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Pulmonary Vascular Disease |

Klippel-Trenaunay Syndrome, Pregnancy, and Pulmonary Embolism: When prophylaxis is not enough!

Vishisht Mehta, MBBS; Karishma Bhatia, MBBS; Vimalkumar Veerappan Kandasamy, MBBS; Zachary DePew, MD
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Creighton University, Omaha, NE


Chest. 2015;148(4_MeetingAbstracts):995A. doi:10.1378/chest.2246403
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Abstract

SESSION TITLE: Pulmonary Vascular Diseases Student/Resident Cases

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Tuesday, October 27, 2015 at 04:30 PM - 05:30 PM

INTRODUCTION: Klippel-Trenaunay Syndrome (KTS) is a rare syndrome comprised of capillary, venous & lymphatic malformations associated with bony/soft-tissue overgrowth affecting a limb or limbs [1]. Deep veinous thrombosis (DVT) is a known potential complication of KTS, but pulmonary embolism (PE) is relatively rare [2]. Herein we describe a postpartum patient with KTS who developed PE despite prophylactic measures.

CASE PRESENTATION: A 39 year old pregnant woman with history of KTS and associated pelvic varicosities delivered her child by emergent cesarean section following placental abruption due to a retroplacental clot. Her history included prior pulmonary embolism (PE) at age 16 with subsequent placement of an IVC filter. Her antenatal course was uncomplicated and she received a low dose aspirin and 40 mg low molecular weight heparin (LMWH), both daily, throughout her pregnancy. LMWH was resumed 6 hours after delivery. On post-operative day 2 she complained of acute severe shortness of breath, and was tachycardic with an oxygen saturation of 94% and non-tender lower limbs. A chest CT angiogram revealed extensive bilateral PE’s. ECG showed a classic S1Q3T3 pattern. Transthoracic echocardiogram showed RV dilatation with reduced systolic function and positive McConnell’s sign. Troponin I was elevated at 1.27 ng/mL (normal <= 0.04 ng/mL). LMWH was increased to therapeutic dosing and warfarin therapy was begun. Upper and lower extremity venous duplex studies were completed and negative for DVT. The patient remained hemodynamically stable and transitioned to the outpatient setting.

DISCUSSION: KTS and pregnancy are both risk factors for DVT/PE. The coexistence of these two prothrombotic states can cause the PE to break through the prophylaxis. PE appears to be rare in KTS [2,3] and reports of KTS complicated by pregnancy are scarce. It has been suggested that aspirin and heparin use during pregnancy in patients with KTS may reduce thromboembolic disease [1]. This did not bear out in our patient, however, Rebarber et al [1] described a case in which therapeutic LMWH was used during pregnancy without thromboembolic complications. Additionally, our patient’s IVC filter was not protective, potentially due to clot bypassing it via the collateral vasculature that develops in the vascular malformations of KTS.

CONCLUSIONS: Patients with KTS need lifelong anticoagulation and/or an IVC filter. Those with additional risk factors predisposing them to thromboembolic disease should have these issues duly addressed and should be monitored closely for DVT/PE.

Reference #1: Rebarber A, Roman AS, Roshan D, Blei F. Obstetric management of Klippel-Trenaunay syndrome. Obstet Gynecol. 2004;104:1205-8

Reference #2: Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. KlippelTrenaunay Syndrome: Its Spectrum and Management. Mayo Clinic Proc 1998; 73: 28-36

Reference #3: Baskerville PA. Thromboembolic disease and congenital venous abnormalities. Phlebologie 1987; 40: 531-6

DISCLOSURE: The following authors have nothing to disclose: Vishisht Mehta, Karishma Bhatia, Vimalkumar Veerappan Kandasamy, Zachary DePew

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