Advertisement

Septal Defects

  • Stephan Waelti
  • Julie Déry
  • Chantale LapierreEmail author
Chapter
Part of the Medical Radiology book series (MEDRAD)

Abstract

Cardiac septal defects are the most common form of congenital heart disease if bicuspid aortic valve is excluded. This category includes atrial septal defect (ASD), atrioventricular septal defect, and ventricular septal defect (VSD). The different types of ASD are: primum, secundum, sinus venosus, and unroofed coronary sinus. The most common forms of atrioventricular septal defect are complete and partial whereas the four main groups of VSDs are inlet, muscular, membranous, and outlet. A good knowledge of the anatomy is necessary to classify adequately the ASDs and VSDs. Transthoracic echocardiography is the primary and most important imaging modality and MRI can be useful when echocardiography is not feasible or not diagnostic.

References

  1. Abaci A, Unlu S, Alsancak Y, Kaya U, Sezenoz B (2013) Short and long term complications of device closure of atrial septal defect and patent foramen ovale: meta-analysis of 28, 142 patients from 203 studies. Catheter Cardiovasc Interv 82:1123–1138PubMedGoogle Scholar
  2. al Zaghal AM, Li J, Anderson RH, Lincoln C, Shore D, Rigby ML (1997) Anatomical criteria for the diagnosis of sinus venosus defects. Heart 78:298–304PubMedPubMedCentralGoogle Scholar
  3. Allen HD, Driscoll DJ, Shaddy RE, Feltes TF (2008) Moss and Adams heart disease in infants, children, and adolescents, 7th edn. Lippincott Williams & Wilkins, PhiladelphiaGoogle Scholar
  4. Alsoufi B, Karamlou T, Osaki M et al (2006) Surgical repair of multiple muscular ventricular septal defects: the role of re-endocardialization strategy. J Thorac Cardiovasc Surg 132:1072–1080PubMedGoogle Scholar
  5. Anderson RH, Ho SY, Falcao S, Daliento L, Rigby ML (1998) The diagnostic features of atrioventricular septal defect with common atrioventricular junction. Cardiol Young 8:33–49PubMedGoogle Scholar
  6. Attie F, Rosas M, Granados N, Zabal C, Buendia A, Calderon J (2001) Surgical treatment for secundum atrial septal defects in patients >40 years old. A randomized clinical trial. J Am Coll Cardiol 38:2035–2042PubMedGoogle Scholar
  7. Bacha EA, Cao QL, Galantowicz ME et al (2005) Multicenter experience with perventricular device closure of muscular ventricular septal defects. Pediatr Cardiol 26:169–175PubMedGoogle Scholar
  8. Baumgartner H, Bonhoeffer P, De Groot NM et al (2010) ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 31:2915–2957PubMedGoogle Scholar
  9. Blom NA, Ottenkamp J, Jongeneel TH, DeRuiter MC, Gittenberger-de Groot AC (2005) Morphogenetic differences of secundum atrial septal defects. Pediatr Cardiol 26:338–343PubMedGoogle Scholar
  10. Botto LD, Correa A, Erickson JD (2001) Racial and temporal variations in the prevalence of heart defects. Pediatrics 107:E32PubMedGoogle Scholar
  11. Bremerich J, Reddy GP, Higgins CB (1999) MRI of supracristal ventricular septal defects. J Comput Assist Tomogr 23:13–15PubMedGoogle Scholar
  12. Bricker AO, Avutu B, Mohammed TL et al (2010) Valsalva sinus aneurysms: findings at CT and MR imaging. Radiographics 30:99–110PubMedGoogle Scholar
  13. Brickner ME, Hillis LD, Lange RA (2000) Congenital heart disease in adults. Second of two parts. N Engl J Med 342:334–342PubMedGoogle Scholar
  14. Brizard CP, Olsson C, Wilkinson JL (2004) New approach to multiple ventricular septal defect closure with intraoperative echocardiography and double patches sandwiching the septum. J Thorac Cardiovasc Surg 128:684–692PubMedGoogle Scholar
  15. Calkoen EE, Hazekamp MG, Blom NA et al (2016) Atrioventricular septal defect: from embryonic development to long-term follow-up. Int J Cardiol 202:784–795PubMedGoogle Scholar
  16. Campbell M (1970) Natural history of atrial septal defect. Br Heart J 32(6):820PubMedPubMedCentralGoogle Scholar
  17. Corno AF, Kandakure PR, Dhannapuneni RR, Gladman G, Venugopal P, Alphonso N (2013) Multiple ventricular septal defects: a new strategy. Front Pediatr 1:16PubMedPubMedCentralGoogle Scholar
  18. Davia JE, Cheitlin MD, Bedynek JL (1973) Sinus venosus atrial septal defect: analysis of fifty cases. Am Heart J 85:177–185PubMedGoogle Scholar
