Cardiovascular System - Atrial Septal Defects

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LB20 Congenital Anomaly of Atrioventricular Valves or Septum

LB20 Congenital Anomaly of Atrioventricular Valves or Septum
A congenital cardiovascular malformation in which there is an abnormality of the atrioventricular valve or atrioventricular septum.
ICD-11 Structural developmental anomalies of the circulatory system (draft) 
ICD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

20 Developmental Anomalies - Structural Developmental Anomalies

Beta coding and tree structure for "structural developmental anomalies" within this section are shown in the table below.

Structural developmental anomalies of the circulatory system  
  • Structural developmental anomaly of heart and great vessels
    • LB00 Congenital heart or great vessel related acquired abnormality
    • LB01 Congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.1 Transposition of the great arteries
      • LB01.2 Double outlet right ventricle
      • LB01.3 Double outlet left ventricle
      • LB01.4 Common arterial trunk
      • LB01.Y Other specified congenital anomaly of atrioventricular or ventriculo-arterial connections
      • LB01.Z Congenital anomaly of atrioventricular or ventriculo-arterial connections, unspecified
    • LB02 Congenital anomaly of the mediastinal veins Congenital anomaly of atria or atrial septum
    • LB20 Congenital anomaly of atrioventricular valves or septum
    • LB21 Congenital anomaly of ventricles and ventricular septum
      • LB21.1 Congenital right ventricular outflow tract obstruction  
      • LB21.2 Double-chambered right ventricle  
      • LB21.3 Tetralogy of Fallot
      • LB21.4 Congenital left ventricular outflow tract obstruction  
      • LB21.5 Congenital ventricular septal defects 
      • LB21.Y Other specified congenital anomaly of ventricles and ventricular septum
      • LB21.Z Congenital anomaly of ventricles and ventricular septum, unspecified  
    • LB22 Functionally univentricular heart
    • LB23 Congenital anomaly of ventriculo-arterial valves and adjacent regions
    • LB24 Congenital anomaly of great arteries including arterial duct
      • LB.1 Congenital aorto-pulmonary window
      • LB.2 Congenital anomaly of pulmonary arterial tree
      • LB.3 Congenital anomaly of aorta and its branches
      • LB.4 Tracheo-oesophageal compressive syndrome
      • LB.5 Patent arterial duct
      • LB.Y Other specified congenital anomaly of great arteries including arterial duct
      • LB.Z Congenital anomaly of great arteries including arterial duct, unspecified
    • LB25 Anomalous position-orientation of heart
    • LB26 Total mirror imagery
    • LB27 Left isomerism
    • LB28 Congenital anomaly of coronary arteries
    • LB29 Structural developmental anomalies of the pericardium
    • LB2Y Other specified structural developmental anomaly of heart and great vessels
    • LB2Z Structural developmental anomaly of heart and great vessels, unspecified
  • LB30 Structural developmental anomalies of the peripheral vascular system
    • LB30.1 Capillary malformations
    • LB30.2 Lymphatic malformations
      • LB30.21 Macrocystic lymphatic malformation
      • LB30.22 Microcystic lymphatic malformation
      • LB30.23 Cystic hygroma in fetus
      • BD23.1 Primary lymphoedema
          • EK91 Yellow nail syndrome
          • LC5F.26 Noonan syndrome
      • LB30.2Y Other specified lymphatic malformations
      • LB30.2Z Lymphatic malformations, unspecified
    • LB30.3 Peripheral venous malformations
    • LB30.4 Peripheral arteriovenous malformations
    • LB30.5 Peripheral arterial malformations
    • LB30.6 Pulmonary arteriovenous fistula
    • LB30.Y Other specified structural developmental anomalies of the peripheral vascular system
    • LB30.Z Structural developmental anomalies of the peripheral vascular system, unspecified
  • LB3Y Other specified structural developmental anomalies of the circulatory system
  • LB3Z Structural developmental anomalies of the circulatory system, unspecified
CD-11 Beta Draft - NOT FINAL, updated on a daily basis, It is not approved by WHO, NOT TO BE USED for CODING except for agreed FIELD TRIALS.

See also International Classification of Diseases | Abnormalities


Atrial Septal Defect cartoon
Atrial Septal Defect

The atrial septal defects (ASD) are a group of common (1% of cardiac) congenital anomolies defects occuring in a number of different forms and more often in females. Patients have been reported living to old age with forms of this cardiac abnormality[1], that is also common in Trisomy 21.

