Persistent right superior vena cava in a patient with dextrocardia: Case report and review of the literature

Abstract

Introduction: Systemic venous circulation anomalies are uncommon; they are often incidental findings during echocardiography. Case: A 56-year-old man, with dextrocardia, was evaluated for dyspnea. The patient's medical history included diabetes mellitus requiring insulin treatment, hypertension, and tobacco use. Physical examination revealed normal jugular venous pulsations and clear lungs. Cardiac examination revealed normal heart sounds, and grade II/VI systolic ejection murmur over the right precordium. Echocardiography revealed normal chamber size and systolic function, without significant valvular lesions. The coronary sinus was dilated. It was evaluated using intravenous agitated saline contrast to rule out anomalous venous drainage or shunting. When injected into the left antecubital vein, contrast appeared initially in the right atrium followed by the right ventricle. However, when injected into the right antecubital vein, contrast appeared initially in the dilated coronary sinus followed by the right atrium and right ventricle. There was no evidence of intracardiac shunting. These findings were consistent with persistent right superior vena cava in the setting of situs inversus dextrocardia, with normally draining left superior vena cava. Conclusion: Persistent superior vena cava connection to the coronary sinus is often incidental but an important finding which helps in planning safe invasive procedures. <Learning objective: Understand the importance of identifying anomalous venous connections with regard to catheter-based procedures. Appreciate the incidence of these vascular anomalies in the normal population and in congenital heart disease. Understand how echocardiography with intravenous agitated saline contrast can be helpful in the diagnosis of such anomalous venous connections.>

Situs inversus totalis: A challenge for cardiac resynchronization therapy

“…2 Anomalies of the venous vascular system (double, absent vena cava or persistent vena cava connected to the CS), single ventricle, double-outlet or double-inlet ventricles, and tricuspid atresia have been reported. 3,4 Therefore, SIT-DXC is usually associated with specific technical difficulties and there is not a standardized surgical approach due to the fact that every patient has an individual anatomy. Moreover, the association with congenital heart block and acquired degeneration of the conduction system have been previously described.…”

“…Indeed, less than 10% of individuals with SIT‐DXC have associated congenital cardiac defects 2 . Anomalies of the venous vascular system (double, absent vena cava or persistent vena cava connected to the CS), single ventricle, double‐outlet or double‐inlet ventricles, and tricuspid atresia have been reported 3,4 . Therefore, SIT‐DXC is usually associated with specific technical difficulties and there is not a standardized surgical approach due to the fact that every patient has an individual anatomy.…”

A 20-year follow-up of successful surgical management for a complex case of pentalogy of fallot and dextrocardia with systemic and pulmonary venous anomalies: A rare case report

“…A recent study estimated the incidence of partial anomalous pulmonary venous connection (PAPVC) in about 0.34 % of TOF cases [ 4 ]. Abnormal systemic venous circulation like additional right or left vena cava is rare, and it has been described in some studies; however, the co-existence of these anomalies with dextrocardia is extremely rare, especially in terms of persistent right superior vena cava (PRSVC) [ 5 ]. DORV occurs when both the aorta and pulmonary vessels arise from the right ventricle; very limited data linked between the presence of DORV with TOF or POF [ 7 ].…”

“…Partial anomalous pulmonary venous connection (PAPVC) is a clinical condition in which one or more of the pulmonary veins are not draining into the right atrium. Preoperative imaging is highly recommended for a precise diagnosis, and this can be done through several diagnostic methods [ 4 , 5 ]. Total surgical repair is the standard treatment, with pulmonary-preserving techniques being the preferred surgical technique [ 6 ].…”

Trans–coronary sinus puncture for catheter ablation and left atrial appendage closure device implantation in a patient with dextrocardia and persistent right superior vena cava

“…3 The combination of dextrocardia with persistence of a second (right) SVC has been described. 4 In patients with dextrocardia, knowledge of the anatomical details is vital because it allows the electrophysiologist to plan the technical approach. Thus, we ordered a chest and abdomen CT and integrated the images with ultrasound images from ICE and the 3D mapping system.…”