• Aso F. Salih Department of Pediatrics, College of Medicine, University of Sulaimani, Kurdistan Region, Iraq.
  • Diyar H. Waly Sulaimani Pediatric Teaching Hospital, Sulaimaniyah, Kurdistan Region, Iraq.
  • Adnan Mohammed Hasan Department of Pediatrics, College of Medicine, University of Sulaimani, Kurdistan Region, Iraq.



Tricuspid atresia, Echocardiography, Single ventricle



A “single ventricle” or “univentricular heart” was characterized by lacking two well-developed ventricles, which thereby excluded hearts with nonseptable but well-formed ventricles. The proposed definition of univentricular heart encompassed double-inlet atrioventricular connection, absence of atrioventricular connection (mitral or tricuspid atresia), double-outlet right ventricle, common atrioventricular valve with only one well-developed ventricle, and heterotaxy syndrome (single ventricle heterotaxy syndrome).


To determine the echocardiographic morphological classification of patients with univentricular heart disease and the subclassification of each type in Sulaimani PediatricTeaching Hospital-Cardiology Unit–Kurdistan-Iraq

Patients and Methods

A retrospective study conducted in pediatric teaching hospital in Suaimani city from September 2009 to January 2014. All children with the confirmed diagnosis of single ventricle were included in the study, hypoplastic left heart syndrome have been excluded from the study. The source of information was the medical and echocardiography records from cardiology unit of the pediatric teaching hospital in Sulaimani by the same pediatric cardiologist as an operator.


Among 100 cases, 73 cases had left ventricle morphology, 24 cases had right ventricle morphology, and 3 cases of indeterminate type. 50 cases were tricuspid atresia, 22 cases were double inlet left ventricle, 17 cases were double outlet right ventricle, 10 cases were common atrioventricular valve, and one case was double inlet right ventricle. Type (I) tricuspid atersia was the most common subtype of tricuspid atresia  (80%). Transposition of great arteries was the most common type of double inlet left ventricle (95%). Tetrallogy of Fallot was the most common subtype of double outlet right ventricle (59%). Ventricular septal defect was the most common associated anomaly, followed by atrial septal defect. 


Tricuspid atresia is the most common type of univentricular heart (with exclusion of hypoplastic left heart synsdrome). Tricuspid atersia with normally related great arteries is the most common subtype of tricuspid atersia. Right isomerism is more common in male patients, and right isomerism is more common than left isomerism. Ventricular septal defect & atrial septal defect are the most common associated anomalies. There is a possibility of a genetic role in patients with tricuspid atersia that increases its incidence in other siblings.


Tchervenkov CI, Jacobs ML, Tahta SA. Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome. Ann Thorac Surg. 2000;69(4 Suppl):S170 –S179. DOI:

Khairy P, Poirier N and Mercier L.A. Univentricular Heart. Circulation AHA 2007;115:800-812. DOI:

Van Praagh R, Van Praagh S, Vlad P, D.Keith J. . Diagnosis of the anatomic types of single or common ventricle. Am J Cardiol 1965; 15: 345-366. DOI:

Anderson RH, Becker AE, Wilkinson JL. Proceedings: morphogenesis and nomenclature of univentricular hearts. Br Heart J 1975;37:781–782.

Larsen WJ. Development of the heart. In: Human embryology. New York: Churchill Livingstone, 1993: 131-65.

Visser D.R. Outcome, Hemodynamic and Genetic Assessment in Patients with Functionally Univentricular Hearts after the Fontan Operation at Young Age.

PhD thesis Erasmus University Rotterdam, Faculty of Medicine, the Netherlands, NARCIS 2012;1:10-11.

Fyler DC, Buckley LP, Hellenbrand WE,et al. Report of the New

England Regional Infant Cardiac Program. Pediatrics. 1980;65:375– 461.

Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890 –1900. DOI:

Rao PS. Tricuspid atresia. Curr Treat Options Cardiovasc Med. 2000;2: 507–520 DOI:

Van Praagh R, Ando M, Dungan WT. Anatomic types of tricuspid atresia: clinical and developmental implications [abstract]. Circulation. 1971;44:115

Van Praagh R. Discussion after paper by Vlad P: Pulmonary atresia with intact ventricular septum. In: Barrett-Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy: Diagnosis and Surgical Treatment. 2nd ed. London: Churchill Livingstone; 1992:1973:236.

Astley R, Oldham JS, Parsons C. Congenital tricuspid atresia. Br Heart J. Jul 1953;15(3):287-97. DOI:

Dick M, Fyler DC, Nadas AS. Tricuspid atresia: clinical course in 101 patients. Am J Cardiol. Sep 1975;36(3):327-37. DOI:

Edwards JE, Burchell HB. Congenital tricuspid atresia: a classification. Med Clin North Am. 1949;33:1117. DOI:

Keith J, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. In: Tricuspid Atresia. 434. 2nd ed. New York: Macmillian; 1967:664.

