Thromboendarterectomy as Treatment in the Antiphospholipid Syndrome

Suzette W. Peng, MD; Jeanne P. Mitchell, MD

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Case Report

A 19-year-old female with a history of APS was admitted for evaluation of hemoptysis. She reported 100 mL of hemoptysis and a recent history of sore throat, nonproductive cough, and rhinitis. She also reported chronic dyspnea on exertion, fatigue, and intermittent right lower extremity swelling. She denied rash, photosensitivity, fever, arthritis, chest pain, or mucosal ulcerations. Daily medications included warfarin 5 mg and propranolol 20 mg, which was prescribed for migraine headache prophylaxis.

Past medical history was significant for a diagnosis of APS at age 14 after she presented with a right lower extremity deep venous thrombosis, pulmonary embolism, and high titer IgG aCL. She had no family history of a clotting disorder. Serologic tests performed with normal or negative findings included antinuclear antibody, anti-Ro, anti-La, anti-ribonucleoprotein, and anti-Smith antibodies. She was treated with warfarin without complication until her current admission 5 years later.

Physical examination revealed a blood pressure of 109/49 mm Hg, heart rate of 83 BPM and respirations 25/min. Mild jaundice of the skin and sclera were present. A cardiopulmonary examination revealed a widely split S2, prominent P2 and a holosystolic murmur at the left sternal border. There was mild splenomegaly and the lower leg circumference measured at the midcalf was greater on the right side (35 cm; left 34 cm).

Laboratory studies revealed a white blood cell count of 3.0 mm3 (normal 4.5-11.0 mm3) with a normal differential, hemoglobin 5.1 g/dL (normal 13.2-16.8 g/dL), and platelet count of 137 mm3 (normal 130-400 mm3). Mean corpuscular volume (MCV) was low normal at 82 fL (normal 82.0-101.0) and RDW was elevated at 21% (normal 11.5-14.5%). Prothrombin time (PT) was 41.4 seconds (normal 11.4-14.5 seconds), partial thromboplastin time (PTT) was 65.3 seconds (normal 24.6-34.6 seconds), and INR was 7.8. Transaminases were normal except for a total bilirubin of 3.3 mg/dL (normal 0.2-1.3 mg/dL) with a normal direct bilirubin. LA and aCL of the IgG 945 GPL (normal 0.0-12.3 GPL) and IgM 113 MPL (normal 0.0-12.9 MPL) subtypes were also positive. An arterial blood gas revealed an alveolar-arterial gradient of 58 mm Hg. A chest radiograph showed fullness in the aorta-pulmonary window and bilateral perihilar opacities. A transthoracic echocardiogram revealed moderate enlargement of the right atrium and ventricle, severe tricuspid regurgitation, and an estimated pulmonary artery systolic pressure of 80 mm Hg. Left ventricular size and function were normal. A ventilation-perfusion (V/Q) scan was remarkable for large defects involving the entire right middle and lower lobes, findings that were unchanged from a scan performed 5 years earlier. A spiral computed tomography (CT) angiography was ordered to evaluate for acute or chronic pulmonary embolism (PE) and showed marked dilation of the left sided pulmonary arteries and a large, organized filling defect in the right proximal pulmonary artery consistent with chronic thromboembolism (Figure 1). Filling defects of the right lower lobe branch of the pulmonary artery felt to be consistent with acute thrombus were also seen on the study.

CT scan of the chest from the 19-year-old female with APS and SLE who presented with severe pulmonary hypertension. The pulmonary arteries are markedly enlarged and a large, organized filling defect consistent with chronic thromboembolism of the right pulmonary artery is present (arrow).

No further episodes of hemoptysis occurred during the patient's hospitalization. Treatment was implemented with intravenous heparin and an inferior vena cava filter was placed, due to an abnormal right lower limb venography that showed evidence of old thrombus. Over the next week, the hemoglobin and platelet counts continued to decline. She was subsequently diagnosed with Evan's syndrome (autoimmune hemolytic anemia and thrombocytopenia). Other laboratory studies obtained were a positive ANA (1:640), positive SS-A, positive SS-B, and mildly depressed complement (C4) level of 10.2 mg/dL (normal 15-45 mg/dL), consistent with the diagnosis of SLE.

Treatment was implemented with 1 mg/kg/d oral prednisone and 400 mg/kg/d intravenous gamma-globulin, administered for 5 days, which resulted in an increase and stabilization in hemoglobin and platelet levels. On hospital day 12, azathioprine 100 mg/d and hydroxychloroquine 200 mg twice daily were initiated as steroid-sparing agents and oral prednisone was tapered. On hospital day 14, the patient was transferred to the University of California at San Diego for further evaluation and treatment of CTEPH.

A right heart catheterization revealed elevations in right ventricular pressure to 68 mm Hg (15-30 mm Hg), pulmonary artery pressures of 65/25 mm Hg (15-30/6-12 mm Hg), and pulmonary vascular resistance index of 327 dynes sec/cm5m2 (80-240 dynes sec/cm5m2). The pulmonary capillary wedge pressure was normal at 12 mm Hg. An arteriogram of the pulmonary arteries revealed total occlusion of the right interlobar artery and partial occlusion of the apical right and left upper lobe vessels and anteriomedial left lower lobe arteries. Pulmonary thromboendarterectomy was performed successfully without complications and an uneventful perioperative course. A postsurgical echocardiogram revealed mild right atrial and ventricular enlargement but with an improvement of her estimated PA systolic pressure to 32 mm Hg. A follow-up VQ scan was obtained and showed dramatic resolution of her previous defects (Figure 2). The patient was discharged on warfarin therapy 10 days following surgery. The patient reported continued improvement in her symptoms when she had follow-up at Walter Reed.

Ventilation-perfusion scans. Top image represents initial scan with large perfusion defect involving entire right middle and lower lobes. Bottom image represents post-thromboendarterectomy scan with resolution of previous defects.

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