The long-term outcomes of physiologic repair for ccTGA (congenitally corrected transposition of the great arteries)

Hirose, Katsumi; Nishina, Tomohiro; Kanemitsu, Naoki; Mizuno, Akihiro; Yasumizu, Daisuke; Yada, Masashi; Onga, Yohei; Yamanaka, Kazuhiro

doi:10.1007/s11748-015-0550-y

Cited by 15 publications

(8 citation statements)

References 14 publications

(13 reference statements)

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“…This improved longer term survival outcome over physiological repair needs to be weighed over the apparent short-term safety of only correcting associated abnormalities in a physiologic repair strategy[ 34 ]. However, the surgical mortality in physiologic repair population is also not negligible, being reported at 3% in operations done after 1986 in a Mayo Clinic series (16% overall) and 6.7% in the Dutch series by Bogers et al[ 35 , 36 ].…”

Section: Discussionmentioning

confidence: 99%

Systematic review and meta-analysis of outcomes of anatomic repair in congenitally corrected transposition of great arteries

Chatterjee

Miller

Cribbs

et al. 2020

WJC

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BACKGROUND Treatment of congenitally corrected transposition of great arteries (cc-TGA) with anatomic repair strategy has been considered superior due to restoration of the morphologic left ventricle in the systemic circulation. However, data on long term outcomes are limited to single center reports and include small sample sizes. AIM To perform a systematic review and meta-analysis for observational studies reporting outcomes on anatomic repair for cc-TGA. METHODS MEDLINE and Scopus databases were queried using predefined criteria for reports published till December 31, 2017. Studies reporting anatomic repair of minimum 5 cc-TGA patients with at least a 2 year follow up were included. Meta-analysis was performed using Comprehensive meta-analysis v3.0 software. RESULTS Eight hundred and ninety-five patients underwent anatomic repair with a pooled follow-up of 5457.2 patient-years (PY). Pooled estimate for operative mortality was 8.3% [95% confidence interval (CI): 6.0%-11.4%]. 0.2% (CI: 0.1%-0.4%) patients required mechanical circulatory support postoperatively and 1.7% (CI: 1.1%-2.4%) developed post-operative atrioventricular block requiring a pacemaker. Patients surviving initial surgery had a transplant free survival of 92.5% (CI: 89.5%-95.4%) per 100 PY and a low rate of need for pacemaker (0.3/100 PY; CI: 0.1-0.4). 84.7% patients (CI: 79.6%-89.9%) were found to be in New York Heart Association (NYHA) functional class I or II after 100 PY follow up. Total re-intervention rate was 5.3 per 100 PY (CI: 3.8-6.8). CONCLUSION Operative mortality with anatomic repair strategy for cc-TGA is high. Despite that, transplant free survival after anatomic repair for cc-TGA patients is highly favorable. Majority of patients maintain NYHA I/II functional class. However, monitoring for burden of re-interventions specific for operation type is very essential.

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Section: Discussionmentioning

confidence: 99%

Systematic review and meta-analysis of outcomes of anatomic repair in congenitally corrected transposition of great arteries

Chatterjee

Miller

Cribbs

et al. 2020

WJC

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“…Among this group, overall survival was 82% at 10 years. 16 Similarly, Hirose et al, 17 Lim et al, 18 and Dobson et al 19 reported excellent longterm survival (upward of 90%) among ccTGA patients undergoing physiologic repair, though nearly half required tricuspid valve intervention. 17 Although we recognize the uncertain fate of the systemic RV among the totality of anatomic subtypes in the spectrum of ccTGA, and the potential selection bias inherent in noninception cohort studies of ccTGA outcomes, it is clear that, on balance, the systemic RV in a ccTGA circulation provides a more durable alternative than either a RV-based, LV-based, or 2-ventricle univentricular circulation.…”

Section: Cctga and The Fate Of The Systemic Rvmentioning

confidence: 95%

Biventricular Conversion in Unseptatable Hearts: “Ventricular Switch”

