Persistent Truncus Arteriosus

A congenital anomaly caused by the failed development of TRUNCUS ARTERIOSUS into separate AORTA and PULMONARY ARTERY. It is characterized by a single arterial trunk that forms the outlet for both HEART VENTRICLES and gives rise to the systemic, pulmonary, and coronary arteries. It is always accompanied by a ventricular septal defect.

Also Known As:

Networked: 36 relevant articles (0 outcomes, 3 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Ventricular Heart Septal Defects (Ventricular Septal Defect)
2.	Tetralogy of Fallot (Fallot Tetralogy)
3.	Pulmonary Atresia (Pulmonary Valve Atresia)
4.	Pulmonary Valve Stenosis (Pulmonary Stenosis)
5.	Double Outlet Right Ventricle

Experts

1.	Epstein, Jonathan A: 2 articles (02/2015 - 11/2006)
2.	Yu, Qing: 2 articles (02/2015 - 12/2004)
3.	Lo, Cecilia W: 2 articles (02/2015 - 12/2004)
4.	Kirby, Margaret L: 2 articles (06/2009 - 12/2004)
5.	Mallek, Souad: 1 article (07/2015)
6.	Fourati, Hela: 1 article (07/2015)
7.	Abid, Leila: 1 article (07/2015)
8.	Daoud, Emna: 1 article (07/2015)
9.	Mnif, Zeineb: 1 article (07/2015)
10.	Kammoun, Samir: 1 article (07/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Persistent Truncus Arteriosus:

