Fetal Cardiac Malposition: Incidence and Outcome of Associated Cardiac and Extracardiac Malformations

 

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ABSTRACT

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed. The presence of congenital heart disease (CHD), extracardiac anomalies, and outcome were noted. Cardiac malposition was present in 101 fetuses among a total of 3313 (3%) pregnancies. In 78 (78%) patients, the heart was positioned in the right hemithorax. Of those, 26 (33%) had dextrocardia (CHD = 21), and 52 (67%) had dextroposition (CHD = 14). Sixteen (16%) patients had mesocardia (CHD = 8), and 7 (7%) had ectopia cordis (CHD = 6). The majority (58%) of fetal cardiac malposition was caused by intrathoracic masses. Concomitant CHD occurred in 50%. Outcome was available in 97%. The perinatal and neonatal mortality rate was 30%; the elective termination rate was 4%. Patients with CHD had higher mortality rates. The highest mortality rates occurred in ectopia cordis and combined disease of diaphragmatic hernia and CHD.

REFERENCES

• 1 Allan L D, Lockhart S. Intrathoracic cardiac position in the fetus.  Ultrasound Obstet Gynecol. 1993;  3 93-96
  CrossrefPubMedGoogle Scholar
• 2 Comstock C H. Normal fetal heart axis and position.  Obstet Gynecol. 1987;  70 255-259
  PubMedGoogle Scholar
• 3 Bernasconi A, Azancot A, Simpson J M, Jones A, Sharland G K. Fetal dextrocardia: diagnosis and outcome in two tertiary centers.  Heart. 2005;  91 1590-1594
  CrossrefPubMedGoogle Scholar
• 4 Walmsley R, Hishitani T, Sandor G G et al.. Diagnosis and outcome of dextrocardia diagnosed in the fetus.  Am J Cardiol. 2004;  94 141-143
  PubMedGoogle Scholar
• 5 Comstock C H, Smith R, Lee W, Kirk J S. Right fetal cardiac axis: clinical significance and associated findings.  Obstet Gynecol. 1998;  91 495-499
  CrossrefPubMedGoogle Scholar
• 6 Abdullah M M, Lacro R V, Smallhorn J et al.. Fetal cardiac dextroposition in the absence of an intrathoracic mass: sign of significant right lung hypoplasia.  J Ultrasound Med. 2000;  19 669-676
  PubMedGoogle Scholar
• 7 Crane J M, Ash K, Fink N, Desjardins C. Abnormal fetal cardiac axis in the detection of intrathoracic anomalies and congenital heart disease.  Ultrasound Obstet Gynecol. 1997;  10 90-93
  CrossrefPubMedGoogle Scholar
• 8 Fogel M, Copel J A, Cullen M T, Hobbins J C, Kleinman C S. Congenital heart disease and fetal thoracoabdominal anomalies: associations in utero and the importance of cytogenic analysis.  Am J Perinatol. 1991;  8 411-416
  Article in Thieme ConnectPubMedGoogle Scholar
• 9 Gibbin C, Touch S, Broth R E, Berghella V. Abdominal wall defects and congenital heart disease.  Ultrasound Obstet Gynecol. 2003;  21 334-337
  CrossrefPubMedGoogle Scholar
• 10 Garg N, Agarwal B L, Modi N. Dextrocardia: an analysis of cardiac structures in 125 patients.  Int J Cardiol. 2003;  88 143-155
  CrossrefPubMedGoogle Scholar
• 11 Tometzki A J, Suda K, Kohl T, Kovalchin J P, Silverman N H. Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies.  J Am Coll Cardiol. 1999;  33 1696-1701
  CrossrefPubMedGoogle Scholar
• 12 Boldt T, Andersson S, Eronen M. Outcome of structural heart disease diagnosed in utero.  Scand Cardiovasc J. 2002;  36 73-79
  CrossrefPubMedGoogle Scholar
• 13 Eronen M. Outcome of fetuses with heart disease diagnosed in utero.  Arch Dis Child Fetal Neonatal Ed. 1997;  77 F41-F46
  PubMedGoogle Scholar
• 14 Cannon C, Dildy G A, Ward R, Varner M W, Dudley D J. A population-based study of congenital diaphragmatic hernia in Utah: 1988-1994.  Obstet Gynecol. 1996;  87 959-963
  CrossrefPubMedGoogle Scholar
• 15 Gallot D, Boda C, Ughetto S et al.. Prenatal detection and outcome of congenital diaphragmatic hernia: a French registry-based study.  Ultrasound Obstet Gynecol. 2007;  29 276-283
  CrossrefPubMedGoogle Scholar
• 16 Cohen M S, Rychik J, Bush D M et al.. Influence of congenital heart disease on survival in children with congenital diaphragmatic hernia.  J Pediatr. 2002;  141 25-30
  CrossrefPubMedGoogle Scholar
• 17 Bedoyan J K, Blackwell S C, Treadwell M C, Johnson A, Klein M D. Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis. Outcome-related variables at two Detroit hospitals.  Pediatr Surg Int. 2004;  20 170-176
  CrossrefPubMedGoogle Scholar
• 18 Taketazu M, Lougheed J, Yoo S J, Lim J S, Hornberger L K. Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome.  Am J Cardiol. 2006;  97 720-724
  CrossrefPubMedGoogle Scholar
• 19 Lim J S, McCrindle B W, Smallhorn J F et al.. Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes.  Circulation. 2005;  112 2454-2461
  CrossrefPubMedGoogle Scholar
• 20 Lin J H, Chang C I, Wang J K et al.. Intrauterine diagnosis of heterotaxy syndrome.  Am Heart J. 2002;  143 1002-1008
  CrossrefPubMedGoogle Scholar

John P KovalchinM.D. 

Nationwide Children's Hospital, Ohio State University, Heart Center, 6th Floor Education Bldg.

700 Children's Drive, Columbus, OH 43205