Vlad P. Tricuspid atresia. In: Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infancy and Childhood. 3rd ed. New York: Macmillian; 1977:518-41.

Rao PS. A unified classification for tricuspid atresia. Am Heart J. 1980;99(6):799-804. DOI:

Kuhne M. Uber zwei falle kongenitaler atresie des ostium venosum dextrum. Jahrb F Kinderh 1906;63:235-249.( transilated)

Tandon R, Edwards JE. Tricuspid atresia: a re-evaluation and classification. J Thorac Cardiovasc Surg 1974;67:530-542. DOI:

Franklin RC, Spiegelhalter DJ, Anderson RH, Macartney FJ, Rossi Filho RI, Douglas JM, Rigby ML, Deanfield JE. Double-inlet ventricle presenting in infancy: I: Survival without definitive repair. J Thorac Cardiovasc Surg. 1991;101:767–776. DOI:

Van Praagh R, Ongley PA, Swan HJ. Anatomic types of single or common ventricle in man: morphologic and geometric aspects of 60 necropsied cases. Am J Cardiol 1964;13:367–386. DOI:

Ferencz C, Rubin JD, McCarter RJ, Brenner JI, Neill CA, Perry LW, Hepner SI, Downing JW. Congenital heart disease: prevalence at livebirth. The baltimore-washington infant study. Am J Epidemiol 1985;121:31-36. DOI:

Earing MG, Hagler DJ, Edwards WD. Univentricular atrioventricular connection. In: Allen HD, Driscoll DJ, Shaddy RE, eds. Moss and Adams’ Heart disease in infants, children, and adolescents including the fetus and young adult. 8th ed. 2013. By Lippincott Williams and Wilkins, pages 1175-1194.

Wright GE, Maeda K, Silverman NH. Double outlet right ventricle. In: Allen HD, Driscoll DJ, Shaddy RE, eds. Moss and Adam’s Heart disease in infants, children, and adults including the fetus and young adult.8th ed. 2013. By Lippincott Williams and Wilkins, pages 1161-1173.

Walters HL III, Mavroudis C, Tchervenkov CI , et al. Congenital heart surgery nomenclature and database project: double outlet right ventricle. Ann Thorac Surg 2000;69:S249-S263. DOI:

Ueda M, Becker AE. Classification of hearts with overriding aortic and pulmonary valves. Int J Cardiol 1985;9:357-369. DOI:

Guller B. and Titus J.L . Morphological Studies in Tricuspid Atresia. American heart association Circulation .1968;38:977-986. DOI:

Epstein M.L. Tricuspid atresia, stenosis, regurgitation and Uhl’s anomaly. in: Allen H.D, Driscoll D.J, Shaddy R.E. eds. Moss and Adam’s Heart disease in infants, children, and adults including the fetus and young adult. 8th ed. 2013. By Lippincott Williams and Wilkins, pages 877-887.

Shiraishi H, Silverman NH, Echocardiographic spectrum of double inlet ventricle: evaluation of the interventricular communication. J Am Coll Cardiol 1990;15:1401. DOI:

Bevilacqua M, Sanders SP, Van Praagh S, Colan SD, Parness I. double inlet single ventricle: echocardiographic anatomy with emphasis on morphology of the AV valves VSD. J Am Coll Cardiol 1991;18:559-568. DOI:

Lev M, Bharati S, Meng CC, Liberthson RR, Paul MH, Idriss F. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg 1972;64:271-281. DOI:

Sridaromont S, Feldt RH, Ritter DG, Davis GD, Edwards JE. double-outlet right ventricle. Anatomic and angiocardiographic correlations. Mayo Clin Proc. 1978;53:555-577.

Van Praagh R, Weinberg P, Smith S. Malpositions of the heart. In: Adams F, Emmanouilides G, Riemenschnieder T, eds. Moss’ Heart Disease in Infants, Children and Adolescents. 4th ed. Baltimore, MD: Williams& Wilkins, 1989:530.

Behera M, Mishra BR. Clinical approach to diagnosis of cardiac malpositions. In: Satpathy M. eds. Clinical diagnosis of congenital heart disease. 1st ed. Jitendar P Vij , 2008:47. DOI:

Rahimtoola S.H , Ongley P.A. and Swan H J. The Hemodynamics of Common (or Single) Ventricle. Circulation AHA 1966;34:14-23. DOI:

Sarkozy A, Conti E, D’Agostino R, et al. ZFPM2/FOG2 and HEY2 genes analysis in nonsyndromic tricuspid atresia. Am J Med Genet 2005:113A:68-70. DOI:



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