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Corrected transposition of great vessels — Clinical Guidelines

Overview

Congenitally corrected transposition of the great arteries (ccTGA) is a congenital heart defect characterized by the aorta arising from the morphologic right ventricle and the pulmonary artery from the morphologic left ventricle, leading to potential hemodynamic challenges and associated anomalies. 1 2

Diagnosis

Key Diagnostic Criteria: Echocardiography is essential for confirming the anatomy, including ventricular morphology, atrioventricular connections, and presence of associated anomalies like ventricular septal defect (VSD) and tricuspid valve abnormalities. 1 2 15

Recommended Tests: Fetal echocardiography for prenatal diagnosis, myocardial perfusion imaging (MPI) can reveal characteristic patterns indicative of ccTGA. 4 2

Electrocardiogram (ECG): Useful for identifying conduction abnormalities and preexcitation patterns, particularly in cases with Ebsteinoid dysplasia or Kent bundles. 14 16

Management

First-Line Treatments: Surgical interventions vary based on patient age and anatomy:

- Conventional Repair: For younger patients with significant VSD. 3 - Anatomical Repair: Aimed at optimizing ventricular function and addressing tricuspid valve issues, often including valvuloplasty if necessary. 3 6 - Single Ventricular Palliation: Considered for patients with complex anatomy unsuitable for biventricular repair. 3

Adjunctive Treatments:

- Pacing: Para-Hisian pacing (PPHP) and atrioventricular sequential pacing can mitigate dyssynchrony and improve systemic ventricular function in patients with heart block. 7 8 - Management of Tricuspid Regurgitation: Valvuloplasty during anatomical repair may be necessary to preserve tricuspid valve function. 6

Special Populations

Pregnancy: Maternal and fetal outcomes vary; careful monitoring is required, especially in patients with residual cyanosis or previous surgical interventions. 11

Pediatrics: Early surgical intervention can improve long-term outcomes, with anatomical repair showing promising results in pediatric cohorts. 3 9

Comorbidities: Chronic subpulmonary left ventricular pacing can adversely affect systemic right ventricular function, necessitating close surveillance and potential pacing strategy adjustments. 5

Key Recommendations

Surgical Repair Timing and Type: Tailor surgical repair based on patient age and anatomy, favoring anatomical repair when feasible to optimize long-term outcomes. (Evidence: Moderate 3 9)

Pacing Strategies: Implement advanced pacing techniques such as para-Hisian pacing to manage dyssynchrony and improve systemic ventricular function in patients with complete heart block. (Evidence: Moderate 7 8)

Monitoring and Management of Tricuspid Valve: Consider tricuspid valvuloplasty during anatomical repair to prevent late-onset tricuspid regurgitation and maintain right ventricular function. (Evidence: Moderate 6)

References

1 Barrios PA, Zia A, Pettersson G, Najm HK, Rajeswaran J, Bhimani S et al.. Outcomes of treatment pathways in 240 patients with congenitally corrected transposition of great arteries. The Journal of thoracic and cardiovascular surgery 2021. link 2 Krummholz A, Gottschalk I, Geipel A, Herberg U, Berg C, Gembruch U et al.. Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries. Archives of gynecology and obstetrics 2021. link 3 Hsu KH, Chang CI, Huang SC, Chen YS, Chiu IS. 17-year experience in surgical management of congenitally corrected transposition of the great arteries: a single-centre's experience. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2016. link 4 Al-faham Z, Jolepalem P, Wong CY. The appearance of congenitally corrected transposition of the great arteries on myocardial perfusion imaging. Journal of nuclear medicine technology 2015. link 5 Yeo WT, Jarman JW, Li W, Gatzoulis MA, Wong T. Adverse impact of chronic subpulmonary left ventricular pacing on systemic right ventricular function in patients with congenitally corrected transposition of the great arteries. International journal of cardiology 2014. link 6 Myers PO, Bautista-Hernandez V, Baird CW, Emani SM, Marx GR, del Nido PJ. Tricuspid regurgitation or Ebsteinoid dysplasia of the tricuspid valve in congenitally corrected transposition: is valvuloplasty necessary at anatomic repair?. The Journal of thoracic and cardiovascular surgery 2014. link 7 Takemoto M, Nakashima A, Muneuchi J, Yamamura K, Shiokawa Y, Sunagawa K et al.. Para-Hisian pacing for a pediatric patient with a congenitally corrected transposition of the great arteries (SLL). Pacing and clinical electrophysiology : PACE 2010. link 8 Kondoh H, Ichikawa H, Shintani H. Reduction of atrioventricular valve regurgitation by atrioventricular sequential pacing for an adult with corrected transposition of the great arteries. Pediatric cardiology 2009. link 9 Bautista-Hernandez V, Marx GR, Gauvreau K, Mayer JE, Cecchin F, del Nido PJ. Determinants of left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries. The Annals of thoracic surgery 2006. link 10 Morita K, Kurosawa H, Koyanagi K, Nomura K, Uno Y, Naganuma H et al.. Atrioventricular groove patch plasty for anatomically corrected malposition of the great arteries. The Journal of thoracic and cardiovascular surgery 2001. link 11 Therrien J, Barnes I, Somerville J. Outcome of pregnancy in patients with congenitally corrected transposition of the great arteries. The American journal of cardiology 1999. link00444-0) 12 Hawkins IF, Hawkins MC. A new deflector handle for catheter exchange. Radiology 1984. link 13 Doty DB, Truesdell SC, Marvin WJ. Techniques to avoid injury of the conduction tissue during the surgical treatment of corrected transposition. Circulation 1983. link 14 Bharati S, Rosen K, Steinfield L, Miller RA, Lev M. The anatomic substrate for preexcitation in corrected transposition. Circulation 1980. link 15 Attie F, Cuaron A, Marquez M, Buendia A, Zamora C, Acero A. Qualitative radionuclide angiocardiography in the diagnosis of corrected transposition. American heart journal 1979. link90347-8) 16 Foster JR, Damato AN, Kline LE, Akhtar M, Ruskin JN. Congenitally corrected transposition of the great vessels: localization of the site of complete atrioventricular block using his bundle electrograms. The American journal of cardiology 1976. link90181-8)

Corrected transposition of great vessels