A 30-year-old woman was referred with epigastralgia. Physical examination was normal, electrocardiography detected a low atrial rhythm of 45 beats per minute ( Fig. 1 , arrow ), chest X-ray showed a slight cardiomegaly and echocardiography highlighted the existence of a dilated coronary sinus (13 × 17 mm). A thoracoabdominal computed tomography scan detected the presence of heterotaxy syndrome, consisting of:
- •
left atrial isomerism (LAI; Fig. 2 A ) and left lung and bronchial isomerism ( Fig. 2 C);
Figure 2
(A) Chest X-ray; (B–D) thoracoabdominal computed tomography scan images. A: left atrial isomerism; B: left bronchial isomerism; L: liver; S: spleen; ST: stomach; V: persistent left superior vena cava.
- •
anomalous venous system (dilated coronary sinus draining to a persistent left superior vena cava ( Fig. 2 B), absence of intrahepatic segment of the inferior vena cava and abdominal drainage pouring to the hemiazygos system and from there to the persistent left superior vena cava);
- •
liver placed in middle position, occupying both hypochondria but with a predominantly left lateralization ( Fig. 2 B) and finally;
- •
stomach ( Fig. 2 A and D) and dysmorphic spleen ( Fig. 2 D) placed on the right side; the rest of the study was normal.
