GORE® CARDIOFORM ASD occluder thrombus in a patient of rheumatoid arthritis occurred three days after deployment

Abstract

A 67-year-old female patient with rheumatoid arthritis that was well suppressed by medications except for steroids had catheter closure of the secundum atrial septal defect (ASD). After the administration of aspirin and intravenous heparin, a 32-mm of GORE® CARDIOFORM ASD occluder was successfully deployed. On postprocedural day 3, transthoracic echocardiography showed mobile echogenic mass of 9 mm on the right side of the device. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel was added. On the following day, the mobile mass was not visible. Even though the occurrence rate of device thrombus is not high, device thrombosis can become a major complication of device closure of ASD because it may cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.

1. Introduction

Thrombus formation after atrial septal defect (ASD) transcatheter closure is one of the major complications. Periprocedural heparinization and antiplatelet therapy are highly recommended because thrombus on the device can cause systemic or pulmonic embolization. We herein report an early-onset thrombus formation after deployment of GORE® CARDIOFORM ASD occluder (W.L. Gore & Associates, Inc., Flagstaff, Arizona) in an adult with rheumatoid arthritis despite adequate heparinization and single antiplatelet therapy.

2. Case report

A female patient was diagnosed with rheumatoid arthritis when she was 65 years old and was prescribed with iguratimod, folic acid, methotrexate, and bisphosphonate. The activity of rheumatoid arthritis was well suppressed by these medications. Two years later, computed tomography, which was performed to examine the condition of rheumatoid arthritis, revealed an enlargement of the pulmonary artery and significant right heart volume overload.

The patient had been diagnosed with a secundum ASD previously in her early forties, for which she underwent catheter examination. The pulmonary-to-systemic blood flow ratio (Qp/Qs) was miscalculated as 1.18 (instead of using oxygen saturation, the partial pressure of arterial oxygen was used for calculating the Qp/Qs; the correct Qp/Qs was 2.5), and she thus was excluded from being a candidate for ASD closure.

Repeat catheter examination revealed that Qp/Qs was 2.7, and she was referred to our institution for catheter closure of the ASD secundum when she was 67 years old.

Aspirin 100 mg was administrated a day before the procedure. In the catheter laboratory, transesophageal echocardiography (TEE) showed that ASD diameter was 15.4 mm, with enough rim around the defect. Immediately after obtaining venous access, heparin 5500 IU (in accordance with the patient's body weight of 53.5 kg) was administered. Balloon sizing using the stop-flow technique indicated a diameter of 18.1 mm by TEE, and a 32 mm of GORE® CARDIOFORM ASD occluder was selected. The activated clotting time measured just before insertion of a delivery sheath was 188 seconds, and an additional 3000 IU of heparin was administered. Initial deployment ended in failure when partial right arterial disk prolapsed into the left atrium across the posterior portion of the septum. The device was recaptured into the delivery sheath once and redeployed. After TEE and fluoroscopy showed adequate positioning of the device and no residual shunt, the device was released. The duration from insertion of the delivery sheath to the complete deployment of the device was 27 minutes.

On postprocedural day 1, transthoracic echocardiography (TTE) showed good device position and shape without residual shunt. There were no abnormal findings on the device. Blood examination showed a D-dimer level of 1.1 mg/L (normal rage, <1.0mg/L), and C-reactive protein (CRP) level of 0.65 mg/dl (normal rage, <0.14 mg/dL). Although she was afebrile, reddish eruptions, without itch, appeared on the upper extremities and trunk and oral antihistamine and steroid ointment were given.

On postprocedural day 3, TTE showed a mobile echogenic mass of 9 mm on the right side of the device (Fig. 1). The patient was asymptomatic, and chest X-ray revealed no abnormal findings. Blood examination showed the following results: white blood cell count, 5.4 × 10,000/μL (normal range, 3.3–8.6 × 10,000/μL), platelet count, 179 × 10,000k/μL (normal range, 158–348 × 10,000/μL), CRP, 3.3 mg/dL, and D‐dimer, 4.7 mg/L. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel of 75 mg and lansoprazole were added. Blood cultures were done to rule out infective endocarditis, and the patient was started on sulbactam/ampicillin despite being afebrile. Blood culture results were negative, and the antibiotics were discontinued. The mobile mass was not visible on the following day in TTE (Fig. 2). There was no relapse of thrombus, and heparin was tapered off. On postprocedural day 6, the patient was prescribed with aspirin 100 mg and clopidogrel 75mg and then discharged. Aspirin and clopidogrel were discontinued after 6 months of procedure without relapse of thrombus.

Fig. 1.

Transthoracic echocardiography on postoperative day 3 showed mobile echogenic mass of 9 mm (arrow) on the right atrial side of the device.

Fig. 2.

The mobile mass was not visible on the following day by transthoracic echocardiography.

