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DOI: 10.1055/s-0028-1083840
Diagnosis and Prognosis in Double-Outlet Right Ventricle
Publication History
Publication Date:
21 August 2008 (online)
ABSTRACT
The aim of this study was to examine prenatal diagnosis of double-outlet right ventricle (DORV)-associated anomalies and prognosis of each case. Medical records were reviewed of fetuses with DORV who had fetal echocardiography at our institution from 2002 to 2006. Pre- and postnatal diagnosis and outcome were compared and evaluated. Twenty-one fetuses were diagnosed with DORV. The pregnancy was terminated in seven cases. Three cases had chromosomal abnormalities; three cases, hypoplastic left ventricle; and one case, encephalocele. Accurate prenatal diagnosis of the ventricular septal defect, outflow obstruction, and great artery relationship was achieved in 14 of 16 cases (87.5%). Only 2 of 13 live-born cases survived beyond 6 months. The overall prognosis for fetuses with DORV is poor. DORV is found in fetuses with a huge spectrum of associated cardiac and extracardiac anomalies. Careful assessment by fetal echocardiography can determine important anatomic details with adequate correctness for precise counseling.
KEYWORDS
Fetal echocardiography - double-outlet right ventricle - prenatal diagnosis - prognosis
REFERENCES
-
1 Graham T P, Gutgesell H P.
Conotruncal abnormalities . In: Long WA Fetal and Neonatal Cardiology. Philadelphia; WB Saunders 1990: 561-570 -
2 Keane J F, Fyler D C.
Double-outlet right ventricle . In: Kean JF, Fyler DC, Lock J Nadas' Pediatric Cardiology. 2nd ed. Philadelphia, PA; Elsevier 2006 - 3 Hoffman J I, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002; 39 1890-1900
- 4 Kim N, Friedberg M K, Silverman N H. Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenat Diagn. 2006; 26 740-745
- 5 Wladimiroff J W, Stewart P A, Reuss A, Sachs E S. Cardiac and extracardiac anomalies as indicators for trisomy 13 and 18: a prenatal study. Prenat Diagn. 1989; 9 515-520
- 6 Goldmuntz E, Clark B J, Mitchell L E et al.. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. 1998; 32 492-498
-
7 Drose J A. Double outlet right ventricle and double outlet left ventricle.
In: Fetal Echocardiography . Philadelphia; WB Saunders 1998 - 8 Kim N, Friedberg M K, Silverman N H. Diagnosis and prognosis with double outlet right ventricle. Prenat Diagn. 2006; 26 740-745
- 9 Allan L D, Sharland G K, Milburn A et al.. Prospective diagnosis of 1006 consecutive cases of congenital heart disease in the fetus. J Am Coll Cardiol. 1994; 23 1452-1458
- 10 Friedberg M K, Silverman N H. Changing indications for fetal echocardiography in a university center population. Prenat Diagn. 2004; 24 781-786
- 11 Gelehrter S, Owens S T, Russell M W, van der Velde M E, Gomez-Fifer C. Accuracy of the fetal echocardiogram in double-outlet right ventricle. Congenit Heart Dis. 2007; 2 32-37
- 12 Brick D H, Allan L D. Outcome of prenatally diagnosed congenial heart disease. An update. Pediatr Cardiol. 2002; 23 449-453
- 13 Paladini D, Russo M G, Teodoro A et al.. Prenatal diagnosis of congenital heart disease in the Naples area during the years 1994–1999—the experience of a joint fetal-pediatric cardiology unit. Prenat Diagn. 2002; 22 545-552
- 14 Fesslova V, Nava S, Villa L. Fetal Cardiology Study Group of the Italian Society of Pediatric Cardiology . Evolution and long-term outcome in cases with fetal diagnosis of congenital heart disease: Italian multicentre study. Heart. 1999; 82 594-599
- 15 Smith R S, Comstock C H, Kirk J S, Lee W, Riggs T, Weinhouse E. Double-outlet right ventricle. An usual diagnostic dilemma. Ultrasound Obstet Gynecol. 1999; 14 315-319
- 16 Kleinert S, Sano T, Weintraub R G, Mee R BB, Karl T R, Wilkinson J L. Anatomic features and surgical strategies in double-outlet right ventricle. Circulation. 1997; 96 1233-1239
- 17 Brown J W, Ruzmetov M, Okada Y, Vijay P, Turrentine M W. Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg. 2001; 72 1630-1635
Ali GedikbasiM.D.
Atakoy 11, Kisim, Lale Apt. D.40
34710 Istanbul, Turkey
Email: alged_1971@yahoo.com