Open Access

ARTICLE

The long‐term functional outcome in Mustard patients study: Another decade of follow‐up

Nayan T. Srivastava^1,2, Roger Hurwitz³, W. Aaron Kay⁴, George J. Eckert⁵, Alisha Kuhlenhoelter⁶, Nicole DeGrave², Eric S. Ebenroth^2,6

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

* Corresponding Author: Nayan T. Srivastava, MD, Pediatrics, Eskenazi Health, 705 Eskenazi Hospital Dr., OCC 2, Indianapolis, IN 46202. Email:

Congenital Heart Disease 2019, 14(2), 176-184. https://doi.org/10.1111/chd.12698

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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