A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava

BackgroundTo explore the diagnostic clues and abnormality spectrum of heterotaxy syndrome by prenatal ultrasonography and postnatal verification.MethodsThe prenatal ultrasonic data of 88 heterotaxy syndrome fetuses were analyzed retrospectively as left isomerism (LI) and right isomerism (RI). Prenatal ultrasound compared with the anatomical casting of the fetal body after labor induction, and the confirmatory postnatal diagnosis after delivery.ResultsFetal LI showed typical malformations of gastric vesicles on different sides from the heart, absence of hepatic segment of the inferior vena cava (IVC), abdominal aorta (AO) parallel with the azygos vein (AV), bilateral left bronchus, bilateral left atrial appendages, and polysplenia; intracardiac malformations of AV septal defects (AVSD), single atrium (SA), left ventricular outflow tract obstruction (LVOTO), and double-outlet right ventricle (DORV); and cardiac conduction abnormalities of sinus bradycardia and AV blockage. Fetal RI reported typical malformations of gastric vesicles on different sides from the heart, juxtaposition of the IVC with AO, anomalous pulmonary venous connection (APVC), asplenia, and bilateral right atrial appendages; intracardiac malformations of AVSD, SA, single ventricle, pulmonary atresia and stenosis, and DORV. The postnatal verification revealed 3 malformations misdiagnoses and 4 malformations missed diagnoses in LI fetuses and 10 misdiagnoses and 8 missed diagnoses in RI fetuses.ConclusionsThe proposed five-step prenatal ultrasonography has an important diagnostic value for the identification and classification of heterotaxy syndrome. The different sides of gastric vesicles and cardiac apex are important diagnostic clues for heterotaxy syndrome, featuring disconnected or hypoplastic IVC, typical complex cardiac malformation, and atrioventricular block in fetal LI, and shown APVC, juxtaposition of IVC and AO, and intracardiac malformations such as AVSD, DORV, and LVOTO in fetal RI.

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Prenatal diagnosis and clinical outcomes of isolated interruption of the inferior vena cava: an analysis of 12 cases

ACAR,

TURAN BAKIRCI,

ACAR

et al. 2023

J Med Palliat Care / JOMPAC / jompac

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Objectives: This study aimed to comprehensively investigate the clinical manifestations and outcomes of prenatally diagnosed isolated interrupted inferior vena cava (IIVC) with azygos continuation to shed light on the significance of prenatal diagnosis. Methods: A longitudinal study involved 12 fetuses prenatally diagnosed with IIVC and azygos continuation. Detailed fetal anomaly scans and echocardiography were performed, and pediatric cardiologists confirmed postnatal diagnoses. Genetic testing and extensive follow-ups were also performed. Results: The study confirmed the high diagnostic accuracy of prenatal identification, with 100% postnatal confirmation. Most patients exhibited favorable outcomes, emphasizing the importance of prenatal diagnosis. Genetic testing revealed normal chromosomal arrangements in all tested cases. Conclusion: Our study underscores the vital role of prenatal diagnosis in managing isolated IIVC cases and highlights their generally favorable prognosis. This study contributes to understanding this rare vascular anomaly and its implications for clinical practice.

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A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava

Cited by 3 publications

References 9 publications

Isomerism: When right and left are similar, and why it matters

Isomerism: When right and left are similar, and why it matters

Characterization of ultrasound and postnatal pathology in fetuses with heterotaxy syndrome

Prenatal diagnosis and clinical outcomes of isolated interruption of the inferior vena cava: an analysis of 12 cases

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