PMC9887705_01
(A) CT angiography: Puncture site identified (single headed, yellow arrow) 3-4cm from IVC/extracardiac Fontan anastomosis to allow Mitraclip device to enter systemic atrium (double headed, green arrow) approximately 4-5cm above mitral valve (MV- single line, pink). (B) 3-D printed model: Demonstration of anatomic relationship of extracardiac Fontan conduit relative to systemic atrium, IVC, and MV annulus (single line, pink). (C) 3-D printed model: Demonstration of extracardiac Fontan baffle relative to right pulmonary vein (RPV), left atrium (LA), left upper pulmonary vein (LPV) and left atrial appendage (LAA). (D) TEE: Commissural view of MV with color Doppler demonstrating severe MR (E) TEE: 3-D surgeon view (left atrial view) of MV with pulmonary artery (PA) stump used for anterior orientation. (F) TEE: Pulse wave Doppler of left upper pulmonary vein (LUPV) showing systolic flow reversal.
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A chest computed tomography (CT) scan with contrast was obtained of the patient's cardiac anatomy (Figure 1). From this scan, a 3D model was created to help guide planning (Figure 1). To allow for optimal function of the mitral clip delivery system, a Fontan conduit puncture was planned that would place the delivery system approximately 4-5 cm above the plane of the MV (Figure 1).
(A) Fluroscopy: contrast injection of extracardiac Fontan conduit. Puncture site identified 3-4 cm above the initial MitraClip XTW clip was manipulated into a medial position along A2/P2 and deployed after securing both the anterior and posterior leaflets within the clip. Following initial deployment, the regurgitation remained significant due to the broad span of the regurgitation jet and the myxomatous leaflets. In total, three MitraClips were placed to ensure a sufficient reduction in regurgitation. Two Abbott XTW clips were secured A2/P2, and 1 Abbott NTW clip was placed across A1/P1 (Figure 3). IVC/extracardiac Fontan anastomosis (single headed, yellow arrow) to allow the Mitraclip device to enter systemic atrium (double headed, green arrow) approximately 4-5 cm above the MV (single line, pink). (B) TEE: relationship of extracardiac Fontan conduit relative to systemic atrium. (C) Fluoroscopy: puncture through extracardiac Fontan conduit into the systemic atrium approximately 4-5 cm above MV with wire advanced to maintain position. (D) TEE: bi-plane image of BRK-1 Brockenbrough needle in extracardiac Fontan conduit (single headed, large red arrow) with puncture into systemic atrium.
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The puncture of the Fontan conduit into the systemic atrium was difficult and required the use of Bovie cautery and manual force, but was successfully performed and a wire was placed into the systemic ventricle (Figure 2).
(A) Fluoroscopy: balloon dilation of Fontan puncture site into systemic atrium to facilitate smooth passage of MitraClip system. (B) Fluroscopy: Mitraclip positioned under mitral valve. (C) Fluroscopy: release of first Mitraclip. (D) TEE: 3D left atrial view of Mitraclip (*) positioned over anterior (A2) and posterior (P2) leaflets with the PA stump used for device orientation. (E) TEE: Mitraclip (*) positioned under anterior and posterior leaflets of MV to grasp leaflets. (F) TEE: post-procedural commissural view of MV following placement of three Mitraclips (*) with colour Doppler demonstrating moderate, residual MR. (G) TEE: pulse wave Doppler of LUPV demonstrating normalized systolic pulmonary vein flow.
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Two Abbott XTW clips were secured A2/P2, and 1 Abbott NTW clip was placed across A1/P1 (Figure 3).
(A) Fluroscopy: three Mitraclip devices successfully deployed. Sheath positioned to deploy an Amplatzer Atrial Septal Occluder (ASO) device to close right to left shunt across Fontan conduit into systemic atrium. (B) Fluroscopy: release of 8 mm ASO device. (C) TEE: colour Doppler demonstrates right to left shunt from extracardiac Fontan baffle into systemic atrium causing significant desaturation in patient. (D) TEE: final position of the 8 mm ASO device (*) across Fontan conduit and systemic atrium.
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Amplatzer atrial septal defect occluder device was placed (Figure 4). Final mean Fontan pressure decreased to 16 mmHg following TEER intervention and final systemic atrial pressure was similarly reduced to 8 mmHg with a v-wave to 12 mmHg. A follow-up transthoracic echo showed a decrease in MR from severe to moderate regurgitation (Figure 5).
(A) TTE: pre-procedural commissural view of MV with colour Doppler demonstrating severe MR (B) TTE: 1-year follow-up scan of commissural view of MV with colour Doppler demonstrating mild to moderate residual MR with three Mitraclips.
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Amplatzer atrial septal defect occluder device was placed (Figure 4). Final mean Fontan pressure decreased to 16 mmHg following TEER intervention and final systemic atrial pressure was similarly reduced to 8 mmHg with a v-wave to 12 mmHg. A follow-up transthoracic echo showed a decrease in MR from severe to moderate regurgitation (Figure 5).