  19. Didier D, Higgins CB (1986) Identification and localization of ventricular septal defect by gated magnetic resonance imaging. Am J Cardiol 57:1363–1368PubMedGoogle Scholar
  20. Didier D, Higgins CB, Fisher MR, Osaki L, Silverman NH, Cheitlin MD (1986) Congenital heart disease: gated MR imaging in 72 patients. Radiology 158:227–235PubMedGoogle Scholar
  21. Driscoll A, Worrall-Carter L, Stewart S (2006) Rationale and design of the National Benchmarking and evidence-based National Clinical Guidelines for chronic heart failure management programs study. J Cardiovasc Nurs 21:276–282PubMedGoogle Scholar
  22. Gersony WM (2001) Natural history and decision-making in patients with ventricular septal defect. Prog Pediatr Cardiol 14:125–132Google Scholar
  23. Geva T, Martins JD, Wald RM (2014) Atrial septal defects. Lancet 383:1921–1932PubMedGoogle Scholar
  24. Hagen PT, Scholz DG, Edwards WD (1984) Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. Mayo Clin Proc 59:17–20PubMedGoogle Scholar
  25. Hanslik A, Pospisil U, Salzer-Muhar U, Greber-Platzer S, Male C (2006) Predictors of spontaneous closure of isolated secundum atrial septal defect in children: a longitudinal study. Pediatrics 118:1560–1565PubMedGoogle Scholar
  26. Helgason H, Jonsdottir G (1999) Spontaneous closure of atrial septal defects. Pediatr Cardiol 20:195–199PubMedGoogle Scholar
  27. Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900PubMedPubMedCentralGoogle Scholar
  28. Hoffman JI, Kaplan S, Liberthson RR (2004) Prevalence of congenital heart disease. Am Heart J 147:425–439PubMedGoogle Scholar
  29. Holzer R, Balzer D, Cao QL, Lock K, Hijazi ZM (2004) Amplatzer muscular ventricular Septal defect I. Device closure of muscular ventricular septal defects using the Amplatzer muscular ventricular septal defect occluder: immediate and mid-term results of a U.S. registry. J Am Coll Cardiol 43:1257–1263PubMedGoogle Scholar
  30. Hugh D, Allen RES, Daniel J (2016) Penny, Timothy F. Feltes, Frank Cetta. Moss and Adams’ Heart Disease in infants, children and adolescents. 9th ednGoogle Scholar
  31. Humenberger M, Rosenhek R, Gabriel H et al (2011) Benefit of atrial septal defect closure in adults: impact of age. Eur Heart J 32:553–560PubMedGoogle Scholar
  32. Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C (2000) Congenital heart surgery nomenclature and database project: ventricular septal defect. Ann Thorac Surg 69:S25–S35PubMedGoogle Scholar
  33. Kafka H, Mohiaddin RH (2009) Cardiac MRI and pulmonary MR angiography of sinus venosus defect and partial anomalous pulmonary venous connection in cause of right undiagnosed ventricular enlargement. AJR Am J Roentgenol 192:259–266PubMedGoogle Scholar
  34. Kirklin JK, Castaneda AR, Keane JF, Fellows KE, Norwood WI (1980) Surgical management of multiple ventricular septal defects. J Thorac Cardiovasc Surg 80:485–493PubMedGoogle Scholar
  35. Kitagawa T, Durham LA III, Mosca RS, Bove EL (1998) Techniques and results in the management of multiple ventricular septal defects. J Thorac Cardiovasc Surg 115:848–856PubMedGoogle Scholar
  36. Ko SF, Liang CD, Yip HK et al (2009) Amplatzer septal occluder closure of atrial septal defect: evaluation of transthoracic echocardiography, cardiac CT, and transesophageal echocardiography. AJR Am J Roentgenol 193:1522–1529PubMedGoogle Scholar
  37. Konstantinides S, Geibel A, Olschewski M et al (1995) A comparison of surgical and medical therapy for atrial septal defect in adults. N Engl J Med 333:469–473PubMedGoogle Scholar
  38. Kucher N, Seiler C, Allemann Y, Eberli FR (2001) Double-chambered right ventricle. Circulation 103:E105–E106PubMedGoogle Scholar
  39. Langer C, Horstkotte D, Piper C (2007) Aneurysm of the membranous septum causes pre-syncopes and transient bilateral blindness. Eur Heart J 28:784PubMedGoogle Scholar
  40. Lapierre C, Hugues N, Dahdah N, Dery J, Raboisson MJ, Miro J (2012) Long-term follow-up of large atrial septal occluder (Amplatzer device) with cardiac MRI in a pediatric population. AJR Am J Roentgenol 199:1136–1141PubMedGoogle Scholar
