Obstet Gynecol Sci.  2014 May;57(3):193-200. 10.5468/ogs.2014.57.3.193.

Prenatal diagnosis of atrial isomerism in the Korean population

• Lee MY
• Won HS
• Shim JY
• Lee PR
• Lee BS
• Kim EA
• Kim YH
• Park JJ
• Yun TJ
• Kim A

• KMID: 1704425
• DOI: http://doi.org/10.5468/ogs.2014.57.3.193

Abstract

OBJECTIVE
To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes.
METHODS
A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution.
RESULTS
Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%.
CONCLUSION
Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

Keyword

Echocardiography; Heart defects, congenital; Heterotaxy syndrome; Prenatal diagnosis

MeSH Terms

Atrioventricular Block
Bradycardia
Double Outlet Right Ventricle
Echocardiography
Fetus
Heart Block
Heart Defects, Congenital
Heart Septal Defects, Ventricular
Heterotaxy Syndrome
Humans
Infant
Isomerism*
Live Birth
Lost to Follow-Up
Prenatal Diagnosis*
Prevalence
Pulmonary Atresia
Retrospective Studies
Survival Rate
Vena Cava, Inferior
Vena Cava, Superior

Figure

• 

  Fig. 1 Flow diagram showing the postnatal outcomes of fetuses diagnosed with right atrial isomerism (RAI, A) and left atrial isomerism (LAI, B).

• 

  Fig. 2 Ultrasonographic diagnostic clues for both forms of atrial isomerism. (A) Right atrial isomerism with juxtaposition of the aorta and inferior vena cava (IVC) on the same side of the spine (arrows). Right-sided stomach and midline liver are also noted. (B) Left atrial isomerism with interruption of the IVC and azygos vein continuation, showng a 'double vessel' sign (arrows). Lee MY, et al. Obstet Gynecol Sci 2013;56:217-26, according to the Creative Commons Attribution License [27].

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