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

Cuypers, Judith A.A.E.; Eindhoven, Jannet A.; Slager, Maarten A.; Opić, Petra; Utens, Elisabeth M. W. J.; Helbing, Willem A.; Witsenburg, Maarten; Bosch, Annemien E. van den; Ouhlous, Mohamed; Domburg, Ron T. van; Rizopoulos, Dimitris; Meijboom, Folkert J.; Bogers, Ad J.J.C.; Roos‐Hesselink, Jolien W.

doi:10.1093/eurheartj/ehu102

Cited by 165 publications

(140 citation statements)

References 30 publications

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“…These include systemic venous and pulmonary venous channel obstruction, atrial baffle leak, sick-sinus syndrome and atrial tachyarrhythmias 44. In a long-term outcome study up to 40 years of patients after the Mustard procedure for d-TGA, long-term survival was diminished and morbidity substantial 45. The cumulative incidence of reinterventions was 46%, mainly for baffle stenosis or leakage.…”

Section: Postsurgical Outcomesmentioning

confidence: 99%

Contemporary management and outcomes in congenitally corrected transposition of the great arteries

Kutty

Danford

Diller³

et al. 2018

Heart

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Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex 'double switch' repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care.

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Section: Postsurgical Outcomesmentioning

confidence: 99%

Contemporary management and outcomes in congenitally corrected transposition of the great arteries

Kutty

Danford

Diller³

et al. 2018

Heart

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“…Due to extensive scarring, patients with transposition of the great arteries (TGA) and atrial switch surgery have a high incidence of atrial arrhythmias 11. Approximately 30% of patients after Mustard or Senning repair develop atrial arrhythmias within 10 years,5 and 28%–40% within 30–35 years after surgery 12 13. With the advent of the arterial switch surgery that does not require atrial incisions, SVT incidence has dropped to approximately 5% 14 15…”

Section: Pathophysiology Of Svt In Achdmentioning

confidence: 99%

Management of supraventricular arrhythmias in adults with congenital heart disease

Wasmer

Eckardt

2016

Heart

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Supraventricular arrhythmias are a frequent complication in adults with congenital heart disease (ACHD). The prevalence increases with time since surgery, complexity of the underlying defect, type of repair and older age at surgery. Arrhythmias are the most frequent reason for hospital admission and along with heart failure the leading cause of death. The arrhythmia-associated increase in morbidity and mortality makes their management a key task in patients with ACHD. Intra-atrial re-entry is the most frequent arrhythmia mechanism. Less common arrhythmia mechanisms are supraventricular tachycardias in the presence of an accessory pathway, atrioventricular nodal re-entrant tachycardia or focal tachycardias. Patient management includes stroke prevention, acute termination and prevention of arrhythmia recurrence. Acute treatment depends on patients' symptoms. In cases of haemodynamic instability, immediate cardioversion is warranted. For stable patients, acute treatment includes rate control and termination by antiarrhythmic drugs or electrical cardioversion. Following a symptomatic arrhythmia, catheter ablation or treatment with antiarrhythmic drugs is recommended to prevent recurrences. Advances in mapping and ablation technology are now associated with high success rates of catheter ablation. In patients with a complex substrate recurrence rates of 50% remain high. However, in the presence of side effects and complications associated with long-term antiarrhythmic drug therapy, redo procedures are encouraged by current guidelines.

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“…3-13 Atrial tachyarrhythmia has been reported as the most common early clinical complication, 5,7,11,13,14 is associated with significant clinical morbidity, has been suggested to reflect systemic RV dysfunction, and is a recognized marker of elevated atrial pressure and increased mortality. 5,7,13,15 Thereafter, RV dysfunction precedes the onset of symptoms, clinical heart Background-We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance predicts outcomes in patients with transposition of the great arteries post atrial redirection surgery.…”

mentioning

confidence: 99%

Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries

Rydman

Gatzoulis

et al. 2015

Circ: Cardiovascular Imaging

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M yocardial fibrosis is implicated in late right ventricular (RV) dysfunction in patients who have undergone atrial redirection surgery (Mustard/Senning operation) for transposition of the great arteries.1,2 The RV in these patients functions as the systemic ventricle which predisposes to arrhythmia, premature systemic RV failure, and sudden death.3-13 Atrial tachyarrhythmia has been reported as the most common early clinical complication, 5,7,11,13,14 is associated with significant clinical morbidity, has been suggested to reflect systemic RV dysfunction, and is a recognized marker of elevated atrial pressure and increased mortality. 5,7,13,15 Thereafter, RV dysfunction precedes the onset of symptoms, clinical heart Background-We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance predicts outcomes in patients with transposition of the great arteries post atrial redirection surgery. These patients have a systemic right ventricle (RV) and are at risk of arrhythmia, premature RV failure, and sudden death. Methods and Results-Fifty-five patients (aged 27±7 years) underwent LGE cardiovascular magnetic resonance and were followed for a median 7.8 (interquartile range, 3.8-9.6) years in a prospective single-center cohort study. RVLGE was present in 31 (56%) patients. The prespecified composite clinical end point comprised new-onset sustained tachyarrhythmia (atrial/ventricular) or decompensated heart failure admission/transplantation/death. Univariate predictors of the composite end point (n=22 patients; 19 atrial/2 ventricular tachyarrhythmia, 1 death) included RV LGE presence and extent, RV volumes/mass/ejection fraction, right atrial area, peak Vo 2 , and age at repair. In bivariate analysis, RVLGE presence was independently associated with the composite end point (hazard ratio, 4.95 [95% confidence interval, 1.60-15.28]; P=0.005), and only percent predicted peak Vo 2 remained significantly associated with cardiac events after controlling for RV LGE (hazard ratio, 0.80 [95% confidence interval, 0.68-0.95]; P=0.009/5%). In 8 of 9 patients with >1 event, atrial tachyarrhythmia, itself a known risk factor for mortality, occurred first. There was agreement between location and extent of RV LGE at in vivo cardiovascular magnetic resonance and histologically documented focal RV fibrosis in an explanted heart. There was RV LGE progression in a different case restudied for clinical indications. Conclusions-Systemic RV LGE is strongly associated with adverse clinical outcome especially arrhythmia in transposition of the great arteries, thus LGE cardiovascular magnetic resonance should be incorporated in risk stratification of these patients. (Circ Cardiovasc Imaging. 2015;8:e002628.

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

Abstract: 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.

Cited by 165 publications

References 30 publications

Contemporary management and outcomes in congenitally corrected transposition of the great arteries

Contemporary management and outcomes in congenitally corrected transposition of the great arteries

Management of supraventricular arrhythmias in adults with congenital heart disease

Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries

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