A case of an uncorrected double outlet right ventricle with ventricular septal defect in pregnancy


  • Kedar M. Tilak Department of Pediatrics, PGI-YCM Hospital, Pune, Maharashtra, India
  • Uma N. Wankhede Department of Obstetrics and Gynaecology, Sassoon General Hospitals, Pune, Maharashtra, India




DORV, Heart disease, Pregnancy, VSD


Congenital Heart Diseases (CHD) lead to various changes in the normal mechanisms of hemodynamics. Pregnancy in women with CHDs is rare. Double Outlet Right Ventricle (DORV) is a rare disorder, in which both the aorta and the pulmonary artery arise from the right ventricle. We present a case of a thirty-year-old pregnant woman who presented to us with 22 weeks of amenorrhea. She had DORV with VSD, which was undiagnosed till she came to our hospital. Double Outlet Right Ventricle (DORV) is a rare congenital heart disease. Pregnancy in a patient with DORV needs early diagnosis and a stepwise multidisciplinary approach for successful outcomes.


Fesslova VM, Villa L, Chessa M. Prospective evaluation from single centre of pregnancy in women with congenital heart disease. Int J Cardiol. 2009;131:257-64.

Sawhney H, Suri V, Vasishta K. Pregnancy and congenital heart disease-maternal and fetal outcome. Aust NZ J Obstet Gynaecol. 1998;38(3):266.

Stout K. Pregnancy in women with congenital heart disease: the importance of evaluation and counseling. Heart. 2005;91(6):713-4.

McFaul PB, Dornan JC, Lamki H, Boyle D. Pregnancy complicated by maternal heart disease. A review of 519 women. Br J Obstet Gynaecol. 1988;95(9):861-7.

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(2):271-81.

Pradat P, Francannet C, Harris JA, Robert E. The epidemiology of cardiovascular defects, part I: a study based on data from three large registries of congenital malformations. Pediatr Cardiol. 2003;24(3):195-221.

Walters HL, Mavroudis C, Tchervenkov CI, Jacobs JP, Lacour-Gayet F, Jacobs ML. Congenital heart surgery nomenclature and database project: double-outlet right ventricle. Ann Thorac Surg. 2000;69(4 Suppl):S249-63.

Gu J, Cai Y, Liu B, Lv S. Anesthetic management for cesarean section in a patient with uncorrected double-outlet right ventricle. Springerplus. 2016;5:415.

Pang KJ, Meng H, Hu SS. Echocardiographic classification and surgical approaches to double-outlet right ventricle for great arteries arising almost exclusively from the right ventricle. Tex Heart Inst J. 2017;44(4):245-51.

Madhavi, Panchbudhe S, Mali K, Satia MN. Uncorrected double outlet right ventricle with Fallot’s physiology in pregnancy. JPGO. 2015;2:6.

Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F. Pregnancy in cyanotic congenital heart disease: outcome of mother and fetus. Circulation. 1994;89:2673-6.

Cardiovascular Disorders. In F. Gary Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL, Casey BM, Sheffield JS, editors. Williams Obstetrics. 24th ed. New York: McGraw-Hill Education; 2014:973-999.

Raisky O, Vouhé PR. Pitfalls in repair of conotruncal anomalies. Thoracic Cardiovascular Surgery Pediatric Cardiology Surgery Annual. 2013;16:7-12.






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