Arrhythmia and Mortality After the Mustard Procedure: A 30-Year Single-Center Experience

Gelatt, Mark; Hamilton, Robert M.; McCrindle, Brian W.; Connelly, Michael S.; Davis, Andrew M.; Harris, Louise; Gow, Robert M.; Williams, William G.; Trusler, George A.; Freedom, Robert M.

doi:10.1016/s0735-1097(96)00424-x

Cited by 436 publications

(263 citation statements)

References 20 publications

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“…14 There were 77 late deaths (16%), with unexpected sudden death occurring in 31 patients at a median of 3.2 years after the operation. 13 Sudden presumed arrhythmic death and myocardial failure were the most frequent causes of late death, a finding that agrees with other reports. 15 Atrial arrhythmias can precipitate a significant deterioration in systemic ventricular function, and, in general, sinus rhythm should be restored when possible.…”

Section: Arrhythmiassupporting

confidence: 90%

“…[7][8][9][10] Sinus node dysfunction is common in adults, 11 and the cause is probably damage to the sinus node and atrial conduction tissue or interruption of sinus node blood flow at the time of operation. 12 In the series of 534 children by Gelatt et al, 13 sinus rhythm was present in 77% at 5 years and only 40% at 20 years. Fifty-three patients (11%) needed pacemaker implantation.…”

Section: Arrhythmiasmentioning

confidence: 96%

“…19 Late attrition from RV failure and arrhythmias is an ongoing issue, however, that warrants continued follow-up. 13 The long-term prognosis must be guarded, and in later decades it is possible that many will require cardiac transplantation. 20 The assessment of RV function is challenging, and the most commonly used modality is still 2-dimensional echocardiography, albeit qualitative rather than quantitative because of the complex geometry of the RV.…”

Section: Systemic Ventricular Dysfunction and Tricuspid Regurgitationmentioning

confidence: 99%

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Transposition of the Great Arteries

2006

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Abstract-Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and hemodynamic assessment of adult congenital heart disease is available.

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Section: Arrhythmiassupporting

confidence: 90%

Section: Arrhythmiasmentioning

confidence: 96%

Section: Systemic Ventricular Dysfunction and Tricuspid Regurgitationmentioning

confidence: 99%

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Transposition of the Great Arteries

2006

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“…Survival functional status and quality of life of patients with transposition of the great arteries (TGA), after an atrial switch operation (Senning or Mustard procedure) are reported to be reasonably good after 2 decades of life [1][2][3][4][5][6][7][8]. However, in many cases, It's possible to detect the function of the systemic right ventricle deteriorates, severe tricuspid regurgitation develops or the patients suffer from arrhythmias.…”

Section: Introductionmentioning

confidence: 99%

Senning operation for correction of the transposition of the great arteries, results, long-term outcome and quality of life

Maluf¹

2012

WJCD

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“…[11][12][13] Multiple mechanisms for RV dysfunction late after atrial switch have been postulated. Previously published nuclear medicine data have demonstrated both fixed and reversible perfusion defects in patients up to 25 years after atrial redirection surgery.…”

Section: Introductionmentioning

confidence: 99%

Late Gadolinium Enhancement Cardiovascular Magnetic Resonance of the Systemic Right Ventricle in Adults With Previous Atrial Redirection Surgery for Transposition of the Great Arteries

et al. 2005

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Background-Patients treated for transposition of the great arteries by atrial redirection surgery have a right ventricle (RV) that sustains systemic pressures long term. Late RV dysfunction occurs in these patients; the reasons for this are unclear, but myocardial fibrosis may be important. Myocardial fibrosis can be visualized by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR). We hypothesized that LGE would be present in the systemic RV and relate to adverse clinical features. Methods and Results-We performed CMR on 36 consecutive adult patients (mean age, 27 years) after atrial redirection surgery for transposition of the great arteries. Late gadolinium RV enhancement was seen in 22 patients (61%) with various patterns. Patients with RV LGE were older (30 versus 22 years; PϽ0.001) and had increased RV end-systolic volume index (43 versus 35 mL/m 2 ; Pϭ0.03), decreased RV ejection fraction (57% versus 62%; Pϭ0.02), increased QRS duration (108 versus 97 ms; Pϭ0.01), and increased QT dispersion (93 versus 71 ms; Pϭ0.002). The extent of LGE correlated with age (rϭ0.59, PϽ0.001) and QRS duration (rϭ0.67, PϽ0.001) and inversely with RV ejection fraction (rϭϪ0.76, PϽ0.001). The incidence of documented arrhythmia and/or syncope (10 of 36) was significantly higher in the late gadolinium-positive group (9/22 versus 1/14; Pϭ0.03). Key Words: heart ventricles Ⅲ magnetic resonance imaging Ⅲ heart defects, congenital Ⅲ transposition of great vessels Ⅲ fibrosis B efore use of the arterial switch operation, patients with transposition of the great arteries were palliated by redirection of blood at the atrial level with the Senning 1 or Mustard operation. 2 There is a contemporary adult population in whom the right ventricle (RV) must sustain systemic pressure long term. Late RV dysfunction is common in these patients 3 and may be related to myocardial fibrosis. In patients with a systemic left ventricle (LV), cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) can detect myocardial fibrosis in both ischemic and nonischemic cardiomyopathies. 4,5 We hypothesized that LGE would be present in the systemic RV and that it would be associated with markers of adverse clinical outcome. 4 Conclusions-LGE Methods Patient PopulationConsecutive patients with transposition of the great arteries who had undergone either the Mustard or Senning procedure were invited to participate. Patients with a permanent pacemaker in situ were excluded. The local research ethics committee approved the study, and all patients gave written informed consent. Cardiovascular Magnetic ResonanceA 1.5-T scanner was used (Siemens Sonata). After routine assessment of anatomy, the patency of atrial pathways and function of valves were assessed by steady-state free precession (SSFP) cine imaging in a sequence of specified oblique planes. They were aligned with each atrial pathway, the inflow valves in long-axis planes, and the outflow tracts. Peak velocity was measured at the narrowest point of each caval pathwa...

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Arrhythmia and Mortality After the Mustard Procedure: A 30-Year Single-Center Experience

Abstract: Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Cited by 436 publications

References 20 publications

Transposition of the Great Arteries

Transposition of the Great Arteries

Senning operation for correction of the transposition of the great arteries, results, long-term outcome and quality of life

Late Gadolinium Enhancement Cardiovascular Magnetic Resonance of the Systemic Right Ventricle in Adults With Previous Atrial Redirection Surgery for Transposition of the Great Arteries

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