Page 10 - Journal of Structural Heart Disease Volume 2, Issue 5

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Original Research Article
pressure, indicating that interventional occlusion was feasible. During balloon occlusion, the diameter of the connecting vein was 15.3 mm, with a stenotic segment of 10.4 × 12.7 mm (Figure 3E). Subsequently, a 20-mm Amplatzer Vascular Plug II (AVP II) was chosen to occlude this portion of the scimitar vein. The stenosis was used as the anchor for the distal waist of the device. After release from the delivery system, there was no residual shunt through the de- vice after 10 min. Absence of obstruction of hepatic venous ow into the IVC and of the right lower pul- monary vein could be documented (Figure 3F). Repeat hemodynamic evaluation did not reveal any di erence of pulmonary wedge pressure to the end-diastolic left ventricular pressure. However, the mean right pulmonary artery pressure remained elevated at 26 mmHg.
Echocardiography postimplantation showed no residual ow along the vessel occluder into the IVC. Before hospital discharge, the patient was started on a vitamin K antagonist for 6 months. No oral pulmo- nary vasodilator therapy was initiated after the inter- vention. At the 6-month follow-up, the patient was well, and the device was in an unchanged position without residual ow as assessed by echocardiogra- phy. Repeat cardiac catheterization 19 months after the intervention revealed improved hemodynamics with a slightly increased mean pulmonary artery pressure at 18 mmHg, underscoring the need for anomalous vessel closure.
Discussion
Unless patients diagnosed with scimitar syndrome are symptomatic or have a signi cant left-to-right
shunt leading to pulmonary hypertension and right ventricular failure, clinical surveillance has been rec- ommended, because surgery may be challenging and associated with signi cant complications and morbidity [8-10]. In the present case, moderate left- to-right-shunting and mild pulmonary hypertension required treatment. There was partial anomalous pul- monary venous drainage of the right lung into the IVC without further abnormalities of the right lung, including blood supply. The patient’s individual anat- omy with double drainage of the right lung allowed successful interventional occlusion of the inferior portion of the scimitar vein without open surgery.
Unfortunately, very few patients diagnosed with classical scimitar syndrome ful ll anatomic requirements for catheter-based rerouting of the anomalously draining vein. This case illustrates that interventional therapy should be considered and may be successful in selected patients with unusual anatomical variants.
Conclusion
Particular attention to anatomic variants of scimitar syndrome may identify novel interventional treatment options.
Con ict of Interest
The authors have no con ict of interest relevant to this publication.
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Interventional Treatment for Scimitar Syndrome