Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial "boxcarring," retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient's vision improved and remained stable at 20/200 and 20/80.
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PMID:Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions. 2572 4

This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the possibility of a diffuse thrombotic process.
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PMID:A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI. 2601 99

The direct oral anticoagulants (DOACs) represent a major advance in oral anticoagulant therapy and have replaced the vitamin K antagonists as the preferred treatment for many indications. By simplifying long-term anticoagulant therapy and improving its safety, the DOACs have the potential to reduce the global burden of thrombosis. Postmarketing studies suggest that the favorable results achieved with DOACs in the randomized controlled trials can be readily translated into practice, but highlight the need for appropriate patient, drug and dose selection, and careful follow-up. Leveraging on their success to date, ongoing studies are assessing the utility of DOACs for the prevention of thrombosis in patients with embolic stroke of unknown source, heart failure, coronary artery disease, peripheral artery disease, antiphospholipid syndrome, and cancer. The purpose of this article is to (1) review the pharmacology of the DOACs, (2) describe the advantages of the DOACs over vitamin K antagonists, (3) summarize the experience with the DOACs in established indications, (4) highlight current challenges and limitations, (5) highlight potential new indications; and (6) identify future directions for anticoagulant therapy.
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PMID:Evolving Treatments for Arterial and Venous Thrombosis: Role of the Direct Oral Anticoagulants. 2712 50

Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder characterized by thrombophilia, vascular thrombosis, and recurrent abortions associated with persistent antiphospholipid antibodies. APS may exist in its primary form, or more commonly is found to be associated with variety of rheumatic disorders, such as systemic lupus erythematosus. Cardiac involvement is not an uncommon complication in primary antiphospholipid patients. Libman-Sacks lesions are typically small, sessile, and wart-like, varying in size from 1-4 mm. Here we present an unusual case of a 37 year-old pregnant woman who suffered from heart failure associated with primary antiphospholipid syndrome and Libman-Sacks endocarditis, with large vegetations involving the mitral valve. The patient underwent mitral valve replacement with a mechanical prosthesis.
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PMID:Libman-Sacks Endocarditis with Unusual Large Size Vegetation Involving the Mitral Valve. 2805 1

Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most cases, patients do not have significant valvular dysfunction. However, patients with significant valvular dysfunction may present with serious complications such as cardiac failure, arrhythmias, and thromboembolic events. Recently, association of Libman-Sacks endocarditis with antiphospholipid antibody syndrome (APS) has been made. APS is most commonly defined by venous and arterial thrombosis, recurrent pregnancy loss, and thrombocytopenia. While the syndrome can be a primary syndrome, it is usually secondary to systemic lupus erythematosus. Catastrophic antiphospholipid syndrome (CAPS) can be a life-threatening presentation of APS and can occur in 1% of patients with antiphospholipid syndrome. We present a very rare case of a young female patient with lupus-negative Libman-Sacks endocarditis complicated by CAPS.
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PMID:Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome. 2851 23

Valve vegetations in nonbacterial thrombotic endocarditis consist of fibrin and platelet aggregates and can be related to circulating immune complexes, such as in the case of antiphospholipid syndrome. In patients with primary antiphospholipid syndrome, echocardiographic studies have disclosed heart valve abnormalities in about a third of patients. Unusual associations between antiphospholipid syndrome and nonbacterial thrombotic endocarditis include presentation as an intracardiac mass compatible with a myxoma on imaging studies, as well as isolated involvement of the tricuspid valve. Both of these scenarios have been previously reported in female patients. This article presents the case of a 53-year-old Hispanic male with antiphospholipid syndrome who presented to the hospital with symptoms of heart failure and persistent right calf pain. An intracardiac mass attached to the anterior leaflet of the tricuspid valve was found through transthoracic echocardiography. Further imaging studies suggested the mass to be a myxoma and the patient underwent mass excision with tricuspid valve replacement. Pathology report of the surgical specimen was consistent with a diagnosis of nonbacterial thrombotic endocarditis. This case highlights the importance of considering nonbacterial thrombotic endocarditis as a key differential diagnosis in patients with concomitant antiphospholipid syndrome and intracardiac masses, as well as challenges encountered in diagnosis and management.
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PMID:Nonbacterial Thrombotic Endocarditis of the Tricuspid Valve in a Male Patient with Antiphospholipid Syndrome. 3006 69