1.	beta-cyclodextrin tetradecasulfate (CTDS)IBA 04/01/2005 - "Earlier studies in the keeshond breed of dogs established that isolated conotruncal defects (CTDs) are a group of genetically and embryologically related cardiac malformations, including sub-clinical defects of the conal septum, conal ventricular septal defects, tetralogy of Fallot, and persistent truncus arteriosus. " 02/01/1993 - "A large subset consisting of a group of malformations of the ventricular outflow region, termed "conotruncal defects" (CTDs), include subarterial ventricular septal defects, tetralogy of Fallot, and persistent truncus arteriosus. "
2.	NimustineIBA 03/01/1989 - "The objective of this study was to confirm the teratogenic effects of ACNU on the cardiovascular system of chick embryos and to determine whether ACNU induces persistent truncus arteriosus by interfering with neural crest cells. " 03/01/1989 - "These results suggest that persistent truncus arteriosus in chick embryos treated with ACNU is induced by neural crest cell death." 03/01/1989 - "Nimustine hydrochloride (ACNU) is a nitrosourea derivative anticancer agent which has been shown to cause persistent truncus arteriosus in chick embryos. " 03/01/1989 - "Pathogenesis of persistent truncus arteriosus induced by nimustine hydrochloride in chick embryos."
3.	X-linked visceral HeterotaxyIBA 01/01/2010 - "This study shows an increased likelihood of specific heart anomalies, namely transposition of the great arteries, persistent truncus arteriosus, visceral heterotaxia and single ventricle, among offspring of diabetic mothers. "
4.	Coarctation of the aortaIBA 09/01/1983 - "The following lesions were repaired: coarctation of the aorta in infancy (n = 28), total anomalous pulmonary venous drainage (n = 6), aortic valvular/subvalvular lesions (n = 11), mitral and tricuspid valve lesions (n = 4), simple transposition of the great arteries (n = 3), vegetations (n = 3), persistent truncus arteriosus (n = 2), and others (n = 13). " 05/01/1970 - "Persistent truncus arteriosus is unusually common, while coarctation of the aorta and congenital aortic stenosis are rare, as compared with series reported from other places." 01/01/2013 - "The most common heart malformations are: stenosis (coarctation) of the aorta and pulmonary trunk, bicuspid aortic valve, atrial and/or ventricular septal defect, persistent truncus arteriosus (Botallo duct), transposition of the great vessels, tricuspid atresia, hypoplastic left and right heart, as well as syndrome of Lutembachera, Cantrell, Ebstein, Eisenmenger and Shone and trilogy, tetralogy, pentalogy of Fallot. "
5.	Semaphorins (Semaphorin)IBA 08/01/2001 - "Targeted disruption of semaphorin 3C leads to persistent truncus arteriosus and aortic arch interruption." 02/03/2015 - "Vital roles for semaphorin signaling in vascular patterning and cardiac morphogenesis have been demonstrated, and impaired semaphorin signaling has been associated with various human cardiovascular disorders, including persistent truncus arteriosus, sinus bradycardia and anomalous pulmonary venous connections. "
6.	CalciumIBA 04/01/1992 - "Calcium currents in hearts with persistent truncus arteriosus." 06/01/1990 - "Calcium currents were examined in an experimental model in the embryonic chick heart with a congenital malformation known as persistent truncus arteriosus. " 06/01/1990 - "Reduced L-type calcium current in the embryonic chick heart with persistent truncus arteriosus."
7.	retinoic acid receptor alphaIBA 09/01/2002 - "Mouse embryos lacking the retinoic acid (RA) receptors RARalpha1 and RARbeta suffer from a failure to properly septate (divide) the early outflow tract of the heart into distinct aortic and pulmonary channels, a phenotype termed persistent truncus arteriosus. " 06/01/1997 - "Malformations of the ventricular chamber (chamber hypoplasia and muscular ventricular septal defects), conotruncus (double-outlet right ventricle, transposition, and membranous ventricular septal defects), aortic sac (persistent truncus arteriosus and aorticopulmonary window), and aortic arch-derived arteries were recovered in various combinations of the RAR alpha 1, RAR beta, and RXR alpha gene mutations. "
8.	GATA6 Transcription FactorIBA 08/18/2009 - "Here we show that mutations in the gene encoding the transcription factor GATA6 cause CHD characteristic of a severe form of cardiac outflow tract (OFT) defect, namely persistent truncus arteriosus (PTA). "
9.	Neuropilin-1IBA 11/01/1999 - "This paper reported that the neuropilin-1 mutant mouse embryos exhibited various types of vascular defects, including impairment in neural vascularization, agenesis and transposition of great vessels, insufficient aorticopulmonary truncus (persistent truncus arteriosus), and disorganized and insufficient development of vascular networks in the yolk sac. "
10.	Connexin 43 (Connexin43)IBA 12/01/2004 - "In two other families, the ENU-induced mutation was identified--Sema3CL605P was associated with persistent truncus arteriosus with interrupted aortic arch, and the Gja1W45X connexin43 mutation caused conotruncal malformation and coronary aneurysms. "

Therapies and Procedures

1.	Homologous Transplantation (Allograft) 05/01/1994 - "Repair of persistent truncus arteriosus has been achieved with low early mortality and no late mortality, which reflects excellent long-term function of the homograft. " 07/26/1992 - "[Successful surgical correction of persistent truncus arteriosus by an aortic homograft]."
2.	Parasympathectomy 09/01/1990 - "The pattern of actin expression was found to be abnormal in hearts which were sympathetically aneural and those which had persistent truncus arteriosus combined with parasympathetic denervation. " 09/01/1990 - "The neural crest provides both neural (sympathetic and parasympathetic) and ectomesenchymal components to the heart, and selective removal of various areas results in embryos with sympathetically aneural hearts, or persistent truncus arteriosus with or without parasympathetic denervation. "
3.	Replantation 03/01/1989 - "[Successful reimplantation of a conduit 7 years after radical correction of a persistent truncus arteriosus]."
4.	Anesthesia 06/01/2009 - "Persistent truncus arteriosus (PTA) was diagnosed in an 8-year-old neutered male Poodle referred for echocardiographic examination prior to anesthesia for surgical correction of bilateral cataract. "