PMC5803982_01
Simultaneous two-dimensional and color Doppler echocardiographic images of the suprasternal view showing severe discrete narrowing of the confluence of the right-sided pulmonary veins (arrow). Individual right-sided pulmonary veins were unobstructed
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F1
Two-dimensional echocardiography with color Doppler revealed severe PA hypertension secondary to a severe stenosis involving the right common pulmonary vein [Figure 1].
(a) Angiogram in the right lower pulmonary vein depicting severe stenosis (arrow) of the right pulmonary venous confluence. (b) Right pulmonary venous confluence being dilated with a 3.5 mm x 15 mm sprinter coronary balloon (Medtronic, Inc., Minneapolis, MN, USA). Note the tight waist being formed in the center of the balloon. (c) Angiogram of the right common pulmonary venous confluence showing the presence of residual discrete narrowing. (d) Right common pulmonary venous confluence being dilated with an 8 mm x 3 cm Opta  Pro Balloon (Cordis, Miami, FL, USA). (e) Final angiogram showing well open segment of the right common pulmonary vein without any residual narrowing
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F2
After recording the pressures, diagnostic angiography with a hand injection of contrast performed in the right upper and lower pulmonary veins confirmed severe right pulmonary venous confluence stenosis [Figure 2a]. A 3.5 mm x 15 mm Sprinter coronary balloon (Medtronic, Inc., Minneapolis, MN, USA) was positioned across the stenosed segment and dilated [Figure 2b]. As repeat angiography revealed a residual waist [Figure 2c] the 0.014" angioplasty wire was then exchanged for a 0.035" J tipped regular guide wire and a 8 mms x 3 cm Opta  Pro Balloon (Cordis, Miami, FL, USA) was placed across the narrow segment, and two inflation deflation cycles were performed at 6 atmospheres pressure [Figure 2d]. Repeat angiography and pressure measurements revealed an excellent angiographic and hemodynamic result with the disappearance of the gradient and no residual stenosis [Figure 2e].
(a) Angiogram in the vertical vein showing a wide open vertical vein draining into the right superior vena cava through the left innominate vein. (b) Contrast injection through left hand peripheral vein after the deployment of the Amplatzer Vascular Plug II (St. Jude Medical, St. Paul, MN, USA). Angiogram shows unobstructed flow of the left subclavian vein into the innominate vein. (c) Final angiogram in the innominate vein after release of the device shows no residual flow across the vertical vein and unobstructed left internal jugular venous flow
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F3
Angiogram in the venous confluence now incorporated into the LA revealed a left VV measuring 9 mm draining freely into the left innominate vein [Figure 3a]. The catheter was adjusted to avoid blocking the entrance of the pulmonary veins distally and avoid interfering with the entry of the left jugular or subclavian vein into the innominate vein proximally [Figure 3b]. The final angiogram revealed near total occlusion of flow in the left VV and unobstructed flow from the left subclavian vein into the innominate vein [Figure 3c].
Simultaneous two-dimensional and color Doppler echocardiographic image of the suprasternal view at 1 month after procedure showing the vascular plug in position and absence of flow from the vertical vein into the innominate vein
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F4
Follow-up echocardiography a month after the procedure revealed a marked decrease in the PA pressure (mean = 20 mmHg), ventricular remodeling with involution of the right vertical (RV), and total absence of flow in the left VV [Figure 4].