Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience

Lee, Alice; Zannino, Diana; Davis, Andrew M.; Fricke, Tyson A.; d’Udekem, Yves; Konstantinov, Igor E.; Brink, Johann; Cheung, Michael

doi:10.1016/j.jtcvs.2017.03.072

Cited by 41 publications

(25 citation statements)

References 20 publications

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“…In addition, the procedure of anatomic correction is described to be a challenging complex surgery providing excellent functional outcomes in survivors, but with a significant rate of reoperation and a definite risk of death or transplantation as well as a relevant rate of postoperative AVB. [24].…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries

Krummholz

Gottschalk

Geipel

et al. 2020

Arch Gynecol Obstet

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Purpose To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up. Methods Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports. Results Isolated ccTGA without associated cardiac anomalies was found in 13.5% (5/37), in all other fetuses additional defects such as VSD (73.0%), pulmonary obstruction (35.1%), tricuspid valve anomalies (18.9%), aortic arch anomalies (13.5%), ventricular hypoplasia (5.4%) or atrioventricular block (5.4%) were present. The rate of extracardiac malformations or chromosomal aberrations was low. There were 91.9% (34/37) live births and postnatal survival rates reached 91.2% in a mean follow-up time of 4.98 years. The prenatal diagnosis of ccTGA was confirmed postnatally in all but one documented live birth and the prenatal counselling regarding the expected treatment after birth (uni- versus biventricular repair) was reassured in the majority of cases. The postnatal intervention rate was high, 64.7% (22/34) received surgery, the intervention-free survival was 36.7%, 35.0% and 25.0% at 1 month, 1 year and 10 years, respectively. Conclusions ccTGA is a rare heart defect often associated with additional heterogeneous cardiac anomalies that can be diagnosed prenatally. The presented study demonstrates a favorable outcome in most cases but the majority of patients require surgical treatment early in life.

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Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries

Krummholz

Gottschalk

Geipel

et al. 2020

Arch Gynecol Obstet

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“…Since then, numerous centers have reported favorable outcomes following anatomic repair. 12 –18 When considering the strategy of anatomic correction, the incomplete natural history data on the systemic RV in this condition confounds the decision to routinely proceed with anatomic correction (with or without LV training) at the time of diagnosis, even during childhood, particularly in the absence of signs or symptoms of RV or Tricuspid value (TV) dysfunction. For purposes of management strategy, three basic decision points might be considered at the time of presentation: No or minor associated cardiac lesions that don’t require surgical intervention with preserved RV function; Major associated cardiac lesions that merit operation with preserved RV function; The development of major RV dysfunction at any time, but without symptoms; and The development of major RV dysfunction with heart failure symptoms. …”

Section: Discussionmentioning

confidence: 99%

Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle

Smood

Kirklin

Pavnica

et al. 2019

World J Pediatr Congenit Heart Surg

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Introduction: Knowledge gaps exist in the life expectancy and functional outcome of patients with congenitally corrected transposition (ccTGA) presenting early in life, which is relevant in the evaluation of early anatomic repair. Methods: In a singlecenter analysis, 91 patients with ccTGA were identified over 25 years, of which 31 presented with biventricular anatomy in the first year of life and formed the study cohort. End points for analysis included survival, moderate or worse tricuspid valve regurgitation, and systemic right ventricle (RV) dysfunction. Median follow-up was 4.9 years (range: 7 days to 20 years). Results: Among 31 patients presenting in the first year of life, 9 (29%) never received cardiac surgery, while 22 (71%) underwent 36 cardiac operations. Overall freedom from moderate or severe systemic RV dysfunction was 75% at 10 years. Overall survival was 82% at 10 years. Surgical mortality was 5.6% (2/36). Among survivors with a systemic RV, 23 (100%) of 23 were Ross or NYHA class I or II at last follow-up. Conclusions: Congenitally corrected transposition presenting in the first year of life and maintaining a systemic RV can expect (1) long-term survival of more than 80% at 10 years, (2) low expected surgical mortality (overall 6%), and (3) 75% late freedom from major RV dysfunction at 10 years. Pending multi-institutional analyses, this experience with a systemic RV in ccTGA provides an initial benchmark for comparison when considering early elective anatomic correction.

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“…Strategies including application of the hemi-Mustard atrial switch simplify the operation and may provide a more predictable pathway for patients undergoing anatomic repair, and potentially improve long-term outcomes in the anatomic group through preservation of atrial function. 19 Other approaches such as the Bex-Nikaidoh approach to aortic root relocation, 20 with a high rate of acquired heart block, 21 might not be superior to the Fontan procedure.…”

Section: Discussionmentioning

confidence: 99%

Congenitally corrected transposition: complex anatomic repair or Fontan pathway?

Marathe

Jones

Ayer

et al. 2017

Asian Cardiovasc Thorac Ann

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Background Successful anatomic repair of congenitally corrected transposition of the great arteries achieves excellent outcomes. Several centers report excellent long-term survival with the Fontan pathway as well. We have selectively applied both approaches depending on individual patient morphology, with anatomic repair preferred but utilizing the Fontan pathway when high technical complexity or operative risk is anticipated. Methods Hospital records over an 18-year period (1998-2016) were reviewed to identify patients with congenitally corrected transposition of the great arteries who underwent surgical management. Physiological repairs and hypoplastic ventricles were excluded. Patient- and procedure-related variables were reviewed. Results We identified 19 patients. Group 1 consisted of 12 anatomic repairs, of which 10 (83.3%) required prior interim staging procedures. Mean age at anatomic repair was 2.6 ± 1.3 years, mean follow-up was 8.7 ± 5.3 years. Nine (75%) patients experienced important complications and 4 (33.3%) required reintervention during follow-up. There were no deaths; one patient required heart transplantation. Group 2 (7 patients) underwent Fontan palliation. Mean age at Fontan completion was 7.2 ± 3.8 years, mean follow-up was 6.3 ± 4 years. There was no reintervention, death, or transplant. Conclusion Patients with congenitally corrected transposition of the great arteries and two adequate-sized ventricles do well with both anatomic repair and the Fontan pathway in the medium term. Excellent outcomes with reduced early complication and reintervention rates can be achieved for this cohort of patients when a strategy of avoiding complex anatomic repair in favor of the Fontan pathway is used.

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Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience

Cited by 41 publications

References 20 publications

Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries

Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries

Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle

Congenitally corrected transposition: complex anatomic repair or Fontan pathway?

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