Najm

Karamlou

Ahmad

et al. 2021

Seminars in Thoracic and Cardiovascular Surgery

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Patients with complex systemic and pulmonary venous anatomy, common atrioventricular canal defects and conotruncal anomalies have traditionally been routed to univentricular palliation and labeled as "unseptatable." This report describes our initial experience in septation/biventricular conversion ("ventricular switch"), utilizing the left ventricle (LV) as the subpulmonary ventricle, essentially recapitulating the physiology of congenitally corrected transposition of the great arteries. Five consecutive patients with challenging anatomic configuration underwent septation. All patients were severely cyanotic and had important functional limitations. All patients required complex atrial septation. Ventricular septation was precluded by fixed pulmonary vascular resistance in 2 patients. Systemic venous return was diverted to the morphologic LV as part of physiological 2V (n = 4) or 1.5 V repair (n = 1). Median conversion age was 9 years (range 11 monthsÀ46 years). Four patients had 12 previous cardiac surgical procedures in preparation for univentricular repair elsewhere. Three dimensional-printed heart models evaluated feasibility of septation. All patients are alive at a median follow-up of 0.6 years (range 0.08À2.7 years). Median hospital stay was 13 (range 10À60) days. LV recruitment improved functional status and significantly increased systemic oxygen saturation in all patients (79 § 7% vs 95 § 5%, P = 0.003). We report a novel paradigm for successfully utilizing both ventricles with the morphologic LV as the subpulmonary ventricle, in a complex population thought to be unseptatable. This approach is versatile and can likely be extrapolated to other complex anatomic configurations. Although we utilized this strategy in patients of variable age, earlier ventricular switch may yield the best results.

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“…The LVOTO and positional heart anomalies made it very difficult to construct the traditional atrial baffle of the Senning and Mustard procedures, so significant technical challenges would be encountered with biventricular repair. Early survival rates are good with physiological repair, but mid-and long-term outcomes were disappointing in some reported studies (2)(3)(4)(5). Fontan operation has the advantage of low operative risk and superior early surgical outcomes but carried with it the inherent features of chronic systemic venous hypertension, low cardiac output, and liver fibrosis, which could translate into poor quality of life and outcomes in the long term (6,7).…”

Section: Introductionmentioning

confidence: 99%

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: One-and-a-half ventricular repair vs. Fontan pathway?

Pang²,

Zhang³

et al. 2022

Front. Cardiovasc. Med.

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ObjectivesThis study was to assess the mid-term results of the one-and-a-half ventricular repair (hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli procedure) and Fontan pathway for correcting congenitally corrected transposition of great artery (ccTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malposition.MethodsIn this retrospective study, 74 consecutive ccTGA with LVOTO and cardiac malposition underwent the one-and-a-half ventricular repair (group A; 33 cases) and Fontan operation (group B; 41 cases) between October 2011 and March 2018. The Median follow-up time was 49 (20–84) and 42 (7–85) months in groups A and B, respectively. To estimate excise tolerance the 6-min walk test (MWT) was performed.ResultsNo in-hospital death. Compared with group A, group B have significantly less CPB, mechanical ventilation time, and intensive care unit stay, but prolonged pleural effusions developed more frequently in Group B. The survival probability was 90.2% (95% CI, 80.2–100%) and 97.2% (95% CI, 92–100%) at 7 years (p = 0.300) in group A and B. The probability of freedom from re-intervention were 80.6% (95% CI, 66.5–97.6%) and 97.2% (95% CI, 92–100%) at 7 years (p = 0.110). Longitudinal repeated measured echo data at every follow-up time shows that group A has more systemic ventricular EF% (p < 0.001) and less moderate systemic ventricular valve regurgitation (p < 0.001) compared with group B. Estimated by 6 MWT, group A has better outcomes for 6-min walk distance.ConclusionsFor correction of ccTGA with LVOTO and cardiac malposition, the one-and-a-half ventricular repair had superior midterm heart function and excise tolerance.

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The long-term outcomes of physiologic repair for ccTGA (congenitally corrected transposition of the great arteries)

Abstract: An analysis of the results of physiologic repair for ccTGA showed that the long-term outcome was overall favorable. To maintain RV function, early TVR may be a reasonable option, even in the management of patients during childhood.

Cited by 15 publications

References 14 publications

Systematic review and meta-analysis of outcomes of anatomic repair in congenitally corrected transposition of great arteries

Systematic review and meta-analysis of outcomes of anatomic repair in congenitally corrected transposition of great arteries

Biventricular Conversion in Unseptatable Hearts: “Ventricular Switch”

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: One-and-a-half ventricular repair vs. Fontan pathway?

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