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__NOTOC__
{{DiseaseDisorder infobox |
{{DiseaseDisorder infobox |
   Name          = Persistent Truncus Arteriosus |
   Name          = Persistent Truncus Arteriosus |
   ICD10          = {{ICD10|Q|20|0|q|20}} |
   ICD10          = {{ICD10|Q|20|0|q|20}} |
   ICD9          = {{ICD9|745.0}} |
   ICD9          = {{ICD9|745.0}} |
  Image          = Gray469.png |
  Caption        = Diagrams to illustrate the transformation of the [[bulbus cordis]]. Ao. [[Truncus arteriosus]]. Au. [[Atrium (heart)|Atrium]]. B. [[Bulbus cordis]]. RV. [[Right ventricle]]. LV. [[Left ventricle]]. P. [[Pulmonary artery]]. |
   ICDO          = |
   ICDO          = |
   OMIM          = 217095 |
   OMIM          = 217095 |
   DiseasesDB    = 32081 |
   DiseasesDB    = 32081 |
   MedlinePlus    = 001111 |
   MedlinePlus    = 001111 |
  eMedicineSubj  = ped |
  eMedicineTopic = 2316 |
   MeshID        = D014339 |
   MeshID        = D014339 |
}}
}}


{{WikiDoc Cardiology Network Infobox}}
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{SI}}
{{Persistent truncus arteriosus}}


{{CMG}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{Fs}}, [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com], [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; [[Kristin Feeney|Kristin Feeney, B.S.]] [[mailto:kfeeney@elon.edu]]


'''Associate Editor-in-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
'''''Synonyms and Keywords:''''' Truncus arteriosus, TA, Buchanan's Syndrome, common arterial trunk, common aortico-pulmonary trunk


{{Editor Join}}
==[[Persistent truncus arteriosus overview|Overview]]==


==Overview==
==[[Persistent truncus arteriosus historical perspective|Historical Perspective]]==
'''Persistent truncus arteriosus''' (or '''Truncus arteriosus''') is a rare form of [[congenital heart disease]] that presents at birth. In this condition, the [[embryological]] structure known as the [[truncus arteriosus (embryology)|truncus arteriosus]] never properly divides into the [[pulmonary artery]] and [[aorta]].


==Classification==
==[[Persistent truncus arteriosus classification|Classification]]==
The most well-known classification was the fourfold system developed by Collett and Edwards in 1949.<ref>Collett RW, Edwards JE: Persistent truncus arteriosus: a classification according to anatomic types. Surg Clin North Am 1949; 29: 1245-70.</ref> Collett/Edwards Types I, II, and III are distinguished by the branching pattern of the pulmonary arteries:<ref>{{cite web |url=http://www.merck.com/mmpe/sec19/ch287/ch287k.html |title=Persistent Truncus Arteriosus: Congenital Cardiovascular Anomalies: Merck Manual Professional |accessdate=2007-11-04 |format= |work=}}</ref><ref name="McElhinney">{{cite web |url=http://www.emedicine.com/ped/topic2316.htm |title=eMedicine - Truncus Arteriosus : Article by Doff McElhinney, MD |accessdate=2007-11-04 |format= |work=}}</ref>


* Type I: truncus -> one pulmonary artery -> two lateral pulmonary arteries
==[[Persistent truncus arteriosus pathophysiology & etiology|Pathophysiology]]==
* 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.<ref name="McElhinney"> </ref>
==[[Persistent truncus arteriosus common causes|Causes]]==


Another well-known classification was defined by Van Praaghs in 1965.<ref name="pmid5828135">{{cite journal |author=Van Praagh R, Van Praagh S |title=The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases |journal=Am. J. Cardiol. |volume=16 |issue=3 |pages=406–25 |year=1965 |pmid=5828135 |doi=}}</ref><ref name="McElhinney"> </ref>
==[[Persistent truncus arteriosus differential diagnosis|Differentiating Persistent truncus arteriosus from other Disorders]]==


