Not every TIA is a PFO – the importance of other shunts
Presenter
Natalie E Caryl, University of Arizona College of Medicine – Tucson, Tucson, AZ
Natalie E Caryl1, Andrew W Hoyer, MD2, Kavitha Yaddanapudi, MD2, Swati Chandra1, Manuel Camarena, RN3, Omar Z Meziab, MD2, Arka Chatterjee, M.D., FSCAI1 and Mike D. Seckeler, M.D., FSCAI2, (1)University of Arizona College of Medicine – Tucson, Tucson, AZ, (2)The University of Arizona, Tucson, AZ, (3)Banner University Medical Center - Tucson, Tucson, AZ
Title
Not every TIA is a PFO – the importance of other shunts
Introduction
We present a patient with Erdheim-Chester Disease (ECD) who developed transient ischemic attacks (TIAs) initially thought to be secondary to shunting through a patent foramen ovale (PFO). ECD is a rare multisystem non-Langerhans histiocytic disorder associated with significant cardiac involvement. This can include right atrial pseudotumor, infiltration of the right atrioventricular sulcus and obstruction of large vessels.
Clinical Case
A 39-year-old female with long-standing ECD was referred for palliative transcatheter treatment of SVC stenosis secondary to external tumor compression and chronic indwelling catheters. She was also found to have TIAs and a transthoracic echocardiogram bubble study suggested atrial level shunting, so concomitant PFO closure was also planned. A TEE showed significant SVC obstruction from tumor and a bubble study with injection directly in the right atrium (RA) showed no shunting and no PFO. No intervention was performed. A cardiac CT confirmed complete SVC occlusion and showed multiple venous collaterals from the innominate vein decompressing to the pulmonary veins as the likely source of right to left shunting. Repeat catheterization was performed with femoral and left IJ venous access. A bubble study from the IJ showed rapid return of bubbles to the left atrium, confirming the source of shunting. Angiography revealed severe innominate vein stenosis and complete SVC occlusion with decompression via a markedly dilated azygous vein with retrograde flow and numerous venous collaterals to the pulmonary veins and left atrium. The largest collateral was embolized with a combination of plugs and coils. It was felt that recanalization of the SVC would stop shunting to the decompressing collaterals by re-establishing normal venous return to the heart. A long sheath with a glide catheter, Corsair® microcatheter (Ashai Intecc USA, Inc, Irvine, CA) and Mongo® wire (Ashai) were positioned in the SVC with a snare in the RA. The stiff end of the wire was carefully advanced out of the Corsair toward the RA, followed by the Corsair; position was confirmed angiographically. An exchange length coronary wire was advanced through the Corsair and snared to pull the glide catheter into the RA. The wire and Corsair were exchanged for an Amplatz Super Stiff™ wire (Boston Scientific, Marlborough, MA) and the wire externalized to make a veno-venous loop. The tract was serially dilated with ultra-high pressure balloons and then a 4010 Palmaz XL stent (Cordis, Miami Lakes, FL) placed to maintain patency. The innominate vein was treated with a 3910 Palmaz Genesis stent (Cordis). Final angiography demonstrated a widely patent SVC and marked reduction in flow to the decompressing veins; repeat bubble study from the IJ was negative. She was discharged the next day with no complications and no further TIAs at three-month follow-up. A CT showed stent patency with a marked decrease in the size of the remaining collaterals.
Discussion
This case highlights the importance of considering other potential sources of shunting as causes of paradoxical emboli as well as the value of advanced imaging when planning complex transcatheter interventions.