Advertisement

Introduction to Congenital Heart Disease Anatomy

  • Pierangelo RenellaEmail author
  • J. Paul Finn
Chapter

Abstract

Imaging of the complex anatomy associated with many forms of congenital heart disease (CHD) requires knowledge of the morphology of the various cardiac chambers and extra-cardiac vessels. For the cardiac diagnostician, assembling together the pieces of disordered anatomy is best done with the so-called “segmental approach.” This approach breaks down the cardiovascular anatomy sequentially, considering first the position of the abdominal viscera, next the cardiac atria, then the looping pattern of the ventricles, and finally, the position of the semilunar valves and great arteries. In this manner, the various forms of CHD may be precisely identified and the proper management applied by the clinician. The large unrestricted field of view, coupled with three-dimensional multiplanar reconstruction and volume rendering capability, as well as the lack of ionizing radiation exposure to the patient, make cardiac magnetic resonance (CMR) the ideal imaging modality for the initial evaluation and serial follow-up of patients with CHD. This holds particularly true for adult and post-operative patients who may have suboptimal echocardiographic imaging windows. This chapter introduces the segmental approach to the diagnosis of CHD. Each “segment” of the cardiovascular system is described with particular attention paid to the distinguishing features of normal structures so that abnormal features may be more clearly identified. Salient examples of pathology in each segment are also presented with their relevant clinical features.

Keywords

Inferior Vena Cava Pulmonary Vein Cardiac Magnetic Resonance Left Atrium Right Ventricle 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Movie 3.1

Balanced AVCD (MOV 1328 KB)

218193_1_En_3_MOESM2_ESM.mov (3.3 mb)
Movie 3.2 RAA with Ab LSCA (MOV 3376 KB)
218193_1_En_3_MOESM3_ESM.mov (2.3 mb)
Movie 3.3 3D VR full body infant angiogram – CoA (MOV 2376)

