Massive coronary thrombosis: An atypical presentation of antiphospholipid syndrome

Vadillo Martín, Pablo; Pastor Pueyo, Pablo; Rivera, Kristian

doi:10.1016/j.medcle.2023.09.021

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Rivera" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Pablo" "apellidos" => "Vadillo Martín" "email" => array:1 [ 0 => "pablovadillomartin@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Pablo" "apellidos" => "Pastor Pueyo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Kristian" "apellidos" => "Rivera" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Universitari Arnau de Vilanova, Institut de Reserca Biomèdica de Lleida (IRBLleida), Lleida, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Trombosis coronaria masiva: una presentación atípica del síndrome antifosfolipídico" ] ] "textoCompleto" => "Antiphospholipid syndrome (APS) is an autoimmune disorder characterised by recurrent vascular thrombosis in patients with persistently positive antiphospholipid antibodies (APA).<a class="elsevierStyleCrossRefs" href="#bib0005">1–4</a> Thrombosis, the defining feature of this disease, occurs in any vascular site,<a class="elsevierStyleCrossRefs" href="#bib0015">3–5</a> and may occur in the coronary arteries in a minority of cases. However, acute myocardial infarction as the first manifestation of this syndrome and, moreover, simultaneous thrombosis of all three coronary arteries is a rare event.<a class="elsevierStyleCrossRefs" href="#bib0005">1,2</a>We report the case of a 60-year-old male, obese and an ex-smoker; with a history of positive lupus anticoagulant antibodies in two determinations separated by more than 12 months (strongly positive lupus anticoagulant; anti-beta2-GP Ab (IgG) 138 - 99 U/ml), following screening for a diagnosis of pulmonary thromboembolism in a first-degree relative.He attended the emergency department for chest pain suggestive of angina, with an electrocardiogram documenting ST-segment elevation in inferior leads and specular decrease in lateral leads (Image 1A). Dual antiplatelet therapy (DAPT) with acetylsalicylic acid (ASA) and prasugrel was initiated. Emergency coronary angiography showed atherosclerotic disease with lesions of mild-moderate severity in the middle anterior descending coronary artery, first obtuse marginal branch and in the middle right coronary artery (RCA), with significant thrombotic burden in all three arteries, although more abundant in the RCA, which was partially occluded (Image 1B–D). Percutaneous coronary intervention was performed on the RCA with manual thrombus aspiration and implantation of a sirolimus-eluting stent, with normal electrocardiogram and resolution of symptoms. Subsequently, he was admitted to the Cardiac Critical Care Unit with DAPT and enoxaparin at therapeutic doses.Six hours after admission, he experienced a new episode of chest pain with ST-segment elevation in inferior leads, with incipient signs of cardiogenic shock. A new coronary angiography revealed acute stent thrombosis (AST), and mechanical aspiration thrombectomy was performed, resulting in a significant thrombotic load (Image 1E) and intracoronary fibrinolysis with Tenecteplase (25mg) due to persistence of the thrombotic load, with normal RCA flow (Image 1F), with no evidence of technical causes related to AST and with very distal embolisation of the posterolateral branch.In view of the thrombotic event, in addition to the history of persistently positive lupus anticoagulant, in conjunction with Internal Medicine and Haematology, a diagnosis of definite APS was made, classified as primary when its association with other autoimmune diseases was ruled out.Due to the severity of the patient's condition and early thrombotic recurrence it was decided to manage as a catastrophic APS (CAPS), despite not fulfilling the diagnostic criteria. Therefore, treatment was started with unfractionated heparin, high doses of glucocorticoids (methylprednisone 500mg for three days with subsequent de-escalation to oral prednisone 60mg/day), plasmapheresis (six sessions) with administration of immunoglobulins (180mg divided into five doses interspersed with the plasma exchanges) and cyclophosphamide (1g).The patient had a favourable outcome, with no new thrombotic events. Once treatment was completed, he was discharged with chronic oral vitamin K antagonist anticoagulants and temporary dual antiplatelet therapy with ASA and clopidogrel.APS is a prothrombotic disease for which the optimal treatment has not yet been fully established. One of the most morbid and fatal presentations of this syndrome is CAPS. There are cases of APS that do not meet the criteria for definite or probable APS, but due to their presentation and need for aggressive treatment, they are considered to be similar to CAPS, such as the case reported.<a class="elsevierStyleCrossRefs" href="#bib0015">3,4</a>Simultaneous multiple coronary artery thrombosis as an initial presentation of APS is rare. Depending on the clinical presentation, the diagnosis can be complex and a high degree of suspicion is required. In cases of diagnostic uncertainty, intracoronary imaging techniques may provide additional value in confirming the presence and extent of thrombi, identifying the culprit lesion and establishing or ruling out concomitant atherosclerotic disease. In case of percutaneous coronary intervention with stent implantation, the addition of DAPT treatment during the first months should be considered.<a class="elsevierStyleCrossRefs" href="#bib0005">1–4</a>Finally, a multidisciplinary approach involving specialists from cardiology, internal medicine, rheumatology and haematology is essential due to the complexity involved in treating this serious condition." "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The “prumary” antiphospholipid síndrome: major clinical and serological features" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "R.A. Asherson" 1 => "M.A. Khamashta" 2 => "J. Ordi-Ros" 3 => "R.H. Derksen" 4 => "S.J. Machin" 5 => "J. Barquinero" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Medicine (Baltimore)" "fecha" => "1989" "volumen" => "68" "paginaInicial" => "366" "paginaFinal" => "374" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/2509856" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J.L. Vianna" 1 => "M.A. Khamashta" 2 => "J. Ordi-Ros" 3 => "J. Font" 4 => "R. Cervera" 5 => "A. Lopez-Soto" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/9343(94)90108-2" "Revista" => array:6 [ "tituloSerie" => "Am J Med" "fecha" => "1994" "volumen" => "96" "paginaInicial" => "3" "paginaFinal" => "9" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8304360" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "C.L. Aguiar" 1 => "D. Erkan" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1177/1759720X13502919" "Revista" => array:6 [ "tituloSerie" => "Ther Adv Musculoskelet Dis" "fecha" => "2013" "volumen" => "5" "paginaInicial" => "305" "paginaFinal" => "314" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24294304" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Recomendations of the Spanish Rheumatology Society for Primary Antiphospholipid Syndrome. Part I: diagnosis, evaluation and treatment" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "R. Cáliz" 1 => "P. Díaz del Campo" 2 => "M. Galindo" 3 => "F.J. López" 4 => "M.A. Martínez" 5 => "A. Santamaría" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.reuma.2018.11.003" "Revista" => array:6 [ "tituloSerie" => "Reumatol Clin (Engl Ed)" "fecha" => "2020" "volumen" => "16" "paginaInicial" => "71" "paginaFinal" => "86" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30713012" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Thrombotic storm and cardiovascular manifestations of probable catastrophic antiphospholipid syndrome: The usefulness of multimodal imaging" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "P. Monteagudo" 1 => "M. Zielonka" 2 => "A.T. Peiro" 3 => "E.V. Pascual" 4 => "K. Rivera" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1093/ehjcr/ytad066" "Revista" => array:5 [ "tituloSerie" => "Eur Heart J Case Rep" "fecha" => "2023" "volumen" => "7" "paginaInicial" => "ytad066" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/36819888" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000016200000005/v1_202403010948/S2387020624000536/v1_202403010948/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000016200000005/v1_202403010948/S2387020624000536/v1_202403010948/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020624000536?idApp=UINPBA00004N"]

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Vol. 162. Issue 5.

Pages 253-254 (March 2024)

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