The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years

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

doi:10.1093/eurheartj/ehu102

The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years

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

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

AIMS: 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.

METHODS AND RESULTS: 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)].

CONCLUSION: 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.

Original language	English
Pages (from-to)	1666-74
Number of pages	9
Journal	European Heart Journal
Volume	35
Issue number	25
DOIs	https://doi.org/10.1093/eurheartj/ehu102
Publication status	Published - 1 Jul 2014

Keywords

Adult
Arrhythmias, Cardiac
Biomarkers
Cardiac Surgical Procedures
Child, Preschool
Echocardiography
Electrocardiography
Electrocardiography, Ambulatory
Epidemiologic Methods
Exercise Test
Female
Heart Failure
Humans
Infant
Magnetic Resonance Angiography
Male
Natriuretic Peptide, Brain
Peptide Fragments
Postoperative Complications
Reoperation
Transposition of Great Vessels
Ventricular Dysfunction, Right

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10.1093/eurheartj/ehu102

http://www.ncbi.nlm.nih.gov/pubmed/24644309

Cite this

Cuypers, J. A. A. E., Eindhoven, J. A., Slager, M. A., Opić, P., Utens, E. M. W. J., Helbing, W. A., Witsenburg, M., van den Bosch, A. E., Ouhlous, M., van Domburg, R. T., Rizopoulos, D., Meijboom, F. J., Bogers, A. J. J. C., & Roos-Hesselink, J. W. (2014). The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years. European Heart Journal, 35(25), 1666-74. https://doi.org/10.1093/eurheartj/ehu102

@article{2fe44392bbd24106addce3ad5e18c079,

title = "The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years",

abstract = "AIMS: 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.METHODS AND RESULTS: 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)].CONCLUSION: 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.",

keywords = "Adult, Arrhythmias, Cardiac, Biomarkers, Cardiac Surgical Procedures, Child, Preschool, Echocardiography, Electrocardiography, Electrocardiography, Ambulatory, Epidemiologic Methods, Exercise Test, Female, Heart Failure, Humans, Infant, Magnetic Resonance Angiography, Male, Natriuretic Peptide, Brain, Peptide Fragments, Postoperative Complications, Reoperation, Transposition of Great Vessels, Ventricular Dysfunction, Right",

author = "Cuypers, {Judith A A E} and Eindhoven, {Jannet A} and Slager, {Maarten A} and Petra Opi{\'c} and Utens, {Elisabeth M W J} and Helbing, {Willem A} and Maarten Witsenburg and {van den Bosch}, {Annemien E} and Mohamed Ouhlous and {van Domburg}, {Ron T} and Dimitris Rizopoulos and Meijboom, {Folkert J} and Bogers, {Ad J J C} and Roos-Hesselink, {Jolien W}",

year = "2014",

month = jul,

day = "1",

doi = "10.1093/eurheartj/ehu102",

language = "English",

volume = "35",

pages = "1666--74",

number = "25",

}

Cuypers, JAAE, Eindhoven, JA, Slager, MA, Opić, P, Utens, EMWJ, Helbing, WA, Witsenburg, M, van den Bosch, AE, Ouhlous, M, van Domburg, RT, Rizopoulos, D, Meijboom, FJ, Bogers, AJJC & Roos-Hesselink, JW 2014, 'The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years', European Heart Journal, vol. 35, no. 25, pp. 1666-74. https://doi.org/10.1093/eurheartj/ehu102

TY - JOUR

T1 - The natural and unnatural history of the Mustard procedure

T2 - long-term outcome up to 40 years

AU - Cuypers, Judith A A E

AU - Eindhoven, Jannet A

AU - Slager, Maarten A

AU - Opić, Petra

AU - Utens, Elisabeth M W J

AU - Helbing, Willem A

AU - Witsenburg, Maarten

AU - van den Bosch, Annemien E

AU - Ouhlous, Mohamed

AU - van Domburg, Ron T

AU - Rizopoulos, Dimitris

AU - Meijboom, Folkert J

AU - Bogers, Ad J J C

AU - Roos-Hesselink, Jolien W

PY - 2014/7/1

Y1 - 2014/7/1

N2 - AIMS: 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.METHODS AND RESULTS: 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)].CONCLUSION: 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.

AB - AIMS: 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.METHODS AND RESULTS: 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)].CONCLUSION: 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.

KW - Adult

KW - Arrhythmias, Cardiac

KW - Biomarkers

KW - Cardiac Surgical Procedures

KW - Child, Preschool

KW - Echocardiography

KW - Electrocardiography

KW - Electrocardiography, Ambulatory

KW - Epidemiologic Methods

KW - Exercise Test

KW - Female

KW - Heart Failure

KW - Humans

KW - Infant

KW - Magnetic Resonance Angiography

KW - Male

KW - Natriuretic Peptide, Brain

KW - Peptide Fragments

KW - Postoperative Complications

KW - Reoperation

KW - Transposition of Great Vessels

KW - Ventricular Dysfunction, Right

U2 - 10.1093/eurheartj/ehu102

DO - 10.1093/eurheartj/ehu102

M3 - Article

C2 - 24644309

SN - 0195-668X

VL - 35

SP - 1666

EP - 1674

JO - European Heart Journal

JF - European Heart Journal

IS - 25

ER -

The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years

Abstract

Keywords

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