Subscribe to RSS
![](/products/assets/desktop/img/oa-logo.png)
DOI: 10.1055/s-0031-1293515
Prenatal Diagnosis and Outcome of Fetuses with Double-Inlet Left Ventricle
Publication History
Publication Date:
07 November 2011 (online)
![](https://www.thieme-connect.de/media/ajpr/201102/lookinside/thumbnails/10.1055-s-0031-1293515-1.jpg)
Abstract
The aim of this study is to characterize the in utero presentation of the subtype of double-inlet left ventricle (DILV), a rare congenital heart disease, and assess the postnatal outcome. We retrospectively studied fetuses diagnosed prenatally with DILV between 2007 and 2011. We reviewed the prenatal and postnatal echocardiograms, clinical presentations, karyotypes, and the postnatal outcomes. There were eight fetuses diagnosed with DILV with L-transposition of the great vessels (S, L, L). Mean gestational age at diagnosis was 24.7 weeks. Of these, four fetuses (50%) had pulmonary atresia. One fetus (12.5%) also had tricuspid atresia and coarctation of the aorta and died at 17 months of age. Complete heart block and long QT syndrome was present in one fetus (12.5%), who died shortly after birth. There were no extracardiac or karyotypic abnormalities. Six (75%) infants are alive and doing well. Double-inlet left ventricle with varied presentation can be accurately diagnosed prenatally. The outcome of fetuses is good in the absence of associated rhythm abnormalities with surgically staged procedures leading to a Fontan circulation.
-
References
- 1 Allen LD, Hornberger LK, Sharland GK. Double-inlet left ventricle in the fetus. In: Textbook of Fetal Cardiology. London, United Kingdom: Greenwich Medical Media; 2000: 174-182
- 2 Rosenberg HS, Donnelly WH. The cardiovascular system. In: Wigglesworth JS, Singer DB, eds. Textbook of Fetal and Perinatal Pathology. Cambridge, MA: Blackwell Scientific Publications; 1991: 716-
- 3 Cook AC, Anderson RH. The functionally univentricular circulation: anatomic substrates as related to function. Cardiol Young 2005; 15 (Suppl. 03) 7-16
- 4 Uemura H, Ho SY, Adachi I, Yagihara T. Morphologic spectrum of ventriculoarterial connection in hearts with double inlet left ventricle: implications for surgical procedures. Ann Thorac Surg 2008; 86: 1321-1327
- 5 Saleeb SF, Juraszek A, Geva T. Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. Am J Cardiol 2010; 105: 542-549
- 6 Hornberger LK, Sahn DJ, Kleinman CS, Copel J, Silverman NH. Antenatal diagnosis of coarctation of the aorta: a multicenter experience. J Am Coll Cardiol 1994; 23: 417-423
- 7 Srinivasan S, Strasburger J. Overview of fetal arrhythmias. Curr Opin Pediatr 2008; 20: 522-531
- 8 Peters M, Crowe J, Piéri JF , et al. Monitoring the fetal heart non-invasively: a review of methods. J Perinat Med 2001; 29: 408-416
- 9 Hamada H, Horigome H, Asaka M , et al. Prenatal diagnosis of long QT syndrome using fetal magnetocardiography. Prenat Diagn 1999; 19: 677-680
- 10 Menéndez T, Achenbach S, Beinder E , et al. Prenatal diagnosis of QT prolongation by magnetocardiography. Pacing Clin Electrophysiol 2000; 23: 1305-1307
- 11 Lin MT, Hsieh FJ, Shyu MK, Lee CN, Wang JK, Wu MH. Postnatal outcome of fetal bradycardia without significant cardiac abnormalities. Am Heart J 2004; 147: 540-544
- 12 Tei C, Ling LH, Hodge DO , et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function—a study in normals and dilated cardiomyopathy. J Cardiol 1995; 26: 357-366
- 13 Tei C, Dujardin KS, Hodge DO, Kyle RA, Tajik AJ, Seward JB. Doppler index combining systolic and diastolic myocardial performance: clinical value in cardiac amyloidosis. J Am Coll Cardiol 1996; 28: 658-664
- 14 Anderson PAW, Sleeper LA, Mahony L , et al; Pediatric Heart Network Investigators. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol 2008; 52: 85-98
- 15 Clarke AJB, Kasahara S, Andrews DR , et al. Mid-term results for double inlet left ventricle and similar morphologies: timing of Damus-Kaye-Stansel. Ann Thorac Surg 2004; 78: 650-657 ; discussion 657
- 16 Earing MG, Cetta F, Driscoll DJ , et al. Long-term results of the Fontan operation for double-inlet left ventricle. Am J Cardiol 2005; 96: 291-298
- 17 Tham EBC, Wald R, McElhinney DB , et al. Outcome of fetuses and infants with double inlet single left ventricle. Am J Cardiol 2008; 101: 1652-1656