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
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 82:1123–1138
al Zaghal AM, Li J, Anderson RH, Lincoln C, Shore D, Rigby ML (1997) Anatomical criteria for the diagnosis of sinus venosus defects. Heart 78:298–304
Allen HD, Driscoll DJ, Shaddy RE, Feltes TF (2008) Moss and Adams heart disease in infants, children, and adolescents, 7th edn. Lippincott Williams & Wilkins, Philadelphia
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 132:1072–1080
Anderson RH, Ho SY, Falcao S, Daliento L, Rigby ML (1998) The diagnostic features of atrioventricular septal defect with common atrioventricular junction. Cardiol Young 8:33–49
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 38:2035–2042
Bacha EA, Cao QL, Galantowicz ME et al (2005) Multicenter experience with perventricular device closure of muscular ventricular septal defects. Pediatr Cardiol 26:169–175
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 31:2915–2957
Blom NA, Ottenkamp J, Jongeneel TH, DeRuiter MC, Gittenberger-de Groot AC (2005) Morphogenetic differences of secundum atrial septal defects. Pediatr Cardiol 26:338–343
Botto LD, Correa A, Erickson JD (2001) Racial and temporal variations in the prevalence of heart defects. Pediatrics 107:E32
Bremerich J, Reddy GP, Higgins CB (1999) MRI of supracristal ventricular septal defects. J Comput Assist Tomogr 23:13–15
Bricker AO, Avutu B, Mohammed TL et al (2010) Valsalva sinus aneurysms: findings at CT and MR imaging. Radiographics 30:99–110
Brickner ME, Hillis LD, Lange RA (2000) Congenital heart disease in adults. Second of two parts. N Engl J Med 342:334–342
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 128:684–692
Calkoen EE, Hazekamp MG, Blom NA et al (2016) Atrioventricular septal defect: from embryonic development to long-term follow-up. Int J Cardiol 202:784–795
Campbell M (1970) Natural history of atrial septal defect. Br Heart J 32(6):820
Corno AF, Kandakure PR, Dhannapuneni RR, Gladman G, Venugopal P, Alphonso N (2013) Multiple ventricular septal defects: a new strategy. Front Pediatr 1:16
Davia JE, Cheitlin MD, Bedynek JL (1973) Sinus venosus atrial septal defect: analysis of fifty cases. Am Heart J 85:177–185
Didier D, Higgins CB (1986) Identification and localization of ventricular septal defect by gated magnetic resonance imaging. Am J Cardiol 57:1363–1368
Didier D, Higgins CB, Fisher MR, Osaki L, Silverman NH, Cheitlin MD (1986) Congenital heart disease: gated MR imaging in 72 patients. Radiology 158:227–235
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 21:276–282
Gersony WM (2001) Natural history and decision-making in patients with ventricular septal defect. Prog Pediatr Cardiol 14:125–132
Geva T, Martins JD, Wald RM (2014) Atrial septal defects. Lancet 383:1921–1932
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 59:17–20
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 118:1560–1565
Helgason H, Jonsdottir G (1999) Spontaneous closure of atrial septal defects. Pediatr Cardiol 20:195–199
Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900
Hoffman JI, Kaplan S, Liberthson RR (2004) Prevalence of congenital heart disease. Am Heart J 147:425–439
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 43:1257–1263
Hugh D, Allen RES, Daniel J (2016) Penny, Timothy F. Feltes, Frank Cetta. Moss and Adams’ Heart Disease in infants, children and adolescents. 9th edn
Humenberger M, Rosenhek R, Gabriel H et al (2011) Benefit of atrial septal defect closure in adults: impact of age. Eur Heart J 32:553–560
Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C (2000) Congenital heart surgery nomenclature and database project: ventricular septal defect. Ann Thorac Surg 69:S25–S35
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 192:259–266
Kirklin JK, Castaneda AR, Keane JF, Fellows KE, Norwood WI (1980) Surgical management of multiple ventricular septal defects. J Thorac Cardiovasc Surg 80:485–493
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 115:848–856
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 193:1522–1529
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 333:469–473
Kucher N, Seiler C, Allemann Y, Eberli FR (2001) Double-chambered right ventricle. Circulation 103:E105–E106
Langer C, Horstkotte D, Piper C (2007) Aneurysm of the membranous septum causes pre-syncopes and transient bilateral blindness. Eur Heart J 28:784
