Truncus Arteriosus: Pathophysiology, Investigations, and Treatment
Omar Elsaka *
Department of Cardiology, Mansoura University, Faculty of Medicine, Mansoura Manchester Medical Program (MMMP), Mansoura, Egypt.
Moneer Ayman Noureldean
Department of Cardiology, Mansoura University, Faculty of Medicine, Mansoura Manchester Medical Program (MMMP), Mansoura, Egypt.
Mohamed Adel Gamil
Department of Cardiology, Mansoura University, Faculty of Medicine, Mansoura Manchester Medical Program (MMMP), Mansoura, Egypt.
Mostafa Tarek Ghazali
Department of Cardiology, Mansoura University, Faculty of Medicine, Mansoura Manchester Medical Program (MMMP), Mansoura, Egypt.
Ashraf Hamada Abd Al-Razik
Department of Cardiology, Mansoura University, Faculty of Medicine, Mansoura Manchester Medical Program (MMMP), Mansoura, Egypt.
Dalia Hisham
Department of Cardiology, Mansoura University, Faculty of Medicine, Mansoura Manchester Medical Program (MMMP), Mansoura, Egypt.
*Author to whom correspondence should be addressed.
Abstract
Background: The failure of the primitive arterial trunk to divide into the aorta and the pulmonary artery causes persistent truncus arteriosus, an uncommon heart abnormality. As a result, the heart has only one outflow, which is an arterial vessel that supplies the systemic, pulmonary, and coronary circulations. The truncal valve serves both ventricles and overrides the ventricular septal defect at the output. Truncal valve dysplasia is common, resulting in stenosis and/or regurgitation in variable degrees. Three forms of truncus arteriosus can be recognized based on the origin of the branch pulmonary arteries. In about 15% of cases, and in one-third of people with DiGeorge syndrome, persistent truncus arteriosus is linked to an interrupted aortic arch. Infants with a chronic truncus arteriosus exhibit early signs of heart failure and require surgery within the first few weeks of life.
Conclusion: Children with congenital heart disease are living longer and longer thanks to developments in surgery and post-operative treatment, as well as new technology advancements.
Keywords: Truncus arteriosus, congenital heart anomalies, fetal echocardiography, neonatal cyanosis, common arterial trunk, congestive heart failure
How to Cite
Downloads
References
Puri K, Allen HD, Qureshi AM. Congenital Heart Disease. Pediatr Rev. 2017 Oct;38(10):471-486.
Moorman A, Webb S, Brown NA, Lamers W, Anderson RH. Development of the heart: (1) formation of the cardiac chambers and arterial trunks. Heart. 2003 Jul;89(7):806-14.
McElhinney DB, Driscoll DA, Emanuel BS, Goldmuntz E. Chromosome 22q11 deletion in patients with truncus arteriosus. Pediatr Cardiol. 2003 Nov-Dec;24(6):569-73.
Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008 Dec;153(6):807-13.
Lenox CC, Debich DE, Zuberbuhler JR. The role of coronary artery abnormalities in the prognosis of truncus arteriosus. J Thorac Cardiovasc Surg. 1992 Dec;104(6):1728-42.
Konstantinov IE, Karamlou T, Blackstone EH, Mosca RS, Lofland GK, Caldarone CA, Williams WG, Mackie AS, McCrindle BW. Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study. Ann Thorac Surg. 2006 Jan;81(1):214-22.
Butto F, Lucas RV, Edwards JE. Persistent truncus arteriosus: pathologic anatomy in 54 cases. Pediatr Cardiol. 1986;7(2):95-101.
Van Praagh R. Truncus arteriosus: what is it really and how should it be classified? Eur J Cardiothorac Surg. 1987;1(2):65-70.
Marcelletti C, McGoon DC, Mair DD. The natural history of truncus arteriosus. Circulation. 1976 Jul;54(1):108-11.
Niwa K, Perloff JK, Kaplan S, Child JS, Miner PD. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol. 1999 Jul;34(1):223-32.
Calder L, Van Praagh R, Van Praagh S, Sears WP, Corwin R, Levy A, Keith JD, Paul MH. Truncus arteriosus communis. Clinical, angiocardiographic, and pathologic findings in 100 patients. Am Heart J. 1976 Jul;92(1):23-38.
