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Transplantation after Mustard operation for transposition of the great arteries

ABSTRACT: Abstract As long‐term outcomes of congenital heart diseases improve, the probability of adult patients presenting for heart transplantation for late failure of congenitally corrected heart disease also increases. In patients with dextro‐transposition of the great arteries (d‐TGA) who were initially treated in the era of Mustard or Senning procedures and before Jatene procedure was introduced, progressive systemic right ventricular failure represents a problem in the very long‐term follow‐up. We report a rare case of heart transplantation as a third operation 36 years after Mustard procedure in a patient with d‐TGA experiencing late failure of the systemic right ventricle. Heart transplantations are becoming more common after surgery for congenital heart disease. Many heart transplantations have been performed also after Mustard surgery, but few have reported detailed surgical procedures with figures or video. This report is useful because of the variations in d‐TGA.

SUBMITTER: Katahira S

PROVIDER: S-EPMC8502455 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BACKGROUND:The arterial switch operation (ASO) is the gold standard operative correction of neonates with transposition of the great arteries and intact ventricular septum, with excellent operative survival. The associations between patient and surgeon characteristics and outcomes are well understood, but the associations between variation in preoperative care and outcomes are less well studied. METHODS:A multicenter retrospective cohort study of infants undergoing neonatal ASO between January 2010 and September 2015 at hospitals contributing data to the Pediatric Health Information Systems database was performed. The association between preoperative care (timing of ASO, preoperative use of balloon atrial septostomy, prostaglandin infusion, mechanical ventilation, and vasoactive agents) and operative outcomes (mortality, length of stay, and cost) was studied with multivariable mixed-effects models. RESULTS:Over the study period, 2159 neonates at 40 hospitals were evaluated. Perioperative mortality was 2.8%. Between hospitals, the use of adjuvant therapies and timing of ASO varied broadly. At the subject level, older age at ASO was associated with higher mortality risk (age >6 days: odds ratio, 1.90; 95% CI, 1.11-3.26; P=0.02), cost, and length of stay. Receipt of a balloon atrial septostomy was associated with lower mortality risk (odds ratio, 0.32; 95% CI, 0.17-0.59; P<0.001), cost, and length of stay. Later hospital median age at ASO was associated with higher odds of mortality (odds ratio, 1.15 per day; 95% CI, 1.02-1.29; P=0.03), longer length of stay ( P<0.004), and higher cost ( P<0.001). Other hospital factors were not independently associated with the outcomes of interest. CONCLUSIONS:There was significant variation in preoperative care between hospitals. Some potentially modifiable aspects of perioperative care (timing of ASO and septostomy) were significantly associated with mortality, length of stay, and cost. Further research on the perioperative care of neonates is necessary to determine whether modifying practice on the basis of the observed associations translates into improved outcomes.

Project description:Introduction: Adult survivors of the atrial switch operation for transposition of the great arteries present with a systemic morphologic right ventricle and a subpulmonary morphologic left ventricle. This physiology can be considered a model for the effects of long-term right ventricular pressure overload and of decreased left ventricular afterload. We aimed to determine the impact of these chronically altered loading conditions on myocardial deformation of the ventricles. Materials and methods: Two-dimensional steady state free precession cine images of 29 patients after atrial repair (age 29 ± 7 years) and 19 controls (24 ± 10 years; n.s.) were post-processed with feature tracking software (TomTec 2D CPA). Volumes, ejection fractions, global and free wall longitudinal and circumferential strains of both ventricles were compared between both groups. Results: Systemic right ventricular global longitudinal strain was decreased in patients compared to controls (-12.9 ± 3.3% vs. -18.9 ± 4.6%, p < 0.001), while right ventricular circumferential strain was unchanged (-15.8 ± 3.4% vs. -15.1 ± 5%; n.s.). Left ventricular longitudinal strain was similar in both groups (-17 ± 5.6% vs. -17.5 ± 4.6%; n.s.), but global left ventricular circumferential strain was lower in patients (-20.7 ± 4.1% vs. -27.3 ± 4.5%, p < 0.001). The systemic right ventricle, compared to the systemic left ventricle, showed decreased global longitudinal (p < 0.001) and circumferential strain (p < 0.001). The subpulmonary left ventricle, compared to the subpulmonary right ventricle, demonstrated similar longitudinal (p = 0.223) but higher circumferential strain (p < 0.001). Conclusions: In patients after atrial switch repair for transposition of the great arteries, the systemic right ventricle shows poor longitudinal strain, but maintains normal right ventricular circumferential strain. The left ventricle shows higher circumferential strain than the right ventricle, in both systemic and subpulmonary positions.

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Transplantation after Mustard operation for transposition of the great arteries

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