Difference between revisions of "Cardiovascular System - Ventricular Septal Defects"

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[[Category:Cardiovascular]] [[Category:Heart]] [[Category:Abnormal Development]]
[[Category:Cardiovascular]] [[Category:Heart]] [[Category:Abnormal Development]]
[[Category:Ventricular Septal Defect]]

Revision as of 10:19, 6 June 2017

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LB21.5 Congenital ventricular septal defects

ICD-11 Beta Draft code: LB21.5. Current ICD-10 code: Q21.0 Ventricular septal defect

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


Ventricular Septal Defect

The Ventricular Septal Defect (VSD) is the most common form of congenital cardiovascular anomaly, occurring in nearly 50% of all infants with a congenital heart defect. Usually occurs in the membranous (perimembranous) rather than muscular interventricular septum, and is more frequent in males that females. This defect can also contribute to outflow tract (OFT) malformations.

Perimembranous defects are located close to the aortic and tricuspid valves and adjacent to atrioventricular conduction bundle.

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

  • Complete Repair of Coarctation of the Aorta and a Ventricular Septal Defect in a 1,480 g Low Birth Weight Neonate[1] "Although outcomes of neonatal cardiac surgery have dramatically improved in the last two decades, low body weight still constitutes an important risk factor for morbidity and mortality. In particular, cardiac surgery in neonates with very low birth weight (≤1.5 kg) is carried out with greater risk because most organ systems are immature. We report here on a successful case of early one-stage total repair of coarctation of the aorta and a ventricular septal defect in a 1,480 gram neonate."
More recent papers  
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<pubmed limit=5>Ventricular Septal Defect</pubmed>


Anderson2016-fig10.jpg Anderson2016-fig11a.jpg Anderson2016-fig11b.jpg
The image shows a congenitally malformed heart in which both the right and left atrioventricular valves (RAVV, LAVV) are connected with the dominant left ventricle. The right ventricle is incomplete, and is supplied through a ventricular septal defect. Note that, in this heart, the aorta arises from the incomplete right ventricle, and the pulmonary trunk from the dominant left ventricle. This is the arrangement usually described as “transposition”, but better accounted for in terms of discordant ventriculo-arterial connections. The image shows the lesion known as classical tricuspid atresia. There has been failure of expansion of the atrioventricular junctions, so that the floor of the right is separated from the roof of the right ventricle by the right atrioventricular groove (dashed black lines). Only the dominant left ventricle has an inlet, with the blood entering the incomplete right ventricle through the ventricular septal defect (star). The image shows a heart dissected to reveal the structure of the incomplete right ventricle. In this heart, as in most example of tricuspid atresia, it gives rise to the pulmonary trunk. The ventricular septal defect (VSD) is restrictive in this heart.

Images from Anderson (2016)[2]


Le Gros Clark (1847)[3] Heart autopsy drawing.

The first western clinical description of ventricular septal defects was made by Henri Roger in 1879[4], which later became known as maladie de Roger.

His description was based upon 6 acyanotic patients and autopsy finding of a child with ventricular septal defect.


Ventricular septal defect 01.jpg

There is a defect in the ventricular septum adjacent to the atrioventricular valves. Blood flow is seen across the defect on Colour Doppler imaging.

Pulmonary Changes

Heath-Edwards classification

Artery normal histology

(Heath-Edward grade) A pathological grading system for pulmonary artery structural changes that occur with congenital cardiac septal defects. The classification is named after the two original paper authors Donald HEATH and Jessee EDWARDS[5] and grades from I to VI with increasing severity of the arterial changes.

  1. Grade I - hypertrophy of the media of small muscular arteries and arterioles.
  2. Grade II - intimal cellular proliferation in addition to medial hypertrophy.
  3. Grade III - advanced medial thickening with hypertrophy and hyperplasia including progressive intimal proliferation and concentric fibrosis. Results in an obliteration of the arterioles and small arteries.
  4. Grade IV - "plexiform lesions" of the muscular pulmonary arteries and arterioles with a plexiform network of capillary-like channels within a dilated segment.
  5. Grade V - complex plexiform, angiomatous and cavernous lesions and hyalinization of intimal fibrosis.
  6. Grade VI - necrotizing arteritis.