3. Discussion

Device thrombosis is a major complication of device closure of ASD because it can cause systemic and/or pulmonary embolism. According to previous reports, the occurrence rate of device thrombus is quite low [[1], [2], [3]]. Device thrombosis as a periprocedural (occurring during the procedure) or postprocedural (until discharge) complication was observed in 3 (0.022 %) of 13,526 cases of device closure of ASD using different types of devices, including Amplatzer occluders, CardioSEAL, Starflex, Helex, Figulla etc., and the pooled estimated rate of device thrombosis as late complication during follow-up was 1.0 % [1]. Krumsdorf et al., using TEE, found 5 cases (1.2 %) of device thrombus in 407 patients of ASD [2]. They pointed out that the difference in occurrence rate depends on the type of devices. The difference between Amplatzer (0 %) and Cardio SEAL (7.1 %), and between Amplatzer (0 %) and StarFLEX (5.7 %) were significant, but the difference between Amplatzer (0 %) and Helex (0.8 %), a previous model of GORE® CARDIOFORM ASD occluder, was not significant at 4 weeks [2].

About the current model of GORE® CARDIOFORM ASD occluder, there is a report of a 14-year-old patient who experienced thrombus formation on this device 2 days after the procedure [3]. She had an asymptomatic COVID-19 infection, confirmed by positive polymerase chain reaction, 7 weeks before the ASD closure, and the thrombus formation was attributed to an ongoing COVID-19-related hypercoagulable state.

The ASSURED study, which included 125 cases and only used GORE® CARDIOFORM ASD occluder, reported 1 case (0.8 %) of device thrombus. In this study, thrombus on the right atrial disc was found 28 days after the closure and was resolved by medical therapy at 6 months [4]. Post-implantation antithrombotic therapy was mentioned for 117 cases out of 125, and the most frequent therapy was dual antiplatelet therapy (105 [89.7 %]), followed by single antiplatelet therapy (10 [8.5 %]) and oral anticoagulant therapy (1 [0.8 %]) [4]. The predominance of dual antiplatelet therapy appears to be a characteristic of the ASSURED study because single antiplatelet therapy is the most used after implantation of Amplatzer like self-centering occluder.

On the other hand, Oliva et al. reported that right disc thrombosis was observed in three out of 44 cases (6.8 %) [5]. They pointed out that the occurrence of thrombosis might be under recognized and emphasized the importance of clinical attention for this problem during a follow-up period. They hypothesized the mechanism of the thrombus formation. The metallic part of the device does not adhere strictly to the septum, avoiding the two e-PTFE surfaces of the right disc to remain in contact between them. As a result, the fenestration in the right disk membrane keeps open. The blood stasis inside the disc activates the coagulation cascade, resulting in thrombus formation and thrombus progression into the right chambers through the fenestration. And larger devices have higher risks of the thrombus formation because they give more chances to the development of a retrograde flow in the virtual cavity [5].

Even though our case's device was not large one, our case had a notable characteristic of concomitant rheumatoid arthritis without administration. Several reports pointed out that patients with rheumatoid arthritis are at increased risk of venous thromboembolism, including deep vein thrombosis and pulmonary embolism [[6], [7], [8]]. The etiology of thrombotic tendency in rheumatoid arthritis remains unclear but the inflammation in rheumatoid arthritis can possibly increase the thromboembolic risk [9]. Viktor et al. reported the evidence of a strong association between rheumatoid arthritis disease activity measured by the Disease Activity Score 28 for RA (DAS28) with erythrocyte sedimentation rate (ESR) and the subsequent risk of venous thromboembolism [10]. Although we did not check ESR of our case, DAS28 using CRP, which is equivalent to DAS28 ESR was calculated as 1.21 in our case, (no swelling and tenderness of 28 joints; CRP, 0.1 mg/dL; visual analog scale, 0), indicating rheumatoid arthritis remission. Meanwhile, Viktor et al. reported that patients in remission are at an increased risk compared with the general population [10]. Therefore, our case had a higher risk of thrombosis, at least compared with the general population, and the further increased risk of thrombosis due to the implantation of a foreign body in the heart cavity. We administrated aspirin to prevent thrombus formation, but dual antiplatelet therapy might be better for patients who have a higher risk of thrombosis.

4. Conclusion

We herein reported a case of device thrombus that occurred 3 days after ASD closure using GORE® CARDIOFORM ASD occluder. Thrombus formation on the device can cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.

CRediT authorship contribution statement

Naomi Nakagawa: Data curation, Investigation, Writing – original draft, Writing – review & editing. Yusuke Shigemitsu: Investigation. Kengo Okamoto: Data curation.

Patient consent statement

The authors confirm that patient consent forms have been obtained for this article.

Data availability statement

Data sharing is not applicable to this article as no data sets were generated or analyzed during the current study.

Funding sources

The authors declare that there is no Funding Source.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.