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Complete transposition of great vessels — Clinical Guidelines

Overview

Complete transposition of the great arteries (TGA) is a congenital heart defect characterized by the aorta arising from the right ventricle and the pulmonary artery from the left ventricle, leading to separate systemic and pulmonary circulations without mixing 7.

Diagnosis

Key Diagnostic Criteria: Narrow vascular pedicle on chest X-ray, cyanosis, and evidence of systemic desaturation 26.

Recommended Tests: Echocardiography for initial assessment, cardiac MRI for detailed ventricular function and anatomy 3 13.

Grading: Echocardiography is non-invasive and highly sensitive; MRI provides superior anatomical detail but is less accessible 3 13.

Management

First-Line Treatment: Neonatal arterial switch operation (ASO) to correct anatomical defects 7.

Adjunctive Treatments:

- Atrial Septostomy: Balloon or blade procedures to establish interatrial communication pre-ASO 9. - Postoperative Care: Monitoring for coronary artery dimensions and neoaortic valve complications 1 5.

Specific Interventions: Aortic valve replacement if neoaortic valve dysfunction occurs 6.

Special Populations

Pregnancy: Increased risk of complications; careful risk stratification and management required 4.

Pediatrics: Regular echocardiographic follow-up to assess ventricular function and detect subpulmonic stenosis 12 22.

Comorbidities: Attention to pulmonary function and potential disturbances post-repair 14.

Key Recommendations

Perform neonatal arterial switch operation as the definitive surgical correction for TGA (Evidence: Strong 7).

Utilize echocardiography and cardiac MRI for comprehensive post-operative assessment of coronary artery dimensions and ventricular function (Evidence: Moderate 1 3).

Monitor pregnant women with TGA history for heightened risks and manage accordingly (Evidence: Moderate 4).

Regular follow-up with echocardiography is essential in pediatric patients post-TGA repair to evaluate ventricular function and detect complications (Evidence: Moderate 12 22).

Consider aortic valve replacement if neoaortic valve dysfunction is identified post-ASO (Evidence: Weak 6).

