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.
Our patient was an 18-year-old girl with recent onset right leg pain and swelling with extension to her inguinal area. She also had dyspnea with respiratory distress. At the time of presentation, she was ill and febrile. She also had pitting edema of right leg and significant size difference between circumferences of her two legs. On the day of admission, she suddenly presented with dyspnea and severe thoracic pain on the right side. All other physical examination was normal.\nShe had no predisposing factor for venous thrombosis. Besides, she had no known a history of DVT, clotting disorders, recurrent abortion, peripheral vascular disease, non-healing ulcerations on the extremities, cerebrovascular accidents or taking oral contraceptive pills.\nHer laboratory data showed only mild normochromic, normocytic anemia, elevated erythrocyte sedimentation rate (ESR) to 75 (normal range less than 25) and CRP = 16 (normal less than 8). Other laboratory test including renal, electrolytes, liver, and urine analysis and culture were normal.\nColor duplex ultrasound study demonstrated deep venous thrombi in the right popliteal, superficial and common femoral veins, with external iliac vein.\nIn addition, hypercoagulable workup was revealed no abnormal finding and also negative for antiphospholipid syndrome and lupus anticoagulant antibodies.\nA multi-detector computed tomography (CT) pulmonary angiography of the chest defined pulmonary emboli in the territory of right main and segmental branch pulmonary artery and suggesting extensive infarction of the right middle pulmonary lobe. She was subsequently started on a low molecular weight heparin (Clexane®: 60 mg, every 12 h SC). Subsequently, CT of the abdomen and CT angiography with IV contrast injection carried out after the procedure confirmed duplication of the IVC []. Both two IVC originate in the pelvis, run parallel to each other, bordering the aorta and left-sided IVC drained into the left renal vein. There was no other pathologic finding in thoracic and abdominopelvic CT studying.\nAfter fully anticoagulated with low molecular weight heparin for 5 days and starting warfarin international normalized ratio (INR) range was between 2 and 3, she was discharged from hospital and was not symptomatic in her follow-up until next 3 months. According to the previous studies, for this patient we planned to continue anticoagulant therapy for 6 months.[]