediatric Surgical InnovationChildren’s National Health SystemWashingtonUSA
  3. 3.Departments of Anesthesia and Surgery, Boston Children’s HospitalHarvard Medical SchoolBostonUSA

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