The long‐term functional outcome in Mustard patients study: Another decade of follow‐up |
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Authors: | Nayan T Srivastava Roger Hurwitz W Aaron Kay George J Eckert Alisha Kuhlenhoelter Nicole DeGrave Eric S Ebenroth |
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Institution: | 1.Department of Pediatrics, Eskenazi Health,
Indianapolis, Indiana
2 Pediatric Cardiology, Riley Hospital for
Children, Indianapolis, Indiana
3 Department of Pediatrics, Indiana
University, Indianapolis, Indiana
4 Indiana University School of Medicine,
Indianapolis, Indiana
5 Department of Biostatistics, Indiana
University‐Purdue University Indianapolis,
Indianapolis, Indiana
6 Department of Pediatrics, Pediatric
Cardiology, Indiana University, Indianapolis,
Indiana |
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Abstract: | Objective: For over 20 years, we have followed a cohort of patients who underwent
the Mustard procedure for d‐transposition of the great arteries. The current study
follows the same cohort from our last study in 2007 to reassess their functional ca‐
pacity and quality of life.
Participants: Of the original 45 patients, six patients have required cardiac transplant
and 10 patients have died, including two of the transplanted patients. Twenty‐five of
the remaining patients agreed to participate in this current study.
Design: Patients underwent comparable testing to the previous studies when possi‐
ble including exercise stress testing, echocardiography, MRI or CT evaluation of car‐
diac anatomy and function, Holter monitor, and quality of life questionnaire.
Results: Thirty‐one percent of patients have experienced cardiac death either in the
form of mortality or cardiac transplantation. The major cause of death was systemic
right ventricular failure. Sixty‐five percent have continuing abnormalities of rhythm.
Exercise time and workload showed a statistically significant decrease from the origi‐
nal study (Time 1) to both 10‐year (Time 2) and 20‐year (Time 3) follow‐up points.
Right ventricular ejection fraction decreased significantly from the Time 1 to Time 2,
and again to this current follow‐up. Quality of life measures of energy level decreased
significantly from the original study to both the Time 2 and Time 3.
Conclusion: Cardiac mortality for Mustard patients remains high, and over time, sys‐
temic right ventricular ejection fraction, rhythm, exercise tolerance, and quality of
life assessments show deterioration. There does not appear to be a single clear pre‐
dictor of poor outcome. |
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Keywords: | adult congenital heart disease cardiac diseases d‐transposition of the great arteries Mustard procedure |
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