- Brian Soriano, MD
Brian Soriano, MD
- Associate Professor of Pediatrics & Adjunct Associate Professor of Radiology
- Heart Center, Seattle Children’s Hospital
- University of Washington School of Medicine
- David R Fulton, MD
David R Fulton, MD
- Section Editor — Pediatric Cardiology
- Associate Professor of Pediatrics
- Harvard Medical School
Truncus arteriosus (TA), also known as common arterial trunk, is a cyanotic congenital heart defect. In this condition, blood is pumped from the heart through a single truncal valve into a truncal artery, which gives rise to the aorta and the pulmonary arteries (figure 1).
The anatomy, clinical manifestations, diagnosis, and management of TA will be reviewed here.
The reported incidence of TA ranges from 6 to 10 per 100,000 live births [1,2]. Although only 0.7 percent of all congenital heart lesions are due to TA, it accounts for 4 percent of all critical congenital heart disease cases .
During the early stages of a normally developing heart, the great arteries originate from a single truncal root. A wall forms within this root (truncoconal septum) that divides the root into a fully developed main pulmonary artery and ascending aorta by the end of the fifth week of gestation. Over the next two weeks of development, there is closure of the conal (infundibular) septum as the truncoconal septum fuses with the endocardial cushions and the interventricular septum, which separates the newly formed right and left ventricle.
Disturbances in the development of the truncoconal septum result in conotruncal abnormalities including TA. The lack of wall development also impairs the creation of separate aortic and pulmonary valves, resulting in the single truncal valve associated with TA .
- Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008; 153:807.
- Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890.
- Pexieder T. Prenatal development of the endocardium: a review. Scan Electron Microsc 1981; :223.
- Scholl AM, Kirby ML. Signals controlling neural crest contributions to the heart. Wiley Interdiscip Rev Syst Biol Med 2009; 1:220.
- Kirby ML, Gale TF, Stewart DE. Neural crest cells contribute to normal aorticopulmonary septation. Science 1983; 220:1059.
- Fuglestad SJ, Puga FJ, Danielson GK, Edwards WD. Surgical pathology of the truncal valve: a study of 12 cases. Am J Cardiovasc Pathol 1988; 2:39.
- Konstantinov IE, Karamlou T, Blackstone EH, et al. Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study. Ann Thorac Surg 2006; 81:214.
- Butto F, Lucas RV Jr, Edwards JE. Persistent truncus arteriosus: pathologic anatomy in 54 cases. Pediatr Cardiol 1986; 7:95.
- Marcelletti C, McGoon DC, Danielson GK, et al. Early and late results of surgical repair of truncus arteriosus. Circulation 1977; 55:636.
- Nath PH, Zollikofer C, Castaneda-Zuniga W, et al. Persistent truncus arteriosis associated with interruption of the aortic arch. Br J Radiol 1980; 53:853.
- Mair DD, Ritter DG, Davis GD, et al. Selection of patients with truncus arteriosus for surgical correction; anatomic and hemodynamic considerations. Circulation 1974; 49:144.
- de la Cruz MV, Cayre R, Angelini P, et al. Coronary arteries in truncus arteriosus. Am J Cardiol 1990; 66:1482.
- Bharati S, Karp R, Lev M. The conduction system in truncus arteriosus and its surgical significance. A study of five cases. J Thorac Cardiovasc Surg 1992; 104:954.
- Bharati S, McAllister HA Jr, Rosenquist GC, et al. The surgical anatomy of truncus arteriosus communis. J Thorac Cardiovasc Surg 1974; 67:501.
- Jacobs ML. Congenital Heart Surgery Nomenclature and Database Project: truncus arteriosus. Ann Thorac Surg 2000; 69:S50.
- COLLETT RW, EDWARDS JE. Persistent truncus arteriosus; a classification according to anatomic types. Surg Clin North Am 1949; 29:1245.
- Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol 1965; 16:406.
- Kirby ML. Pulmonary atresia or persistent truncus arteriosus: is it important to make the distinction and how do we do it? Circ Res 2008; 103:337.
- Alverson CJ, Strickland MJ, Gilboa SM, Correa A. Maternal smoking and congenital heart defects in the Baltimore-Washington Infant Study. Pediatrics 2011; 127:e647.
- Long J, Ramadhani T, Mitchell LE. Epidemiology of nonsyndromic conotruncal heart defects in Texas, 1999-2004. Birth Defects Res A Clin Mol Teratol 2010; 88:971.
- Momma K. Cardiovascular anomalies associated with chromosome 22q11.2 deletion syndrome. Am J Cardiol 2010; 105:1617.
