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

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.

KEYWORDS

Dextrocardia - dextroposition - heterotaxy - fetal echocardiography

REFERENCES

• 1 Allan L D, Lockhart S. Intrathoracic cardiac position in the fetus.  Ultrasound Obstet Gynecol. 1993;  3 93-96
• 2 Comstock C H. Normal fetal heart axis and position.  Obstet Gynecol. 1987;  70 255-259
• 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
• 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
• 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
• 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
• 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
• 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
• 9 Gibbin C, Touch S, Broth R E, Berghella V. Abdominal wall defects and congenital heart disease.  Ultrasound Obstet Gynecol. 2003;  21 334-337
• 10 Garg N, Agarwal B L, Modi N. Dextrocardia: an analysis of cardiac structures in 125 patients.  Int J Cardiol. 2003;  88 143-155
• 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
• 12 Boldt T, Andersson S, Eronen M. Outcome of structural heart disease diagnosed in utero.  Scand Cardiovasc J. 2002;  36 73-79
• 13 Eronen M. Outcome of fetuses with heart disease diagnosed in utero.  Arch Dis Child Fetal Neonatal Ed. 1997;  77 F41-F46
• 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
• 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
• 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
• 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
• 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
• 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
• 20 Lin J H, Chang C I, Wang J K et al.. Intrauterine diagnosis of heterotaxy syndrome.  Am Heart J. 2002;  143 1002-1008