Autopsy findings of a child with left atrial isomerism associated with pulmonary agenesis

Achados de autópsia de uma criança com isomerismo atrial esquerdo associado à agenesia pulmonar

Camilla M. V. Pereira Larissa V. Cruz Marina C. Girotto André C. Cunha Jorge Alberto B. Telles Bruna L. Diniz Rosana C. M. Rosa Paulo Ricardo G. Zen Rafael Fabiano M. Rosa About the authors

ABSTRACT

Isomerism is a rare laterality defect. Our aim was to describe the autopsy findings of a child with left atrial isomerism (LAI) and pulmonary agenesis (PA), an association still not described in the literature. Fetal ultrasound revealed right renal agenesis, single umbilical artery and polyhydramnios. Echocardiography revealed a complex heart defect with LAI. The child died minutes after birth. Autopsy confirmed the prenatal findings and revealed the presence of unilateral PA, complex vascular abnormalities and polysplenia. Despite its rarity, health professionals should be aware for the possibility of such an association.

Key words:
isomerism; heterotaxy syndrome; lung; congenital heart defects; autopsy

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