Fetal diagnosis of left isomerism not associated with other cardiac malformations

Pedro Pires; Sandra Mattos; Sônia Araújo Hinrichsen; Nathalie Bravo-Valenzuela; Gabriele Tonni; Edward Araujo Júnior

doi:10.5114/pcard.2021.112313

eISSN: 2449-6731
ISSN: 2449-6723

Prenatal Cardiology

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abstract:

Case report

Fetal diagnosis of left isomerism not associated with other cardiac malformations

Pedro Pires

¹

,

Sandra Silva Mattos

²

,

Sônia Cristina Araújo Hinrichsen

³

,

Nathalie Jeanne Bravo-Valenzuela

⁴

,

Gabriele Tonni

⁵

,

Edward Araujo Júnior

⁶

Department of Obstetrics and Gynaecology, University of Pernambuco (UPE), Recife-PE, Brazil
Maternal Fetal Cardiac Unit, Real Portuguęs Hospital, Recife-PE, Brazil
Sector of Obstetrics and Gynaecology, Pernambuco Maternal and Child Institute (IMIP), Recife-PE, Brazil
Department of Paediatrics, Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro-RJ, Brazil
Department of Obstetrics and Neonatology, Istituto di Ricerca a Carattere Clinico Scientifico (IRCCS), AUSL di Reggio Emilia, Reggio Emilia, Italy
Department of Obstetrics, Paulista School of Medicine-Federal University of São Paulo (EPM-UNIFESP), São Paulo-SP, Brazil

Prenat Cardio 2021; 11(1): 51–54

DOI: https://doi.org/10.5114/pcard.2021.112313

Online publish date: 2021/12/31

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Left atrial isomerism is a rare and complex congenital condition characterized by bilateral left-sidedness of atria. It is frequently associated with the loss of normal asymmetry of thoracoabdominal organs (heterotaxy syndrome) and congenital heart defects. The authors studied a rare case of left atrial isomerism with bradycardia and no associated cardiac defects. The authors reviewed important features to improve this diagnosis by applying ultrasound. A healthy pregnant woman was referred for fetal echocardiography at 20 weeks of gestation due to persistent fetal bradycardia. The fetal echocardiogram confirmed arrhythmia (fetal heart rate ranging from 95 to 100 beats per minute) with a 1:1 atrioventricular (AV) relationship and a normal AV interval time. In addition, the fetal echocardiogram showed left-sided atrial morphology with no associated cardiac defects. Absence of the hepatic segment of the inferior vena cava with azygous continuation drew attention for the diagnosis of left isomerism. The fetal heart rate remained stable until term, and a male baby was born healthy. The diagnosis of left isomerism can be very difficult to achieve prenatally, especially in the absence of associated heart defects. An interrupted inferior vena cava with azygous and hemi-azygous continuation should draw attention to this diagnosis.

keywords:

Fetal diagnosis of left isomerism not associated with other cardiac malformations

Pedro Pires 1 , Sandra Silva Mattos 2 , Sônia Cristina Araújo Hinrichsen 3 , Nathalie Jeanne Bravo-Valenzuela 4 , Gabriele Tonni 5 , Edward Araujo Júnior 6

left isomerism, heterotaxy, bradycardia, ultrasound imaging, fetal echocardiography

Pedro Pires

¹

,

Sandra Silva Mattos

²

,

Sônia Cristina Araújo Hinrichsen

³

,

Nathalie Jeanne Bravo-Valenzuela

⁴

,

Gabriele Tonni

⁵

,

Edward Araujo Júnior

⁶