References

  1. 1.
    VanPraagh R. Segmental approach to diagnosis. In: Keane JE, Lock JE, Flyer DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 39–46.Google Scholar
  2. 2.
    Cohen MS, Anderson RH, Cohen MI, Atz AM, Fogel M, Gruber PJ, Lopez L, Rome JJ, Weinberg PM. Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 2007;17 Suppl 2:29–43.PubMedGoogle Scholar
  3. 3.
    Jacobs JP, Anderson RH, Weinberg PM, Walters 3rd HL, Tchervenkov CI, Del Duca D, Franklin RC, Aiello VD, Beland MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young. 2007;17 Suppl 2:1–28.PubMedGoogle Scholar
  4. 4.
    Bartram U, Wirbelauer J, Speer CP. Heterotaxy syndrome – ­asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 2005;88(4):278–90.PubMedCrossRefGoogle Scholar
  5. 5.
    Van Mierop L, Gessner IH, Schiebler GL. Asplenia and polysplenia syndrome. Birth Defects. 1972;8:74–84.Google Scholar
  6. 6.
    Peoples WM, Moller JH, Edwards JE. Polysplenia: a review of 146 cases. Pediatr Cardiol. 1983;4(2):129–37.PubMedCrossRefGoogle Scholar
  7. 7.
    Bartz PJ, Driscoll DJ, Dearani JA, Puga FJ, Danielson GK, O’Leary PW, Earing MG, Warnes CA, Hodge DO, Cetta F. Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol. 2006;48(11):2301–5.PubMedCrossRefGoogle Scholar
  8. 8.
    Geva T, Vick 3rd GW, Wendt RE, Rokey R. Role of spin echo and cine magnetic resonance imaging in presurgical planning of heterotaxy syndrome. Comparison with echocardiography and catheterization. Circulation. 1994;90(1):348–56.PubMedCrossRefGoogle Scholar
  9. 9.
    Hong YK, Park YW, Ryu SJ, Won JW, Choi JY, Sul JH, Lee SK, Cho BK, Choe KO. Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. J Comput Assist Tomogr. 2000;24(5):671–82.PubMedCrossRefGoogle Scholar
  10. 10.
    Edwards WD. Cardiac anatomy and examination of cardiac specimens. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents, vol. 1. Philadelphia: Lippincott, Williams, and Wilkins; 2007. p. 5–8.Google Scholar
  11. 11.
    Mazzucco A, Bortolotti U, Stellin G, Gallucci V. Anomalies of the systemic venous return: a review. J Card Surg. 1990;5(2):122–33.PubMedCrossRefGoogle Scholar
  12. 12.
    Celentano C, Malinger G, Rotmensch S, Gerboni S, Wolman Y, Glezerman M. Prenatal diagnosis of interrupted inferior vena cava as an isolated finding: a benign vascular malformation. Ultrasound Obstet Gynecol. 1999;14(3):215–8.PubMedCrossRefGoogle Scholar
  13. 13.
    Edwards WD. Cardiac anatomy and examination of cardiac specimens. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents, vol. 1. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 2–33.Google Scholar
  14. 14.
    Pappone C, Rosanio S, Oreto G, Tocchi M, Gugliotta F, Vicedomini G, Salvati A, Dicandia C, Mazzone P, Santinelli V, Gulletta S, Chierchia S. Circumferential radiofrequency ablation of pulmonary vein ostia: a new anatomic approach for curing atrial fibrillation. Circulation. 2000;102(21):2619–28.PubMedCrossRefGoogle Scholar
  15. 15.
    Stein P. Total anomalous pulmonary venous connection. AORN J. 2007;85(3):509–20; quiz 521–504.PubMedCrossRefGoogle Scholar
  16. 16.
    Keane JF, Flyer DC. Total anomalous pulmonary venous return. In: Keane JF, Lock JE, Flyer DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 773–81.Google Scholar
  17. 17.
    Dorosz JL, Bolson EL, Waiss MS, Sheehan FH. Three-dimensional visual guidance improves the accuracy of calculating right ventricular volume with two-dimensional echocardiography. J Am Soc Echocardiogr. 2003;16(6):675–81.PubMedCrossRefGoogle Scholar
  18. 18.
    Kuhl HP, Schreckenberg M, Rulands D, Katoh M, Schafer W, Schummers G, Bucker A, Hanrath P, Franke A. High-resolution transthoracic real-time three-dimensional echocardiography: quantitation of cardiac volumes and function using semi-automatic border detection and comparison with cardiac magnetic resonance imaging. J Am Coll Cardiol. 2004;43(11):2083–90.PubMedCrossRefGoogle Scholar
  19. 19.
    Owens GE, Gomez-Fifer C, Gelehrter S, Owens ST. Outcomes for patients with unbalanced atrioventricular septal defects. Pediatr Cardiol. 2009;30(4):431–5.PubMedCrossRefGoogle Scholar
  20. 20.
    Rogers HM, Edwards JE. Incomplete division of the atrioventricular canal with patent inter-atrial foramen primum, persistent common atrioventricular ostium; report of five cases and review of the literature. Am Heart J. 1948;36(1):28–54.PubMedCrossRefGoogle Scholar
