Long-Term Outcome After Mustard Repair at Young Age

Sahra Ünlütürk, MD; Robert M. Kauling, MD; Judith A.A.E. Cuypers, MD, PhD; Annemien E. van den Bosch, MD, PhD; Alexander Hirsch, MD, PhD; Mieke M.P. Driessen, MD, PhD; Chiara Pelosi, MD, PhD; Daniel J. Bowen, PhD; Ad J.J.C. Bogers, MD, PhD; Willem A. Helbing, MD, PhD; Isabella Kardys, MD PhD; Jolien W. Roos-Hesselink, MD, PhD

doi:10.1016/j.jacadv.2025.101984

JACC: Advances (Aug 2025)

Long-Term Outcome After Mustard Repair at Young Age

Sahra Ünlütürk, MD,
Robert M. Kauling, MD,
Judith A.A.E. Cuypers, MD, PhD,
Annemien E. van den Bosch, MD, PhD,
Alexander Hirsch, MD, PhD,
Mieke M.P. Driessen, MD, PhD,
Chiara Pelosi, MD, PhD,
Daniel J. Bowen, PhD,
Ad J.J.C. Bogers, MD, PhD,
Willem A. Helbing, MD, PhD,
Isabella Kardys, MD PhD,
Jolien W. Roos-Hesselink, MD, PhD

Affiliations

Sahra Ünlütürk, MD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands
Robert M. Kauling, MD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, The Netherlands; Address for correspondence: Dr Martijn Kauling, Department of Cardiology, Room Rg-419, Erasmus Medical Center Rotterdam, Dr. Molewaterplein 40, 3015 GD Rotterdam, Post Office Box: 2040, Rotterdam 3000, the Netherlands.
Judith A.A.E. Cuypers, MD, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, The Netherlands
Annemien E. van den Bosch, MD, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, The Netherlands
Alexander Hirsch, MD, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; Department of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, the Netherlands
Mieke M.P. Driessen, MD, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, The Netherlands
Chiara Pelosi, MD, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands
Daniel J. Bowen, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands
Ad J.J.C. Bogers, MD, PhD: Department of Cardiothoracic Surgery, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands
Willem A. Helbing, MD, PhD: Department of Paediatrics, Division of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands
Isabella Kardys, MD PhD: Clinical Epidemiology and Innovation Unit, Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands
Jolien W. Roos-Hesselink, MD, PhD: Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, The Netherlands

DOI: https://doi.org/10.1016/j.jacadv.2025.101984
Journal volume & issue: Vol. 4, no. 8
p. 101984

Abstract

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Background: Concerns remain regarding the high incidence of morbidity in transposition of the great arteries (TGA) patients who underwent Mustard repair. Objectives: The purpose of this study was to evaluate the long-term outcome and quality of life of patients with TGA up to 48 years. Methods: Single-center, longitudinal cohort study evaluating consecutive patients with TGA who underwent Mustard repair between 1973 and 1980 at young age with extensive cardiac and subjective quality of life evaluation every decade. Results: Of the original cohort of 91 patients, 31 died and 2 underwent heart transplantation. Cumulative survival at 48 years was 61% (51% to 73%), with a shift in cause of death from sudden death to heart failure (HF) after 35 years of follow-up. Of the 44 eligible survivors, 34 (77%) were evaluated (71% male, median age 46 years) with a median follow-up of 46 (range: 40-48) years. Event-free survival was 14%, with cardiac reintervention (39%), symptomatic arrhythmia (39%), and HF (37%) being the most common events. Systemic right ventricular function (sRVF) declined over time, with 73% having an ejection fraction below 40%, and 95% having a reduced VO2max. Prolonged QRS duration was found to be a predictor for HF, early postoperative arrhythmias for late symptomatic arrhythmias, and a reduced sRVF for mortality. Self-perceived quality of life was good and remained stable over time. Conclusions: Mortality and morbidity after Mustard repair are substantial, with an event-free survival of only 14% at 48 years. The clinical condition deteriorated over time, necessitating timely consideration of advanced treatment options.

Published in JACC: Advances

ISSN: 2772-963X (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the circulatory (Cardiovascular) system; Medicine: Internal medicine: Medical emergencies. Critical care. Intensive care. First aid
Website: https://www.sciencedirect.com/journal/jacc-advances

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