Pulmonary Endarterectomy in Patients with Antiphospholipid Syndrome-Associated Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Antiphospholipid syndrome is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis. Chronic thromboembolism is one of the known established pathogenesis of pulmonary hypertension, known as chronic thrombo-embolic pulmonary hypertension. Pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension. The aim of this study is to evalu-ate the efficacy and risk of pulmonary endarterectomy in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. METHODS: Data were prospectively collected and retrospectively analyzed, for patientswho underwent pulmonary endarterectomy between March 2011 and March 2020. RESULTS: Seventeen patients (4 male and 13 female) were identified. Thirteen patients had primary antiphospholipid syndrome and 4 had secondary antiphospholipid syndrome. The mean age was 34.82 ± 10.07 years and the mean time interval between the diagno-sis and surgery was 26.94 ± 17.35 months. Dyspnea on exertion was the main symptom in all patients. Seven patients had previous deep vein thrombosis, 5 patients had a history of recurrent abortions, and 2 patients had hemoptysis. Following surgery, mean pulmo-nary artery pressure decreased from 47.82 ± 13.11 mm Hg to 22.24 ± 4.56 mm Hg (P < .001), and pulmonary vascular resistance improved from 756.50 ± 393.91 dyn/s/cm-5 to 298.31 ±132.84 dyn/s/cm-5 (P < .001). There was no in-hospital mortality with a mean follow-up of 75.29 ± 40.21 months. The functional capacity of all patients improved from 269.46 ±111.7 m to 490 ± 105.34 m on a 6-minute walking test. CONCLUSIONS: Pulmonary endarterectomy is a safe and curative treatment in patientswith antiphospholipid syndrome-associated chronic thromboembolic pulmonary hyper-tension. It has a favorable outcome by increasing the quality of life. A multidisciplinary experienced chronic thromboembolic pulmonary hypertension team is critical in the management of these unique patients.

Combined coronary artery bypass graft and liver/kidney transplantation in a liver failure patient with acute on chronic kidney failure and antiphospholipid syndrome.

Coexisting coronary artery disease (CAD), end-stage liver disease (ESLD), renal failure, and hypercoagulable state poses a formidable clinical challenge. Here, we discuss the first known case of a patient with antiphospholipid syndrome (APLS), ESLD complicated by hepatorenal syndrome (HRS), and severe CAD who successfully underwent combined coronary artery bypass grafting (CABG) and simultaneous liver/kidney (SLK) transplant.

Spinal epidural hematoma in antiphospholipid syndrome: case report and review of the literature.

INTRODUCTION: Spinal epidural hematomas are a surgical emergency, the delay in diagnosis can develop devastating sequelae due to its acute and progressive course. If not treated properly, it may lead to death or permanent neurological deficit. It is a rare condition that can occur in patients with hematologic pathology. CASE PRESENTATION: We report a case report and literature review of a patient with antiphospholipid syndrome, who undergoes a diagnostic lumbar puncture for probable fungal meningitis. Developed a spinal acute epidural hematoma with neurological involvement that is evidenced in MRI. Urgent surgical decompression was performed with good results. DISCUSSION: Despite the low incidence of an epidural hematoma in patients who undergo lumbar puncture, it is important to perform a thorough evaluation in any patient with coagulation abnormalities prior and after a lumbar puncture, by reason of the inherent possibility of developing an epidural hematoma at the site of the procedure. In the same way, early diagnosis and aggressive treatment is necessary in patients who develop progressive neurological symptoms to limit the damage and improve the prognosis for neurological recovery.

Safety and effectiveness of transjugular renal biopsy for systemic lupus erythematosus and antiphospholipid antibody syndrome patients taking antithrombotics.

BACKGROUND: Renal biopsy is the cornerstone of systemic lupus erythematosus (SLE) nephritis and antiphospholipid syndrome (APS) nephropathy management. However, transcutaneous renal biopsy (TCRB) is hampered by the antithrombotic treatment frequently prescribed for those diseases. Transjugular renal biopsy (TJRB) offers an attractive alternative for patients at increased risk of bleeding. The primary objective of the study was to describe the safety profile and diagnostic performance of TJRB in SLE and APS patients. METHODS: All SLE and/or APS patients who underwent a renal biopsy in our department (between January 2004 and October 2016) were retrospectively reviewed. Major complications were death, haemostasis nephrectomy, renal artery embolization, red blood cell transfusion, sepsis and vascular thrombosis; macroscopic haematuria, symptomatic perirenal/retroperitoneal bleeding and renal arteriovenous fistula without artery embolization were considered as minor complications. RESULTS: Two hundred and fifty-six TJRBs-119 without antithrombotics (untreated), 69 under aspirin and 68 on anticoagulants and 54 TCRBs without antithrombotics-were analysed. Their major and minor complication rates, respectively, did not differ significantly for the four groups: 0 and 8% for untreated TJRBs, 1 and 6% for aspirin-treated, 6 and 10% for anticoagulant-treated and 2 and 2% for TCRBs. The number of glomeruli sampled and the biopsy contribution to establishing a histological diagnosis was similar for the four groups. CONCLUSIONS: TJRBs obtained from SLE and APS patients taking antithrombotics had diagnostic yields and safety profiles similar to those of untreated TCRBs. Thus, TJRB should be considered for SLE and APS patients at risk of bleeding.