  • patent foramen ovale - allows a continuation of the atrial shunting of blood, in 25% of people a probe patent foramen ovale (allowing a probe to bepassed from one atria to the other) exists.
  • ostium secundum defect
  • endocardial cushion defect involving ostium primum
  • sinus venosus defect - contributes about 10% of all ASDs and occurs mainly in a common and less common form.
    • Common ("usual type") - in upper atrial septum which is contiguous with the superior vena cava.
    • Less common - at junction of the right atrium and inferior vena cava.
  • common atrium

Heart Abnormal: Tutorial Abnormalities | atrial septal defects | double outlet right ventricle | hypoplastic left heart | patent ductus arteriosus‎ | transposition of the great vessels | Tetralogy of Fallot | ventricular septal defects | coarctation of the aorta | Category ASD | Category PDA | Category ToF | Category VSD | ICD10 - Cardiovascular | ICD11

Cardiovascular Links: cardiovascular | Heart Tutorial | Lecture - Early Vascular | Lecture - Heart | Movies | 2016 Cardiac Review | heart | coronary circulation | heart valve | heart rate | Circulation | blood | blood vessel | blood vessel histology | heart histology | Lymphatic | ductus venosus | spleen | Stage 22 | cardiovascular abnormalities | OMIM | 2012 ECHO Meeting | Category:Cardiovascular
Historic Embryology - Cardiovascular 
1902 Vena cava inferior | 1905 Brain Blood Vessels | 1909 Cervical Veins | 1909 Dorsal aorta and umbilical veins | 1912 Heart | 1912 Human Heart | 1914 Earliest Blood-Vessels | 1915 Congenital Cardiac Disease | 1915 Dura Venous Sinuses | 1916 Blood cell origin | 1916 Pars Membranacea Septi | 1919 Lower Limb Arteries | 1921 Human Brain Vascular | 1921 Spleen | 1922 Aortic-Arch System | 1922 Pig Forelimb Arteries | 1922 Chicken Pulmonary | 1923 Head Subcutaneous Plexus | 1923 Ductus Venosus | 1925 Venous Development | 1927 Stage 11 Heart | 1928 Heart Blood Flow | 1935 Aorta | 1935 Venous valves | 1938 Pars Membranacea Septi | 1938 Foramen Ovale | 1939 Atrio-Ventricular Valves | 1940 Vena cava inferior | 1940 Early Hematopoiesis | 1941 Blood Formation | 1942 Truncus and Conus Partitioning | Ziegler Heart Models | 1951 Heart Movie | 1954 Week 9 Heart | 1957 Cranial venous system | 1959 Brain Arterial Anastomoses | Historic Embryology Papers | 2012 ECHO Meeting | 2016 Cardiac Review | Historic Disclaimer

Some Recent Findings

Australia Congenital heart disease 2016–17
Australia Congenital heart disease 2016–17
  • Atrial septal defect devices used in the cardiac catheterization laboratory[3] "An atrial septal defect (ASD) is a hole in the atrium of the heart. There are 3 types of ASDs; sinus venosus (high in the atrial septum), secundum ASD (middle of septum), and ostium primum (low in the septum). The most common ASD is a secundum ASD. Secundum ASDs are caused by a failure of the atrial septum to close completely during the development of the heart. The most common reported symptoms are fatigue and shortness of breath. Most patients are found to have an ASD after evaluation for a murmur. All ASDs used to be repaired by open heart surgery. However, with advances in the cardiac catheterization lab and development of new devices, some secundum ASDs are able to be closed in the catheterization lab by an interventional cardiologist. There are various types of devices that may be used for closure of an ASD in the cardiac catheterization laboratory. This paper will address 2 of the devices most commonly used. Anticoagulation therapy will need to be followed for approximately 6 months and echocardiograms will need to be obtained at follow-up visits. Nurses have an important role in preparing and teaching the patient and family about the ASD closure procedure and follow-up care."
More recent papers  
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Search term: Atrial Septal Defect | Congenital Anomaly of Atrioventricular Valves


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 ‎‎Atrial Septation
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 ‎‎Cardiac Septation
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 ‎‎Cardiac Septation
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Oval Fossa Defect

Anderson2016-fig19.jpg Anderson2016-fig20.jpg
The cross-section of a human heart shows defects (patent foramen ovale) in the floor of the oval fossa (fossa ovalis, annulus ovalis) shown by double headed white arrow due to deficiency and perforation of the flap valve derived from the primary atrial septum.
The white arrow with black borders shows the intact infolded cranial rim of the fossa.
Human heart has been sectioned across the short axis of the atrial chambers, and photographed from above, to show a persistently patent oval foramen (double headed red arrow).
The double headed white arrow shows the normal rims of the oval fossa, which are overlapped by the flap valve, but in absence of fusion with the left atrial aspect of the rims.
Heart specimen images produced by Diane Spicer.

Sinus Venosus Atrial Septal Defect

Sinus venosus atrial septal defects (SV-ASDs) are inter-atrial abnormality caused by a deficiency of the common wall between the superior or inferior vena cava and the right-sided pulmonary veins. The images below are MRI from a 25-year-old asymptomatic male[4] SSFP - Breath-held fat suppressed three-dimensional steady-state free precession.

Sinus venosus atrial septal defect 02.jpg Sinus venosus atrial septal defect 03.jpg
MRI SSFP sagittal view SSFP axial view
Sinus venosus atrial septal defect 04.jpg Sinus venosus atrial septal defect 05.jpg
Turbo spin-echo axial plane SSFP dilated right ventricle
Links: Magnetic Resonance Imaging




"Patent foramen ovale and atrial (or auricular) septal defect (A.S.D.), though both characterised by an aperture in the atrial septum, are embryologically and pathologically different conditions."


