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Judith A.A.E. Cuypers, Jannet A. Eindhoven, Maarten A. Slager, Petra Opić, Elisabeth M.W.J. Utens, Willem A. Helbing, Maarten Witsenburg, Annemien E. van den Bosch, Mohamed Ouhlous, Ron T. van Domburg, Dimitris Rizopoulos, Folkert J. Meijboom, Ad J.J.C. Bogers, Jolien W. Roos-Hesselink, The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years, European Heart Journal, Volume 35, Issue 25, 1 July 2014, Pages 1666–1674, https://doi.org/10.1093/eurheartj/ehu102
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Abstract
To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair.
This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age <15 years, and were evaluated in-hospital every 10 years. Survival status was obtained of 86 patients. Median follow-up was 35 (IQR 34–38) years. Cumulative survival was 84% after 10 years, 80% after 20 years, 77% after 30 years, and 68% after 39 years. Cumulative survival free of events (i.e. heart transplantation, arrhythmias, reintervention, and heart failure) was 19% after 39 years. Reinterventions were mainly required for baffle-related problems. Supraventricular and ventricular arrhythmias occurred in 28 and 6% of the patients, respectively. Pacemaker and/or ICD implantation was performed in 39%. Fifty survivors participated in the current in-hospital investigation including electrocardiography, 2D-echocardiography, cardiopulmonary-exercise testing, NT-proBNP measurement, Holter monitoring, and cardiac magnetic resonance. Right ventricular systolic function was impaired in all but one patient at last follow-up, and 14% developed heart failure in the last decade. NT-proBNP levels [median 31.6 (IQR 22.3–53.2) pmol/L] were elevated in 92% of the patients. Early postoperative arrhythmias were a predictor for late arrhythmias [HR 3.8 (95% CI 1.5–9.5)], and development of heart failure [HR 8.1 (95% CI 2.2–30.7)]. Also older age at operation was a predictor for heart failure [HR 1.26 (95% CI 1.0–1.6)].
Long-term survival after Mustard repair is clearly diminished and morbidity is substantial. Early postoperative arrhythmias are a predictor for heart failure and late arrhythmias.
- cardiac arrhythmia
- artificial cardiac pacemaker
- holter electrocardiography
- electrocardiogram
- heart transplantation
- echocardiography
- transposition of great vessels
- heart failure
- two-dimensional echocardiography
- exercise
- ergometry
- follow-up
- heart ventricle
- mustard (food)
- survivors
- systole
- morbidity
- treatment outcome
- ventricular arrhythmia
- implantable defibrillator insertion
- cardiac mri
- nt-probnp
- adverse event
- cumulative survival rate
- mustard procedure
- cardiac development