Autologous Hematopoietic Stem Cell Transplantation for Antiphospholipid Syndrome: Case Report and Review of the Literature.

Refractory antiphospholipid syndrome represents a challenge for preventing thrombosis that may occur despite adequate anticoagulation and immunomodulation therapy. Here, we report a 35-year-old male patient who presented with variable venous thromboembolic events. Autologous hematopoietic stem cell transplant was performed after conditioning with cyclophosphamide and antithymocyte globulin. Five years after transplant, the treatment continued to show a successful outcome in preventing new thrombotic events. Autologous hematopoietic stem cell transplant represents a chance for cure from antiphospholipid syndrome despite the paucity of reported data so far.

Antiphospholipid syndrome combined with acute coronary syndrome: Case report.

RATIONALE: Antiphospholipid syndrome (APS) combined with acute coronary syndrome (ACS) is rarely reported. PATIENT CONCERNS: One male patient with APS was admitted to our hospital, who had recent unstable angina (UA). DIAGNOSIS: The preliminary diagnosis of ACS and UA (BraunwaldiB) was then made. INTERVENTIONS: This patient received secondary preventative therapy for coronary heart disease (CHD) in combination with percutaneous transluminal coronary angioplasty (PTCA) and implantation of NeoVas Bioresorbable Coronary Scaffold. OUTCOMES: The patient was followed up, without new UA episodes were observed at 6 months, 1 year, and 2 year after surgery, respectively. LESSONS: It was thus concluded that percutaneous coronary intervention (PCI) is effective for APS patients and NeoVas scaffold implantation is presumed safe.

Anticoagulation management during cardiopulmonary bypass in patients with antiphospholipid syndrome.

Antiphospholipid syndrome (APS) is a complex autoimmune disease often related to systemic lupus erythematosus. Although adequate anticoagulation is important for APS patients during cardiopulmonary bypass, clotting tests can be potentially misleading due to antiphospholipid antibodies. We performed cardiac surgery safely in two APS patients under anticoagulation monitoring determined using preoperative heparin titration. We performed heparin titration for activated clotting time to determine the appropriate heparin concentration during cardiac surgery. We changed the targeted heparin concentration considering each patient's thrombotic risks: 3 U/ml of heparin for a normal-risk APS patient and 5 U/ml for a high-risk APS patient with a history of antiphospholipid-antibody-associated thrombocytopenia. A higher targeted heparin concentration might be necessary for patients with high thrombotic risks.

Reversible Parkinsonism and Multiple Cerebral Infarctions after Pulmonary Endarterectomy in a Patient with Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is a cause of chronic thromboembolic pulmonary hypertension (CTEPH) and it is associated with an increased risk of postoperative neurological complications. We experienced a case of reversible parkinsonism after pulmonary endarterectomy (PEA) and subsequent multiple cerebral infarctions under standard anticoagulation therapy in a patient with CTEPH associated with APS. Strict management using a combination of antiplatelet and anticoagulation therapy should be considered in patients with a high titer of triple antiphospholipid antibodies in the perioperative period. We should be aware of the high risk of postoperative neurologic manifestations in patients with APS.

Antiphospholipid syndrome, antiphospholipid antibodies and solid organ transplantation.

Antiphospholipid syndrome is considered a high risk factor for any kind of surgery. Considering that all solid organ transplants are critically dependent on the patency of vascular anastomosis, there is much concern about the consequences this pro-thrombotic condition may have on transplantation. Relatively little information is available in the literature assessing the real risk that antiphospholipid syndrome or the presence of antiphospholipid antibodies represent in solid organ transplantation. The aim of this article is to review the literature related to transplantation of solid organs in patients diagnosed with antiphospholipid syndrome or patients with positive antiphospholipid antibodies.