TAUSSIG HB. Clinical and pathological findings in cases of truncus arteriosus in infancy. Am J Med. 1947 Jan;2(1):26-34.
Tlaskal T, Hucin B, Kucera V, Vojtovic P, Gebauer R, Chaloupecky V, Skovranek J. Repair of persistent truncus arteriosus with interrupted aortic arch. Eur J Cardiothorac Surg. 2005 Nov;28(5):736-41.
Chew C, Halliday JL, Riley MM, Penny DJ. Population-based study of antenatal detection of congenital heart disease by ultrasound examination. Ultrasound Obstet Gynecol. 2007 Jun;29(6):619-24.
Mawad W, Mertens LL. Recent Advances and Trends in Pediatric Cardiac Imaging. Curr Treat Options Cardiovasc Med. 2018 Feb 21;20(1):9.
Samyn MM. A review of the complementary information available with cardiac magnetic resonance imaging and multi-slice computed tomography (CT) during the study of congenital heart disease. Int J Cardiovasc Imaging. 2004 Dec;20(6):569-78.
Naimo PS, Fricke TA, Yong MS, d'Udekem Y, Kelly A, Radford DJ, Bullock A, Weintraub RG, Brizard CP, Konstantinov IE. Outcomes of Truncus Arteriosus Repair in Children: 35 Years of Experience From a Single Institution. 2016 SummerSemin Thorac Cardiovasc Surg. 28(2):500-511.
Sandrio S, Rüffer A, Purbojo A, Glöckler M, Dittrich S, Cesnjevar R. Common arterial trunk: current implementation of the primary and staged repair strategies. Interact Cardiovasc Thorac Surg. 2015 Dec;21(6):754-60.
Huff C, Mastropietro CW, Riley C, Byrnes J, Kwiatkowski DM, Ellis M, Schuette J, Justice L. Comprehensive Management Considerations of Select Noncardiac Organ Systems in the Cardiac Intensive Care Unit. World J Pediatr Congenit Heart Surg. 2018 Nov;9(6):685-695.
Gotsch F, Romero R, Espinoza J, Kusanovic JP, Erez O, Hassan S, Yeo L. Prenatal diagnosis of truncus arteriosus using multiplanar display in 4D ultrasonography. J Matern Fetal Neonatal Med. 2010 Apr;23(4):297-307.
Kalavrouziotis G, Purohit M, Ciotti G, Corno AF, Pozzi M. Truncus arteriosus communis: early and midterm results of early primary repair. Ann Thorac Surg. 2006 Dec;82(6):2200-6.
Asagai S, Inai K, Shinohara T, Tomimatsu H, Ishii T, Sugiyama H, Park IS, Nagashima M, Nakanishi T. Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years. Congenit Heart Dis. 2016 Dec;11(6):672-677.
Alfieris GM, Swartz MF. The Initial Glimpse at Long-term Outcomes Following the Repair of Truncus Arteriosus. Semin Thorac Cardiovasc Surg. 2016 Summer;28(2):512-513.
Graziani F, Delogu AB. Evaluation of Adults With Congenital Heart Disease. World J Pediatr Congenit Heart Surg. 2016 Mar;7(2):185-91.
Williams JM, de Leeuw M, Black MD, Freedom RM, Williams WG, McCrindle BW. Factors associated with outcomes of persistent truncus arteriosus. J Am Coll Cardiol. 1999 Aug;34(2):545-53.
Gómez O, Soveral I, Bennasar M, Crispi F, Masoller N, Marimon E, Bartrons J, Gratacós E, Martinez JM. Accuracy of Fetal Echocardiography in the Differential Diagnosis between Truncus Arteriosus and Pulmonary Atresia with Ventricular Septal Defect. Fetal Diagn Ther. 2016;39(2):90-9.
Balachandran R, Nair SG, Kumar RK. Establishing a pediatric cardiac intensive care unit - Special considerations in a limited resources environment. Ann Pediatr Cardiol. 2010 Jan;3(1):40-9.
Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 02;73(12):1494-1563.
Kodo K, Nishizawa T, Furutani M, et al. GATA6 mutations cause human cardiac outflow tract defects by disrupting semaphorin-plexin signaling. Proc Natl Acad Sci U S A. 2009 Aug 18. 106(33):13933-8.
