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 ,
A. Louise Calder, MD*,
Ralph M. L. Whitlock, MB ,
Ronald N. Easthope, MB and
John M. Neutze, MD*
* Cardiology Department, Green Lane Hospital, Auckland, New Zealand
Cardiothoracic Surgical Unit, Green Lane Hospital, Auckland, New Zealand
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.
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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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