A 44-year-old fit and active female was referred by her general practitioner with palpitations, mostly occurring at rest. Electrocardiography performed in the community demonstrated sudden onset of an atrial tachycardia of 115 beats per min. When assessed in hospital, she was asymptomatic with a blood pressure of 110/62 and a regular pulse rate of 50 beats per min. Resting electrocardiography revealed sinus rhythm with poor R wave progression with no evidence of left ventricular hypertrophy or ischemia. A 24-h electrocardiography demonstrated four beats of atrial tachycardia.\nA transthoracic echocardiogram demonstrated a prominent coronary sinus, moderate mitral regurgitation, and a bicuspid aortic valve []. Transesophageal echocardiography showed normal biventricular systolic function, left atrial dilatation, mild to moderate mitral regurgitation, and tricuspid regurgitation. The aortic valve was bicuspid with trivial aortic regurgitation but no significant stenosis. The ascending aorta was dilated for her age and body surface area, with a maximum diameter of 4.0 cm. There was no evidence of aortic coarctation or dissection.\nSaline contrast echocardiography was performed via the left and right arms [], [], [] and []. This confirmed PLSVC with no obvious shunt. Cardiac magnetic resonance imaging (MRI) demonstrated bicuspid aortic valve with dilated aortic root. A PLSVC was seen draining into the coronary sinus and then the right atrium. A separate right-sided non-hypoplastic superior vena cava also emptied into the right atrium. It was noted that a left-sided IVC drained into the coronary sinus and right atrium. Three hepatic veins were seen emptying into a short right-sided IVC, again draining into the right atrium.
A 35-year-old man with the diagnosis of mitral atresia, a double outlet right ventricle, pulmonary atresia with interrupted inferior caval vein, and azygous continuation to the right superior caval vein underwent a Blalock-Taussig shunt in early childhood and a Kawashima operation at the age of 17 at another institution. He presented to our clinic with progressive cyanosis and fatigue 18 years after his last surgical procedure. His oxygen saturation was 65%.\nCardiac catheterization revealed left pulmonary artery stenosis and a decompressing vein from the right superior caval vein to the left superior caval vein which directly drains to the left atrium. Left pulmonary artery stenting was done via right internal jugular approach (Figures , , and ).\nHe underwent a second catheterization for occlusion of the left superior caval vein via left internal jugular approach. The anomalous vein measured 22 and 20 mm in AP and lateral projections, respectively. Balloon test occlusion resulted in immediate increase in oxygen saturation from 65% to 82% without drop of blood pressure. The mean pressure in the azygous vein remained at 9 mmHg; pressure in the left internal jugular vein increased to 11 mmHg and dropped within a few minutes to 10 mmHg. Angiography showed good venous collateral drainage.\nThe largest available vascular plug, 24 mm in diameter, failed to completely occlude the vessel, which was successfully occluded using a 24 mm ASD occluder (Cera Lifetech, China) via a 12 Fr sheath. Upon completion of the procedure the patient had oxygen saturation of 80%, left internal jugular pressure of 10 mmHg, and right superior caval vein and pulmonary artery pressure of 9 mmHg.\nAfter occlusion, the patient had significant improvement of his exercise intolerance and fatigue. Follow-up hemodynamic study 12 months later showed no increase in venous pressure, no development of venous collaterals, and no decrease in his oxygen saturation.