The surgical repair requires a cardiopulmonary bypass and is recommended in most cases of ostium secundum ASD, even though there is a significant risk involved. Ostium primum defects tend to present earlier and are often associated with endocardial cushion defects and defective mitral or tricuspid valves. In such cases, valve replacement may be necessary and the extended operation has a considerable chance of mortality.

  • Increasingly closure by a transcatheter device closure has been applied.
  • Repair of atrial septal defects on the perfused beating heart (atrial septal defect size 2 cm - 4.5 cm)[6]

Cardiovascular Abnormalities

Data shown as a percentage of all major abnormalities based upon published statistics using the same groupings as Congenital Malformations Australia 1981-1992 P. Lancaster and E. Pedisich ISSN 1321-8352.

Heart defects and preterm birth are the most common causes of neonatal and infant death. The long-term development of the heart combined with extensive remodelling and post-natal changes in circulation lead to an abundance of abnormalities associated with this system.

A UK study literature showed that preterm infants have more than twice as many cardiovascular malformations (5.1 / 1000 term infants and 12.5 / 1000 preterm infants) as do infants born at term and that 16% of all infants with cardiovascular malformations are preterm. (0.4% of live births occur at greater than 28 weeks of gestation, 0.9% at 28 to 31 weeks, and 6% at 32 to 36 weeks. Overall, 7.3% of live-born infants are preterm)[7]

"Baltimore-Washington Infant Study data on live-born cases and controls (1981-1989) was reanalyzed for potential environmental and genetic risk-factor associations in complete atrioventricular septal defects AVSD (n = 213), with separate comparisons to the atrial (n = 75) and the ventricular (n = 32) forms of partial AVSD. ...Maternal diabetes constituted a potentially preventable risk factor for the most severe, complete form of AVSD."[8]

In addition, there are in several congenital abnormalities that exist in adults (bicuspid aortic valve, mitral valve prolapse, and partial anomalous pulmonary venous connection) which may not be clinically recognized.


  1. Matsumoto T, Tamiya E, Kanoh T, Takabe T, Kuremoto KI, Kamiyama T, Yamamoto S & Daida H. (2019). Atrial Septal Defect of the Ostium Secundum Type in A 101-Year-Old Patient. Int Heart J , , . PMID: 30799379 DOI.
  2. Australian Institute of Health and Welfare 2019. Congenital heart disease in Australia. Cat. no. CDK 14. Canberra: AIHW.
  3. Gervasi L & Basu S. (2009). Atrial septal defect devices used in the cardiac catheterization laboratory. Prog Cardiovasc Nurs , 24, 86-9. PMID: 19737165 DOI.
  4. Ganigara M, Tanous D, Celermajer D & Puranik R. (2014). The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect. Ann Pediatr Cardiol , 7, 160-2. PMID: 24987269 DOI.
  5. Bedford DE, Papp C & Parkinson J. (1941). ATRIAL SEPTAL DEFECT. Br Heart J , 3, 37-68. PMID: 18609869
  6. Pendse N, Gupta S, Geelani MA, Minhas HS, Agarwal S, Tomar A & Banerjee A. (2009). Repair of atrial septal defects on the perfused beating heart. Tex Heart Inst J , 36, 425-7. PMID: 19876418
  7. Tanner K, Sabrine N & Wren C. (2005). Cardiovascular malformations among preterm infants. Pediatrics , 116, e833-8. PMID: 16322141 DOI.
  8. Loffredo CA, Hirata J, Wilson PD, Ferencz C & Lurie IW. (2001). Atrioventricular septal defects: possible etiologic differences between complete and partial defects. Teratology , 63, 87-93. PMID: 11241431 <87::AID-TERA1014>3.0.CO;2-5 DOI.


Ng WP & Yip WL. (2012). Successful maternal-foetal outcome using nitric oxide and sildenafil in pulmonary hypertension with atrial septal defect and HIV infection. Singapore Med J , 53, e3-5. PMID: 22252195

Ma ZS, Dong MF, Yin QY, Feng ZY & Wang LX. (2012). Totally thoracoscopic closure for atrial septal defect on perfused beating hearts. Eur J Cardiothorac Surg , 41, 1316-9. PMID: 22219470 DOI.

Scheuerle A. (2011). Clinical differentiation of patent foramen ovale and secundum atrial septal defect: a survey of pediatric cardiologists in Dallas, Texas, USA. J Registry Manag , 38, 4-8. PMID: 22097699

Ağaç MT, Akyüz AR, Acar Z, Akdemir R, Korkmaz L, Kırış A, Erkuş E, Erkan H & Celik S. (2012). Evaluation of right ventricular function in early period following transcatheter closure of atrial septal defect. Echocardiography , 29, 358-62. PMID: 22066780 DOI.

Rodriguez FH, Moodie DS, Parekh DR, Franklin WJ, Morales DL, Zafar F, Graves DE, Friedman RA & Rossano JW. (2011). Outcomes of hospitalization in adults in the United States with atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Am. J. Cardiol. , 108, 290-3. PMID: 21545985 DOI.

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Cite this page: Hill, M.A. (2024, June 20) Embryology Cardiovascular System - Atrial Septal Defects. Retrieved from

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