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.

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.

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.

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.

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.

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.

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.

Comparison of surgical methods in catastrophic antiphospholipid syndrome (CAPS).

Antiphospholipid syndrome (APS) is an autoimmune condition characterized by vascular thromboses and/or pregnancy morbidity in the presence of antiphospholipid antibodies. The variant "catastrophic" (CAPS) is defined as a potential life-threatening disease, characterized by multiple small vessel thromboses that can lead to multiple organ failure. Surgery is between precipitating factors in CAPS International Registry, but it's still unclear the relationship between surgery and syndrome. The present study reports two surgical procedures, performed on two patients with APS admitted with diagnosis of loss of substance of the lower limb needing of reconstructive treatment. We compared and analyzed aggressive and minimally invasive surgery.

Experience with multiple stent implantations in primary antiphospholipid syndrome in childhood: a case report.

The antiphospholipid syndrome (APS) is an autoimmune condition characterized by the persisting presence of antiphospholipid antibodies in association with thrombosis and/or pregnancy morbidity. Primary APS is quite rare in childhood and exact prevalence is not known. However, substantial proportion of thrombotic events in children is being attributed to APS. We herein present a 9-year-old boy presented with impending pericardial tamponade and large pleural effusions likely secondary to transudation of fluid from his gradually developed collateral circulation which was resulted from almost completely occluded vena caval system due to primary APS. He was treated with multiple angioplasty-stenting which offered symptomatic relief and better quality of life. To our knowledge, this is the first reported paediatric case of primary APS presented with extensive occlusive lesions in both caval systems and treated with repeated endovascular stent placements.

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.

Adrenal insufficiency after laparoscopic hysterectomy in a patient with primary antiphospholipid syndrome.

BACKGROUND: We report a case of bilateral adrenal hemorrhage and subsequent adrenal insufficiency after a laparoscopic hysterectomy in a patient with anticardiolipin antibody syndrome. CASE: A 55-year-old woman with a history of anticardiolipin antibody syndrome presented with nausea and vomiting 1 week after laparoscopic hysterectomy and staging for endometrial adenocarcinoma. Based on a diagnosis of adrenal insufficiency, the patient was started on oral hydrocortisone 20 mg in the morning and 10 mg in the afternoon, and fludrocortisone 0.05 mg twice daily on day 5. Her symptoms resolved completely within 24 hours of beginning steroids. CONCLUSION: The diagnosis of adrenal insufficiency should be entertained in any patient with a history of thrombophilias presenting with general abdominal complaints.

The antiphospholipid syndrome and heart valve surgery.

The antiphospholipid syndrome (APLS) is a complex autoimmune disease often connected to systemic lupus erythematodes. Main features are thromboses, fetal loss and specific antibodies. The involved autoantibodies are directed against plasma proteins such as beta2glycoprotein1 (beta2GPI) or prothrombin which depend on negatively charged phospholipids. Direct antibodies against phospholipids are of no importance for APLS. Clotting tests such as activated partial thromboplastin time or diluted Russell's viper venom test (dRVVT) can show a prolonged time for coagulation despite a prothrombotic state in vivo but the investigator needs awareness about disturbing phospholipid sources and other influential factors. Enzyme linked immuno sorbent assay tests for antibodies against cardiolipin, beta2GPI and prothrombin are valuable solid phase tests with different specificity. Antiphospholipid, anticardiolipin or lupus anticoagulant are misnomers in connection with APLS. They are preserved as a reminiscence of the pioneering work on the way to the still not exactly revealed basics of APLS. Valve operations in APLS patients seem to be rare; a meta-analysis of 57 cases proves that the perioperative management is, at the moment, an empirical approach with high morbidity and mortality in these young patients.

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.

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.

Peripheral vascular occlusions leading to gangrene and amputations in antiphospholipid antibody positive patients.

Twenty-one cases from several medical centers (Brazil, Italy, Serbia, South Africa, Israel, and the United Kingdom) with severe peripheral vascular disease progressing to amputations of limbs/digits, all of whom tested positive for antiphospholipid antibodies, are documented. The patients were suffering from either systemic lupus erythematosus, discoid LE, "primary" antiphospholipid syndrome (PAPS), "lupus-like" disease, undifferentiated connective tissue disease. A high frequency of livedo reticularis preceding the arterial occlusions in our series of patients who subsequently progressed to ischemic necrosis and amputation of limbs/digits was noted. Five of the 10 patients, in whom histopathological studies had been performed, demonstrated the typical vasculopathy seen with the antiphospholipid syndrome ("APS vasculopathy"). Complicating vasculitis was present in seven of the patients. Five of the patients developed severe peripheral vascular disease during the course of the catastrophic antiphospholipid (Asherson's) syndrome.

[Acute abdomen in antiphospholipid antibodies syndrome (PAPS)]. / Addome acuto nella sindrome da anticorpi antifosfolipidi (PAPS).

Intestinal ischemia in antiphospholipid antibody syndrome (PAPS) could be due to arterial thrombosis from hypercoagulability. A male patient, 45 years old, was admitted to the hospital with symptoms of acute abdomen and after laparotomy he developed sepsis, right kidney infarction, jejunal ischemia, aortic thrombosis, wide necrosis of both gluteus muscles, left subclavian vein thrombosis. Our therapeutic and diagnostic strategy was delineated after demonstration of antiphospholipid antibodies. The patient was treated with total parenteral nutrition in the presence of 5 enteric fistulas with very high outflow, arterial stent insertion and daily changes of medicated dressings. Outcome was excellent with small residual deficit in walking. Continuous nutritional status monitoring and very high nitrogen supply allowed excellent healing of huge wounds and closure of enteral fistulas.