Transposition of the Great Arteries

  • Michael Hofbeck
  • Karl-Heinz Deeg
  • Thomas Rupprecht
Chapter

Abstract

Transposition of the great arteries is a cardiac malformation, which is characterized by ventriculoarterial discordance. It is easily diagnosed by 2D echo in the precordial and subcostal views, confirming origin of the aorta from the morphologic right and of the pulmonary artery from the morphologic left ventricle. In the majority of cases, transposition of the great arteries is associated with concordant connections of the atria and ventricles. Colour Doppler echocardiography in neonates is essential to visualize shunting between both circulations across the atrial septum and the ductus arteriosus. It is also important in the search for ventricular septal defects, obstruction of the left and right ventricular outflow tract as well as possible obstruction of the aortic arch. PW and CW Doppler can be applied to document flow across an associated VSD, to quantify gradients across the outflow tracts and to assess flow across the aortic arch and ductus arteriosus.

Keywords

Ventriculoarterial discordance Atrioventricular discordance Transposition of the great arteries Congenitally corrected transposition 

Supplementary material

318084_1_En_15_MOESM1_ESM.mp4 (492 kb)
Video 15.1 The parasternal long-axis view in a newborn with transposition of the great arteries shows origin of a great artery from the left ventricle. This view does not reveal the ventriculoarterial discordance (AVI 8764 kb)
318084_1_En_15_MOESM2_ESM.mp4 (271 kb)
Video 15.2 Colour Doppler reveals unobstructed flow from the left ventricle to the transposed pulmonary artery (same patient as in Video 15.1) (AVI 1451 kb)
318084_1_En_15_MOESM3_ESM.mp4 (640 kb)
Video 15.3 A further cranial plane reveals parallel course of the anterior aorta and posterior pulmonary artery in another newborn with transposition of the great arteries (AVI 6130 kb)
318084_1_En_15_MOESM4_ESM.mp4 (1.7 mb)
Video 15.4 This clip in a newborn with transposition is obtained by cranial angulation of the transducer starting in the parasternal long-axis view of the left ventricle. It shows transition of the anterior vessel, which originates from the right ventricle, to the aortic arch confirming the diagnosis of transposition of the great arteries. Note the perimembranous VSD located underneath the pulmonary valve (AVI 25015 kb)
318084_1_En_15_MOESM5_ESM.mp4 (380 kb)
Video 15.5 The apical four-chamber view in a newborn with transposition of the great arteries shows normal anatomy of the ventricles (AVI 7496 kb)
318084_1_En_15_MOESM6_ESM.mp4 (344 kb)
Video 15.6 Colour Doppler in a more anterior plane (apical five-chamber view) shows a great artery originating from the left ventricle (same patient as in Video 15.6) (AVI 1362 kb)
318084_1_En_15_MOESM7_ESM.mp4 (301 kb)
Video 15.7 Further anterior tilt of the transducer reveals a second great artery (aorta) originating from the right ventricle and taking a parallel course to the pulmonary artery that originates from the left ventricle (same patient as in Videos 15.6 and 15.7) (AVI 1423 kb)
318084_1_En_15_MOESM8_ESM.mp4 (247 kb)
Video 15.8 The high parasternal short-axis view in a neonate with transposition of the great arteries reveals the dextroposed and anterior aorta in cross section, while the pulmonary artery and the pulmonary bifurcation are located posterior and to the left (AVI 5956 kb)
318084_1_En_15_MOESM9_ESM.mp4 (233 kb)
Video 15.9 Colour Doppler (same patient as in Video 15.8) reveals unobstructed flow in the pulmonary bifurcation. The vessel displayed anterior to the right pulmonary in cross section is the superior vena cava ( (AVI 1778 kb)
318084_1_En_15_MOESM10_ESM.mp4 (352 kb)
Video 15.10 The parasternal short-axis view at the level of the great arteries shows the anterior and dextroposed aorta. The aortic sinuses facing the pulmonary artery are termed sinus 1 and sinus 2; the sinus without contact to the pulmonary artery (non-facing sinus) is termed sinus 3 (see also Fig. 15.6) (AVI 4809 kb)
