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@#Objective To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P<0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P>0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.
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ABSTRACT Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients. Methods: A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords "congenitally corrected transposition of the great arteries" and "adults". Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study. Results: Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included. Conclusion: Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.
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@#Objective To evaluate the effects of anatomic correction for congenitally corrected transposition of the great arteries (ccTGA) and 10-year follow-up. Methods From January 2008 to December 2018, 48 patients with ccTGA who underwent anatomic correction were reviewed. There were 29 males and 19 females with age of 39.2 (3-91) months. The cohort was divided into two groups: a biventricular anatomic correction group (39 patients) and a 1.5 ventricular anatomic correction group (9 patients). They were followed for in-hospital mortality, late mortality, long-term survival, freedom from reoperation, and heart function. Results There were 3 early deaths and 2 early re-intervention in the biventricular anatomic correction group, but no death and only one re-intervention in the 1.5 ventricular anatomic correction group. Compared with the biventricular anatomic correction group, the operation time, tracheal intubation and ICU time were significantly reduced or shortened in the 1.5 ventricular anatomic correction group (P<0.05). The patients were followed up for 0.5-10.4 years. Four patients were lost. Two patients died in the biventricular anatomic correction group, and two patients received re-intervention. The 1-year, 5-year and 10-year survival rate was 88.2%, 84.0%, and 84.0%, respectively. There was no death or intervention in the 1.5 ventricular anatomic correction group. The quality of life of the other patients in the medium-term follow-up was satisfactory. Only 2 patients were classified as grade Ⅲ in cardiac function, and the other patients were classified as grade Ⅰ-Ⅱ. Conclusion According to the different anatomic characteristics of ccTGA, the individualized strategy of anatomic correction can achieve satisfactory surgical results, and the medium-term quality of life was good. Especially, 1.5 ventricular anatomic correction may obtain better therapeutic effects because of its lower operative mortality and less postoperative complications.
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Objective To review the early-and mid-term results of anatomic repair or Fontan pathway for congenitally corrected transposition of the great arteries(ccTGA) in a single institution of China.Methods Hospital records over a 9-year period(2009-2017) were reviewed to identify patients with ccTGA who underwent anatomic repair or Fontan pathway.Pa-tient-and procedure-related variables were reviewed.Results We identified 37 patients.Group 1 consisted of 10 anatomic re-pairs, of which 4 required prior pulmonary artery banding.Median age at anatomic repair was 1.0 years( range: 0.3 -7.8 years).There was one early death, and one patients experienced ECMO support.The mean follow-up was(3.0 ±2.8)years (range:0.7-8.4 years).Five(55.5％) patients showed arrhythmias, and one required permanent pacemaker implantation during follow-up.Group 2(27 patients) underwent Fontan palliation, of which 23(85.2％) underwent prior bidirectional Glenn shunt.Median age at Fontan completion was 3.8years(range:2.2-14.3 years).there was one early death with a mor-tality of 3.7％.The mean follow-up was(2.8 ±1.6) years(range:0.8-8.2 years).There was 4(15.4％) cases of arrhyth-mias, but none required reintervention.The arrhythmias incidence in Fontan group was significantly lower than the anatomic re-pair group.The early-and mid-term survival rate were 90.0％ and 96.3％in the two groups.The difference was not statistical-ly significant(P=0.458).Conclusion Patients with ccTGA do well with both anatomic repair and the Fontan pathway in the medium term.Pulmonary artery banding can be used effectively for morphological left ventricular retraining , and extenuate tri-cuspid regurgitation.Excellent outcomes with reduced early complication and arrhythmias incidence can be achieved for this co-hort of patients when a strategy of avoiding complex anatomic repair in favor of the Fontan pathway is used .
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Objective To review and compare various types of operations for congenitally corrected transposition of the great arteries (ccTGA),to provide more suitable surgical procedure and improve surgical results.Methods Analysing 203 patients with ccTGA between June 1999 and June 2014,there were thirten patients who had undergone palliative procedure.Thirty-nine patients had received conventional repair operation.Eighty-eight patients had received double switch operation in which there were three patients of Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure.Sixty-three patients had received other surgical treatments including functional univentricular repair operation.Results There were 13 hospital deaths in all patients,consisted of 2 by conventional repair operation,9 by double switch operation and 2 by other procedure.Conclusion The operative procedure depend on the anatomy and physiology in ccTGA.The double switch operation have relatively higher mortality,more complication,the indication of Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure should be more acceptable,but the long-term outcomes will be followed-up.
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A 62-year-old woman was admitted to our hospital because of dextrocardia on her chest X-ray film. She had been in good health though the X-ray abnormality had been pointed out from her childhood. Echocardiogram, magnetic resonance imaging, and cardiac catheterization revealed situs inversus, congenitally corrected transposition of the great arteries, and severe tricuspid valve (systemic atrioventricular valve) regurgitation with mild systemic ventricular dysfunction. The surgeon stood on the patient's left side during the operation. On cardiopulmonary bypass, the tricuspid valve, facing almost dorsally, was exposed through a superior transseptal approach. Tricuspid valve replacement with a mechanical valve was performed with leaflet preservation. Systemic ventricular function is preserved at one year after operation.
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Objective To evaluated the early and mid-term results of arterial switch operation (ASO) for patients with intramural coronary artery.Methods From September 2008 to March 2012,75 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.Among these patients,7patients (9.3％) had an intramural coronary artery.Mean age at operation was 2.4 months (2 days to 1 year) and mean body weight was(4.3 ±2.2) kg.The TGA and VSD in 3 cases,TGA/IVS in 2,and Taussig-Bing anomaly in 2.Among them 3 patients had an aortic arch anomaly,interruption of the aortic arch in 1 and coarctation of the aorta in 1.The individual coronary button technique was used in coronary transfer in 7 patients,of whom one patient required to unroof the intramural segment,an-other one required to unroof the intramural segment and enlarge with autologous pericardium the because of myocardial ischemia.There was 1 operative death because of low cardiac output syndrome.This patient underwent a coronary transfer combining aortic arch repair but without unroofing the stenotic intramural segment.The mortality was 14.2％.In the same period the mortality for 68 patients without an intramural coronary artery was 4.4％ (3/68).There was no statistical difference in mortalitv between the patients with and without an intramural coronary artery (P ＞ 0.05).Results 6 patients follow-up 4 to 47months.There was no late death.No intramural coronary artery obstruction was identified by cardiac computerize temography.All patients had normal ventricular function and were in NYHA class Ⅰ during follow-up.The intramural coronary artery is well known as a risk factor of ASO.Conclusion The technique of coronary transfer should be individually adapted to each anatomical situation.Individual technique for coronary transfer has excellent results.