Autopsy findings of an isolated persistent left superior vena cava in an intrauterine dead fetus

Abstract

Persistent left superior vena cava (PLSVC) is one of the cardiac system abnormalities with a 0.3–0.5% incidence and caused by inadequate obliteration of the left anterior cardinal vein during embryonic development. Prognosis of PLSVC is generally assumed to be good if it is not accompanied by other cardiac system abnormalities. During the routine ultrasound control of a patient at 25th week of pregnancy at the Obstetrics and Gynecology Department of Mersin University, PLSVC anomaly was detected in an intrauterine fetus. Then, intrauterine death occurred and after removal of the deceased fetus, PLSVC diagnosis was confirmed by autopsy. According to the autopsy findings, right superior vena cava (SVC) and azygos vein were found in normal course. PLSVC opened into the right atrium via enlarged coronary sinus. There was no connection between the two SVCs. On the left side of posterior mediastinum, instead of hemiazygos or accessory hemiazygos veins, a vein symmetrical to azygos was opened into PLSVC, similar to the one on the right. No other cardiac anomaly associated with PLSVC or any other pathology in the other parts of body that could be responsible for death was discovered during autopsy. There was no evidence indicating that PLSVC played any role in intrauterine exitus of the present case. However, as mentioned in the literature, the ectopic beats in the atrium wall of patients with isolated PLSVC and enlarged coronary sinus may lead to pathologies in the conduction system of the heart. Considering the intrauterine death of an isolated PLSVC case associated with cardiac conduction pathologies, we recommend that the common assumption of ‘isolated PLSVC is not associated with death’ should be reviewed by studies on large series and even intrauterine cases should be closely monitored for cardiac arrhythmia.