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never-ceasing; "the relentless beat of the drums" (同)relentless, unrelenting
retained; not shed; "persistent leaves remain attached past maturity"; "the persistent gills of fishes" (同)lasting

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(反対・困難にもくじけず)『あくまでもやり抜く』,ねばり強い,執ような / いつまでも続く,持続する(lasting)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/12 02:28:50」(JST)

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Persistent truncus arteriosus (or patent truncus arteriosus), also known as 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:

single artery arising from the two 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
right-to-left shunting of blood

Clinical manifestations

Cyanosis presents at birth
Heart failure may occur 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)

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

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

Additional images

Diagrams to illustrate the transformation of the bulbus cordis. Ao. Truncus arteriosus. Au. Atrium. B. Bulbus cordis. RV. Right ventricle. LV. Left ventricle. P. Pulmonary artery.

References

^ ^a ^b Barata, IA (Aug 2013). "Cardiac emergencies.". Emergency medicine clinics of North America 31 (3): 677–704. doi:10.1016/j.emc.2013.04.007. PMID 23915599.
^ Collett RW, Edwards JE: Persistent truncus arteriosus: a classification according to anatomic types. Surg Clin North Am 1949; 29: 1245-70.
^ "Persistent Truncus Arteriosus: Congenital Cardiovascular Anomalies: Merck Manual Professional". Retrieved 2007-11-04.
^ ^a ^b ^c "eMedicine - Truncus Arteriosus : Article by Doff McElhinney, MD". Retrieved 2007-11-04.
^ Van Praagh R, Van Praagh S (1965). "The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases". Am. J. Cardiol. 16 (3): 406–25. doi:10.1016/0002-9149(65)90732-0. PMID 5828135.
^ Kirby ML, Gale TF, and Stewart DE. (1983). "Neural crest cells contribute to normal aorticopulmonary septation.". Science 220 (4061): 1059–61. doi:10.1126/science.6844926. PMID 6844926.
^ Jiang X, Rowitch DH, Soriano P, McMahon AP, Sucov HM.. (2000). "Fate of the mammalian cardiac neural crest...". Development (Cambridge, England) 127 (8): 1607–16. PMID 10725237.
^ Hutson MR, Kirby ML.. (2003). "Neural crest and cardiovascular development: a 20-year perspective.". Birth Defects Res C Embryo Today. 69 (1): 2–13. doi:10.1002/bdrc.10002. PMID 12768653.
^ Waller BR 3rd, McQuinn T, Phelps AL, Markwald RR, Lo CW, Thompson RP, Wessels A. (2000). "Conotruncal anomalies in the trisomy 16 mouse: an immunohistochemical analysis with emphasis on the involvement of the neural crest.". Anat. Rec. 260 (3): 279–93. doi:10.1002/1097-0185(20001101)260:3<279::AID-AR65>3.0.CO;2-2. PMID 11066038.
^ Franz T. (1989). "Persistent truncus arteriosus in the Splotch mutant mouse.". Anat. Embryol. (Berlin). 180 (5): 457–64. doi:10.1007/BF00305120. PMID 2619088.
^ Rodefeld M, Hanley F (2002). "Neonatal truncus arteriosus repair: surgical techniques and clinical management.". Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5 (1): 212–7. doi:10.1053/pcsu.2002.31497. PMID 11994881.
^ http://sfx.lis.curtin.edu.au/sfx_local?sid=Entrez%3APubMed&id=pmid%3A15872317
^ Parker, SE; Mai, CT; Canfield, MA; Rickard, R; Wang, Y; Meyer, RE; Anderson, P; Mason, CA; Collins, JS; Kirby, RS; Correa, A; National Birth Defects Prevention, Network (Dec 2010). "Updated National Birth Prevalence estimates for selected birth defects in the United States, 2004-2006.". Birth defects research. Part A, Clinical and molecular teratology 88 (12): 1008–16. doi:10.1002/bdra.20735. PMID 20878909.