318084_1_En_15_MOESM11_ESM.mp4 (782 kb)
Video 15.11 The high parasternal short-axis view in this neonate with transposition of the great arteries shows anteroposterior position of the aorta and pulmonary artery (AVI 20674 kb)
318084_1_En_15_MOESM12_ESM.mp4 (1.1 mb)
Video 15.12 This slightly oblique parasternal short-axis view in a newborn with transposition of the great arteries visualizes origin of the right coronary artery from the aorta. Note the ectopic cranial origin of this coronary artery just above the sinotubular junction (AVI 16235 kb)
318084_1_En_15_MOESM13_ESM.mp4 (353 kb)
Video 15.13 The parasternal short-axis view displays the anteroposterior position of the great arteries and the facing sinuses of the aorta in a newborn with transposition of the great arteries. Note that both coronary arteries originate in close proximity from sinus 2 of the aorta. The left coronary artery takes an intramural course between the great arteries to reach the left ventricle (AVI 17305 kb)
318084_1_En_15_MOESM14_ESM.mp4 (386 kb)
Video 15.14 The ductal view in a neonate shows a large ductus arteriosus connecting the aorta and pulmonary artery. Note the fibrous shelf at the pulmonary end of the ductus (AVI 17882 kb)
318084_1_En_15_MOESM15_ESM.mp4 (255 kb)
Video 15.15 Obstruction of the pulmonary end of the ductus by the fibrous shelf is confirmed by colour Doppler interrogation (same patient as in Video 15.14) (AVI 3905 kb)
318084_1_En_15_MOESM16_ESM.mp4 (428 kb)
Video 15.16 Colour Doppler in the ductal view of this newborn with transposition of the great arteries shows significant hypoplasia of the distal aortic arch. Note that in the presence of a widely patent ductus arteriosus, there is no acceleration of flow in the distal aortic arch and aortic isthmus (AVI 2253 kb)
318084_1_En_15_MOESM17_ESM.mp4 (521 kb)
Video 15.17 The ductal view in another newborn with transposition shows severe preductal isthmic coarctation with a fibrous shelf distal to the left subclavian artery and just proximal to the widely patent ductus arteriosus (AVI 7924 kb)
318084_1_En_15_MOESM18_ESM.mp4 (271 kb)
Video 15.18 Colour Doppler (same patient as in Video 15.17) shows absence of turbulent flow in the distal aortic arch due to the presence of the widely patent ductus arteriosus. Due to the isthmic coarctation, flow from the distal aortic arch is oriented anteriorly towards the ductus arteriosus (AVI 2020 kb)
318084_1_En_15_MOESM19_ESM.mp4 (599 kb)
Video 15.19 Colour Doppler in the subcostal coronal view of the atrial septum in a neonate with transposition of the great arteries shows a restrictive foramen ovale with left to right shunting (AVI 4021 kb)
318084_1_En_15_MOESM20_ESM.mp4 (1.2 mb)
Video 15.20 This video clip in a slightly oblique subcostal coronal view from a newborn with transposition of the great arteries shows how a Rashkind balloon is advanced from the inferior vena cava to the right atrium and passing the foramen ovale to enter the left atrium (AVI 25062 kb)
318084_1_En_15_MOESM21_ESM.mp4 (709 kb)
Video 15.21 This sequence in the subcostal coronal view shows how the inflated Rashkind balloon is retracted from the left atrium to the right atrium (same patient as in Video 15.20) (AVI 24939 kb)
318084_1_En_15_MOESM22_ESM.mp4 (427 kb)
Video 15.22 The subcostal coronal view in a newborn with transposition of the great arteries shows origin of the pulmonary artery from the left ventricle (AVI 5342 kb)
318084_1_En_15_MOESM23_ESM.mp4 (384 kb)
Video 15.23 Colour Doppler confirms unobstructed flow from the left ventricle to the pulmonary artery (same patient as in Video 15.22). Retrograde flow in the pulmonary bifurcation is due to a patent ductus arteriosus (AVI 2426 kb)
318084_1_En_15_MOESM24_ESM.mp4 (926 kb)
