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J Am Coll Cardiol, 1999; 34:545-553
© 1999 by the American College of Cardiology Foundation
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CLINICAL STUDIES

Factors associated with outcomes of persistent truncus arteriosus

Joyce M. Williams, MSca,b, Maaike de Leeuwa,b, Michael D. Black, MDa,b, Robert M. Freedom, MD, FACCa,b, William G. Williams, MDa,b and Brian W. McCrindle, MD, MPH, FACCa,b

a Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
b Division of Cardiovascular Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

Manuscript received September 28, 1998; revised manuscript received January 25, 1999, accepted April 26, 1999.

Reprint requests and correspondence: Dr. Brian W. McCrindle, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8
brian.mccrindle{at}sickkids.on.ca

OBJECTIVES

The purpose of this study was to identify trends and factors associated with outcomes of persistent truncus arteriosus (PTA).

BACKGROUND

Although there have been significant improvements, PTA continues to be associated with significant morbidity and mortality.

METHODS

We undertook a review of all consecutive cases of PTA (n = 205) presenting at our institution from 1953 to 1997. Data were collected regarding demographics, anatomy, management (surgical palliation and repair) and outcomes (mortality and reoperation).

RESULTS

Significant trends (p ≤ 0.001) related to groups defined by year of birth were as follows: number of cases (1953–1967, n = 13; 1968–1977, n = 42; 1978–1987, n = 69; 1988–1997, n = 81), median age at first assessment (8 months, 42 days, 7 days and 2 days, respectively), proportion who did not have any surgery (58%, 27%, 22% and 11%), proportion who had an initial palliative procedure (25%, 37%, 6% and 2%), proportion who underwent PTA repair (31%, 59%, 72% and 88%), median age at PTA repair (11.2 years, 1.1 years, 1.6 months and 12 days) and proportion dying before hospital discharge after repair (50%, 63%, 56% and 41%). Since 1995, mortality before hospital discharge after repair has further decreased to 2/11 (18%). Increasing time to initial conduit replacement in hospital survivors was significantly related to larger sized conduit at repair (p = 0.02) and use of pulmonary homografts (vs. aortic homografts or xenografts; p = 0.002). Interventional catheterization to address conduit obstructions significantly increased conduit longevity.

CONCLUSIONS

Significant improvements in PTA outcomes are evident with trends toward earlier age at assessment and complete repair.

Abbreviations and Acronyms
  PA = pulmonary artery
  PTA = persistent truncus arteriosus
  RV = right ventricle




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