Persistent left superior vena cava (PLSVC) with anomalous left hepatic vein drainage into the right atrium: role of imaging and clinical relevance

Abstract

An 86-year-old white male with a history of inferior wall myocardial infarction 25 years ago, chronic atrial fibrillation and hyperlipidemia presented with shortness of breath, dizziness of several weeks' duration, and chronic stable angina. Physical exam was remarkable for bradycardia with regular rhythm and normal S1 and S2 with a systolic ejection murmur. ECG on admission showed complete heart block with a ventricular rate of 37 beats/min. Cardiac biomarkers were negative. His heart rate then decreased to 29 beats/min and atropine 0.5 mg followed by 1 mg was given without any response.During pacemaker implantation a fluoroscopic examination of the thoracic anatomy demonstrated normal structure. Initially, the left subclavian vein approach was employed. Multiple attempts to locate the left subclavian vein percutaneously in the usual infra-clavicular fashion under fluoroscopic guidance were not successful. Therefore, a left brachial venogram was performed using 15 cc of nonionic contrast injected into the left antecubital vein. The cineangiogram demonstrated patency of the subclavian vein but with very slow flow and a suggestion of a vertical course of the subclavian vein along the left mediastinum. The left brachial vein was accessed using a micropuncture needle and a flexible guide wire was advanced which confirmed the suspicion of a persistence of left superior vena cava (Figure 1). Therefore, the left subclavian vein approach for the pacing lead insertion was abandoned. Next, under fluoroscopic guidance, the right subclavian vein was accessed with the micropuncture needle followed by placement of a guide wire into the right atrium without difficulty.Echocardiography showed markedly dilated left and right atria (8.9 ϫ 8.1 cm, 9.3 ϫ 8.5 cm, respectively) with an intact interatrial septum. An enlarged coronary sinus draining into the right atrium was observed. Abstract: Persistent left superior vena cava (PLSVC) is a very rare and yet the most commonly described thoracic venous anomaly in medical literature. It has a 10-fold higher incidence with congenital heart disease. PLSVC often becomes apparent when an unknown PLSVC is incidentally discovered during central venous line placement, intracardiac electrode/pacemaker placement or cardiopulmonary bypass, where it may cause technical difficulties and life-threatening complications. PLSVC is also associated with disturbances of cardiac impulse formation and conduction including varying degrees of heart blocks, supraventricular arrhythmias and Wolff Parkinson White syndrome. We describe the case of an 86-year-old male with a history of coronary artery disease and chronic atrial fibrillation who presented with worsening dyspnea and syncopal episodes. An ECG was consistent with complete heart block. During lead placement for the pacemaker, a left subclavian approach was unsuccessful. A left venogram was performed through the brachial vein that demonstrated a left superior vena cava. The diagnosis was confirmed with echocardiography using a bubble study and also a c...

Study of the Association Between an Anomalous Superior Vena Cava and Horseshoe Kidney

“…11,22,23 CVC placement or access through the persistent left SVC may pose several serious problems, such as hypotension, angina, arrhythmias, and cardiac arrest. 11,20, 23 In cases of persistent left SVC with an absent right SVC, pacemaker implantation is extremely difficult, even impossible.…”

Persistent Left Superior Vena Cava (PLSVC) with Anomalous Hepatic Venous Drainage

“…Surgical repair for ASD was performed under cardiopulmonary bypass with four venous cannulas. PLSVC is a rare but important congenital anomaly reported to be present in approximately 0.4% of the population, and complication with anomalous hepatic venous drainage is remarkably uncommon (1,2). CT angiography is a preferred modality for diagnosing PLSVC and evaluating the full extent of vascular anomalies.…”

Atresia of the Coronary Sinus Ostium to the Right Atrium with a Persistent Left Superior Vena Cava

“…When this ostium is not present, this is a rare congenital anomaly named atresia of the CS ostium (Zimand et al, 1999;Jha et al, 2003), considered one of the most rare congenital anomalies of the thoracic circulatory system (Bhatti et al, 2007;Stylianou et al, 2007). This anomaly is commonly associated with others of the cardiocirculatory system, including those of the heart itself (Santoscoy et al, 1996;AlexiMeskishvili et al, 1999).…”

“…Also, CS anomalies might be associated to alterations of the conduction system of the heart, leading to arrhythmias (Ratliff et al, 2006;Bhatti et al;Stylianou et al) and these anomalies should be considered when patients present with inexplicable arrhythmias (Heather et al, 2006). Atresia of the CS ostium is commonly associated with a persistent left superior vena cava (LSVC) (Salminen et al, 2006;Stylianou et al) and both, when associated to the univentricular anomaly, are not life compatible (Salminen et al).…”