Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension Due to Subclavian Vein Thrombosis From Bilateral First Rib Anomaly

Abstract

SESSION TITLE: Pulmonary Vascular Disease Cases ISESSION TYPE: Affiliate Case Report SlidePRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PMINTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved pulmonary emboli originating from a site of venous thrombosis. Development of CTEPH from subclavian vein (SCV) thrombosis due to thoracic outlet syndrome has been rarely reported and none yet associated with bilateral first rib fractures.CASE PRESENTATION: A 19-year-old previously healthy female noted to have acute dyspnea and hemoptysis was diagnosed with acute pulmonary embolism. She was treated with low molecular weight heparin then warfarin at therapeutic levels. However, developed recurrent pulmonary embolism with severe right heart failure 4 months after her initial embolic event. A large left SCV thrombosis associated with thoracic outlet syndrome was identified. She was also noted to have symmetric bilateral healed first rib fractures on chest x-ray without any relatable trauma history. This was deemed to be the likely underlying cause of her thoracic outlet syndrome. She underwent successful directed thrombolysis and angioplasty of her left SCV. Right heart catheterization (RHC) was performed due to persistent dyspnea, which showed mean pulmonary artery pressure (mPAP) of 37mmHg. A computed tomography angiogram revealed abnormalities consistent with CTEPH. Patient was referred to a pulmonary thromboendarterectomy (PTE) specialty center. At the time of referral, her functional status was WHO class III with repeated presyncopal episodes and exertional dyspnea. A ventilation/perfusion (V/Q) scan showed multiple mismatched perfusion defects consistent with CTEPH. A hypercoagulability evaluation was unremarkable. A RHC preoperatively showed mPAP of 47mmHg and a pulmonary vascular resistance (PVR) of 775 dyne-second/cm5. Pulmonary angiography confirmed the presence of surgically-accessible chronic thromboembolic disease. She underwent a successful bilateral PTE with postoperative PVR of 227 dyne-second/cm5 and mPAP of 20mmHg. Postoperative V/Q scan showed improvement in the areas of preoperative perfusion defects. She was discharged home after an uneventful postoperative course.DISCUSSION: This illustrates an unusual case of CTEPH from upper extremity thrombosis associated with first rib anomaly. Also, hemodynamic and clinical improvement can be achieved with PTE for CTEPH due to this condition.CONCLUSIONS: Consideration for first rib anomaly should be included as part of evaluation of CTEPH secondary to SCV thrombosis.Reference #1: Kim N et al. Chronic Thromboembolic Pulmonary Hypertension. J Am Coll Cardiol. 2013;62:S92-9.DISCLOSURE: The following authors have nothing to disclose: Stephanie Shin, Peter Fedullo, Theodore Frank, William AugerNo Product/Research Disclosure Information SESSION TITLE: Pulmonary Vascular Disease Cases I SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PM INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved pulmonary emboli originating from a site of venous thrombosis. Development of CTEPH from subclavian vein (SCV) thrombosis due to thoracic outlet syndrome has been rarely reported and none yet associated with bilateral first rib fractures. CASE PRESENTATION: A 19-year-old previously healthy female noted to have acute dyspnea and hemoptysis was diagnosed with acute pulmonary embolism. She was treated with low molecular weight heparin then warfarin at therapeutic levels. However, developed recurrent pulmonary embolism with severe right heart failure 4 months after her initial embolic event. A large left SCV thrombosis associated with thoracic outlet syndrome was identified. She was also noted to have symmetric bilateral healed first rib fractures on chest x-ray without any relatable trauma history. This was deemed to be the likely underlying cause of her thoracic outlet syndrome. She underwent successful directed thrombolysis and angioplasty of her left SCV. Right heart catheterization (RHC) was performed due to persistent dyspnea, which showed mean pulmonary artery pressure (mPAP) of 37mmHg. A computed tomography angiogram revealed abnormalities consistent with CTEPH. Patient was referred to a pulmonary thromboendarterectomy (PTE) specialty center. At the time of referral, her functional status was WHO class III with repeated presyncopal episodes and exertional dyspnea. A ventilation/perfusion (V/Q) scan showed multiple mismatched perfusion defects consistent with CTEPH. A hypercoagulability evaluation was unremarkable. A RHC preoperatively showed mPAP of 47mmHg and a pulmonary vascular resistance (PVR) of 775 dyne-second/cm5. Pulmonary angiography confirmed the presence of surgically-accessible chronic thromboembolic disease. She underwent a successful bilateral PTE with postoperative PVR of 227 dyne-second/cm5 and mPAP of 20mmHg. Postoperative V/Q scan showed improvement in the areas of preoperative perfusion defects. She was discharged home after an uneventful postoperative course. DISCUSSION: This illustrates an unusual case of CTEPH from upper extremity thrombosis associated with first rib anomaly. Also, hemodynamic and clinical improvement can be achieved with PTE for CTEPH due to this condition. CONCLUSIONS: Consideration for first rib anomaly should be included as part of evaluation of CTEPH secondary to SCV thrombosis. Reference #1: Kim N et al. Chronic Thromboembolic Pulmonary Hypertension. J Am Coll Cardiol. 2013;62:S92-9. DISCLOSURE: The following authors have nothing to disclose: Stephanie Shin, Peter Fedullo, Theodore Frank, William Auger No Product/Research Disclosure Information