Occurrence of paradoxical arterial embolism may cause the ﬁrst symptoms in patients with a coexisting hypercoagulable state and patent foramen ovale (PFO). This can result in signiﬁcant morbidity and mortality depending on the location of the embolism. The risks and beneﬁts of closure of small PFOs have not been well elucidated in prior studies. We describe a patient with a history of Factor V Leiden heterozygosity who presented with left arm pain secondary to arterial embolism. The patient was a 51-year-old male who initially presented to the emergency department after awaking from sleep with progressive, severe, burning left arm pain. He had also noted intermittent shortness of breath over the 2 weeks prior to admission. Temperature was 97.4 F, pulse 86, respiratory rate 20 and blood pressure 121/87. Oxygen saturation was 94% on supplemental oxygen. He had a cool left upper extremity and the patient described subjective paresthesias in this extremity. Left radial pulse was difﬁcult to palpate. Physical exam was otherwise unremarkable. Troponin I was mildly elevated at 0.217 ng/l. White blood cell count was 11.8 and INR 1.1. EKG showed sinus tachycardia with non-speciﬁc T abnormalities in the anterior leads. His past medical history was notable for only hypertension and hyperlipidemia. Current recommendation is for antiplatelet or anticoagulation for those with hypercoaguable states who suffer a stroke; there is currently no absolute indication for closure device. We describe the case of a 51-year-old male who had presented with left arm pain and shortness of breath. The computed tomography (CT) angiography of chest showed pulmonary emboli with heavy clot burden bilaterally. Heparin was started, but patient was found to have occlusion along large arteries of the left arm. Emergent left axillary, brachial, radial and ulnar embolectomy for acute critical arm ischemia were performed. The transthoracic echocardiogram done the next day with bubble study was positive for patent foramen ovale. Hypercoaguability showed factor V Leiden heterozygosity. Decision was made for the patient to initiate long-term anticoagulation with rivaroxaban and closure was performed. Patient was advised that closure is off label but opted to proceed with closure in light of hypercoaguable state. Chest X-ray did not show any process that could explain the emboli extending from the right and left main pulmonary arter- acute hypoxic episode and patient sounded clear on lung exam. ies into the segmental and subsegmental branches with heavy Hence, computed tomography (CT) angiography of the chest was clot burden bilaterally (Fig. 1). Transthoracic echocardiogram performed with higher suspicion for pulmonary embolism (PE). (TTE) was done showing ejection fraction of 65% and severely The CT angiography showed multiple large bilateral pulmonary elevated pulmonary artery systolic pressure with right ventricular Received: September 20, 2017. Revised: November 25, 2017. Accepted: December 13, 2017 © The Author(s) 2017. Published by Oxford University Press. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact email@example.com Downloaded from https://academic.oup.com/omcr/article-abstract/2018/3/omx105/4935135 by Ed 'DeepDyve' Gillespie user on 16 March 2018 90 A. Parikh and T.P. Vacek Figure 1: CT angiography of the chest was done showing multiple large bilateral pulmonary emboli extending from the right and main pulmonary arteries into the segmental and subsegmental branches with heavy clot burden bilaterally. Arrows show areas of PE. Figure 2: TTE was done showing ejection fraction of 65% and improved pulmonary artery systolic pressure with RVSP of 46 mmHg after 3 days of therapy. systolic pressure (RVSP) of 72 mmHg (Fig. 2). There was no free- was not felt to be a candidate for thrombectomy since he was not ﬂoating cardiac thrombi detected, and echo did not show concomi- showing hemodynamic instability characteristic of massive PE, so tant septal ﬂattening; moreover, McConnell’s sign was not present therapeutic heparin drip was initiated and the patient was admit- Workup thus far was discussed with pulmonology and the patient ted to the intensive care unit for submassive PE. