Percutaneous closure of an anomalous vertical vein

Abstract

We report the case of a 39-year-old male with a history of untreated arterial hypertension. He presented to a community hospital with pulmonary edema and anasarca, and was transferred to our heart center for further investigation and therapy. Echocardiography revealed the enlargement of all four heart chambers including a severely compromised systolic function of both ventricles. Coronary angiography ruled out coronary artery disease. Computer tomography of the thorax was performed to exclude pulmonary embolism. Surprisingly, an anomalous left vertical vein was found, connecting left upper pulmonary veins to the left innominate vein as well as to the left atrium (Fig. 1a). Magnetic resonance tomography confirmed the diagnosis of this anatomical large left partial anomalous pulmonary venous connection (PAPVC) with a pulmonary stroke volume of 86 ml and a systemic stroke volume of 68 ml. The resulting left-to-right shunt was relatively small with a pulmonary to systemic cardiac index (Qp:Qs) of just 1.26 and a shunt stroke volume in the vertical vein of 12 ml. Furthermore, a mild coarctation of the aorta and a minimal late enhancement of the left ventricle could be demonstrated, the latter finding was consistent with hypertensive heart disease. Right heart catheterization revealed moderate pulmonary hypertension with a mean pulmonary pressure of 34 mmHg and a mean pulmonary capillary wedge pressure of 29 mmHg. We decided to close the vertical vein percutaneously. After retrograde intubation of the vertical vein, repetitive angiograms at various heights of the vein were performed to define size and target zone of the closure device. Thereby, a further connection to the right atrium via the accessory hemiazygos vein was found, explaining the missing 6 ml of calculated shunt stroke volume (Fig. 2a). Afterwards, this finding of a strictly posterior originating additional connection could be confirmed by re-evaluation of the initial CT scan (Fig. 1b, c). We decided to use an 18-mm Amplatzer vascular plug II (St. Jude Medical, St. Paul, MN, USA) to occlude both anomalous connections. After positioning the Amplatzer TorqVue 2 Delivery Sheath (St. Jude Medical) in the left atrium, the vascular plug II was advanced. While pull-back of the sheath the plug was positioned in the target zone, which was the distal vertical vein at the orifice of the accessory hemiazygos vein, just above the orifice of the left pulmonary veins. After deployment of the device, there was no more washout into the left innominate vein: neither from the accessory hemiazygos vein nor from pulmonary veins (Fig. 2b), and a pulmonary angiogram confirmed successful closure of the anomalous vertical vein also. Fetal venous circulation is very variable and complex. During normal development of the human fetal venous system the left supracardinal vein connects the accessory hemiazygos vein and the left atrium with the left innominate vein. Normally, this vein occludes before birth. However, in cases of left PAPVC, one or more left pulmonary veins can drain into the left innominate vein via a Electronic supplementary material The online version of this article (doi:10.1007/s00392-013-0597-7) contains supplementary material, which is available to authorized users.