International Classification of Diseases

The International Classification of Diseases (ICD) World Health Organization's classification used worldwide as the standard diagnostic tool for epidemiology, health management and clinical purposes. This includes the analysis of the general health situation of population groups. It is used to monitor the incidence and prevalence of diseases and other health problems. Within this classification "congenital malformations, deformations and chromosomal abnormalities" are (Q00-Q99) but excludes "inborn errors of metabolism" (E70-E90).

Congenital malformations of the circulatory system (Q20-Q28)

ICD-10 Code: Q21.0 Ventricular septal defect

Q21 Congenital malformations of cardiac septa

Excl.: acquired cardiac septal defect (I51.0)

  • Q21.0 Ventricular septal defect
  • Q21.1 Atrial septal defect Coronary sinus defect Patent or persistent: foramen ovale ostium secundum defect (type II) Sinus venosus defect
  • Q21.2 Atrioventricular septal defect Common atrioventricular canal Endocardial cushion defect Ostium primum atrial septal defect (type I)
  • Q21.3 Tetralogy of Fallot Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right ventricle.
  • Q21.4 Aortopulmonary septal defect Aortic septal defect Aortopulmonary window
  • Q21.8 Other congenital malformations of cardiac septa Eisenmenger's defect Pentalogy of Fallot Excl.: Eisenmenger's complex (I27.8) syndrome (I27.8)
  • Q21.9 Congenital malformation of cardiac septum, unspecified Septal (heart) defect NOS


Heart1 realign icon.jpg
 ‎‎Heart Realign
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Heart1 ventricle icon.jpg
 ‎‎Outflow Septation
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Heart septation 001 icon.jpg
 ‎‎Cardiac Septation
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Outflow tract 001 icon.jpg
 ‎‎Outflow Tract
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 ‎‎Ventricular Septum 1
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 ‎‎Ventricular Septum 2
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 ‎‎Ventricular Septum 3
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Clinical Classification

Number of parameters are considered when classifying:

  • Size of the defect.
  • Location of the defect.
  • Number of defects.
  • Presence or absence of a ventricular septal aneurysm.

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)[6]

"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." [7]

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. <pubmed>22263148</pubmed>
  2. Anderson RH. Teratogenecity in the setting of cardiac development and maldevelopment. (2016)
  3. <pubmed>20895864</pubmed>| PMC2104017 | PDF
  4. <pubmed>382810</pubmed>
  5. <pubmed>13573570</pubmed>Circulation
  6. <pubmed>16322141</pubmed>
  7. <pubmed>11241431</pubmed>




<pubmed>22263148</pubmed> <pubmed>22229525</pubmed> <pubmed>22163127</pubmed> <pubmed>3808993</pubmed> <pubmed>2938464</pubmed> <pubmed>6179217</pubmed>