Double-valve replacement for mitral and aortic regurgitation in a Patient with Libman-Sacks endocarditis.

A 53-year-old woman with systemic lupus erythematosus and antiphospholipid syndrome presented with central nervous system (CNS) lupus and vegetation of the mitral and aortic valves. Her CNS lupus was relieved with methylprednisolone pulse therapy; however, her mitral regurgitation worsened, and she developed acute decompensated heart failure. The mitral and aortic valves were replaced with mechanical heart valves. Microscopic examination of the excised valves showed no bacterial invasion, and Libman-Sacks (LS) endocarditis of both valves was confirmed. This was a case of LS endocarditis with clear vegetation that spread over the mitral and aortic valves.

Neurosurgical management for complicated catastrophic antiphospholipid syndrome.

Antiphospholipid syndrome (APS) is an autoimmune condition involving arterial and venous thrombosis. An unusual APS variant, catastrophic antiphospholipid syndrome (CAPS), includes rapid multi-organ failure from widespread small vessel thrombosis. Central nervous system complications arise in one-third of CAPS patients. In rare cases, CAPS co-manifests with cerebellar hemorrhage presenting a neurosurgical emergency. We present a 65-year-old woman with CAPS-related cerebellar hematoma, co-morbid idiopathic thrombocytopenic purpura, deep vein thrombosis and altered mental status, with treatment complicated by thrombocytopenia. The patient suddenly deteriorated, secondary to a cerebellar subdural hematoma, and underwent decompression and excision of the hematoma. After recovery in the intensive care unit, she developed a new spontaneous epidural hematoma requiring additional surgery. Management of these patients is hematologically complex and often requires a multi-disciplinary team of physicians. This patient provides an important learning point for clinicians - consider CAPS when hemorrhage and thrombosis are present.

Mitral valve surgery for marantic endocarditis and multiple cerebral embolisation.

Valvular involvement is common in antiphospholipid syndrome (APS) with increased risk of thrombo-embolic events. We report a patient with APS and multiple cerebral infarcts. Echocardiography demonstrated verrucous vegetations of the mitral valve in keeping with marantic endocarditis. The patient underwent successful mitral valve replacement. Post-operative clinical and echocardiographic follow-up showed excellent short term results.

Cardiac valve replacement in patients with antiphospholipid syndrome.

OBJECTIVE: To analyze the results of cardiac valve replacement in a multicenter cohort of patients with antiphospholipid syndrome (APS) and to identify prognostic factors of poor outcome. METHODS: We performed a retrospective analysis of clinical manifestations (cardiac involvement and APS characteristics), operative and early postoperative courses, and long-term followup. All of the patients fulfilled the Sapporo criteria for APS. Logistic regression analyses were performed to identify those variables associated with adverse outcomes. RESULTS: Between 1981 and 2008, 33 valvular replacements were carried out in 32 patients with APS. The mean ± SD age at the time of surgery was 43.09 ± 14.08 years. Thirty patients were women. Primary APS was present in 21 patients. The median followup time after surgery was 33.5 months (range 0-192 months). The mitral valve was the most frequently replaced (22 of 33). Mechanical valve replacement was performed in 23 patients (71.9%). The mortality rate was 12.5% (1 cardiogenic shock, 1 septic shock, 1 following renal transplantation, and 1 hemorrhagic stroke). Fourteen patients experienced 20 complications (8 major bleeding, 5 thrombotic events, 2 valvular deteriorations, 2 third-degree atrioventricular block, 1 endocarditis, 1 cardiac tamponade, and 1 cardiac failure). Fifty percent of the patients had an uneventful outcome. CONCLUSION: Morbidity and mortality were high in APS patients undergoing valve replacement surgery. Most complications were related to thrombosis and bleeding. Anticoagulation must be carefully monitored to prevent hemorrhagic and thrombotic complications.

[Kidney allograft: a target for systemic disease]. / Le rein transplanté : une cible des maladies systémiques.

Recurrence of disease after transplantation is frequent and represents the third cause of allograft loss. Recurrence of lupus nephritis after transplantation is rare. Kidney transplantation in patients with antiphospholipid syndrome or lupus anticoagulant is challenging due to the high risk of immediate post-transplant thrombosis and bleeding risk associated to the subsequent anticoagulation. Moreover, vascular changes associated to the presence of antiphospholipid antibodies negatively impact allograft rate survival. Recurrence of pauci immune glomerulonephritis or Goodpasture syndrome is exceptional.