The syndrome of right isomerism - Prenatal diagnosis and outcome

Ultraschall in der Medizin

Volumn 27, Issue 3, 2006, Pages 225-233

  Berg, Christoph ^a   Geipel, A ^a   Kamil, D ^a   Krapp, M ^b   Breuer, J ^a   Baschat, A A ^c   Knopfle C ^a   Germer, U ^d   Hansmann, M ^a   Gembruch, U ^a  

^a UNIVERSITY OF BONN   (Germany)

^b UNIVERSITY HOSPITAL SCHLESWIG HOLSTEIN   (Germany)

^c UNIVERSITY OF HAMBURG   (Germany)

^d UNIVERSITY OF REGENSBURG   (Germany)

Author keywords

Asplenia; Cardiac defects; Echocardiography; Foetus; Heterotaxy; Isomerism; Ivemark syndrome; Juxtaposition; Prenatal diagnosis

Indexed keywords

ASPLENIA; CAVA VEIN DISEASE; CLINICAL ARTICLE; DIAGNOSTIC ACCURACY; ECHOGRAPHY; FETUS; GERMANY; HEART RIGHT VENTRICLE FAILURE; HEART SEPTUM DEFECT; HUMAN; INFERIOR CAVA VEIN; ISOMERISM; LUNG VEIN DRAINAGE ANOMALY; MITRAL VALVE REPLACEMENT; PRENATAL DIAGNOSIS; PROGNOSIS; REVIEW; RIGHT ISOMERISM SYNDROME; TACHYCARDIA;

ADOLESCENT; ADULT; AUTOPSY; ECHOCARDIOGRAPHY; FEMALE; FOLLOW-UP STUDIES; FUNCTIONAL LATERALITY; HEART DEFECTS, CONGENITAL; HUMANS; INFANT, NEWBORN; MATERNAL AGE; PREGNANCY; REPRODUCIBILITY OF RESULTS; RETROSPECTIVE STUDIES; ULTRASONOGRAPHY, PRENATAL;

EID: 33745698193     PISSN: 01724614     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-2005-858639     Document Type: Review

References (39)