Video 15.24 The video clip in the subcostal coronal view of a neonate with transposition of the great arteries starts in a posterior plane depicting origin of the pulmonary artery from the left ventricle. Anterior sweep of the transducer depicts origin of the aorta from the right ventricle. Note the parallel course of the great arteries (AVI 20218 kb)
318084_1_En_15_MOESM25_ESM.mp4 (380 kb)
Video 15.25 Colour Doppler following some clockwise rotation of the transducer from the coronal view nicely depicts the parallel orientation of the great arteries in a newborn with transposition of the great arteries (AVI 2421 kb)
318084_1_En_15_MOESM26_ESM.mp4 (583 kb)
Video 15.26 The slightly oblique subcostal coronal view in this newborn with transposition depicts parallel course of the great vessels and a large VSD that is located underneath the pulmonary valve (AVI 9476 kb)
318084_1_En_15_MOESM27_ESM.mp4 (338 kb)
Video 15.27 Colour Doppler in the subcostal sagittal (short axis) view in this newborn with transposition of the great arteries shows discrepancy in size between the smaller anterior aorta and large posterior pulmonary artery (AVI 2651 kb)
318084_1_En_15_MOESM28_ESM.mp4 (778 kb)
Video 15.28 The apical four-chamber view in a newborn with congenitally corrected transposition shows connection of the right atrium with a morphologic left ventricle and connection of the left atrium with a morphologic right ventricle. The morphologic right ventricle is characterized by the moderator band in the apex and more apical insertion of its respective atrioventricular valve (left-sided tricuspid valve). In addition the patient has a large perimembranous inlet ventricular septal defect (AVI 17952 kb)
318084_1_En_15_MOESM29_ESM.mp4 (198 kb)
Video 15.29 Colour Doppler in the apical four-chamber view (same patient as in Video 15.28) reveals moderate regurgitation of the left-sided systemic tricuspid valve (AVI 1920 kb)
318084_1_En_15_MOESM30_ESM.mp4 (3 mb)
Video 15.30 This video sequence from a child with congenitally corrected transposition shows a sweep in a high parasternal long-axis view starting from the pulmonary artery, which originates posteriorly from the left ventricle. Leftward tilt of the transducer displays the anterior aorta and its transition to the aortic arch. Note the parallel course of both vessels (AVI 38670 kb)
318084_1_En_15_MOESM31_ESM.mp4 (418 kb)
Video 15.31 The high parasternal short-axis view in a child with congenitally corrected transposition (same patient as in Video 15.30) displays the transposed aorta in an anterior and leftward position (AVI 7577 kb)
318084_1_En_15_MOESM32_ESM.mp4 (362 kb)
Video 15.32 The subcostal coronal view in a child with congenitally corrected transposition shows posterior origin of the pulmonary artery from the right-sided left ventricle. The pulmonary valve is stenotic. The aorta originates in a left anterior position from the left-sided right ventricle. Both ventricles are connected by a large VSD (AVI 5661 kb)
318084_1_En_15_MOESM33_ESM.mp4 (1.3 mb)
Video 15.33 The subcostal coronal view in a neonate with congenitally corrected transposition reveals mesocardia. The pulmonary artery originates from the left ventricle and exhibits subvalvular and valvular stenosis. The aorta originates in a left anterior position from the ventricle. The patient has a large perimembranous VSD (AVI 16418 kb)
318084_1_En_15_MOESM34_ESM.mp4 (402 kb)
Video 15.34 Colour Doppler in the subcostal coronal view confirms severe obstruction of the pulmonary outlet (same patient as in Video 15.33) (AVI 2429 kb)
318084_1_En_15_MOESM35_ESM.mp4 (229 kb)
Video 15.35 Colour Doppler in the subcostal coronal view of a newborn with transposition of the great arteries reveals obstruction of flow from the left ventricle to the transposed pulmonary artery due to pulmonary valve stenosis. The connection of the right ventricle to the aorta appears with obstruction (AVI 1120 kb)

