Cardiovascular System - Tetralogy of Fallot
Embryology - 15 Jun 2024 Expand to Translate |
---|
Google Translate - select your language from the list shown below (this will open a new external page) |
العربية | català | 中文 | 中國傳統的 | français | Deutsche | עִברִית | हिंदी | bahasa Indonesia | italiano | 日本語 | 한국어 | မြန်မာ | Pilipino | Polskie | português | ਪੰਜਾਬੀ ਦੇ | Română | русский | Español | Swahili | Svensk | ไทย | Türkçe | اردو | ייִדיש | Tiếng Việt These external translations are automated and may not be accurate. (More? About Translations) |
LB21.3 Tetralogy of Fallot
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 |
|
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.
|
Introduction
Tetralogy of Fallot named after Etienne-Louis Arthur Fallot (1888) who described it as "la maladie blue" and is a common developmental cardiac defect. The syndrome consists of a number of a number of cardiac defects possibly stemming from abnormal neural crest migration. Tetralogy refers to a set of four related symptoms or abnormalities frequently occurring together.
Occurs in about 3 in 10,000 live births and causes 7–10% of all congenital cardiac malformations.
- ventricular septal defect
- obstruction of the right ventricular outflow tract
- override of the ventricular septum by the aortic root
- right ventricular hypertrophy.
ICD-10 Q21.3 Tetralogy of Fallot Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right ventricle.
This cardiovascular abnormality has also been researched as a 2011 Student Project.
Some Recent Findings
|
More recent papers |
---|
This table allows an automated computer search of the external PubMed database using the listed "Search term" text link.
More? References | Discussion Page | Journal Searches | 2019 References | 2020 References Search term: Tetralogy of Fallot <pubmed limit=5>Tetralogy of Fallot</pubmed> |
International Classification of Diseases
ICD11
- LB21 Congenital anomaly of ventricles and ventricular septum
- LB21.3 Tetralogy of Fallot - defined as a group of malformations with biventricular atrioventricular alignments or connections characterized by anterosuperior deviation of the conal or outlet septum or its fibrous remnant, narrowing or atresia of the pulmonary outflow, a ventricular septal defect of the malalignment type, and biventricular origin of the aorta. Tetralogy of Fallot will always have a ventricular septal defect, narrowing or atresia of the pulmonary outflow, aortic override, and most often right ventricular hypertrophy.
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 |
|
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.
|
ICD10
ICD10 Congenital malformations of the circulatory system
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
Anatomy
The aortic root is overriding the crest of the muscular ventricular septum, in presence of a large interventricular communication. The muscular outlet septum is deviated antero-cranially, and is located exclusively within the right ventricle. Together with the hypertrophied trabeculations on the parietal wall of the right ventricle, it produces muscular subpulmonary obstruction. The walls of the right ventricle are hypertrophied, producing the fourth feature of the tetralogy.
Autopsy showing features of Tetralogy of Fallot[3] | Tetralogy of Fallot Echocardiography[3] |
People
Dr Helen Brooke Taussig (1898-1986) was a paediatric cardiologist, who developed a surgical procedure for Tetralogy of Fallot.[4] She also campaigned for blocking introduction of thalidomide into the U.S.A. One of her students had drawn her attention to the data on congenital malformations occurring in Germany and England.
- "Taussig used fluoroscopy, a new x-ray technique, to establish that babies suffering from anoxemia had a leaking septum (the wall that separates the chambers of the heart), and an underdeveloped artery leading from the heart to the lungs. In 1941 Taussig suggested an idea for an operation that might help children with "blue baby" to her colleagues at Hopkins—surgeon Alfred Blalock and surgical technician Vivien Thomas. On November 9, 1944 Taussig and Blalock first performed this new operation on a child with anoxemia, (after Thomas had experimented extensively with the procedure). They later repeated it successfully on two more patients. They published their results in the Journal of the American Medical Association. The technique was named the Blalock-Taussig operation, and was soon used worldwide. Taussig continued her research on cardiac birth defects and published her important work Congenital Malformations of the Heart, in 1947." (excerpt from NLM biography)
- Search PubMed: Taussig HB (Author)
References
- ↑ Cordell HJ, Töpf A, Mamasoula C, Postma AV, Bentham J, Zelenika D, Heath S, Blue G, Cosgrove C, Granados Riveron J, Darlay R, Soemedi R, Wilson IJ, Ayers KL, Rahman TJ, Hall D, Mulder BJ, Zwinderman AH, van Engelen K, Brook JD, Setchfield K, Bu'Lock FA, Thornborough C, O'Sullivan J, Stuart AG, Parsons J, Bhattacharya S, Winlaw D, Mital S, Gewillig M, Breckpot J, Devriendt K, Moorman AF, Rauch A, Lathrop GM, Keavney BD & Goodship JA. (2013). Genome-wide association study identifies loci on 12q24 and 13q32 associated with tetralogy of Fallot. Hum. Mol. Genet. , 22, 1473-81. PMID: 23297363 DOI.