==Causes==
==[[Persistent truncus arteriosus epidemiology and demographics|Epidemiology and demographics]]==
Most of the time, this defect occurs spontaneously. [[Genetic disorders]], and [[teratogen]]s (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 22]]q11 deletions. The [[neural crest]], specifically a population known as the cardiac neural crest, directly contributes to the [[aorticopulmonary septum]].<ref>{{cite journal | author = Kirby ML, Gale TF, and Stewart DE.  | title = Neural crest cells contribute to normal aorticopulmonary septation. | journal = Science | volume = 220 | issue =4061 | pages = 1059-61 | year =1983 | id = PMID 6844926}}</ref> <ref>{{cite journal | author = Jiang X, Rowitch DH, Soriano P, McMahon AP, Sucov HM..| title = Fate of the mammalian cardiac neural crest...journal = Development. | volume = 127| issue =8| pages = 1607-16 | year =2000 | id = PMID  10725237}}</ref>


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.<ref>{{cite journal | author = Hutson MR, Kirby ML..  | title = Neural crest and cardiovascular development: a 20-year perspective. | journal = Birth Defects Res C Embryo Today.  | volume = 69 | issue =1| pages = 2-13 | year =2003 | id = PMID 12768653}}</ref> <ref>{{cite journal | author = Waller BR 3rd, McQuinn T, Phelps AL, Markwald RR, Lo CW, Thompson RP, Wessels A.| title = Conotruncal anomalies in the trisomy 16 mouse: an immunohistochemical analysis with emphasis on the involvement of the neural crest. | journal = Anat. Rec.  | volume = 260 | issue =3| pages = 279-93 | year =2000 | id = PMID 11066038 }}</ref> <ref>{{cite journal | author = Franz T.| title = Persistent truncus arteriosus in the Splotch mutant mouse. | journal = Anat. Embryol. (Berlin).  | volume = 180 | issue =5| pages = 457-64 | year =1989 | id = PMID 2619088 }}</ref>
==[[Persistent truncus arteriosus risk factors|Risk Factors]]==


Numerous perturbations affecting the cardiac neural crest have been associated with persistent truncus arteriosus, some of which include [[growth factor]]s ([[fibroblast growth factor 8]] and [[bone morphogenetic protein]]), [[transcription factor]]s ([[T-box]], [[Pax genes|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]].
==[[Persistent truncus arteriosus screening|Screening]]==


==Anatomical changes ==
==[[Persistent truncus arteriosus natural history|Natural history, Complications, and Prognosis]]==
Anatomical changes associated with this disorder includes:
* single artery arising from the two [[ventricle (heart)|ventricles]] which gives rise to both the aortic and pulmonary vessels
* abnormal [[truncal valve]]
* right sided [[aortic arch]] in about 30% of cases (not shown)
* large [[ventricular septal defect]]
* [[pulmonary hypertension]]
* complete mixing occurring at level of the [[great vessel]]
 
==Clinical manifestations==
* [[Cyanosis]] presents at birth
* [[Heart failure]] occurs within weeks
* [[Systolic]] ejection [[murmur]] is heard at the left sternal border
* Widened [[pulse pressure]]
* Bounding arterial pulses
* Loud second [[heart sound]]
* Biventricular hypertrophy
* [[Cardiomegaly]]
* Increased pulmonary vascularity
* [[Hypocalcemia]] (if associated with [[DiGeorge syndrome]])


==Diagnosis==
==Diagnosis==
 
[[Persistent truncus arteriosus diagnostic study of choice|Diagnostic study of choice]]| [[Persistent truncus arteriosus history and symptoms|History and Symptoms]] | [[Persistent truncus arteriosus physical examination|Physical Examination]] | [[Persistent truncus arteriosus laboratory findings|Laboratory Findings]] | [[Persistent truncus arteriosus electrocardiogram | Electrocardiogram]] | [[Persistent truncus arteriosus chest x ray|Chest X Ray]] | [[Persistent truncus arteriosus echocardiography or ultrasound|Echocardiography]]| [[Persistent truncus arteriosus CT|CT]] | [[Persistent truncus arteriosus MRI|MRI]] | [[Persistent truncus arteriosus other imaging findings|Other Imaging Findings]]| [[Persistent truncus arteriosus other diagnostic studies|Other Diagnostic Studies]]
===Echocardiography===
 