References

  1. Anderson RH, Weinberg PM (2005) The clinical anatomy of transposition. Cardiol Young 15(Suppl 1):76–87CrossRefPubMedGoogle Scholar
  2. Baker EJ, Allan LD et al (1984) Balloon atrial septostomy in the neonatal intensive care unit. Br Heart J 51(4):377–378CrossRefPubMedPubMedCentralGoogle Scholar
  3. Bierman FZ, Williams RG (1979) Prospective diagnosis of d-transposition of the great arteries in neonates by subxiphoid, two-dimensional echocardiography. Circulation 60(7):1496–1502CrossRefPubMedGoogle Scholar
  4. Bonnet D, Coltri A et al (1999) Detection of transposition of the great arteries in fetuses reduces neonatal morbidity and mortality. Circulation 99(7):916–918CrossRefPubMedGoogle Scholar
  5. Chin AJ, Yeager SB et al (1985) Accuracy of prospective two-dimensional echocardiographic evaluation of left ventricular outflow tract in complete transposition of the great arteries. Am J Cardiol 55(6):759–764CrossRefPubMedGoogle Scholar
  6. D’Orsogna L, Lam J et al (1989) Assessment of bedside umbilical vein balloon septostomy using two-dimensional echocardiographic guidance in transposition of great arteries. Int J Cardiol 25(3):271–277CrossRefPubMedGoogle Scholar
  7. Freedom RM, Smallhorn JF et al (1992) Transposition of the great arteries. Neonatal Heart Disease. Freedom RM, Benson LN, Smallhorn JF. Springer, London/Berlin/HeidelbergGoogle Scholar
  8. Gittenberger-de Groot AC, Sauer U et al (1983) Coronary arterial anatomy in transposition of the great arteries: a morphologic study. Pediatr Cardiol 4(suppl 1):15–24Google Scholar
  9. Graham TP Jr, Bernard YD et al (2000) Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol 36(1):255–261CrossRefPubMedGoogle Scholar
  10. Jaggers JJ, Cameron DE et al (2000) Congenital Heart Surgery Nomenclature and Database Project: transposition of the great arteries. Ann Thorac Surg 69(4 Suppl):S205–S235CrossRefPubMedGoogle Scholar
  11. Jatene AD, Fontes VF et al (1975) Successful anatomic correction of transposition of the great vessels. A preliminary report. Arq Bras Cardiol 28(4):461–464PubMedGoogle Scholar
  12. Kang N, de Leval MR et al (2004) Extending the boundaries of the primary arterial switch operation in patients with transposition of the great arteries and intact ventricular septum. Circulation 110(11 Suppl 1):II123–II127PubMedGoogle Scholar
  13. Kirklin JW, Blackstone EH et al (1992) Clinical outcomes after the arterial switch operation for transposition – patient, support, procedural, and institutional risk-factors. Circulation 86(5):1501–1515CrossRefPubMedGoogle Scholar
  14. Lau KC, Mok CK et al (1987) Balloon atrial septostomy under two-dimensional echocardiographic control. Pediatr Cardiol 8(1):35–37CrossRefPubMedGoogle Scholar
  15. Lecompte Y, Zannini L et al (1981) Anatomic correction of transposition of the great-arteries – new technique without use of a prosthetic conduit. J Thorac Cardiovasc Surg 82(4):629–631PubMedGoogle Scholar
  16. Lindinger A, Schwedler G et al (2010) Prevalence of congenital heart defects in newborns in Germany: results of the first registration year of the PAN Study (July 2006 to June 2007). Klin Padiatr 222(5):321–326CrossRefPubMedGoogle Scholar
  17. Lundstrom U, Bull C et al (1990) The natural and “unnatural” history of congenitally corrected transposition. Am J Cardiol 65(18):1222–1229CrossRefPubMedGoogle Scholar
  18. Mahle WT, Marx GR et al (2006) Anatomy and echocardiography of discordant atrioventricular connections. Cardiol Young 16(Suppl 3):65–71CrossRefPubMedGoogle Scholar
  19. Marino B, de Simone G et al (1985) Complete transposition of the great arteries: visualization of left and right outflow tract obstruction by oblique subcostal two-dimensional echocardiography. Am J Cardiol 55(9):1140–1145CrossRefPubMedGoogle Scholar
  20. Pasquini L, Parness IA et al (1993) Diagnosis of intramural coronary-artery in transposition of the great-arteries using 2-dimensional echocardiography. Circulation 88(3):1136–1141CrossRefPubMedGoogle Scholar
  21. Pasquini L, Sanders SP et al (1987) Diagnosis of coronary artery anatomy by two-dimensional echocardiography in patients with transposition of the great arteries. Circulation 75(3):557–564CrossRefPubMedGoogle Scholar
  22. Pasquini L, Sanders SP et al (1994) Coronary echocardiography in 406 patients with D-loop transposition of the great-arteries. J Am Coll Cardiol 24(3):763–768CrossRefPubMedGoogle Scholar
  23. Qamar ZA, Goldberg CS et al (2007) Current risk factors and outcomes for the arterial switch operation. Ann Thorac Surg 84(3):871–878; discussion 878-9CrossRefPubMedGoogle Scholar
  24. Rashkind WJ, Miller WW (1966) Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. JAMA 196(11):991–992CrossRefPubMedGoogle Scholar
  25. Schuhmacher G, Hess J et al (2008) Klinische Kinderkardiologie, Springer Medizin VerlagGoogle Scholar
  26. Schwedler G, Lindinger A et al (2011) Frequency and spectrum of congenital heart defects among live births in Germany : a study of the Competence Network for Congenital Heart Defects. Clin Res Cardiol Off J German Cardiac Soc 100(12):1111–7CrossRefGoogle Scholar
  27. Sharland G, Tingay R et al (2005) Atrioventricular and ventriculoarterial discordance (congenitally corrected transposition of the great arteries): echocardiographic features, associations, and outcome in 34 fetuses. Heart 91(11):1453–1458CrossRefPubMedPubMedCentralGoogle Scholar
  28. Wilkinson JL, Cochrane AD et al (2000) Congenital Heart Surgery Nomenclature and Database Project: corrected (discordant) transposition of the great arteries (and related malformations). Ann Thorac Surg 69(4 Suppl):S236–S248CrossRefPubMedGoogle Scholar
  29. Williams WG, McCrindle BW et al (2003) Outcomes of 829 neonates with complete transposition of the great arteries 12-17 years after repair. Eur J Cardiothorac Surg 24(1):1–9CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Michael Hofbeck
    • 1
  • Karl-Heinz Deeg
    • 2
  • Thomas Rupprecht
    • 3
  1. 1.Universitätskinderklinik Tübingen Abt. KinderkardiologieTübingenGermany
  2. 2.Pediatric Clinic of the Sozialstiftung BambergBambergGermany
  3. 3.Klinikum Bayreuth GmbH Klinik für Kinderheilkunde/JugendmedizinBayreuthGermany

Personalised recommendations