Cardiac disease is a well-known complication of antiphospholipid syndrome (APS), with many patients presenting with valvular thickening or vegetations, referred to as Libman-Sacks endocarditis (LSE). Because cases of APS with cardiac involvement are relatively rare, paucity of large clinical trials studying this complication has made management challenging. In the absence of acute heart failure and embolic events, a medical approach is usually selected, consisting of anticoagulation and possibly corticosteroids when another underlying autoimmune disease is present. However, the role of various anticoagulant classes and the duration of steroid therapy continue to be debated. Here, we present a 45-year-old woman who developed two vegetations in the setting of secondary APS while taking rivaroxaban before experiencing marked improvement with the use of enoxaparin and steroids.
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PMID:Improvement of Cardiac Vegetations in Antiphospholipid Syndrome with Enoxaparin and Corticosteroids after Rivaroxaban Failure. 3027 42

Abdominal aortic occlusions are rare, but occasionally life threatening. A 48-year-old man was hospitalized due to acute heart failure accompanied by acute kidney injury (AKI). Abdominal ultrasound revealed deteriorating blood flow in the bilateral renal arteries. Subsequent abdominal aortography showed abdominal aortic occlusion just below the right renal artery and an occluded left renal artery. Dilated superior and inferior mesenteric arteries functioning as collateral feeding arteries suggested chronic occlusion. A hypercoagulation workup led to a diagnosis of antiphospholipid antibody syndrome (APS). This case report describes rare chronic juxtarenal abdominal occlusion in a patient with APS. <Leaning objective: Abdominal aortic occlusions are rare, but occasionally life threatening. The proximal propagation of aortic thrombosis might cause prerenal AKI. In our case, the renal arteries' involvement of chronic juxtarenal arteries in a patient with APS gave rise to the onset of acute heart failure associated with prerenal AKI.>.
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PMID:Acute heart failure due to chronic juxtarenal aortic occlusion in a patient with antiphospholipid antibody syndrome. 3054 9

Thrombotic microangiopathic syndromes are characterized by thrombus formation leading to microangiopathic hemolytic anemia, thrombocytopenia, and end-organ injury that most often affects the kidney and brain. Patients with thrombotic microangiopathy can also present with cardiac involvement, which has been shown to worsen their prognosis. We describe the case of a 46-year-old woman who presented with acute congestive heart failure as a manifestation of catastrophic antiphospholipid syndrome, which is characterized by rapidly progressing multiorgan involvement. Targeted therapy improved our patient's cardiomyopathy and saved her life. Increased recognition of thrombotic microangiopathy as an underlying pathophysiologic mechanism in heart failure and initiation of timely treatment may help to prevent death in patients with thrombotic microangiopathy.
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PMID:Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy. 3083 39

Systemic lupus erythematosus (SLE) is associated with several cardiac manifestations but, to our knowledge, there have been no previously published reports on left ventricular (LV) pseudoaneurysm in this disease. We describe a case of a 30-year-old woman with SLE who presented with a disease flare (acute and subacute cutaneous lupus, pericarditis, fever, leukopenia) associated with heart failure syndrome. The patient was diagnosed with a large LV pseudoaneurysm and a bovine pericardium patch closure was performed. Coronary arteries were angiographically normal, and cardiac magnetic resonance imaging did not exhibit detectable myocardial fibrosis or infarction. Trauma, previous cardiac surgery, Chagas disease, and antiphospholipid syndrome were excluded. Histopathology of the pericardium revealed lymphocytic arteriolitis raising the possibility of an autoimmune-mediated mechanism for this complication. The unequivocal concomitant diagnosis of lupus flare, the exclusion of other causes of pseudoaneurysm and the histopathological finding of arteriolitis in this patient reinforces the hypothesis of lupus-mediated lesion.
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PMID:Left ventricular pseudoaneurysm associated with systemic lupus erythematosus. 3090 95


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