*Truncus Arteriosus Type I 1
<googlevideo>8970669383266720423&hl=en</googlevideo>
*Truncus Arteriosus Type I 2
<googlevideo>564681477766752717&hl=en</googlevideo>
*Truncus Arteriosus Type I 3
<googlevideo>564681477766752717&hl=en</googlevideo>
*Truncus Arteriosus Type I 4
<googlevideo>-3202146762215926147&hl=en</googlevideo>
*Truncus Arteriosus Type I 5
<googlevideo>5568593693292079875&hl=en</googlevideo>
*Truncus Arteriosus Type I 6
<googlevideo>-1383871098920679580&hl=en</googlevideo>


==Treatment==
==Treatment==
Treatment is with neonatal surgical repair.<ref>{{cite journal | author = Rodefeld M, Hanley F | title = Neonatal truncus arteriosus repair: surgical techniques and clinical management. | journal = Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu | volume = 5 | issue = | pages = 212-7 | year = | id = PMID 11994881}}</ref> The [[ventricular septal defect]] is closed with a patch. The [[pulmonary artery|pulmonary arteries]] are then detached from the common artery ([[truncus arteriosus]]) and connected to the [[right ventricle]] using a tube (a conduit or tunnel).
[[Persistent truncus arteriosus medical therapy|Medical Therapy]] | [[Persistent truncus arteriosus surgery|Surgery]] | [[Persistent truncus arteriosus primary prevention|Primary Prevention]] | [[Persistent truncus arteriosus secondary prevention|Secondary Prevention]] | [[Persistent truncus arteriosus cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Persistent truncus arteriosus future or investigational therapies|Future or Investigational Therapies]]


==References==
== Case Studies ==
{{Reflist|2}}


==External links==
==External links==
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* [http://pedsccm.wustl.edu/All-Net/english/cardpage/operate/truncus/repair.html surgical repair, at wustl.edu]  
* [http://pedsccm.wustl.edu/All-Net/english/cardpage/operate/truncus/repair.html surgical repair, at wustl.edu]  
* [http://www.med.umich.edu/cvc/mchc/partrun.htm Overview] at University of Michigan
* [http://www.med.umich.edu/cvc/mchc/partrun.htm Overview] at University of Michigan
* [http://www.pediatricheartsurgery.com Pediatric Heart Surgery]
* [http://www.youtube.com/user/Redmond111 The Congenital Heart Surgery Video Project]
* [http://www.youtube.com/watch?v=HXlWeSGIR7A Truncus Arteriosus Repair in a Premature Newborn Baby]
* [http://www.scribd.com/doc/43761253/Repair-of-Truncus-Arteriosus Surgery for Truncus Arteriosus: Miami Children's Hospital]


{{SIB}}
[[pt:Truncus arteriosus]]
[[fr:Tronc artériel commun]]
{{WH}}
{{WS}}


{{Congenital malformations and deformations of circulatory system}}
[[Category:Cardiovascular system]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]
[[Category:Cardiology]]
[[Category:Cardiovascular system]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
 
[[Category:Disease]]
[[pt:Truncus arteriosus]]
[[fr:Tronc artériel commun]]
 
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Latest revision as of 03:10, 16 April 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Fahimeh Shojaei, M.D., Priyamvada Singh, MBBS [2], Keri Shafer, M.D. [3]; Kristin Feeney, B.S. [[4]]

Synonyms and Keywords: Truncus arteriosus, TA, Buchanan's Syndrome, common arterial trunk, common aortico-pulmonary trunk

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Persistent truncus arteriosus from other Disorders

Epidemiology and demographics

Risk Factors

Screening

Natural history, Complications, and Prognosis

Diagnosis

Diagnostic study of choice| History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | Echocardiography| CT | MRI | Other Imaging Findings| Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

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