  41. Loukas M, Tubbs RS, Louis RG Jr, Curry B (2006) Pseudoaneurysm of the membranous septum, case report and review of the literature. Surg Radiol Anat 28:564–568PubMedGoogle Scholar
  42. Lun K, Li H, Leung MP et al (2001) Analysis of indications for surgical closure of subarterial ventricular septal defect without associated aortic cusp prolapse and aortic regurgitation. Am J Cardiol 87:1266–1270PubMedGoogle Scholar
  43. Matsuwaka R, Tomokuni T, Ishikawa S, Watanabe F, Matsushita T, Matsuda H (1987) Partially unroofed coronary sinus associated with tricuspid atresia. An important associated lesion in the Fontan operation. Eur J Cardiothorac Surg 1:180–182PubMedGoogle Scholar
  44. McMahon CJ, Feltes TF, Fraley JK et al (2002) Natural history of growth of secundum atrial septal defects and implications for transcatheter closure. Heart 87:256–259PubMedPubMedCentralGoogle Scholar
  45. Minette MS, Sahn DJ (2006) Ventricular septal defects. Circulation 114:2190–2197PubMedGoogle Scholar
  46. Mongeon FP, Burkhart HM, Ammash NM et al (2010) Indications and outcomes of surgical closure of ventricular septal defect in adults. JACC Cardiovasc Interv 3:290–297PubMedGoogle Scholar
  47. Neumayer U, Stone S, Somerville J (1998) Small ventricular septal defects in adults. Eur Heart J 19:1573–1582PubMedGoogle Scholar
  48. Newfeld EA, Muster AJ, Paul MH, Idriss FS, Riker WL (1976) Discrete subvalvular aortic stenosis in childhood. Study of 51 patients. Am J Cardiol 38:53–61PubMedGoogle Scholar
  49. Ootaki Y, Yamaguchi M, Yoshimura N, Oka S, Yoshida M, Hasegawa T (2003a) Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg 126:1655–1656PubMedGoogle Scholar
  50. Ootaki Y, Yamaguchi M, Yoshimura N, Oka S, Yoshida M, Hasegawa T (2003b) Surgical management of trabecular ventricular septal defects: the sandwich technique. J Thorac Cardiovasc Surg 125:508–512PubMedGoogle Scholar
  51. Parsons JM, Baker EJ, Anderson RH et al (1990) Morphological evaluation of atrioventricular septal defects by magnetic resonance imaging. Br Heart J 64:138–145PubMedPubMedCentralGoogle Scholar
  52. Pennell DJ, Sechtem UP, Higgins CB et al (2004) Clinical indications for cardiovascular magnetic resonance (CMR): consensus panel report. Eur Heart J 25:1940–1965PubMedGoogle Scholar
  53. Penny DJ, Vick GW III (2011) Ventricular septal defect. Lancet 377:1103–1112PubMedGoogle Scholar
  54. Pongiglione G, Freedom RM, Cook D, Rowe RD (1982) Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study. Am J Cardiol 50:776–780PubMedGoogle Scholar
  55. Prasad SK, Soukias N, Hornung T et al (2004) Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation 109:207–214PubMedGoogle Scholar
  56. Rajiah P, Kanne JP (2010) Computed tomography of septal defects. J Cardiovasc Comput Tomogr 4:231–245PubMedGoogle Scholar
  57. Roguin N, ZD D, Barak M, Nasser N, Hershkowitz S, Milgram E (1995) High prevalence of muscular ventricular septal defect in neonates. J Am Coll Cardiol 26:1545–1548PubMedGoogle Scholar
  58. Sachweh JS, Daebritz SH, Hermanns B et al (2006) Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect. Ann Thorac Surg 81:207–213PubMedGoogle Scholar
  59. Sadler TW (2015) Langman’s medical embryology. 13th ednGoogle Scholar
  60. Saremi F (2014) Cardiac CT and MR for adult congenital heart disease. Springer, New YorkGoogle Scholar
  61. Saxena A, Divekar A, Soni NR (2005) Natural history of secundum atrial septal defect revisited in the era of transcatheter closure. Indian Heart J 57:35–38PubMedGoogle Scholar
  62. Seddio F, Reddy VM, McElhinney DB, Tworetzky W, Silverman NH, Hanley FL (1999) Multiple ventricular septal defects: how and when should they be repaired? J Thorac Cardiovasc Surg 117:134–139. discussion 39-40PubMedGoogle Scholar
  63. Serraf A, Lacour-Gayet F, Bruniaux J et al (1992) Surgical management of isolated multiple ventricular septal defects. Logical approach in 130 cases. J Thorac Cardiovasc Surg 103:437–42; discussion 43PubMedGoogle Scholar
  64. Slovis TL (2008). Caffey’s pediatric diagnostic imaging. 11th edition.Google Scholar
  65. Soto B, Becker AE, Moulaert AJ, Lie JT, Anderson RH (1980) Classification of ventricular septal defects. Br Heart J 43:332–343PubMedPubMedCentralGoogle Scholar
