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.>.

Keywords: Anomalous venous connections; Dextrocardia; Echocardiography.

Fig. 1

Electrocardiogram showing sinus rhythm with features of dextrocardia.

Fig. 2

Chest X-ray with situs inversus dextrocardia showing part of the stomach air on the right side.

Fig. 3

Parasternal long-axis 2D echocardiographic view showing the dilated coronary sinus, which measured 2.5 cm in transverse diameter.

Fig. 4

Apical four-chamber view angled to reveal the coronary sinus following injection of intravenous agitated saline in the left arm. Note the appearance on the contrast in the right atrium and right ventricle without opacification of the coronary sinus, indicating normal drainage of the left superior vena cava into the right atrium.

Fig. 5

Apical four-chamber view angled to reveal the coronary sinus following injection of intravenous agitated saline in the right arm. Note the appearance on the contrast in the coronary sinus before opacification of the right atrium and right ventricle, indicating anomalous drainage of the right superior vena cava into the coronary sinus.

Fig. 6

Chest X-ray from Fig. 2 with an overlay of a diagrammatic representation of the patient's anticipated major venous connections.