- ↑ Wei D, Bao H, Liu XY, Zhou N, Wang Q, Li RG, Xu YJ & Yang YQ. (2013). GATA5 loss-of-function mutations underlie tetralogy of fallot. Int J Med Sci , 10, 34-42. PMID: 23289003 DOI.
- ↑ 3.0 3.1 Bailliard F & Anderson RH. (2009). Tetralogy of Fallot. Orphanet J Rare Dis , 4, 2. PMID: 19144126 DOI.
- ↑ TAUSSIG H & BLALOCK A. (1947). Surgery of congenital heart disease. Br Med J , 2, 462. PMID: 20263273
Reviews
Kilner PJ. (2011). Imaging congenital heart disease in adults. Br J Radiol , 84 Spec No 3, S258-68. PMID: 22723533 DOI.
Motta P & Miller-Hance WC. (2012). Transesophageal echocardiography in tetralogy of Fallot. Semin Cardiothorac Vasc Anesth , 16, 70-87. PMID: 22344815 DOI.
Caplan R & Allen PJ. (2011). Physical activity recommendations for adolescents with repaired tetralogy of fallot: review of the literature and guidelines for practitioners. Pediatr Nurs , 37, 191-9. PMID: 21916347
Starr JP. (2010). Tetralogy of fallot: yesterday and today. World J Surg , 34, 658-68. PMID: 20091166 DOI.
Bailliard F & Anderson RH. (2009). Tetralogy of Fallot. Orphanet J Rare Dis , 4, 2. PMID: 19144126 DOI.
Prifti E, Bonacchi M, Bernabei M, Leacche M, Bartolozzi F, Murzi B, Battaglia F, Nadia NS & Vanini V. (2004). Repair of complete atrioventricular septal defect with tetralogy of fallot: our experience and literature review. J Card Surg , 19, 175-83. PMID: 15016061
Articles
Downing TE & Kim YY. (2015). Tetralogy of Fallot: General Principles of Management. Cardiol Clin , 33, 531-41, vii-viii. PMID: 26471818 DOI.
Cha S, Kim GB, Kwon BS, Bae EJ, Noh CI, Lim HG, Kim WH, Lee JR, Kim YJ & Choi JY. (2012). Recent trends in indications of fetal echocardiography and postnatal outcomes in fetuses diagnosed as congenital heart disease. Korean Circ J , 42, 839-44. PMID: 23323122 DOI.
Bittel DC, Butler MG, Kibiryeva N, Marshall JA, Chen J, Lofland GK & O'Brien JE. (2011). Gene expression in cardiac tissues from infants with idiopathic conotruncal defects. BMC Med Genomics , 4, 1. PMID: 21208432 DOI.
Search Pubmed
Search Pubmed: Tetralogy of Fallot
Search OMIM: Tetralogy of Fallot
External Links
External Links Notice - The dynamic nature of the internet may mean that some of these listed links may no longer function. If the link no longer works search the web with the link text or name. Links to any external commercial sites are provided for information purposes only and should never be considered an endorsement. UNSW Embryology is provided as an educational resource with no clinical information or commercial affiliation.
- OMIM Tetralogy of Fallot
- PubMed Health TOF
- NIH What Is Tetralogy of Fallot?
- The Royal Children's Hospital Melbourne Fallots Tetralogy
Glossary Links
- Glossary: A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z | Numbers | Symbols | Term Link
Cite this page: Hill, M.A. (2024, June 15) Embryology Cardiovascular System - Tetralogy of Fallot. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Cardiovascular_System_-_Tetralogy_of_Fallot
- © Dr Mark Hill 2024, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G