External links

Truncus Arteriosus Cincinnati Children's Medical Center
A.D.A.M. Medical Encyclopedia from NCBI site.
Surgery for Truncus Arteriosus: Powerpoint™ Presentation
Truncus Arteriosus Repair in a Premature Newborn Baby
Truncus arteriosus information for parents.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 総動脈幹 truncus arteriosus
2. 新生児におけるチアノーゼの心臓の原因 cardiac causes of cyanosis in the newborn
3. 新生児におけるチアノーゼ性心疾患の診断および初期マネージメント diagnosis and initial management of cyanotic heart disease in the newborn
4. Newborn screening for critical congenital heart disease using pulse oximetry
5. Overview of common causes of cardiac murmurs in infants and children

English Journal

Cardiac outflow morphogenesis depends on effects of retinoic acid signaling on multiple cell lineages.

El Robrini N1,2, Etchevers HC1,2, Ryckebüsch L1,2, Faure E1,2, Eudes N1,2, Niederreither K3, Zaffran S1,2, Bertrand N1,2.
Developmental dynamics : an official publication of the American Association of Anatomists.Dev Dyn.2016 Mar;245(3):388-401. doi: 10.1002/dvdy.24357. Epub 2015 Oct 28.
BACKGROUND: Retinoic acid (RA), the bioactive derivative of vitamin A, is essential for vertebrate heart development. Both excess and reduced RA signaling lead to cardiovascular malformations affecting the outflow tract (OFT). To address the cellular mechanisms underlying the effects of RA signaling
PMID 26442704

The SWI/SNF BAF-A complex is essential for neural crest development.

Chandler RL1, Magnuson T2.
Developmental biology.Dev Biol.2016 Mar 1;411(1):15-24. doi: 10.1016/j.ydbio.2016.01.015. Epub 2016 Jan 22.
Growing evidence indicates that chromatin remodeler mutations underlie the pathogenesis of human neurocristopathies or disorders that affect neural crest cells (NCCs). However, causal relationships among chromatin remodeler subunit mutations and NCC defects remain poorly understood. Here we show tha
PMID 26806701

Dysregulated endocardial TGFβ signaling and mesenchymal transformation result in heart outflow tract septation failure.

Ma MC1, Li P1, Shen H1, Estrada KD1, Xu J2, Kumar SR3, Sucov HM4.
Developmental biology.Dev Biol.2016 Jan 1;409(1):272-6. doi: 10.1016/j.ydbio.2015.09.021. Epub 2015 Oct 29.
Heart outflow tract septation in mouse embryos carrying mutations in retinoic acid receptor genes fails with complete penetrance. In this mutant background, ectopic TGFβ signaling in the distal outflow tract is responsible for septation failure, but it was uncertain what tissue was responsive to ec
PMID 26522286

Japanese Journal

臨床研究・症例報告総動脈幹遺残に気管・気管支軟化症を合併した1例

佐藤智幸,稀代雅彦,清水俊明
小児科臨床 63(11), 2351-2355, 2010-11-00
NAID 40017327269

Right Ventricular Outflow Tract Reconstruction in Patients With Persistent Truncus Arteriosus : A 15-Year Experience in a Single Japanese Center

Honjo Osami,Kotani Yasuhiro,Akagi Teiji,Osaki Satoru,Kawada Masaaki,Ishino Kozo,Sano Shunji
Circulation journal : official journal of the Japanese Circulation Society 71(11), 1776-1780, 2007-10-20
… Background The present study analyzes a 15-year experience of repairing persistent truncus arteriosus (PTA) with a consistent policy of right ventricular outflow tract (RVOT) reconstruction (ie, direct anastomosis). …
NAID 110006448590

Developmental Toxicity Induced by Inhalation Exposure of Pregnant Rats to N,N-Dimethylacetamide

Okuda Hirokazu,Takeuchi Tetsuya,Senoh Hideki [他],ARITO Heihachiro,NAGANO Kasuke,YAMAMOTO Seigo,MATSUSHIMA Taijiro
Journal of occupational health 48(3), 154-160, 2006-05-00
… Observed cardiovascular malformations included ventricular septum defect, persistent truncus arteriosus, malpositioned subclavian branch and retroesophageal subclavian artery. … Persistent truncus arteriosus was accompanied by ventricular septal defect (VSD). …
NAID 110004729535

Related Pictures

★リンクテーブル★

リンク元	「総動脈幹症」「PTA」「総動脈幹」
関連記事	「persistent」「truncus arteriosus」「arteriosus」

「総動脈幹症」

Persistent Truncus Arteriosus
	Illustration of truncus arteriosus
Classification and external resources
ICD-10	Q20.0
ICD-9-CM	745.0
OMIM	217095
DiseasesDB	32081
MedlinePlus	001111
eMedicine	ped/2316
MeSH	D014339

