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 54-year-old male patient with long standing history of type II DM, dyslipidemia and hypertension, with medical therapy of bisoprolol 10 mg daily, valsartan 160 mg daily, HCTZ 25 mg daily, atorvastatin 20 mg nocturnally, insulin and metformin was admitted to a peripheral hospital with bronchopneumonia requiring high dependency unit admission. This was complicated by a transient ischemic attack. Brain computed tomography (CT) was normal. On the second day of admission, he had a significant episode of chest pain. Electrocardiogram (ECG) showed transient ST-segment elevation with spontaneous resolution in the inferior lead. There was mild troponin leak. He was later referred to our center for further workup. His physical exam revealed oxygen saturation at 98% on room air, pulse 70 bpm, BP both upper limb 155/65 mm Hg, and BP lower limb 105/45 mm Hg. There was a brachio-femoral delay. Apex was mildly displaced; he had S1+ S2+ S4 and a systolic murmur at the tip of left scapula; no clicks were appreciated on auscultation.\nECG showed sinus rhythm and left ventricular hypertrophy (LVH) with strain pattern. A 24-h Holter came back with normal sinus rhythm, no atrial fibrillation, no heart blocks or arrhythmia. Transthoracic echocardiogram revealed concentric LVH, borderline left ventricular function of 45-50%, mild to moderate aortic regurgitation, bicuspid aortic valve and a coarctation of the aorta with a 70 mm Hg gradient across it. Brain MRI demonstrated lacunar infarcts and no AV-malformations. Both carotid arteries were free of atherosclerosis on Doppler. Coronary CT angiogram not only demonstrated the tight coarctation but also showed a rare anomaly of a PLSVC draining into the left atrium (). Anomalous pulmonary drainage and pulmonary arteriovenous malformation were excluded. The approach to management of this patient was discussed in a heart team meeting. It was decided to proceed percutaneously.\nThe patient was brought to the catheterization laboratory. Coronary angiography showed normal coronaries. He did have a significant coarctation with a pressure gradient of 40 mm Hg. Angiographic confirmation of the PLSVC draining into the left superior pulmonary vein was confirmed. A 40 mm CP stent mounted on a 20 × 45 mm BIB balloon was deployed successfully (), guided by fluoroscopy and overdrive pacing. There was complete abolition of the gradient. At a later date, the patient was brought for PLSVC exclusion therapy. Selective angiography of the PLSVC was performed to delineate vessel course and venous drainage (). Using a marker pigtail measurement of the vessel was obtained. The PLSVC measured 9 mm proximally, 10.5 mm at its mid-point and 8 mm near the entrance to the left upper pulmonary vein. The PLSVC was then temporarily occluded with an 8 mm peripheral balloon to make sure that the patient would tolerate closure of the vessel. There was no significant change in the patient’s hemodynamic measurements, and the decision was made to proceed with transcatheter occlusion of the PLSVC which was achieved using a 12 mm Amplatzer vascular plug II (AGA Medical Corporation, Golden Valley, MN) ().