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/3/omx105/4935135 by Ed 'DeepDyve' Gillespie user on 16 March 2018 PFO closure in high-risk patient 91 Seven hours after initial presentation, the patient continued of multiple coagulation events, prospect for embolism was having severe left arm pain. It was initially thought that the endorsed. He was sent for ultrasound of upper and lower pain was musculoskeletal in nature; however, after discovery extremities. Ultrasound of bilateral lower extremities revealed acute deep venous thrombosis within the left posterior tibial and peroneal veins. Venous ultrasound of the upper extrem- ities showed no evidence of clot. Arterial ultrasound of the left upper extremity showed occlusion of the left proximal to mid- brachial artery with reconstitution of ﬂow at the level of the distal brachial artery. Vascular surgery was consulted and the patient underwent emergent left axillary, brachial, radial and ulnar embolectomy for acute critical arm ischemia (Fig. 3). TREATMENT A repeat TTE was done the next day and bubble study was posi- tive for a patent foramen ovale (PFO). Cardiology and cardio- thoracic surgery were consulted regarding the need for open heart surgery versus percutaneous closure of the PFO. It was decided that the patient initiate long-term anticoagulation and that percutaneous closure could be performed at a later date. Hematology was consulted and the patient underwent a hyper- coagulability workup which later was remarkable for Factor V Leiden heterozygosity. The patient opted for treatment with rivaroxaban over warfarin, and therefore rivaroxaban 15 mg twice daily was started with plans to adjust dosing to 20 mg daily in three weeks. Another TTE was done on admission Day Figure 3: Patient underwent emergent left axillary, brachial, radial and ulnar embolectomy for acute critical arm ischemia and sample of clot is shown. 3 showing improvement of pulmonary artery systolic pressure Figure 4: One week after discharge, the patient underwent further evaluation with TEE showing a very large PFO with right to left shunt by bubble study. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/3/omx105/4935135 by Ed 'DeepDyve' Gillespie user on 16 March 2018 92 A. Parikh and T.P. Vacek Figure 5: A balloon was placed across the PFO for size measurement and the decision made to deploy a 25 mm cribriform Amplatzer PFO occluder device. Figure 6: Placement was conﬁrmed by TEE showing negative bubble study. with RVSP of 46 mmHg. Pulmonology recommended against OUTCOME AND FOLLOW-UP inferior vena cava ﬁlter placement as patient had rapid clinical One week after discharge, the patient underwent further evalu- improvement with systemic anticoagulation. After 2 more days ation with transesophageal echocardiogram (TEE) showing a of observation and treatment, the patient was discharged from very large PFO with right to left shunt by bubble study (Fig. 4). the hospital with plans for outpatient follow-up. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/3/omx105/4935135 by Ed 'DeepDyve' Gillespie user on 16 March 2018 PFO closure in high-risk patient 93 During outpatient cardiology follow-up, there was a discussion LEARNING POINTS with the patient regarding the very large PFO/atrial septal In summary, paradoxical arterial embolism via PFO should be defect (ASD) and his high risk of further embolic event. He was determined to be a possibility in a patient presenting with deep advised that closure is off-label and that the latest trials did not venous thrombosis and/or PE who also have evidence of an arter- show superiority with closure versus anticoagulation. Given his ial embolic event. Testing may include CT angiogram, venous/ high risk of embolic event, and because he was a scuba diver arterial ultrasound and echocardiography with bubble study. with increased risk for the decompression sickness, he opted to Treatment options include systemic anticoagulation, IVC ﬁlter proceed with closure. placement, percutaneous closure of PFO, surgical closure of PFO Three weeks later, patient underwent percutaneous closure or no closure of PFO depending on the patient’s presentation, his- of the PFO with intraoperative ﬂuoroscopy guidance. A balloon tory, and consideration of indications/contraindications. Pending was placed across the PFO for size measurement and the deci- further randomized trial data, percutaneous PFO closure may be sion made to deploy a 25 mm cribriform Amplatzer PFO occlu- considered as for prevention of paradoxical arterial embolism. der device (Fig. 5). This device consists of two disks, the ﬁrst deployed on the left side of the PFO and then the second along the right side of the PFO. Placement was conﬁrmed by TEE CONFLICT OF INTEREST STATEMENT (Fig. 6). Patient was started on dual antiplatelet therapy