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Cardiovascular Terms  
Cardiovascular System Development See also Heart terms, Immune terms and Blood terms.
  • angioblast - the stem cells in blood islands generating endothelial cells which will form the walls of both arteries and veins. (More? Blood Vessel)
  • angiogenesis - the formation of new blood vessels from pre-existing vessels following from vasculogenesis in the embryo. (More? Blood Vessel)
  • anlage (German, anlage = primordium) structure or cells which will form a future more developed or differentiated adult structure.
  • blood islands - earliest sites of blood vessel and blood cell formation, seen mainly on yolk sac chorion.
  • cardinal veins - paired main systemic veins of early embryo, anterior, common, posterior.
  • cardiogenic region - region above prechordal plate in mesoderm where heart tube initially forms.
  • ectoderm - the layer (of the 3 germ cell layers) which form the nervous system from the neural tube and neural crest and also generates the epithelia covering the embryo.
  • endoderm - the layer (of the 3 germ cell layers) which form the epithelial lining of the gastrointestinal tract (GIT) and accessory organs of GIT in the embryo.
  • endocardium - lines the heart. Epithelial tissue lining the inner surface of heart chambers and valves.
  • endothelial cells - single layer of cells closest to lumen that line blood vessels.
  • extraembryonic mesoderm - mesoderm lying outside the trilaminar embryonic disc covering the yolk sac, lining the chorionic sac and forming the connecting stalk. Contributes to placental villi development.
  • haemocytoblasts - stem cells for embryonic blood cell formation.
  • anastomose - to connect or join by a connection (anastomosis) between tubular structures.
  • chorionic villi - the finger-like extensions which are the functional region of the placental barrier and maternal/fetal exchange. Develop from week 2 onward as: primary, secondary, tertiary villi.
  • estrogens - support the maternal endometrium.
  • growth factor - usually a protein or peptide that will bind a cell membrane receptor and then activates an intracellular signaling pathway. The function of the pathway will be to alter the cell directly or indirectly by changing gene expression. (eg VEGF, shh)
  • intra-aortic hematopoietic cluster - (IAHC) blood stem cells associated with the endothelial layer of aorta and large arteries.
  • maternal decidua - region of uterine endometrium where blastocyst implants. undergoes modification following implantation, decidual reaction.
  • maternal sinusoids - placental spaces around chorionic villi that are filled with maternal blood. Closest maternal/fetal exchange site.
  • Megakaryocytopoiesis - the process of bone marrow progenitor cells developMENT into mature megakaryocytes.
  • mesoderm - the middle layer of the 3 germ cell layers of the embryo. Mesoderm outside the embryo and covering the amnion, yolk and chorion sacs is extraembryonic mesoderm.
  • myocardium - muscular wall of the heart. Thickest layer formed by spirally arranged cardiac muscle cells.
  • pericardium - covers the heart. Formed by 3 layers consisting of a fibrous pericardium and a double layered serous pericardium (parietal layer and visceral epicardium layer).
  • pericytes - (Rouget cells) cells located at the abluminal surface of microvessels close to endothelial cells, mainly found associated with CNS vessels and involved in vessel formation, remodeling and stabilization.
  • pharyngeal arches (=branchial arches, Gk. gill) series of cranial folds that form most structures of the head and neck. Six arches form but only 4 form any structures. Each arch has a pouch, membrane and groove.
  • placenta - (Greek, plakuos = flat cake) refers to the discoid shape of the placenta, embryonic (villous chorion)/maternal organ (decidua basalis)
  • placental veins - paired initially then only left at end of embryonic period, carry oxygenated blood to the embryo (sinus venosus).
  • protein hormone - usually a protein distributed in the blood that binds to membrane receptors on target cells in different tissues. Do not easliy cross placental barrier.
  • sinus venosus - cavity into which all major embryonic paired veins supply (vitelline, placental, cardinal).
  • splanchnic mesoderm - portion of lateral plate mesoderm closest to the endoderm when coelom forms.
  • steroid hormone - lipid soluble hormone that easily crosses membranes to bind receptors in cytoplasm or nucleus of target cells. Hormone+Receptor then binds DNA activating or suppressing gene transcription. Easliy cross placental barrier.
  • syncitiotrophoblast extraembryonic cells of trophoblastic shell surrounding embryo, outside the cytotrophoblast layer, involved with implantation of the blastocyst by eroding extracellular matrix surrounding maternal endometrial cells at site of implantation, also contribute to villi. (dark staining, multinucleated).
  • truncus arteriosus - an embryological heart outflow structure, that forms in early cardiac development and will later divides into the pulmonary artery and aorta. Term is also used clinically to describe the malformation where only one artery arises from the heart and forms the aorta and pulmonary artery.
  • vascular endothelial growth factor - (VEGF) A secreted protein growth factor family, which stimulates the proliferation of vasular endotheial cells and therefore blood vessel growth. VEGF's have several roles in embryonic development. The VEGF family has 7 members (VEGF-A, VEGF-B, VEGF-C, VEGF-D, VEGF-E, VEGF-F, and PlGF) that have a common VEGF homology domain. PIGF is the placental growth factor. They act through 3 VEGF tyrosine kinase membrane receptors (VEGFR-1 to 3) with seven immunoglobulin-like domains in the extracellular domain, a single transmembrane region, and an intracellular tyrosine kinase sequence.
  • vasculogenesis - the formation of new blood vessels from mesoderm forming the endothelium. Compared to angiogenesis that is the process of blood vessel formation from pre-existing vessels.
  • vitelline blood vessels - blood vessels associated with the yolk sac.
  • waste products - products of cellular metabolism and cellular debris, e.g.- urea, uric acid, bilirubin.
Other Terms Lists  
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Cite this page: Hill, M.A. (2021, April 22) Embryology Cardiovascular System - Ventricular Septal Defects. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Cardiovascular_System_-_Ventricular_Septal_Defects

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© Dr Mark Hill 2021, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G