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 199:1136–1141
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 28:564–568
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 87:1266–1270
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 1:180–182
McMahon CJ, Feltes TF, Fraley JK et al (2002) Natural history of growth of secundum atrial septal defects and implications for transcatheter closure. Heart 87:256–259
Minette MS, Sahn DJ (2006) Ventricular septal defects. Circulation 114:2190–2197
Mongeon FP, Burkhart HM, Ammash NM et al (2010) Indications and outcomes of surgical closure of ventricular septal defect in adults. JACC Cardiovasc Interv 3:290–297
Neumayer U, Stone S, Somerville J (1998) Small ventricular septal defects in adults. Eur Heart J 19:1573–1582
Newfeld EA, Muster AJ, Paul MH, Idriss FS, Riker WL (1976) Discrete subvalvular aortic stenosis in childhood. Study of 51 patients. Am J Cardiol 38:53–61
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 126:1655–1656
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 125:508–512
Parsons JM, Baker EJ, Anderson RH et al (1990) Morphological evaluation of atrioventricular septal defects by magnetic resonance imaging. Br Heart J 64:138–145
Pennell DJ, Sechtem UP, Higgins CB et al (2004) Clinical indications for cardiovascular magnetic resonance (CMR): consensus panel report. Eur Heart J 25:1940–1965
Penny DJ, Vick GW III (2011) Ventricular septal defect. Lancet 377:1103–1112
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 50:776–780
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 109:207–214
Rajiah P, Kanne JP (2010) Computed tomography of septal defects. J Cardiovasc Comput Tomogr 4:231–245
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 26:1545–1548
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 81:207–213
Sadler TW (2015) Langman’s medical embryology. 13th edn
Saremi F (2014) Cardiac CT and MR for adult congenital heart disease. Springer, New York
Saxena A, Divekar A, Soni NR (2005) Natural history of secundum atrial septal defect revisited in the era of transcatheter closure. Indian Heart J 57:35–38
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 39-40
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 43
Slovis TL (2008). Caffey’s pediatric diagnostic imaging. 11th edition.
Soto B, Becker AE, Moulaert AJ, Lie JT, Anderson RH (1980) Classification of ventricular septal defects. Br Heart J 43:332–343
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 76:1037–1042
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 69:597–601
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 84:523–542
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 75:2213–2219
Tandon R, Edwards JE (1973) Aneurysmlike formations in relation to membranous ventricular septum. Circulation 47:1089–1097
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 48:1028–1037
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 128:365–379
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–II169
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 82:30–33
Wald RM, Powell AJ (2006) Simple congenital heart lesions. J Cardiovasc Magn Reson 8:619–631
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:ii
Wang ZJ, Reddy GP, Gotway MB, Yeh BM, Higgins CB (2003) Cardiovascular shunts: MR imaging evaluation. Radiographics 23(Spec issue):S181–S194
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–e263
Webb G, Gatzoulis MA (2006) Atrial septal defects in the adult: recent progress and overview. Circulation 114:1645–1653
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 18:2406–2413
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):595
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):363
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 47:319–325
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 11:307–311
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing AG
About this chapter
Cite this chapter
Waelti, S., Déry, J., Lapierre, C. (2017). Septal Defects. In: Ley, S., Ley-Zaporozhan, J. (eds) Congenital Heart Diseases in Adults. Medical Radiology(). Springer, Cham. https://doi.org/10.1007/174_2017_83
Download citation
DOI: https://doi.org/10.1007/174_2017_83
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-61886-9
Online ISBN: 978-3-319-61888-3
eBook Packages: MedicineMedicine (R0)