A case of unusual visceral heterotaxy syndrome with isolated levocardia

Abstract

Situs inversus of the abdominal organs in the presence of normally placed heart on the left side of the thorax is known as situs inversus with isolated levocardia. This rare condition is commonly associated with severe congenital defects of the heart. We report a case of situs inversus with levocardia in a 19-year-old asymptomatic male patient with completely normal heart on the left chest. Spiral computed tomography of the thorax and abdomen and echocardiographic studies revealed situs inversus of abdominal organs, normal heart (levocardia), mirrored left lungs, a midline liver, a left-sided inferior vena cava connecting to the right atrium, multiple splenic masses in the abdominal right upper quadrant, and aneurysmal dilatation of a splenic artery.

Keywords: Heterotaxy syndrome; Levocardia.

Fig. 1

Chest and abdomen plain X-rays reveal a normally located heart (A) and a right-sided stomach gas shadow, which is displaced by the bridging liver and is not directly beneath the diaphragm (B).

Fig. 2

Spiral computed tomography of the abdomen show situs inversus and multiple spleens (white arrow) in the right upper quadrant (A), and aneurysmal dilatation (black arrow) of a splenic artery (B).

Fig. 3

Hepatic veins (empty arrowheads) show unusual drainage into the inferior vena cava (solid arrowheads); the left hepatic vein (A) into the left-sided inferior vena cava, mid-hepatic veins (B) into inferior vena cava crossing the midline, and the right hepatic vein (C) into the inferior vena cava just before the right atrium.

Fig. 4

The inferior vena cava (solid arrowheads) runs on the left side (A), and the suprahepatic segment of inferior vena cava returns to the right atrium directly (B).

Fig. 5

Spiral computed tomography of the thorax and abdomen demonstrated normal positioning of the ascending aorta, the main pulmonary artery and the superior vena cava (A and B). Sagittal images in lung window setting, right lung (C) shows the mirror structure of the bi-lobed normal left lung (D).