　　[★]

英: truncus arteriosus communis TAC
同: 総動脈幹残遺動脈幹遺残症総動脈幹遣残症 persistent truncus arteriosus PTA
関: 総動脈幹

[show details]

en

persistent truncus arteriosus : 約 20,500 件
truncus arteriosus communis : 約 54,500 件

ja

persistent truncus arteriosus : 78 件
truncus arteriosus communis : 56 件

「PTA」

　　[★]

「総動脈幹」

　　[★]

ラ: truncus arteriosus
関: [[]]

臨床関連

動脈幹遺残症総動脈幹症 persistent truncus arteriosus PTA

「persistent」

　　[★]

adj.

持続性の、持続的な、遷延性の、遺残の、残留性の

関: continuous、continuously、insurmountable、long-lasting、permanent、permanently、persistence、persistently、persisting、prolonged、repository、sustained、tonic、tonically

「truncus arteriosus」

　　[★]

総動脈管 TA

同: persistent

「arteriosus」

　　[★]

adj.

動脈性の

関: arterial

[cardiac_emergencies_pmid_23915599-1] Barata, IA (Aug 2013). "Cardiac emergencies.". Emergency medicine clinics of North America 31 (3): 677–704. doi:10.1016/j.emc.2013.04.007. PMID 23915599.

[2] Collett RW, Edwards JE: Persistent truncus arteriosus: a classification according to anatomic types. Surg Clin North Am 1949; 29: 1245-70.

[3] "Persistent Truncus Arteriosus: Congenital Cardiovascular Anomalies: Merck Manual Professional". Retrieved 2007-11-04.

[McElhinney-4] "eMedicine - Truncus Arteriosus : Article by Doff McElhinney, MD". Retrieved 2007-11-04.

[pmid5828135-5] Van Praagh R, Van Praagh S (1965). "The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases". Am. J. Cardiol. 16 (3): 406–25. doi:10.1016/0002-9149(65)90732-0. PMID 5828135.

[6] Kirby ML, Gale TF, and Stewart DE. (1983). "Neural crest cells contribute to normal aorticopulmonary septation.". Science 220 (4061): 1059–61. doi:10.1126/science.6844926. PMID 6844926.

[7] Jiang X, Rowitch DH, Soriano P, McMahon AP, Sucov HM.. (2000). "Fate of the mammalian cardiac neural crest...". Development (Cambridge, England) 127 (8): 1607–16. PMID 10725237.

[8] Hutson MR, Kirby ML.. (2003). "Neural crest and cardiovascular development: a 20-year perspective.". Birth Defects Res C Embryo Today. 69 (1): 2–13. doi:10.1002/bdrc.10002. PMID 12768653.

[9] Waller BR 3rd, McQuinn T, Phelps AL, Markwald RR, Lo CW, Thompson RP, Wessels A. (2000). "Conotruncal anomalies in the trisomy 16 mouse: an immunohistochemical analysis with emphasis on the involvement of the neural crest.". Anat. Rec. 260 (3): 279–93. doi:10.1002/1097-0185(20001101)260:3<279::AID-AR65>3.0.CO;2-2. PMID 11066038.

[10] Franz T. (1989). "Persistent truncus arteriosus in the Splotch mutant mouse.". Anat. Embryol. (Berlin). 180 (5): 457–64. doi:10.1007/BF00305120. PMID 2619088.

[11] Rodefeld M, Hanley F (2002). "Neonatal truncus arteriosus repair: surgical techniques and clinical management.". Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5 (1): 212–7. doi:10.1053/pcsu.2002.31497. PMID 11994881.

[12] ttp://sfx.lis.curtin.edu.au/sfx_local?sid=Entrez%3APubMed&id=pmid%3A15872317

[13] Parker, SE; Mai, CT; Canfield, MA; Rickard, R; Wang, Y; Meyer, RE; Anderson, P; Mason, CA; Collins, JS; Kirby, RS; Correa, A; National Birth Defects Prevention, Network (Dec 2010). "Updated National Birth Prevalence estimates for selected birth defects in the United States, 2004-2006.". Birth defects research. Part A, Clinical and molecular teratology 88 (12): 1008–16. doi:10.1002/bdra.20735. PMID 20878909.

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persistent truncus arteriosus