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Stenosis of the superior limb of the systemic venous baffle following a Mustard procedure: an under-recognized
Natalie A Bottega1, Candice K Silversides, Erwin N Oechslin
1Toronto Congenital Cardiac Centre for Adults, Division of Cardiology, University of Toronto, University Health Network, Toronto General Hospital, Peter Munk Cardiac Centre, Toronto, Ontario, Canada. natalie.bottega@muhc.mcgill.ca
Insights
Stenosis of the superior limb of the systemic venous baffle (SLSVB) affects 44% of adults with d-transposition of the great arteries (DTGA) and Mustard procedure. Non-echocardiographic imaging is more effective for SLSVB stenosis detection.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Patients with d-transposition of the great arteries (DTGA) and Mustard procedure are at risk for superior limb of the systemic venous baffle (SLSVB) stenosis.
- The study evaluated the frequency of this complication in an adult cohort.
Purpose of the Study:
- To determine the prevalence of SLSVB stenosis in adult patients who underwent the Mustard procedure for DTGA.
- To assess the effectiveness of different imaging modalities in detecting SLSVB stenosis.
- To evaluate the impact of pacemaker/implantable cardioverter defibrillator (PM/ICD) presence on SLSVB stenosis.
Main Methods:
- Adult patients (>18 years) with DTGA and a Mustard procedure were identified.
- Cardiac imaging (CT, MRI, venography, catheterization) was used to assess SLSVB narrowing (<10mm).
- Hemodynamically significant narrowing was defined by specific criteria, including azygous vein dilatation or SVC syndrome.
Main Results:
- SLSVB narrowing was observed in 44% of patients (49/112).
- Hemodynamically significant narrowing occurred in 15/49 patients.
- Non-echocardiographic imaging showed 88% sensitivity for detecting SLSVB narrowing, compared to 16% for pulse-wave Doppler.
Conclusions:
- SLSVB stenosis is a common complication in adult Mustard patients with DTGA, with a prevalence of 44%.
- Non-echocardiographic imaging is superior to pulse-wave Doppler for detecting SLSVB stenosis.
- Baffle patency assessment is crucial before transvenous device implantation.
Background:
Patients with atrioventricular concordance and ventriculoarterial discordance (DTGA) and a Mustard procedure may develop stenosis of the superior limb of the systemic venous baffle (SLSVB). The frequency of this complication in an adult cohort was evaluated.
Methods:
Patients >18 years with DTGA and a Mustard procedure with and without a pacemaker (PM)/implantable cardioverter defibrillator (ICD) were identified through an institutional database. Subjects were included following a cardiac imaging study (computed tomography, magnetic resonance imaging, venography or cardiac catheterization) and follow-up in the PM/ICD or congenital cardiac clinics from 2001 to 2007. The primary end-point was narrowing of the SLSVB (<10mm) on cardiac imaging. Hemodynamically significant narrowing was defined by: azygous vein dilatation with retrograde flow or superior vena cava syndrome or the need for dilatation ± stenting of the SLSVB.
Results:
Narrowing of the SLSVB was observed in 49/112 patients (70 males) age 31 ± 6 years (range 18-49) and was hemodynamically significant in 15/49. Of 29 patients with a PM (23) or ICD (6) and cardiac imaging, 17 had narrowing of the SLSVB which was hemodynamically significant in 8. Non-echocardiographic imaging had a sensitivity of 88% at detecting narrowing of the SLSVB in contrast to pulse-wave Doppler, which yielded a sensitivity of 16% (61% negative predictive value, 88% positive predictive value).
Conclusions:
In our adult cohort of Mustard patients, narrowing of the SLSVB had a prevalence of 44% and was more likely to be detected by non-echocardiographic imaging. Baffle patency should be evaluated before transvenous device implantation.
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