Persistent truncus arteriosus

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Persistent Truncus Arteriosus
Truncus arteriosus.jpg
Illustration of truncus arteriosus
Classification and external resources
Specialty Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value).
ICD-10 Q20.0
ICD-9-CM 745.0
OMIM 217095
DiseasesDB 32081
MedlinePlus 001111
eMedicine ped/2316
Patient UK Persistent truncus arteriosus
MeSH D014339
[[[d:Lua error in Module:Wikidata at line 863: attempt to index field 'wikibase' (a nil value).|edit on Wikidata]]]

Persistent truncus arteriosus (or Patent truncus arteriosus or Common arterial trunk), is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. This results in one arterial trunk arising from the heart and providing mixed blood to the coronary arteries, pulmonary arteries, and systemic circulation.[1]

Classification

The most well-known classification was the fourfold system developed by Collett and Edwards in 1949.[2] Collett/Edwards Types I, II, and III are distinguished by the branching pattern of the pulmonary arteries:[3][4]

  • Type I: truncus -> one pulmonary artery -> two lateral pulmonary arteries
  • Type II: truncus -> two posterior/posterolateral pulmonary arteries
  • Type III: truncus -> two lateral pulmonary arteries

The "Type IV" proposed in 1949 is no longer considered a form of PTA by most modern sources.[4]

Another well-known classification was defined by Van Praaghs in 1965.[4][5]

Causes

Most of the time, this defect occurs spontaneously. Genetic disorders, and teratogens (viruses, metabolic imbalance, and industrial or pharmacological agents) have been associated as possible causes. Up to 50% (varies in studies) of cases are associated with chromosome 22q11 deletions (DiGeorge Syndrome). The neural crest, specifically a population known as the cardiac neural crest, directly contributes to the aorticopulmonary septum.[6][7]

Microablation of the cardiac neural crest in developing chick embryos and genetic anomalies affecting this population of cells in rodents results in persistent truncus arteriosus.[8][9][10]

Numerous perturbations affecting the cardiac neural crest have been associated with persistent truncus arteriosus, some of which include growth factors (fibroblast growth factor 8 and bone morphogenetic protein), transcription factors (T-box, Pax, Nkx2-5, GATA-6, and Forkhead), and gap junction proteins (Connexin). The cardiac neural crest also contributes the smooth muscle of the great arteries.

Anatomical changes

Anatomical changes associated with this disorder includes:

Clinical manifestations

Treatment

Treatment is with neonatal surgical repair.[11] The ventricular septal defect is closed with a patch. The pulmonary arteries are then detached from the common artery (truncus arteriosus) and connected to the right ventricle using a tube (a conduit or tunnel). There have been cases where the condition has been diagnosed at birth and surgical intervention is an option. A number of these cases have survived well into adulthood. [12]

Epidemiology

Persistent truncus arteriosus is a rare cardiac abnormality that has a prevalence of less than 1%.[1][13]

Additional images

See also

References

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  2. Collett RW, Edwards JE: Persistent truncus arteriosus: a classification according to anatomic types. Surg Clin North Am 1949; 29: 1245-70.
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  12. http://sfx.lis.curtin.edu.au/sfx_local?sid=Entrez%3APubMed&id=pmid%3A15872317
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External links