Obstet Gynecol Sci.  2016 Nov;59(6):544-547. 10.5468/ogs.2016.59.6.544.

A case of prenatally diagnosed extrapulmonary arteriovenous malformation associated with a complex heart defect

• Jeong BD
• Won HS
• An S
• Kim JY
• Lee MY
• Kim EN
• Kim JS
• Kim CJ

• KMID: 2378582
• DOI: http://doi.org/10.5468/ogs.2016.59.6.544

Abstract

Pulmonary arteriovenous malformations are rare vascular anomalies of the lung, only a few cases of which have been diagnosed prenatally. The diagnostic clue for prenatal diagnosis was cardiomegaly with a particularly enlarged left atrium. All previous cases of pulmonary arteriovenous malformations diagnosed prenatally have been reported as an isolated anomaly or in association with simple heart defects. We here describe the first case of a pulmonary arteriovenous malformation with a complex heart defect that was diagnosed prenatally at 21.0 weeks of gestation and confirmed by postmortem autopsy.

Keyword

Congenital heart defects; Prenatal diagnosis; Pulmonary arteriovenous fistulas

MeSH Terms

Arteriovenous Malformations*
Autopsy
Cardiomegaly
Heart Atria
Heart Defects, Congenital
Heart*
Lung
Pregnancy
Prenatal Diagnosis

Figure

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  Fig. 1 Fetal echocardiographic images obtained at 21.0 weeks of gestation in the current study case demonstrating cardiomegaly with a particularly enlarged left atrium (LA) in the four-chamber view (A), both aorta (Ao) and pulmonary artery (PA) arising from the right ventricle (RV) (B), and an abnormal communication of main pulmonary artery (MPA) and LA with color Doppler in the parasagittal view (C). Postmortem autopsy showing the abnormal root of Ao in front of the pulmonary artery (PA) arising from the RV in the anterior view (D), PA from the RV running between the right upper lobe (RUL) and the right middle lobe (RML) of the lung turning to the left behind the main bronchus in the right superior view (white arrow, opened PA) (E), and PA connecting to the LA in the right posterior view (white arrow, opened PA; black arrow, opened LA) (F). The inside of LA was dyed by red ink flowing through the PA. RA, right atrium; LV, left ventricle; Ant, anterior; Post, posterior; LUL, left upper lobe; LLL, left lower lobe; RLL, right lower lobe.

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