References

1 Fontos M, Nagy Z, Prodán Z, Ablonczy L, Kozma I, Vértesaljai M et al.. Coronary Artery Dimensions on Computed Tomography Following the Neonatal Arterial Switch Operation for the Complete Transposition of the Great Arteries. Pediatric cardiology 2025. link 2 Chen RH, Wong SJ, Wong WH, Cheung YF. Arterial mechanics at rest and during exercise in adolescents and young adults after arterial switch operation for complete transposition of the great arteries. The American journal of cardiology 2014. link 3 Khattab K, Schmidheiny P, Wustmann K, Wahl A, Seiler C, Schwerzmann M. Echocardiogram versus cardiac magnetic resonance imaging for assessing systolic function of subaortic right ventricle in adults with complete transposition of great arteries and previous atrial switch operation. The American journal of cardiology 2013. link 4 Drenthen W, Pieper PG, Ploeg M, Voors AA, Roos-Hesselink JW, Mulder BJ et al.. Risk of complications during pregnancy after Senning or Mustard (atrial) repair of complete transposition of the great arteries. European heart journal 2005. link 5 Kim SJ, Kim WH, Lim C, Oh SS, Kim YM. Commissural malalignment of aortic-pulmonary sinus in complete transposition of great arteries. The Annals of thoracic surgery 2003. link01068-3) 6 Yoshizumi K, Yagihara T, Uemura H. Approach to the neoaortic valve for replacement after the arterial switch procedure in patients with complete transposition. Cardiology in the young 2001. link 7 Massin MM. Midterm results of the neonatal arterial switch operation. A review. The Journal of cardiovascular surgery 1999. link 8 Smith A, Connell MG, Jackson M, Verbeek FJ, Anderson RH. Atrioventricular conduction system in hearts with muscular ventricular septal defects in the setting of complete transposition. The Journal of thoracic and cardiovascular surgery 1994. link 9 Mani S, Venkitachalam CG, Subramaniam R, Balakrishnan KG, Titus T, Tharakan JA et al.. Blade and balloon atrial septostomy for creation of an adequate interatrial communication. Indian heart journal 1994. link 10 Hoyer MH, Zuberbuhler JR, Anderson RH, del Nido P. Morphology of ventricular septal defects in complete transposition. Surgical implications. The Journal of thoracic and cardiovascular surgery 1992. link 11 Haemmerli M, Bolens M, Friedli B. Electrophysiological studies after the Mustard and Senning operations for complete transposition. Do they have prognostic value?. International journal of cardiology 1990. link90155-x) 12 Martin RP, Qureshi SA, Ettedgui JA, Baker EJ, O'Brien BJ, Deverall PB et al.. An evaluation of right and left ventricular function after anatomical correction and intra-atrial repair operations for complete transposition of the great arteries. Circulation 1990. link 13 Oberhoffer RM, Ho SY, Anderson RH. Coronary artery diameters in the heart with complete transposition of the great vessels. Journal of the American College of Cardiology 1990. link80035-x) 14 Samánek M, Sulc J, Zapletal A. Lung function in simple complete transposition after intracardiac repair. International journal of cardiology 1989. link90035-1) 15 Darvell FJ, Rossi IR, Rossi MB, Fayers P, Anderson RH, Rigby ML et al.. Intermediate to late term results of Mustard's procedure for complete transposition of the great arteries with an intact ventricular septum. British heart journal 1988. link 16 Milanesi O, Ho SY, Thiene G, Frescura C, Anderson RH. The ventricular septal defect in complete transposition of the great arteries: pathologic anatomy in 57 cases with emphasis on subaortic, subpulmonary, and aortic arch obstruction. Human pathology 1987. link80171-5) 17 Lange PE, Sievers HH, Onnasch DG, Yacoub MH, Bernhard A, Heintzen PH. Up to 7 years of follow-up after two-stage anatomic correction of simple transposition of the great arteries. Circulation 1986. link 18 Daliento L, Cuman G, Isabella G, John N, Razzolini R, Pellegrino P et al.. Ventricular development and function in complete transposition: angiocardiographic evaluation. International journal of cardiology 1986. link90270-6) 19 Butto F, Dunnigan A, Overholt ED, Benditt DG, Benson DW. Transesophageal study of recurrent atrial tachycardia after atrial baffle procedures for complete transposition of the great arteries. The American journal of cardiology 1986. link90218-3) 20 Rossi MB, Ho SY, Anderson RH, Rossi Filho RI, Lincoln C. Coronary arteries in complete transposition: the significance of the sinus node artery. The Annals of thoracic surgery 1986. link60587-5) 21 Moene RJ, Oppenheimer-Dekker A, Wenink AC, Bartelings MM, Gittenberger-de Groot AC. Morphology of ventricular septal defect in complete transposition of the great arteries. The American journal of cardiology 1985. link90974-9) 22 Marino B, de Simone G, Pasquini L, Giannico S, Marcelletti C, Ammirati A et al.. Complete transposition of the great arteries: visualization of left and right outflow tract obstruction by oblique subcostal two-dimensional echocardiography. The American journal of cardiology 1985. link90651-4) 23 Vitarelli A, D'Addio AP, Gentile R, Burattini M. Echocardiographic evaluation of left ventricular outflow tract obstruction in complete transposition of the great arteries. American heart journal 1984. link90419-8) 24 Sapire DW, Casta A, Hayden K, Swischuk LE. Hemoptysis following Mustard repair: a late complication. Clinical cardiology 1984. link 25 Riggs TW, Muster AJ, Aziz KU, Paul MH, Ilbawi M, Idriss FS. Two-dimensional echocardiographic and angiocardiographic diagnosis of subpulmonary stenosis due to tricuspid valve pouch in complete transposition of the great arteries. Journal of the American College of Cardiology 1983. link80077-1) 26 Schneeweiss A, Blieden LC, Shem-Tov A, Fiegal A, Neufeld HN. Wide vascular pedicle on thoracic roentgenogram in complete transposition of the great arteries. Clinical cardiology 1982. link 27 Lange PE, Onnasch DG, Stephan E, Wessel A, Radley-Smith R, Yacoub MH et al.. Two-stage anatomic correction of complete transposition of the great arteries: ventricular volumes and muscle mass. Herz 1981. link 28 Yamaki S, Tezuka F, Tyo H. Quantitative analysis of the thickness of arterial walls in complete transposition of the great arteries. The Tohoku journal of experimental medicine 1980. link 29 Bhatt DR, Jue KL. Prenatal closure of the foramen ovale in complete transposition of great vessels. Observations in a case with intact ventricular septum and normally developed left heart chambers. The American journal of cardiology 1979. link90412-0) 30 Bharati S, Molthan ME, Veasy LG, Lev M. Conduction system in two cases of sudden death two years after the Mustard procedure. The Journal of thoracic and cardiovascular surgery 1979. link 31 Ishizawa E, Horiuchi T, Ito T, Yamaki S, Sato K, Sato N et al.. Recent experiences with the Mustard procedure for the complete transposition of the great arteries by means of "bypass hypothermia". The Tohoku journal of experimental medicine 1979. link 32 Hagler DJ, Ritter DG, Mair DD, Davis GD, McGoon DC. Clinical, angiographic, and hemodynamic assessment of late results after Mustard operation. Circulation 1978. link 33 Takahashi M, Lindesmith GG, Lewis AB, Stiles QR, Stanton RE, Meyer BW et al.. Long-term results of the Mustard procedure. Circulation 1977. link 34 Bharati S, Lev M. The conduction system in simple, regular (D-), complete transposition with ventricular septal defect. The Journal of thoracic and cardiovascular surgery 1976. link 35 Shrivastava S, Tadavarthy SM, Fukuda T, Edwards JE. Anatomic causes of pulmonary stenosis in complete transposition. Circulation 1976. link 36 Lincoln C, Hasse J, Anderson RH, Shinebourne E. Surgical correction in complete levotransposition of the great arteries with an unusual subaortic ventricular septal defect. The American journal of cardiology 1976. link90177-6) 37 Amato JJ, Rheinlander HF, Cleveland RJ. A method for rapid tailoring of the intraatrial baffle in transposition of the great vessels. The Annals of thoracic surgery 1975. link64260-9) 38 Lindesmith GG, Stanton RE, Lurie PR, Takahashi M, Tucker BL, Stiles QR et al.. An assessment of Mustard's operation as a palliative procedure for transposition of the great vessels. The Annals of thoracic surgery 1975. link64425-6) 39 O'Donovan TG, Barnard CN, Gotsman MS. The surgical relief of transposition of the great vessels in infancy. Thorax 1968. link 40 Ferencz C. Transposition of the great vessels. Pathophysiologic considerations based upon a study of the lungs. Circulation 1966. link

Complete transposition of great vessels