  66. Steele PM, Fuster V, Cohen M, Ritter DG, McGoon DC (1987) Isolated atrial septal defect with pulmonary vascular obstructive disease--long-term follow-up and prediction of outcome after surgical correction. Circulation 76:1037–1042PubMedGoogle Scholar
  67. Stellin G, Padalino M, Milanesi O et al (2000) Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy. Ann Thorac Surg 69:597–601PubMedGoogle Scholar
  68. Studer M, Blackstone EH, Kirklin JW et al (1982) Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg 84:523–542PubMedGoogle Scholar
  69. Sugimoto M, Ota K, Kajihama A, Nakau K, Manabe H, Kajino H (2011) Volume overload and pressure overload due to left-to-right shunt-induced myocardial injury. Evaluation using a highly sensitive cardiac troponin-I assay in children with congenital heart disease. Circ J 75:2213–2219PubMedGoogle Scholar
  70. Tandon R, Edwards JE (1973) Aneurysmlike formations in relation to membranous ventricular septum. Circulation 47:1089–1097PubMedGoogle Scholar
  71. Tatsuno K, Konno S, Ando M, Sakakibara S (1973) Pathogenetic mechanisms of prolapsing aortic valve and aortic regurgitation associated with ventricular septal defect. Anatomical, angiographic, and surgical considerations. Circulation 48:1028–1037PubMedGoogle Scholar
  72. Van Praagh S, Carrera ME, Sanders SP, Mayer JE, Van Praagh R (1994) Sinus venosus defects: unroofing of the right pulmonary veins—anatomic and echocardiographic findings and surgical treatment. Am Heart J 128:365–379PubMedGoogle Scholar
  73. van Son JA, Schaff HV, Danielson GK, Hagler DJ, Puga FJ (1993) Surgical treatment of discrete and tunnel subaortic stenosis. Late survival and risk of reoperation. Circulation 88:II159–II169PubMedGoogle Scholar
  74. Vogel M, Berger F, Kramer A, Alexi-Meshkishvili V, Lange PE (1999) Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects. Heart 82:30–33PubMedPubMedCentralGoogle Scholar
  75. Wald RM, Powell AJ (2006) Simple congenital heart lesions. J Cardiovasc Magn Reson 8:619–631PubMedGoogle Scholar
  76. Wald RM, Silversides CK, Kingdom J et al (2015) Maternal cardiac output and fetal Doppler predict adverse neonatal outcomes in pregnant women with heart disease. J Am Heart Assoc 4:iiGoogle Scholar
  77. Wang ZJ, Reddy GP, Gotway MB, Yeh BM, Higgins CB (2003) Cardiovascular shunts: MR imaging evaluation. Radiographics 23(Spec issue):S181–S194PubMedGoogle Scholar
  78. Warnes CA, Williams RG, Bashore TM et al (2008) ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (writing committee to develop guidelines on the Management of Adults with Congenital Heart Disease). Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 52:e143–e263PubMedGoogle Scholar
  79. Webb G, Gatzoulis MA (2006) Atrial septal defects in the adult: recent progress and overview. Circulation 114:1645–1653PubMedGoogle Scholar
  80. Weber C, Weber M, Ekinci O et al (2008) Atrial septal defects type II: noninvasive evaluation of patients before implantation of an Amplatzer Septal Occluder and on follow-up by magnetic resonance imaging compared with TEE and invasive measurement. Eur Radiol 18:2406–2413PubMedGoogle Scholar
  81. Wollenek G, Wyse R, Sullivan I, Elliott M, de Leval M, Stark J (1996) Closure of muscular ventricular septal defects through a left ventriculotomy. Eur J Cardiothorac Surg 10(8):595PubMedGoogle Scholar
  82. Yavuz S, Eris C, Goncu T, Sezen M, Ata Y, Turk T (2010) An incidental aneurysm of the interventricular membranous septum. Arch Iran Med 13(4):363PubMedGoogle Scholar
  83. YC F, Bass J, Amin Z et al (2006) Transcatheter closure of perimembranous ventricular septal defects using the new Amplatzer membranous VSD occluder: results of the U.S. phase I trial. J Am Coll Cardiol 47:319–325Google Scholar
  84. Yilmaz AT, Ozal E, Arslan M, Tatar H, Ozturk OY (1997) Aneurysm of the membranous septum in adult patients with perimembranous ventricular septal defect. Eur J Cardiothorac Surg 11:307–311PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Stephan Waelti
    • 1
  • Julie Déry
    • 1
  • Chantale Lapierre
    • 1
    Email author
  1. 1.Medical Imaging DepartmentCHU Sainte-JustineMontrealCanada

Personalised recommendations