Korean Circ J.  2017 Mar;47(2):201-208. 10.4070/kcj.2016.0194.

Clinical Outcomes after Anatomic Repair Including Hemi-Mustard Operation in Patients with Congenitally Corrected Transposition of the Great Arteries

• Shim MS
• Jun TG
• Yang JH
• Park PW
• Cho YH
• Kang S
• Huh J
• Song JY

• KMID: 2377460
• DOI: http://doi.org/10.4070/kcj.2016.0194

Abstract

BACKGROUND AND OBJECTIVES
The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure.
SUBJECTS AND METHODS
We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4).
RESULTS
Atrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation.
CONCLUSION
Anatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.

Keyword

Anatomic repair; Congenitally corrected TGA; Congenital heart defect

MeSH Terms

Arterial Switch Operation
Arteries*
Body Weight
Constriction, Pathologic
Follow-Up Studies
Freedom
Heart
Heart Defects, Congenital
Hospital Mortality
Humans
Mortality
Reoperation
Retrospective Studies
Sick Sinus Syndrome
Survival Rate
Tricuspid Valve Insufficiency

Figure

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  Fig. 1 Diagram showing the procedures undertaken according to the left ventricular outflow tract obstruction in 16 patients. ccTGA: congenitally corrected transposition of the great arteries, VSD: ventricular septal defect, PS: pulmonary stenosis, PA: pulmonary atresia, PS: pulmonary stenosis, PAB: pulmonary artery banding, BVP: balloon valvuloplasty, MBTS: modified Blalock-Taussig shunt, BCPS: bidirectional cavopulmonary shunt.

• 

  Fig. 2 Hemi-Mustard and Rastelli operations using a size-reduced bicuspid homograft conduit (black arrow). SVC: superior vena cava, RPA: right pulmonary artery, B: bovine pericardial baffle from the inferior vena cava to the left-sided atrium, MV: mitral valve, H: bovine pericardial hood between the right ventricle and homograft.

• 

  Fig. 3 Progression of TR. TR was classified on a scale of 1 to 5 (1: no to mild, 2: mild to moderate, 3: moderate, 4: moderate to severe, and 5: severe). *Number of patients who underwent tricuspid valve repair. TR: tricuspid regurgitation, F/U: follow-up.

• 

  Fig. 4 Freedom from reoperation rate. Kaplan-Meier survival curves shows freedom from reoperation rate after anatomic repair

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