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 23-year-old female patient with no significant past medical history presented to the hospital complaining of acute shortness of breath, abdominal pain, and severe left lower extremity pain. The patient was in her usual state of health until the evening of her symptoms, when she suddenly turned in her bed and began to experience numbness, tingling, and purplish discoloration of her left lower extremity. On arrival to the emergency department, the patient was in respiratory distress and had bilateral swelling of her lower extremities, the left side larger than the right. A bilateral lower extremity duplex ultrasound revealed a deep vein thrombosis (DVT) in the left lower extremity, the popliteal, and the femoral veins. In addition a CT of the chest utilizing our pulmonary embolism protocol delineated two pulmonary emboli in the first order pulmonary arteries and multiple segmental emboli located in the right upper and right lower lobes. She was subsequently started on a heparin drip.\nThe patient denied any tobacco use, recent immobilization, or travel. She reported the use of an estrogen vaginal delivery device (ethinyl estradiol vaginal ring) for the previous 6 months. Her hypercoagulable workup was revealing for heterozygosity at C677T for factor V Leiden. It should be noted that heterozygous carriers of FVL have been shown to have an overall 3- to 7-fold increased risk of venous thrombosis, while homozygotes to C677T have a 50- to 100-fold increased risk. []\nDue to the acute presentation of her venous thromboembolism, a left pelvic and lower extremity venous thrombectomy was performed. This was followed by stenting of a popliteal venous occlusion, and subsequent thrombolysis with tissue plasminogen activator. Surprisingly, a left lower extremity venogram revealed bilateral inferior vena cavas.\nIn our patient, the left-sided duplicated IVC and iliac veins were found to be thrombosed. This was marked by the stippled appearance of the duplicated left IVC vessel seen in Figure . The decision was made to stent the duplicated IVC given the anatomic irregularity and increased risk of repeat thrombosis. Two wall stents (Figure ) were deployed at the proximal end at the level of the left sided (duplicated) IVC. Next a balloon was used to angioplasty the entire length of the stented segment of the duplicated IVC (Figure ). Figure depicts the duplicated left IVC with wall-stent draining into the left renal vein with a non-occlusive thrombus within it. The orthotopic IVC remained patent. The patient was kept on heparin for 24-hours and then started on Coumadin.