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Persistent truncus arteriosus(PTA)

MedGen UID:
52867
•Concept ID:
C0041207
•
Congenital Abnormality
Synonym: Persistent truncus arteriosus (disease)
SNOMED CT: Common truncus arteriosus (61959006); Persistent truncus arteriosus (61959006); Common aortopulmonary trunk (61959006); Truncus arteriosus communis (61959006); Common arterial trunk (truncus arteriosus) (61959006); Persistent truncus arteriosus (787779000)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
•Concept ID:
C0441748
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Autosomal recessive inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018072
OMIM®: 217095; 601656; 611770
Orphanet: ORPHA3384

Definition

Truncus arteriosus (TA) is a rare congenital cardiovascular anomaly characterized by a single arterial trunk arising from the heart by means of a single semilunar valve (<I>i.e.</I> truncal valve). Pulmonary arteries originate from the common arterial trunk distal to the coronary arteries and proximal to the first brachiocephalic branch of the aortic arch. TA typically overrides a large outlet ventricular septal defect (VSD). The intracardiac anatomy usually displays situs solitus and atrioventricular (AV) concordance. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPersistent truncus arteriosus

Professional guidelines

PubMed

Genotype and cardiovascular phenotype correlations with TBX1 in 1,022 velo-cardio-facial/DiGeorge/22q11.2 deletion syndrome patients.
Guo T, McDonald-McGinn D, Blonska A, Shanske A, Bassett AS, Chow E, Bowser M, Sheridan M, Beemer F, Devriendt K, Swillen A, Breckpot J, Digilio MC, Marino B, Dallapiccola B, Carpenter C, Zheng X, Johnson J, Chung J, Higgins AM, Philip N, Simon TJ, Coleman K, Heine-Suner D, Rosell J, Kates W, Devoto M, Goldmuntz E, Zackai E, Wang T, Shprintzen R, Emanuel B, Morrow B; International Chromosome 22q11.2 Consortium
Hum Mutat 2011 Nov;32(11):1278-89. Epub 2011 Sep 16 doi: 10.1002/humu.21568. PMID: 21796729Free PMC Article
Prenatal diagnosis of conotruncal malformations: diagnostic accuracy, outcome, chromosomal abnormalities, and extracardiac anomalies.
Sivanandam S, Glickstein JS, Printz BF, Allan LD, Altmann K, Solowiejczyk DE, Simpson L, Perez-Delboy A, Kleinman CS
Am J Perinatol 2006 May;23(4):241-5. Epub 2006 Apr 19 doi: 10.1055/s-2006-939535. PMID: 16625498
Persistent truncus arteriosus and pseudotruncus arteriosus. Angiographic criteria for corrective surgery.
Klinkhamer AC, Verheugt AP
Radiol Clin Biol 1972;41(1):36-46. PMID: 5059345

Recent clinical studies

Etiology

Non-Conduit Repair of Truncus Arteriosus.
Kılıç Y, Doyurgan O, İrdem AK, Gül Ö, Borakay D, Aldudak B
Braz J Cardiovasc Surg 2023 Apr 23;38(2):248-251. doi: 10.21470/1678-9741-2022-0029. PMID: 36259996Free PMC Article
Discussion.
Morell VO
J Thorac Cardiovasc Surg 2022 Jan;163(1):235-236. Epub 2021 Mar 13 doi: 10.1016/j.jtcvs.2021.01.137. PMID: 33726910
Long-Term Fate of the Truncal Valve.
Gellis L, Binney G, Alshawabkeh L, Lu M, Landzberg MJ, Mayer JE, Mullen MP, Valente AM, Sleeper LA, Brown DW
J Am Heart Assoc 2020 Nov 17;9(22):e019104. Epub 2020 Nov 9 doi: 10.1161/JAHA.120.019104. PMID: 33161813Free PMC Article
Outcomes of truncus arteriosus repair and predictors of mortality.
Hamzah M, Othman HF, Daphtary K, Komarlu R, Aly H
J Card Surg 2020 Aug;35(8):1856-1864. Epub 2020 Jun 19 doi: 10.1111/jocs.14730. PMID: 32557823
Surgical repair for persistent truncus arteriosus in neonates and older children.
Alamri RM, Dohain AM, Arafat AA, Elmahrouk AF, Ghunaim AH, Elassal AA, Jamjoom AA, Al-Radi OO
J Cardiothorac Surg 2020 May 11;15(1):83. doi: 10.1186/s13019-020-01114-1. PMID: 32393289Free PMC Article

