53. Case Report: CTEPH & May Thurner Syndrome Temple University

Published: Sept. 7, 2020, 6:06 p.m.

CardioNerds\xa0(Amit Goyal\xa0&\xa0Daniel Ambinder) join Temple Cardiology Fellows (Anika Vaidy and Anne- Sophie LaCharite-Roberge) in Philadelphia, PA! They discuss a fascinating case of pulmonary hypertension secondary to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) associated with May Thurner syndrome and large uterine fibroids. Dr. Vaidya provides the E-CPR and message to applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.\xa0\n\n\n\n\n\n\n\n\n\nJump to: Patient summary - Case figures & media - Case teaching - Educational video - References - Production team\n\n\n\nEpisode graphic by Dr. Carine Hamo\n\n\n\n\n\n\n\nThe\xa0CardioNerds Cardiology Case Reports\xa0series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an\xa0\u201cExpert CardioNerd Perspectives & Review\u201d (E-CPR)\xa0for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.\n\n\n\nWe are teaming up with the\xa0ACC FIT Section\xa0to use the\xa0#CNCR episodes\xa0to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an\xa0E-CPR\xa0segment and a message from the program director.\n\n\n\nCardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza\n\n\n\n\n\n\n\n\n\n\n\nPatient Summary\n\n\n\nA\xa0woman in her late 40s\xa0with history of iron deficiency anemia, uterine fibroids, and recurrent DVTs/PEs for the past 6 years\xa0despite different\xa0anticoagulation regimens, presented with syncope and progressive dyspnea on exertion. Family history was negative for DVT/PE or\xa0hypercoagulable states. On exam, she was euvolemic. Labs were significant for anemia, a normal pro-BNP, and a negative extensive hypercoagulable workup. TTE showed interventricular\xa0systolic\xa0septal flattening, right ventricular outflow tract pulse wave doppler with mid-systolic notch, and shortened acceleration time consistent with elevated\xa0pulmonary artery pressure. A VQ scan showed bilateral right greater than left\xa0mis-matched\xa0perfusion defects. CT angiogram showed right greater than left chronic mural thrombus, correlating with pulmonary angiogram which showed severe proximal and mid-vessel disease on the right and distal disease on the left. RHC\xa0corroborated the diagnosis\xa0of chronic thromboembolic pulmonary hypertension (CTEPH) with mean pulmonary artery pressure of 41 and PVR of 5.2 Woods Units\xa0(WU).\xa0\xa0\n\n\n\nTo determine the etiology of her recurrent clots, a lower extremity venogram was performed and showed 80% stenosis of her left common iliac vein by the overlying right common iliac artery confirming May-Thurner syndrome. Lower extremity venogram also showed severe proximal stenosis of right iliac vein thought to be due to\xa0large uterine fibroids. Given her severe proximal and mid-vessel clot burden, she underwent pulmonary thromboendarterectomy with a subsequent drop in pulmonary vascular resistance to 1 WU.\xa0\xa0The etiology of DVTs and CTEPH was determined to be external compression related to both May-Thurner syndrome and uterine fibroids.\xa0To prevent future thromboembolic events, she underwent stenting of her left common iliac vein and hysterectomy. With these interventions, RV function returned to normal, and her symptoms completely resolved!\xa0\n\n\n\n\n\n\n\n\nCase Media\n\n\n\n\n\nA\n\n\n\nB\n\n\n\nC\n\n\n\nD\n\n\n\nE\n\n\n\nF\n\n\n\nG\n\n\n\nH\n\n\n\nI\n\n\n\nJ\nClick to enlarge ???