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J Am Coll Cardiol, 1998; 32:758-765
© 1998 by the American College of Cardiology Foundation
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CLINICAL STUDIES

Long-term outcome after the mustard repair for simple transposition of the great arteries

28-year follow-up

Nigel J. Wilson, MB*, Patricia M. Clarkson, MB*, Brian G. Barratt-Boyes, ChM{dagger}, A. Louise Calder, MD*, Ralph M. L. Whitlock, MB{ddagger}, Ronald N. Easthope, MB§ and John M. Neutze, MD*

* Cardiology Department, Green Lane Hospital, Auckland, New Zealand
{dagger} Cardiothoracic Surgical Unit, Green Lane Hospital, Auckland, New Zealand
{ddagger} Clinical Physiology Department, Green Lane Hospital, Auckland, New Zealand
§ Cardiology Department, Wellington Hospital, Wellington, New Zealand

Manuscript received October 28, 1997; revised manuscript received May 6, 1998, accepted May 20, 1998.

Address for correspondence: Dr. Nigel Wilson, Cardiology Department, Green Lane Hospital, Private Bag 92 189, Auckland 1030, New Zealand
nigelw{at}ahsl.co.nz

Objectives. This study examines the late outcome in patients with simple transposition of the great arteries (TGA) after a Mustard operation.

Background. Continuing medical follow-up for patients after the Mustard procedure, now extending to three decades, is required. The quality of life of adult survivors has not been well documented.

Methods. Survival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assessed by medical review and a lifestyle questionnaire. The incidence of right ventricular failure and echocardiographic right ventricular dysfunction (RVD) were determined. A measure of lifestyle, the ability index, was determined.

Results. Actuarial survival was 90%, 80%, and 80% at 10, 20, and 28 years, respectively, with 76% of survivors being New York Heart Association class 1. Sudden death, with an incidence of 7% without identifiable risk factors, was the most common cause of late demise. RVD was identified in 18% of patients who had echocardiography, but there was right ventricular failure in only two patients. Seventy-five percent of current survivors lead a normal life, 20% have some symptoms or lifestyle modification, and 5% are unable to work.

Conclusions. The survival of patients to 28 years with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date. The quality of life of late survivors is good, most achieving a normal level of education and employment.

Abbreviations and Acronyms
  LVEF = left ventricular ejection fraction
  LVOTO = left ventricular outflow trace obstruction
  NYHA = New York Heart Association
  PDA = patent ductus arteriosus
  PVRI = pulmonary vascular resistance index
  RVD = right ventricular dysfunction
  RVEF = right ventricular ejection fraction
  TGA = transposition of the great arteries
  VSD = ventricular septal defect




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