with None declared. aspirin and plavix for 3 months and advised to continue sys- temic anticoagulation with rivaroxaban. CONSENT DISCUSSION Written consent was obtained from the patient for publication of this article. This patient was found to have a hypercoagulable state from Factor V Leiden heterozygosity. This predisposed him to the development of lower extremity deep vein thrombosis and REFERENCES large bilateral PE . The development of arterial embolus to the left arm was made possible by a large PFO and has been 1. Ridker PM, Hennekens CH, Selhub J, Miletich JP, Malinow MR, cited several times in the literature . Stampfer MJ. Interrelation of hyperhomocyst(e)inemia, factor Paradoxical arterial embolism has been associated with PFO, V Leiden, and risk of future venous thromboembolism. atrial septal aneurysm (ASA), and ostium secundum ASD. Circulation 1997;95:1777–82. Recommended therapy differs depending on the type of anom- 2. Guo S, Roberts I, Missri J. Paradoxical embolism, deep vein aly present and its characteristics that can predispose the thrombosis, pulmonary embolism in a patient with patent patient to increased risk of arterial embolism events. PFO foramen ovale: a case report. J Med Case Rep 2007;1:104. occurs in 25–30% of the general population. It is thought to be 3. Landzberg MJ, Khairy P. Indications for the closure of patent an innocent bystander in many patients with stroke; however, foramen ovale. Heart 2004;90:219–24. in the younger population (<55 years of age), it could be the 4. Kernan WN, Ovbiagele B, Black HR, Bravata DM, Chimowitz MI, underlying etiology for recurrent arterial embolus events, such Ezekowitz MD, et al. Guidelines for the prevention of stroke in as cryptogenic stroke or peripheral embolism . patients with stroke and transient ischemic attack: a guideline for Currently, the usefulness of percutaneous closure of PFO healthcare professionals from the American Heart Association/ versus surgical closure or medical therapy alone remains American Stroke Association. Stroke 2014;45:2160–236. unclear. Further complicating factors to decide on treatment 5. Furlan AJ, Reisman M, Massaro J, Mauri L, Adams H, Albers include hypercoaguable states that were not part of inclusion GW, et al. Closure or medical therapy for cryptogenic stroke criteria when evaluating for efﬁcacy. The current recommen- with patent foramen ovale. N Engl J Med 2012;366:991–9. dation is for antiplatelet or anticoagulation for those with 6. Meier B, Kalesan B, Mattle HP, Khattab AA, Hildick-Smith D, hypercoaguable states who suffer a stroke . Three studies Dudek D, et al. Percutaneous closure of patent foramen ovale (CLOSURE I trial, PC trial and RESPECT trial) have failed to in cryptogenic embolism. N Engl J Med 2013;368:1083–91. demonstrate that percutaneous PFO closure relates signiﬁ- 7. Carroll JD, Saver JL, Thaler DE, Smalling RW, Berry S, cant reduction in primary endpoint (stroke, TIA, peripheral MacDonald LA, et al RESPECT Investigators. Closure of patent embolism, etc.) outcomes [5–7]. However, extended data from foramen ovale versus medical therapy after cryptogenic the RESPECT trial after 10 years have shown some beneﬁtto stroke. N Engl J Med 2013;368:1092–100. closure with 54% relative risk reduction for recurrent crypto- 8. Kasner SE, Thomassen L, Søndergaard L, Rhodes JF, Larsen CC, genic stroke versus those assigned to medical management Jacobson J. Patent foramen ovale closure with GORE HELEX or of anticoagulation. Moreover, two more recent trials have CARDIOFORM Septal Occluder vs. antiplatelet therapy for shown some beneﬁt to closure of PFO closure with aspirin in reduction of recurrent stroke or new brain infarct in patients those with a true cryptogenic stroke: REDUCE and CLOSE [8, with prior cryptogenic stroke: Design of the randomized Gore 9]. Additional randomized trial data are still needed to deter- REDUCE Clinical Study. Int J Stroke 2017;12:998–1004. mine the effectiveness of percutaneous PFO closure as com- 9. Mas JL, Derumeaux G, Guillon B, Massardier E, Hosseini H, pared to other treatment modalities before considering a Mechtouff L, et al. Patent foramen ovale closure or anticoagula- widespread use. tion vs. antiplatelets after stroke. NEnglJMed 2017;377:1011–21. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/3/omx105/4935135 by Ed 'DeepDyve' Gillespie user on 16 March 2018
Oxford Medical Case Reports – Oxford University Press
Published: Mar 1, 2018
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