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 14-month-old female was referred to our hospital with a history of an enlarging abdominal mass noted by her parents for 3 days. Physical examination revealed an abdominal mass with clear and smooth margins extending from the right upper quadrant to the right hemipelvis. Routine blood tests were normal apart from mild anaemia and urine analysis did not show hematuria. Ultrasonography of the abdomen revealed a unilateral 10.8 × 7.2 × 9.2 cm solid tumor in the right kidney, whereas the contralateral kidney was normal. Computed tomography (CT) revealed a large lesion arising from the inferior aspect of the right kidney, occupying the right flank and extending across the midline. Enhanced CT detected duplication of IVC below the renal veins and compression and displacement of the right IVC caused by the enormous tumor (Fig. ). An additional movie file shows this in more detail [see Additional file ].\nNeither intravascular extension nor invasion to adjacent organs and regional lymph nodes was detected by CT. Chest radiography was reported normal.\nWith the presumptive diagnosis of WT, a right-sided radical nephrectomy was performed. Final pathology was consistent with favorable histology, stromal-predominant (60%) WT. The renal vessel and IVC were tumor free. The renal hilar and para-aortic lymph nodes were also free from tumor and the final pathological stage was Stage I. According to the regimen of the National Wilms’ Tumor Study Group 5, the patient received postoperative chemotherapy with dactinomycin and vincristine. CT imaging at 3 months postoperatively showed no evidence of residual or recurrent disease. Interestingly, the right IVC played a dominant role and the left IVC seemed to disappear in postoperative enhanced CT (Fig. ). An additional movie file shows this in more detail [see Additional file ]. During the follow-up of 18 months, no local recurrence or metastasis has been observed.