SESSION TITLE: Pulmonary Vascular Case Report Posters
SESSION TYPE: Case Report Poster
PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM
INTRODUCTION: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital condition characterized by extensive venous malformations and soft tissue and bone hypertrophy. KTWS predisposes to hypercoagulable states, including venous thromboembolism (VTE) and pulmonary embolism (PE). The incidence of PE ranges from 14-22 %; however, recurrent massive PE is unusual. We report the case of an adult female with KTWS who developed recurrent massive PE and was successfully treated with thrombolytic therapy.
CASE PRESENTATION: A 36-year-old female with history of KTWS and previous massive PE one year prior, noncompliant with anticoagulation therapy, presented with sudden onset pleuritic chest pain, dyspnea and diaphoresis. At presentation blood pressure was 80/64mmHg, pulse was 150bpm, respiratory rate was 22 and SpO2 on room air was 87%. Physical examination revealed significant swelling of the left leg with prominent venous irregularities. CT pulmonary angiogram revealed filling defect in both main pulmonary arteries with no pulmonary infarcts. 2D echocardiogram showed increased right ventricular size and bowing of the septum into the left ventricle with elevated PA pressure (64 mmHg). Thrombolytic therapy was administered, hemodynamic improvement was noted over the next 24 hours. Repeat 2D echocardiogram showed resolution of right ventricular overload. The patient was advised to continue lifelong anticoagulation therapy.
DISCUSSION: The common pulmonary manifestations of KTWS include thromboembolic phenomena, pulmonary venous varicosities, pulmonary lymphatic obstruction and cavernous hemangiomas of the pleura. Missed diagnosis of these KTWS associated complications can potentially be life threatening. Due to the rarity of this disease, there are no recommended guidelines for the management of thromboembolic phenomena. However, aggressive therapeutic anticoagulation and early placement of IVC filter have been suggested.
CONCLUSIONS: This rare congenital condition and associated complications can at times be difficult to manage. The management of prophylaxis for PE recurrence is an area that needs special attention.
Reference #1: Adriane Gianlupi, MD; Richart W. Harper, MD; Denis M. Dwyre, MD; Gregory P. Marelich, MD, FCCP, "Recurrent Pulmonary Embolism Associated With Klippel-Trenaunay-Weber Syndrome" Chest. 1999;115(4):1199-1201. doi:10.1378/chest.115.4.1199
Reference #2: Erin E. Huiras, MD, Cheryl J. Barnes, MD, Lawrence F. Eichenfield, MD, Andrew N. Pelech, MD, Beth A. Drolet, MD, "Pulmonary Thromboembolism Associated With Klippel-Trenaunay Syndrome" Published online September 15, 2005 PEDIATRICS Vol. 116 No. 4 October 1, 2005 pp. e596 -e600
Reference #3: Mikula, N, Gupta, SM, Miller, M, et al Klippel-Trenaunay-Weber syndrome with recurrent pulmonary embolism.Clin Nucl Med1991;16,253-255
DISCLOSURE: The following authors have nothing to disclose: Abhay Vakil, Khalid Sherani, Viral Patel, Kelly Cervellione, Mohammed Babury
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