Lisowski LA, Verheijen PM, Copel JA, et al. Congenital heart disease in pregnancies complicated by maternal diabetes mellitus. An international clinical collaboration, literature review, and metaanalysis. Herz. 2010 Jan. 35(1):19-26.
Chaoui R, Bollmann R, Zienert A, et al. [Prenatal diagnosis of truncus arteriosus communis (type I) in diabetic pregnancy]. Zentralbl Gynakol. 1992. 114(4):198-200.
Egbe A, Uppu S, Lee S, Ho D, Srivastava S. Changing prevalence of severe congenital heart disease: a population-based study. Pediatr Cardiol. 2014 Oct. 35(7):1232-8.
Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014 Aug 26. 130(9):749-56.
Swanson TM, Selamet Tierney ES, Tworetzky W, Pigula F, McElhinney DB. Truncus arteriosus: diagnostic accuracy, outcomes, and impact of prenatal diagnosis. Pediatr Cardiol. 2009 Apr. 30(3):256-61.
Turan S, Turan OM, Desai A, Harman CR, Baschat AA. First-trimester fetal cardiac examination using spatiotemporal image correlation, tomographic ultrasound and color Doppler imaging for the diagnosis of complex congenital heart disease in high-risk patients. Ultrasound Obstet Gynecol. 2014 Nov. 44(5):562-7.
Rao PS. Diagnosis and management of cyanotic congenital heart disease: part I. Indian J Pediatr. 2009 Jan. 76(1):57-70.
Saxena A. Consensus on timing of intervention for common congenital heart disease. Indian Pediatr. 2008 Feb. 45(2):117-26.
Lund AM, Vogel M, Marshall AC, et al. Early reintervention on the pulmonary arteries and right ventricular outflow tract after neonatal or early infant repair of truncus arteriosus using homograft conduits. Am J Cardiol. 2011 Jul 1. 108(1):106-13.
Anderson RH, Thiene G. Categorization and description of hearts with a common arterial trunk. Eur J Cardiothorac Surg. 1989. 3(6):481-7.
Gerlis LM, Ho SY, Smith A, et al. The site of origin of nonconfluent pulmonary arteries from a common arterial trunk or from the ascending aorta: its morphological significance. Am J Cardiovasc Pathol. 1990. 3(2):115-20.
Hanley FL, Heinemann MK, Jonas RA, et al. Repair of truncus arteriosus in the neonate. J Thorac Cardiovasc Surg. 1993 Jun. 105(6):1047-56.
Heathcote K, Braybrook C, Abushaban L, et al. Common arterial trunk associated with a homeodomain mutation of NKX2.6. Hum Mol Genet. 2005 Mar 1. 14(5):585-93.
Heinemann MK, Hanley FL, Fenton KN, et al. Fate of small homograft conduits after early repair of truncus arteriosus. Ann Thorac Surg. 1993 Jun. 55(6):1409-11.
Imamura M, Drummond-Webb JJ, Sarris GE, et al. Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair. Ann Thorac Surg. 1999 Apr. 67(4):1142-6.
Jahangiri M, Zurakowski D, Mayer JE, et al. Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus. J Thorac Cardiovasc Surg. 2000 Mar. 119(3):508-14.
Kaartinen V, Dudas M, Nagy A, et al. Cardiac outflow tract defects in mice lacking ALK2 in neural crest cells. Development. 2004 Jul. 131(14):3481-90.
Lacour-Gayet F, Serraf A, Komiya T, et al. Truncus arteriosus repair: influence of techniques of right ventricular outflow tract reconstruction. J Thorac Cardiovasc Surg. 1996 Apr. 111(4):849-56.
Litovsky SH, Ostfeld I, Bjornstad PG, et al. Truncus arteriosus with anomalous pulmonary venous connection. Am J Cardiol. 1999 Mar 1. 83(5):801-4, A10.
Mavroudis C, Backer CL. Surgical management of severe truncal insufficiency: experience with truncal valve remodeling techniques. Ann Thorac Surg. 2001 Aug. 72(2):396-400.
McElhinney DB, Driscoll DA, Emanuel BS, Goldmuntz E. Chromosome 22q11 deletion in patients with truncus arteriosus. Pediatr Cardiol. 2003 Nov-Dec. 24(6):569-73.