A Case of Unusual Visceral Heterotaxy Syndrome with Isolated Levocardia
A 19-year-old male was referred to the Pediatric Cardiology Clinic of Chonbuk National University Hospital for the evaluation of abnormal chest X-ray films showing the position of the stomach gas as being discordant with the location of the heart. The patient had no cardiac symptoms and was a normal developed adolescent. Family and past histories were negative. Physical examination revealed this young man to be acyanotic and was in no distress. Cardiac examinations revealed a grade 1/6 ejection systolic murmur at the upper left sternal border. The liver dullness was presented in the upper left quadrant of the abdomen. The remaining part of the physical examination was entirely normal.\nPlain X-ray films of the chest and abdomen showed a normally located heart and a right-sided stomach (). The electrocardiogram was normal with sinus rhythm. Trans-thoracic echocardiographic study demonstrated no intra-cardiac lesions. The IVC was seen on the left side, crossed the midline at the level of the hepatic dome, and connected directly to the right atrium. Spiral computed tomography of the thorax and abdomen showed situs inversus with levocardia and multiple spleens in the right upper quadrant of the abdomen (), a midline liver, a midline positioned gall bladder, hepatic veins with unusual drainage into the IVC (), and a prominent pancreatic head with associated absence of body and tail of the pancreas. The stomach was located on the right side. Most of small bowel loop was located in the right-lateral portion of the abdomen along the left side ascending colon, with some intestinal malrotation. The splenic artery originated from the celiac trunk of the left sided descending aorta with tortuosity coursing to the spleens. There was a discrete aneurysmal dilatation (2.0×2.6 cm in diameter) in the proximal portion of the artery (). The IVC ran on the left side (), and connected directly to the right atrium (). Azygos and hemiazygos veins, and lumbar veins on both sides were slightly dilated. The ascending aorta, main pulmonary artery, pulmonary veins, and superior vena cava were all normally positioned. The structure of right lung was a mirror to the normal left lung, i.e., bilateral bi-lobed lungs ().