- Galindo A, Mendoza A, Arbues J, et al. Conotruncal anomalies in fetal life: accuracy of diagnosis, associated defects and outcome. Eur J Obstet Gynecol Reprod Biol 2009; 146:55.
- Ziolkowska L, Kawalec W, Turska-Kmiec A, et al. Chromosome 22q11.2 microdeletion in children with conotruncal heart defects: frequency, associated cardiovascular anomalies, and outcome following cardiac surgery. Eur J Pediatr 2008; 167:1135.
- McElhinney DB, Driscoll DA, Emanuel BS, Goldmuntz E. Chromosome 22q11 deletion in patients with truncus arteriosus. Pediatr Cardiol 2003; 24:569.
- Botto LD, May K, Fernhoff PM, et al. A population-based study of the 22q11.2 deletion: phenotype, incidence, and contribution to major birth defects in the population. Pediatrics 2003; 112:101.
- Swanson TM, Selamet Tierney ES, Tworetzky W, et al. Truncus arteriosus: diagnostic accuracy, outcomes, and impact of prenatal diagnosis. Pediatr Cardiol 2009; 30:256.
- Friedberg MK, Silverman NH, Moon-Grady AJ, et al. Prenatal detection of congenital heart disease. J Pediatr 2009; 155:26.
- Porter CJ, Edwards WD. Atrial septal defects. In: Moss and Adams' Heart Disease in Infants, Children and Adolescents, 7th ed, Allen HD, Driscoll DJ, Shaddy RE, Feltes TF (Eds), Lippincott, Williams and Wilkins, Philadelphia 2008. Vol 1, p.632.
- Marcelletti C, McGoon DC, Mair DD. The natural history of truncus arteriosus. Circulation 1976; 54:108.
- Niwa K, Perloff JK, Kaplan S, et al. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 1999; 34:223.
- Duke C, Sharland GK, Jones AM, Simpson JM. Echocardiographic features and outcome of truncus arteriosus diagnosed during fetal life. Am J Cardiol 2001; 88:1379.
- Volpe P, Paladini D, Marasini M, et al. Common arterial trunk in the fetus: characteristics, associations, and outcome in a multicentre series of 23 cases. Heart 2003; 89:1437.
- Thompson LD, McElhinney DB, Reddy M, et al. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg 2001; 72:391.
- Bove EL, Lupinetti FM, Pridjian AK, et al. Results of a policy of primary repair of truncus arteriosus in the neonate. J Thorac Cardiovasc Surg 1993; 105:1057.
- Rodefeld MD, Hanley FL. Neonatal truncus arteriosus repair: surgical techniques and clinical management. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5:212.
- Williams JM, de Leeuw M, Black MD, et al. Factors associated with outcomes of persistent truncus arteriosus. J Am Coll Cardiol 1999; 34:545.
- Russell HM, Pasquali SK, Jacobs JP, et al. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg 2012; 93:164.
- Kaza AK, Burch PT, Pinto N, et al. Durability of truncal valve repair. Ann Thorac Surg 2010; 90:1307.
- 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; 119:508.
- Rajasinghe HA, McElhinney DB, Reddy VM, et al. Long-term follow-up of truncus arteriosus repaired in infancy: a twenty-year experience. J Thorac Cardiovasc Surg 1997; 113:869.
- Tlaskal T, Chaloupecky V, Hucin B, et al. Long-term results after correction of persistent truncus arteriosus in 83 patients. Eur J Cardiothorac Surg 2010; 37:1278.
- O'Byrne ML, Mercer-Rosa L, Zhao H, et al. Morbidity in children and adolescents after surgical correction of truncus arteriosus communis. Am Heart J 2013; 166:512.
- Balady GJ, Arena R, Sietsema K, et al. Clinician's Guide to cardiopulmonary exercise testing in adults: a scientific statement from the American Heart Association. Circulation 2010; 122:191.
- Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012; 126:1143.
- Truncal valve
- Aortic arch anomalies
- Ventricular septal defect
- Coronary artery and conduction system anomalies
- - Other cardiac anomalies
- Collet and Edwards classification
- Van Praagh classification
- STS modification
- CLINICAL MANIFESTATIONS
- Antenatal presentation
- Postnatal presentation
- - Other noncardiac congenital malformations
- Natural history
- - Electrocardiogram
- - Chest radiography
- Fetal diagnosis
- Postnatal diagnosis
- - Echocardiography
- DIFFERENTIAL DIAGNOSIS
- Initial medical management
- Surgical correction
- - Primary repair procedure
- FOLLOW-UP CARE
- Physical examination
- Diagnostic testing
- Endocarditis prophylaxis
- Neurodevelopmental outcome
- SUMMARY AND RECOMMENDATIONS