Primary light-chain (AL) amyloidosis is a plasma cell dyscrasia associated with the deposition of immunoglobulin-derived amyloid in multiple organs. In the heart, this results in an infiltrative cardiomyopathy, with increased left ventricular wall thickness, normal or decreased left ventricular (LV) cavity size and congestive heart failure. Cardiac involvement is a major determinant of prognosis of AL amyloidosis. We report a patient with cardiac amyloidosis proven by cardiac biopsy, and aim to point out at transthoracic echocardiography as the hallmark of diagnostics. Echocardiography revealed increased LV thickness at 20mm, impaired LV ejection fraction (EF) at 35%, enlarged atria, transmitral deceleration time at 156 ms and increased E/A ratio at 4.25. Early diagnosis and intervention can have a significant impact on the patient's response to treatment, especially when the underlying condition involves a malignancy or infiltrative disorder. Standard transthoracic echocardiography as a noninvasive diagnostic tool is valuable and has a significant role in diagnosis and prognosis.
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Arthritis Rheumatol
January 2025
Department of Immunology and inflammation, Imperial College London, UK.
Background: Takayasu arteritis (TAK) and giant cell arteritis (GCA), the most common forms of large-vessel vasculitis (LVV), can result in serious morbidity. Understanding the molecular basis of LVV should aid in developing better biomarkers and treatments.
Methods: Plasma proteomic profiling of 184 proteins was performed in two cohorts.
Narra J
December 2024
Department of Internal Medicine, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia.
Dysregulation of renin-angiotensin-aldosterone system (RAAS) often leads to hypertension and severe cardiorenal complications. Although RAAS-targeted therapies have proven effective, it remains yet optimal in reducing cardiovascular events. The aim of this study was to evaluate the efficacy and safety of angiotensin receptor neprilysin inhibitor (ARNI) compared to control in patients with hypertension.
View Article and Find Full Text PDFBackground: Light-chain cardiac amyloidosis (AL-CA) is associated with structural and functional changes in the left atrium and left ventricle. This study aims to assess the value of the left atrioventricular coupling index (LACI) assessed by three-dimensional echocardiography (3DE) for predicting primary outcome in AL-CA.
Methods: Participants with biopsy-confirmed AL-CA from April 2022 to February 2024 were prospectively analysed.
Heart Fail Rev
January 2025
Duke Clinical Research Institute, 300 West Morgan Street, Durham, NC, 27701, USA.
Strong evidence supports the importance of rapid sequence or simultaneous initiation of quadruple guideline-directed medical therapy (GDMT) for heart failure with reduced ejection fraction (HFrEF) for substantially reducing risk of mortality and hospitalization. Barring absolute contraindications for each individual medication, employing the strategy of rapid sequence, simultaneous, and/or in-hospital initiation at the time of HF diagnosis best ensures patients with HFrEF have the opportunity to benefit from proven medications and achieve large absolute risk reductions for adverse clinical outcomes. However, despite guideline recommendations supporting this approach, implementation in clinical practice remains persistently low, with less than one-fifth of eligible patients being prescribed the quadruple GDMT regimen.
View Article and Find Full Text PDFAdv Clin Exp Med
January 2025
Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland.
Background: We still know little about the effective pharmacological treatment of heart failure (HF) associated with the Fontan circulation. One of the new options may be sodium glucose cotransporter-2 inhibitors (SGLT2i), which have been proven effective in classic forms of left ventricular HF.
Objectives: To evaluate the effect and safety of SGLT2i inclusion in adults with Fontan circulation.
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