  21. 21.
    Dunlop KA, Mulholland HC, Casey FA, Craig B, Gladstone DJ. A ten year review of atrioventricular septal defects. Cardiol Young. 2004;14(1):15–23.PubMedCrossRefGoogle Scholar
  22. 22.
    Geva T, Ayres NA, Pignatelli RH, Gajarski RJ. Echocardiographic evaluation of common atrioventricular canal defects: a study of 206 consecutive patients. Echocardiography. 1996;13(4):387–400.PubMedCrossRefGoogle Scholar
  23. 23.
    Laursen HB. Congenital heart disease in Down’s syndrome. Br Heart J. 1976;38(1):32–8.PubMedCrossRefGoogle Scholar
  24. 24.
    Cetta F, Minich L, Edwards WD, Dearani JA, Puga FJ. Atrioventricular septal defects. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents, vol. 1. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 647–67.Google Scholar
  25. 25.
    Lev M, Liberthson RR, Kirkpatrick JR, Eckner FA, Arcilla RA. Single (primitive) ventricle. Circulation. 1969;39(5):577–91.PubMedCrossRefGoogle Scholar
  26. 26.
    Keane JF, Flyer DC. Single ventricle. In: Keane JF, Lock JE, Flyer DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 743–51.Google Scholar
  27. 27.
    Fulton DR, Flyer DC. D-transposition of the great arteries. In: Keane JF, Lock JE, Flyer DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 645–61.Google Scholar
  28. 28.
    Botto LD, Correa A, Erickson JD. Racial and temporal variations in the prevalence of heart defects. Pediatrics. 2001;107(3):E32.PubMedCrossRefGoogle Scholar
  29. 29.
    Blume ED, Altmann K, Mayer JE, Colan SD, Gauvreau K, Geva T. Evolution of risk factors influencing early mortality of the arterial switch operation. J Am Coll Cardiol. 1999;33(6):1702–9.PubMedCrossRefGoogle Scholar
  30. 30.
    Hornung TS, Calder L. Congenitally corrected transposition of the great arteries. Heart. 2010;96(14):1154–61.PubMedCrossRefGoogle Scholar
  31. 31.
    Van Praagh R, Van Praagh S. Anatomically corrected transposition of the great arteries. Br Heart J. 1967;29(1):112–9.PubMedCrossRefGoogle Scholar
  32. 32.
    Powell AJ, Mandell VS. Vascular rings and slings. In: Keane JF, Lock JE, Flyer DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 811–23.Google Scholar
  33. 33.
    Kellenberger CJ. Aortic arch malformations. Pediatr Radiol. 2010;40(6):876–84.PubMedCrossRefGoogle Scholar
  34. 34.
    Edwards JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am. 1948;32:925–49.PubMedGoogle Scholar
  35. 35.
    Kussman BD, Geva T, McGowan FX. Cardiovascular causes of airway compression. Paediatr Anaesth. 2004;14(1):60–74.PubMedCrossRefGoogle Scholar
  36. 36.
    Dodge-Khatami A, Tulevski II, Hitchcock JF, de Mol BA, Bennink GB. Vascular rings and pulmonary arterial sling: from respiratory collapse to surgical cure, with emphasis on judicious imaging in the hi-tech era. Cardiol Young. 2002;12(2):96–104.PubMedCrossRefGoogle Scholar
  37. 37.
    Edwards JE. Malformations of the aortic arch system manifested as vascular rings. Lab Invest. 1953;2(1):56–75.PubMedGoogle Scholar
  38. 38.
    Fiore AC, Brown JW, Weber TR, Turrentine MW. Surgical treatment of pulmonary artery sling and tracheal stenosis. Ann Thorac Surg. 2005;79(1):38–46; discussion 38–46.PubMedCrossRefGoogle Scholar
  39. 39.
    Beekman RH. Coarctation of the aorta. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents, vol. 2. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 987–1005.Google Scholar
  40. 40.
    Praagh RV, Praagh SV. Morphologic anatomy. In: Keane JF, Lock JE, Flyer DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 27–37.Google Scholar
  41. 41.
    Reardon MJ, Cooley DA, Kubrusly L, Ott DA, Johnson W, Kay GL, Sweeney MS. Total anomalous pulmonary venous return: report of 201 patients treated surgically. Tex Heart Inst J. 1985 Jun;12(2):131–41.Google Scholar

Copyright information

© Springer-Verlag London 2012

Authors and Affiliations

  1. 1.Department of Radiology SciencesDavid Geffen School of Medicine, Ronald Reagan UCLA Medical CenterLos AngelesUSA
  2. 2.Department of Pediatric CardiologyUC-Irvine College of Medicine, Children’s Hospital Orange CountyOrangeUSA
  3. 3.University of California Los Angeles (UCLA)Los AngelesUSA
  4. 4.Department of RadiologyDiagnostic Cardiovascular Imaging Section, UCLALos AngelesUSA

Personalised recommendations