PULMONARY VEIN STENOSIS: A SERIOUS AND UNDERDIAGNOSED COMPLICATION OF PULMONARY VEIN ISOLATION THERAPY

Abstract

SESSION TITLE: Thrombosis Jamboree: Rare and Unique CasesSESSION TYPE: Rapid Fire Case ReportsPRESENTED ON: 10/19/2022 12:45 pm - 1:45 pmINTRODUCTION: Radiofrequency catheter ablation (RFCA) for pulmonary vein isolation (PVI) therapy is a widely utilized interventional technique for drug-refractory atrial fibrillation. Pulmonary vein stenosis (PVS), defined as ≥ 20 % reduction in baseline pulmonary venous diameter, is a rare complication of PVI therapy with an incidence rate of 0 - 42 % [1].CASE PRESENTATION: A 68-year-old asymptomatic man with history of hypertension, diabetes mellitus type II, cerebrovascular accident, paroxysmal atrial fibrillation (CHA₂DS₂-VASc score 5), PVI therapy with RFCA and anticoagulation intolerance was referred for left atrial appendage closure. The patient underwent transesophageal echocardiogram (TEE) for left atrial appendage evaluation. TEE revealed a high velocity jet emanating from the left superior pulmonary vein. Continuous Wave Doppler across the vein recorded a velocity of 1.1 cm/s, while Pulse Wave Doppler prior to the outflow showed a velocity of 0.3 cm/s. CT angiography (CTA) confirmed left superior pulmonary vein stenosis. The other pulmonary veins were unaffected. Pulmonary artery pressure was not elevated. This patient eventually underwent successful left atrial appendage occlusion.DISCUSSION: The severity of PVS and its associated symptoms are directly dependent upon the degree of reduction in pulmonary venous diameter, further classified as mild (20 - 50 % reduction), moderate (50 - 69 % reduction) and severe (≥ 70 % reduction) [2]. Accurate diagnosis of PVS from other disease entities is complicated by the presence of non-specific symptoms (dyspnea on exertion, chest pain, cough, hemoptysis, hypoxemia), vague radiographic manifestations (multifocal opacities, nodular lesions, unilateral effusions, interstitial septal thickening), and overlapping histologic features (interlobular and alveolar septal thickening, arteriopathic changes, hemosiderosis) [3]. Although most patients with PVS remain asymptomatic, management with balloon angioplasty or large stent implantation (> 10 mm) has shown improved clinical outcomes in severe symptomatic cases of PVS. Given the high incidence rate of restenosis, it is strongly recommended that these patients are closely followed with routine CT imaging for prompt intervention to prevent total occlusion if indicated.CONCLUSIONS: PVS is an extremely serious and rare complication of PVI therapy with RFCA. Wide variability in symptomatology of PVS is reflective of the underlying degree of luminal narrowing and number of pulmonary veins affected. Close post-procedural follow-up care and routine monitoring with diagnostic imaging are crucial steps in identifying PVS in the early stages for better clinical outcomes.Reference #1: Edriss H, Denega T, Test V, Nugent K. Pulmonary vein stenosis complicating radiofrequency catheter ablation for atrial fibrillation: A literature review. Respir Med. 2016;117:215-222. doi:10.1016/j.rmed.2016.06.014Reference #2: Saad EB, Rossillo A, Saad CP, et al. Pulmonary vein stenosis after radiofrequency ablation of atrial fibrillation: functional characterization, evolution, and influence of the ablation strategy. Circulation. 2003;108(25):3102-3107. doi:10.1161/01.CIR.0000104569.96907.7FReference #3: Linga KR, Khoor A, Phelan JA, Mira-Avendano I. Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. Case Rep Pulmonol. 