Diagnosis

Surgical Repair of Truncus Arteriosus: A Long-Term Hemodynamic Assessment.
Martín de Miguel I, Jain CC, Egbe AC, Hagler DJ, Connolly HM, Miranda WR
World J Pediatr Congenit Heart Surg 2022 Nov;13(6):716-722. doi: 10.1177/21501351221114779. PMID: 36300270
Discussion.
Morell VO
J Thorac Cardiovasc Surg 2022 Jan;163(1):235-236. Epub 2021 Mar 13 doi: 10.1016/j.jtcvs.2021.01.137. PMID: 33726910
"Micro-Bentall" procedure.
Pelouze A, Prêtre R, Beghetti M, Sologashvili T
Multimed Man Cardiothorac Surg 2020 Dec 24;2020 doi: 10.1510/mmcts.2020.079. PMID: 33577148
Outcomes of truncus arteriosus repair and predictors of mortality.
Hamzah M, Othman HF, Daphtary K, Komarlu R, Aly H
J Card Surg 2020 Aug;35(8):1856-1864. Epub 2020 Jun 19 doi: 10.1111/jocs.14730. PMID: 32557823
Persistent truncus arteriosus on dual source CT.
Sharma A, Priya S, Jagia P
Jpn J Radiol 2016 Jul;34(7):486-93. Epub 2016 Jun 4 doi: 10.1007/s11604-016-0559-x. PMID: 27262857

Therapy

Surgical Repair of Truncus Arteriosus: A Long-Term Hemodynamic Assessment.
Martín de Miguel I, Jain CC, Egbe AC, Hagler DJ, Connolly HM, Miranda WR
World J Pediatr Congenit Heart Surg 2022 Nov;13(6):716-722. doi: 10.1177/21501351221114779. PMID: 36300270
Discussion.
Morell VO
J Thorac Cardiovasc Surg 2022 Jan;163(1):235-236. Epub 2021 Mar 13 doi: 10.1016/j.jtcvs.2021.01.137. PMID: 33726910
Long-term outcomes of truncus arteriosus repair: A modulated renewal competing risks analysis.
Guariento A, Doulamis IP, Staffa SJ, Gellis L, Oh NA, Kido T, Mayer JE, Baird CW, Emani SM, Zurakowski D, Del Nido PJ, Nathan M
J Thorac Cardiovasc Surg 2022 Jan;163(1):224-236.e6. Epub 2021 Feb 12 doi: 10.1016/j.jtcvs.2021.01.136. PMID: 33726908
Modified Repair of Type I and II Truncus Arteriosus Limits Early Right Ventricular Outflow Tract Reoperation.
Louis C, Swartz MF, Simon BV, Cholette JM, Atallah-Yunes N, Wang H, Gensini F, Alfieris GM
Semin Thorac Cardiovasc Surg 2018 Summer;30(2):199-204. Epub 2018 Feb 8 doi: 10.1053/j.semtcvs.2018.02.003. PMID: 29428623
Repair of persistent truncus arteriosus without a conduit: sleeve resection of the pulmonary trunk from the aorta and direct right ventricle-pulmonary artery anastomosis.
Nemoto S, Ozawa H, Sasaki T, Katsumata T, Kishi K, Okumura K, Mori Y
Eur J Cardiothorac Surg 2011 Sep;40(3):563-8. Epub 2011 Jan 14 doi: 10.1016/j.ejcts.2010.11.066. PMID: 21237668

