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Following routine medical screening, a 26-year-old man was found to have aortic coarctation and a partial anomalous pulmonary venous connection. Historically, multiple cardiac pathologies, such as in the present case, required a surgical approach; however, percutaneous treatment of the coarctation with a covered stent and occlusion of the partial anomalous pulmonary venous connection with an Amplatzer device (AGA Medical Corporation, USA) without complications is described.
Partial anomalous pulmonary venous connection is characterized by the failure of one or more pulmonary veins to connect with the left atrium during fetal development. As an alternative, the anomalous pulmonary veins may connect to the right atrium, superior caval vein, inferior caval vein or to one or more of their venous tributaries (1). Partial anomalous pulmonary venous connections are frequently associated with atrial septal defects (2), and are rarely associated with other congenital abnormalities of the heart (3). Generally, patients with a partial anomalous pulmonary venous connection, if symptomatic or showing evidence of significant left-to-right shunting, are treated with surgery. Various surgical techniques have been described to repair this defect (4–6), although such operative procedures are associated with a significant risk of superior cava vein or pulmonary vein stenosis and residual shunting, or may be complicated by nodal and atrial arrhythmias (4,7). Occasionally, it is possible to treat partial anomalous pulmonary venous connections percutaneously by means of an Amplatzer occlusion device (AGA Medical Corporation, USA), although this is only feasible when the anomalous pulmonary veins connect both to the left atrium and the systemic veins. To our knowledge, only one such case has been previously described in the literature (8). We report the first case of percutaneous treatment of a partial anomalous pulmonary venous connection and coarctation of the aorta in a single patient.
A 26-year-old man was referred following a clinical screening examination by the Royal Navy in which he was found to be hypertensive and have an audible ejection systolic murmur. Subsequent investigations revealed evidence of an aortic coarctation beyond the origin of the subclavian artery and partially anomalous drainage of the left upper lobe pulmonary vein via a vertical vein into the systemic left innominate vein. The patient underwent cardiac catheterization, and angiography revealed a coarctation below the subclavian artery level (Figure 1A), with a gradient of 21 mmHg and a minimum coarctation diameter of 7 mm (the diameter just below the subclavian artery was 17 mm, and the diameter of the descending aorta at the level of the diaphragm was 24 mm). A significant left-to-right shunt via the anomalous venous connection from the left upper lobe pulmonary vein via a vertical vein into the systemic left innominate vein was also observed, with oxygen saturations in the left and right pulmonary vein of 98%, in the left innominate vein of 94%, and in the right innominate vein of 82%. A 4.5 cm NuMED CP covered stent (NuMED Inc, USA) on a 16 mm BIB balloon (NuMED Inc, USA) was positioned across the coarctation and deployed (Figure 1B), with no residual gradient after stenting. A repeat procedure was undertaken for occlusion of the partial anomalous pulmonary venous connection. Angiography of the left pulmonary artery confirmed dual drainage of the left upper pulmonary vein via a vertical vein into the innominate vein (Figures 2A and and2B)2B) and the left atrium. Using a left internal jugular vein approach, a 14 mm Amplatzer Vascular Plug II (AGA Medical Corporation, USA) was used to occlude the anomalous connection. Repeat angiography confirmed no residual leak (Figures 2C and and2D).2D). The patient remained healthy at one-year follow-up, and a chest x-ray revealed that both prostheses remained well seated (Figure 2E).
A partial anomalous pulmonary venous connection occurs when at least one (but not all) of the pulmonary veins connect to the right atrium directly or indirectly through one or more of its venous tributaries (1). No definitive classification system has been proposed for this disorder, although Alsoufi et al (7) have proposed five subtypes of partial anomalous pulmonary venous connection, which include the following:
Here we describe a less common, type 4 variety of partial anomalous pulmonary venous connection, in which the anomalous pulmonary vein (vertical vein) ascends into the left innominate vein, in association with coarctation of the aorta, which was discovered during routine assessment of hypertension during a screening medical examination for the Royal Navy. Until more recently, such cases with multiple cardiac pathologies would have been treated using a surgical approach. However, advancement and refinement of percutaneous techniques has meant that such cases involving multiple cardiac pathologies can be treated using interventional percutaneous techniques, obviating the need for surgery with very good outcome data. Endovascular stent implantation has become the modality of choice for the treatment of coarctation of the aorta in older children and adults, and is associated with relatively low morbidity and mortality (9). In contrast, anomalous pulmonary venous connections with a persistent vertical vein can be considered for device occlusion only if there is dual drainage of the pulmonary vein into the left atrium and the systemic venous system, as in the present case. Currently, the vast majority of partial anomalous pulmonary venous connections are treated surgically. Longer-term complications include pulmonary vein obstruction (14%) (7), vena cava obstruction (3%) (7), the need for permanent pacing (1%) (7) and persistent arrhythmias (20%) (4). To our knowledge, there are currently only two other reports of percutaneous treatment of partial anomalous pulmonary venous connection. One case was treated with an Amplatzer occlusion device (8), and the second report described two cases in which a Gianturco-Grifka vascular occlusion device (Cook Group Inc, USA) and Gianturco vascular occlusion coils (Cook Group Inc, USA) were used (10). To our knowledge, there are no reported cases of coarctation in association with partial anomalous pulmonary venous connection treated successfully with a percutaneous approach, thereby obviating the need for surgery and its associated risks.
We describe the percutaneous treatment of the coarctation with a covered stent and occlusion of a partial anomalous pulmonary venous connection with an Amplatzer device without complication. Even in patients with multiple cardiac pathologies, percutaneous treatment options should be considered in close liaison with surgical colleagues before the decision for a surgical approach to treatment is made.