2015;2015:290391. doi:10.1155/2015/290391DISCLOSURES: Consultant relationship with Siemens Medical Please note: 03/01/2021-Current Added 03/30/2022 by Nathaniel Dittoe, value=Grant/Research SupportNo relevant relationships by Osama HallakNo relevant relationships by Akruti PrabhakarNo relevant relationships by EricsonJohn TorralbaNo relevant relationships by Damian Valencia SESSION TITLE: Thrombosis Jamboree: Rare and Unique Cases SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Radiofrequency catheter ablation (RFCA) for pulmonary vein isolation (PVI) therapy is a widely utilized interventional technique for drug-refractory atrial fibrillation. Pulmonary vein stenosis (PVS), defined as ≥ 20 % reduction in baseline pulmonary venous diameter, is a rare complication of PVI therapy with an incidence rate of 0 - 42 % [1]. CASE PRESENTATION: A 68-year-old asymptomatic man with history of hypertension, diabetes mellitus type II, cerebrovascular accident, paroxysmal atrial fibrillation (CHA₂DS₂-VASc score 5), PVI therapy with RFCA and anticoagulation intolerance was referred for left atrial appendage closure. The patient underwent transesophageal echocardiogram (TEE) for left atrial appendage evaluation. TEE revealed a high velocity jet emanating from the left superior pulmonary vein. Continuous Wave Doppler across the vein recorded a velocity of 1.1 cm/s, while Pulse Wave Doppler prior to the outflow showed a velocity of 0.3 cm/s. CT angiography (CTA) confirmed left superior pulmonary vein stenosis. The other pulmonary veins were unaffected. Pulmonary artery pressure was not elevated. This patient eventually underwent successful left atrial appendage occlusion. DISCUSSION: The severity of PVS and its associated symptoms are directly dependent upon the degree of reduction in pulmonary venous diameter, further classified as mild (20 - 50 % reduction), moderate (50 - 69 % reduction) and severe (≥ 70 % reduction) [2]. Accurate diagnosis of PVS from other disease entities is complicated by the presence of non-specific symptoms (dyspnea on exertion, chest pain, cough, hemoptysis, hypoxemia), vague radiographic manifestations (multifocal opacities, nodular lesions, unilateral effusions, interstitial septal thickening), and overlapping histologic features (interlobular and alveolar septal thickening, arteriopathic changes, hemosiderosis) [3]. Although most patients with PVS remain asymptomatic, management with balloon angioplasty or large stent implantation (> 10 mm) has shown improved clinical outcomes in severe symptomatic cases of PVS. Given the high incidence rate of restenosis, it is strongly recommended that these patients are closely followed with routine CT imaging for prompt intervention to prevent total occlusion if indicated. CONCLUSIONS: PVS is an extremely serious and rare complication of PVI therapy with RFCA. Wide variability in symptomatology of PVS is reflective of the underlying degree of luminal narrowing and number of pulmonary veins affected. Close post-procedural follow-up care and routine monitoring with diagnostic imaging are crucial steps in identifying PVS in the early stages for better clinical outcomes. Reference #1: Edriss H, Denega T, Test V, Nugent K. Pulmonary vein stenosis complicating radiofrequency catheter ablation for atrial fibrillation: A literature review. Respir Med. 2016;117:215-222. doi:10.1016/j.rmed.2016.06.014 Reference #2: Saad EB, Rossillo A, Saad CP, et al. Pulmonary vein stenosis after radiofrequency ablation of atrial fibrillation: functional characterization, evolution, and influence of the ablation strategy. Circulation. 2003;108(25):3102-3107. doi:10.1161/01.CIR.0000104569.96907.7F Reference #3: Linga KR, Khoor A, Phelan JA, Mira-Avendano I. Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. Case Rep Pulmonol. 2015;2015:290391. doi:10.1155/2015/290391 DISCLOSURES: Consultant relationship with Siemens Medical Please note: 03/01/2021-Current Added 03/30/2022 by Nathaniel Dittoe, value=Grant/Research Support No relevant relationships by Osama Hallak No relevant relationships by Akruti Prabhakar No relevant relationships by EricsonJohn Torralba No relevant relationships by Damian Valencia