1. Bowers PN, Brueckner M, Yost HJ. The genetics of left-right development and heterotaxia. Semin Perinatol 1996; 20: 577-588
2. Lin AE, Ticho BS, Houde K et al. Heterotaxy: associated conditions and hospital-based prevalence in newborns. Genet Med 2000; 2: 157-172
3. Van Praagh S, Kakou-Guikahue M, Hae-Seong K et al. Atrial situs in patients with visceral heterotaxy and congenital heart disease: conclusions based on findings in 104 postmortem cases. Coeur 1988; 19: 484-502
4. Winer-Muram HT, Tonkin IL. The spectrum of heterotaxic syndromes. Radiol Clin North Am 1989; 27: 1147-1170
5. Ivemark BL. Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood. Acta Paediatr Scand 1955; 44 (suppl 104): 1-116
6. Berg C, Geipel A, Smrcek J et al. Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience. Ultrasound Obstet Gynecol 2003; 22: 451-459
7. Huggon IC, Cook AC, Smeeton NC et al. Atrioventricular septal defects diagnosed in fetal life: associated cardiac and extra-cardiac abnormalities and outcome. J Am Coll Cardiol 2000; 36: 593-601
8. Phoon CK, Villegas MD, Ursell PC et al. Left atrial isomerism detected in fetal life. Am J Cardiol 1996; 77: 1083-1088
9. Atkinson DE, Drant S. Diagnosis of heterotaxy syndrome by fetal echocardiography. Am J Cardiol 1998; 82: 1147-1149, A10
10. Lin JH, Chang CI, Wang JK et al. Intrauterine diagnosis of heterotaxy syndrome. Am Heart J 2002; 143: 1002-1008
11. Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840-852
12. Hashmi A, Abu-Sulaiman R, McCrindle BW et al. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31: 1120-1126
13. Berg C, Geipel A, Kamil D et al. The syndrome of left isomerism - sonographic findings and outcome in prenatally diagnosed cases. J Ultrasound Med 2005; 24: 921-931
14. Okoye BO, Bailey DM, Cusick EL et al. Prophylactic gastropexy in the asplenia syndrome. Pediatr Surg Int 1997; 12: 28-29
15. Nakada K, Kawaguchi F, Wakisaka M et al. Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg 1997; 32: 91-94
16. Machado MV, Crawford DC, Anderson RH et al. Atrioventricular septal defect in prenatal life. Br Heart J 1988; 59: 352-355
17. Allan LD. Atrioventricular septal defect in the fetus. Am J Obstet Gynecol 1999; 181: 1250-1253
18. Patel CR, Lane JR, Muise KL. In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia. Ultrasound Obstet Gynecol 2001; 17: 268-271
19. Comstock CH, Smith R, Lee W et al. Right fetal cardiac axis: clinical significance and associated findings. Obstet Gynecol 1998; 91: 495-499
20. Colloridi V, Pizzuto F, Ventriglia F et al. Prenatal echocardiographic diagnosis of right atrial isomerism. Prenat Diagn 1994; 14: 299-302
21. Mauser I, Deutinger J, Bernaschek G. Prenatal diagnosis of a complex fetal cardiac malformation associated with asplenia. Br Heart J 1991; 65: 293-295
22. Boopathy Vijayaraghavan S, Rao AR, Padmashree G et al. Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism. Ultrasound Obstet Gynecol 2003; 21: 393-396
23. Wu MH, Wang JK, Lin JL et al. Supraventricular tachycardia in patients with right atrial isomerism. J Am Coll Cardiol 1998; 32: 773-779
24. Yasukochi S, Satomi G, Iwasaki Y. Prenatal diagnosis of total anomalous pulmonary venous connection with asplenia. Fetal Diagn Ther 1997; 12: 266-269
25. Huhta JC, Smallhorn JF, Macartney FJ. Two dimensional echocardiographic diagnosis of situs. Br Heart J 1982; 48: 97-108
26. Yagel S, Cohen SM, Achiron R. Examination of the fetal heart by five short-axis views: a proposed screening method for comprehensive cardiac evaluation. Ultrasound Obstet Gynecol 2001; 17: 367-369
27. Freedom RM, Harrington DP, White RI et al. The differential diagnosis of levo-transposed or malposed aorta. An angiocardiographic study. Circulation 1974; 50: 1040-1046
28. Ho SY, Cook A, Anderson RH et al. Isomerism of the atrial appendages in the fetus. Pediatr Pathol 1991; 11: 589-608
29. Berg C, Geipel A, Kohl T et al. Fetal echocardiographic evaluation of atrial morphology and the prediction of laterality in cases of heterotaxy syndromes. Ultrasound Obstet Gynecol 2005; 26: 538-545
30. Yoo SJ, Lee YH, Cho KS et al. Sequential segmental approach to fetal congenital heart disease. Cardiol Young 1999; 9: 430-444
31. Wu MH, Lin JL, Wang JK et al. Electrophysiological properties of dual atrioventricular nodes in patients with right atrial isomerism. Br Heart J 1995; 74: 553-555
32. Sadiq M, Stumper O, De Giovanni JV et al. Management and outcome of infants and children with right atrial isomerism. Heart 1996; 75: 314-319
33. Waldman JD, Rosenthal A, Smith AL et al. Sepsis and congenital asplenia. J Pediatr 1977; 90: 555-559
34. Cheung YF, Cheng VY, Chau AK et al. Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage? Heart 2002; 87: 146-152
35. Wu MH, Wang JK, Lue HC. Sudden death in patients with right isomerism (asplenism) after palliation. J Pediatr 2002; 140: 93-96
36. Noack F, Sayk F, Ressel A et al. Ivemark syndrome with agenesis of the corpus callosum: a case report with a review of the literature. Prenat Diagn 2002; 22: 1011-1015
37. Aylsworth AS. Clinical aspects of defects in the determination of laterality. Am J Med Genet 2001; 101: 345-355
38. Brown DL, Emerson DS, Shulman LP et al. Predicting aneuploidy in fetuses with cardiac anomalies: significance of visceral situs and non-cardiac anomalies. J Ultrasound Med 1993; 12: 153-161
39. Chaoui R, Körner H, Bommer C et al. Prenatal diagnosis of heart defects and associated chromosomal aberrations. Ultraschall Med 1999; 20: 177-184