Prognosis

Surgical Repair of Truncus Arteriosus: A Long-Term Hemodynamic Assessment.
Martín de Miguel I, Jain CC, Egbe AC, Hagler DJ, Connolly HM, Miranda WR
World J Pediatr Congenit Heart Surg 2022 Nov;13(6):716-722. doi: 10.1177/21501351221114779. PMID: 36300270
Long-Term Fate of the Truncal Valve.
Gellis L, Binney G, Alshawabkeh L, Lu M, Landzberg MJ, Mayer JE, Mullen MP, Valente AM, Sleeper LA, Brown DW
J Am Heart Assoc 2020 Nov 17;9(22):e019104. Epub 2020 Nov 9 doi: 10.1161/JAHA.120.019104. PMID: 33161813Free PMC Article
Outcomes of truncus arteriosus repair and predictors of mortality.
Hamzah M, Othman HF, Daphtary K, Komarlu R, Aly H
J Card Surg 2020 Aug;35(8):1856-1864. Epub 2020 Jun 19 doi: 10.1111/jocs.14730. PMID: 32557823
Surgical repair for persistent truncus arteriosus in neonates and older children.
Alamri RM, Dohain AM, Arafat AA, Elmahrouk AF, Ghunaim AH, Elassal AA, Jamjoom AA, Al-Radi OO
J Cardiothorac Surg 2020 May 11;15(1):83. doi: 10.1186/s13019-020-01114-1. PMID: 32393289Free PMC Article
The Blalock-Taussig shunt.
Yuan SM, Shinfeld A, Raanani E
J Card Surg 2009 Mar-Apr;24(2):101-8. Epub 2008 Nov 7 doi: 10.1111/j.1540-8191.2008.00758.x. PMID: 19040408

Clinical prediction guides

Outcome of truncus arteriosus repair: 20 years of single-center experience comparing early versus late surgical repair.
Hrfi A, Ismail M, Mohammed MHA, Hamadah HK, Alhabshan F, Abu-Sulaiman R, Kabbani MS
Cardiol Young 2022 Aug;32(8):1289-1295. Epub 2021 Oct 20 doi: 10.1017/S104795112100408X. PMID: 34666851
Long-Term Fate of the Truncal Valve.
Gellis L, Binney G, Alshawabkeh L, Lu M, Landzberg MJ, Mayer JE, Mullen MP, Valente AM, Sleeper LA, Brown DW
J Am Heart Assoc 2020 Nov 17;9(22):e019104. Epub 2020 Nov 9 doi: 10.1161/JAHA.120.019104. PMID: 33161813Free PMC Article
Outcomes of truncus arteriosus repair and predictors of mortality.
Hamzah M, Othman HF, Daphtary K, Komarlu R, Aly H
J Card Surg 2020 Aug;35(8):1856-1864. Epub 2020 Jun 19 doi: 10.1111/jocs.14730. PMID: 32557823
Surgical repair for persistent truncus arteriosus in neonates and older children.
Alamri RM, Dohain AM, Arafat AA, Elmahrouk AF, Ghunaim AH, Elassal AA, Jamjoom AA, Al-Radi OO
J Cardiothorac Surg 2020 May 11;15(1):83. doi: 10.1186/s13019-020-01114-1. PMID: 32393289Free PMC Article
The Blalock-Taussig shunt.
Yuan SM, Shinfeld A, Raanani E
J Card Surg 2009 Mar-Apr;24(2):101-8. Epub 2008 Nov 7 doi: 10.1111/j.1540-8191.2008.00758.x. PMID: 19040408

Recent systematic reviews

Outcomes in Primary Repair of Truncus Arteriosus with Significant Truncal Valve Insufficiency: A Systematic Review and Meta-analysis.
Mitta A, Vogel AD, Korte JE, Brennan E, Bradley SM, Kavarana MN, Konrad Rajab T, Kwon JH
Pediatr Cardiol 2023 Dec;44(8):1649-1657. Epub 2023 Jul 20 doi: 10.1007/s00246-023-03231-9. PMID: 37474609
Outcomes of truncal valve replacement in neonates and infants: a meta-analysis.
Hardy WA, Kang L, Turek JW, Rajab TK
Cardiol Young 2023 May;33(5):673-680. Epub 2023 Mar 27 doi: 10.1017/S1047951123000604. PMID: 36970855

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