979 results match your criteria: "Imaging in Restrictive Cardiomyopathy"

Background: Cardiac amyloidosis (CA) is an underdiagnosed, progressive and lethal disease. Machine learning applied to common measurements derived from routine echocardiogram studies can inform suspicion of CA.

Objectives: Our objectives were to test a random forest (RF) model in detecting CA.

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Rheumatic heart disease (RHD) and endomyocardial fibrosis (EMF) are major causes of cardiac disease in low-income countries. We present a case of a patient with mitral stenosis and restrictive cardiomyopathy, initially attributed to severe RHD, but with disease progression despite valve replacement, likely secondary to previously undiagnosed EMF.

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Article Synopsis
  • Hypereosinophilia (HE) is characterized by an eosinophil count over 1500 cells/microL in blood tests, confirmed by either blood tests or high eosinophil percentages in bone marrow samples. Hypereosinophilic syndrome (HES) involves organ damage due to eosinophils and can be classified as primary, secondary, or idiopathic.
  • Cardiac issues occur in 5% of acute cases and 20% of chronic cases of HES, presenting symptoms like heart failure and arrhythmias, but the severity of heart problems isn't always proportional to eosinophil levels.
  • Diagnosis of cardiac involvement relies on advanced imaging techniques, particularly
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Partial cavopulmonary shunt is an established procedure for patients with abnormal right ventricular (RV) physiology. Late failure of the Glenn shunt is usually due to further progression of the primary disease process. However, there may be surprises, as in our patient.

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Role of biomarkers in early diagnosis and prognosis of cardiac amyloidosis: A systematic review and meta-analysis.

Curr Probl Cardiol

January 2025

Lung Transplant Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Article Synopsis
  • Cardiac amyloidosis involves amyloid deposits in the heart, leading to serious conditions like heart failure, making early diagnosis important for treatment.
  • A systematic review analyzed studies on biomarkers to detect and track cardiac amyloidosis, focusing on their diagnostic and prognostic value.
  • Key findings highlighted that NT-proBNP and troponins are effective for early detection, while new biomarkers may help differentiate subtypes and predict disease progression, though results varied with imaging correlations.
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  • AL amyloidosis can lead to heart issues (AL-CM), and existing imaging techniques struggle to identify heart dysfunction accurately.* -
  • A study involving cardiac MRIs compared patients with AL-CM to healthy individuals, revealing lower myocardial function index (MFI) in AL-CM patients and indicating MFI as a potential marker for risk assessment.* -
  • The findings showed that a lower MFI correlates with worse survival rates, making it a valuable tool for evaluating heart health in AL-CM patients.*
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  • Mulibrey nanism (MN) is a rare genetic disorder affecting growth and causing serious health issues, with about 150 cases identified globally since 1973.
  • A case study of a male toddler with MN showed symptoms like hypoxia and swelling, leading to a complex diagnostic process that confirmed restrictive cardiomyopathy through invasive testing.
  • The case emphasizes the challenges of diagnosing restrictive cardiomyopathy using echocardiography and underscores the importance of a multidisciplinary, family-centered treatment approach for this condition.
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  • Cardiac amyloidosis is a rare but serious form of systemic amyloidosis that requires early diagnosis for better treatment outcomes.
  • A 56-year-old woman experienced symptoms like shortness of breath and heart palpitations, leading to a diagnosis through cardiac imaging and a bone marrow biopsy.
  • Accurate diagnosis is challenging but vital, as delays can worsen the condition; treatments focus on managing symptoms and modifying the disease, with cardiac MRI being a key diagnostic tool.
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  • Amyloidosis is a disease where misfolded proteins accumulate in tissues, often causing cardiac issues like stiffness and heart failure, particularly in transthyretin amyloidosis.
  • A study involving 60 patients, including those with cardiac ATTR amyloidosis, measured right ventricular (RV) stiffness using shear wave elastography (SWE) and assessed correlations with various health metrics.
  • The findings suggest that SWE is an effective, noninvasive method for evaluating RV stiffness, with an SWE threshold of ≥ 4.6 kPa indicating potential cardiac involvement, thus aiding in diagnosis and treatment.
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  • The case study focuses on a 71-year-old woman with hypertrophic obstructive cardiomyopathy (HOCM) who experienced multiple arrhythmias and complications, including atrial flutter after her treatments.
  • Initially diagnosed with HOCM based on echocardiography, she underwent two transcoronary ablations of septal hypertrophy, with varying success, and later developed atrial fibrillation which required further intervention.
  • The findings support that while the treatment of HOCM can be complicated by arrhythmias, a combination of catheter ablation and targeted therapies can lead to positive long-term outcomes for patients.
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Over the last 20 years, the scientific progresses in molecular biology and genetics in combination with the increasing use in the clinical setting of contrast-enhanced cardiac magnetic resonance (CMR) for morpho-functional imaging and structural myocardial tissue characterization have provided important new insights into our understanding of the distinctive aspects of cardiomyopathy, regarding both the genetic and biologic background and the clinical phenotypic features. This has led to the need of an appropriate revision and upgrading of current nosographic framework and pathobiological categorization of heart muscle disorders. This article proposes a new definition and classification of cardiomyopathies that rely on the combination of the distinctive pathobiological basis (genetics, molecular biology and pathology) and the clinical phenotypic pattern (morpho-functional and structural features), leading to the proposal of three different disease categories, each of either genetic or non-genetic etiology and characterized by a combined designation based on both "anatomic" and "functional" features, i.

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Imaging modalities in cardiac amyloidosis.

Curr Probl Cardiol

December 2024

Department of Cardiovascular Medicine, Johns Hopkins University, Baltimore, MD, USA. Electronic address:

Article Synopsis
  • Cardiac amyloidosis (CA) is a condition where amyloid fibrils infiltrate the heart muscle, leading to restrictive cardiomyopathy, with the two main types being transthyretin (ATTR) and immunoglobulin light chain (AL).
  • ATTR is divided into wild-type (ATTRwt) and hereditary (ATTRv) forms, and the disease is becoming a recognized cause of heart failure.
  • Advances in diagnostic methods such as electrocardiography, echocardiography, and scintigraphy are improving the early detection of CA, essential for timely treatment and better patient outcomes.
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[BAG3 gene related restrictive cardiomyopathy: a case report].

Zhonghua Xin Xue Guan Bing Za Zhi

September 2024

Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases of China, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.

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Cardiac amyloidosis is a rare but increasingly recognized condition characterized by the deposition of amyloid fibrils in cardiac tissue, leading to structural and functional heart impairment. This infiltrative cardiomyopathy often mimics more common cardiac conditions, posing significant diagnostic challenges. Particularly deceptive is its presentation as non-ST-segment elevation myocardial infarction (NSTEMI), where the clinical overlap necessitates considering amyloidosis in differential diagnoses.

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Primary cardiac angiosarcoma presenting as restrictive cardiomyopathy.

Lancet Oncol

August 2024

Department of Pathology, University of Texas Medical Branch, Galveston, TX, USA; Department of Pathology & Laboratory Medicine, Cleveland Clinic Florida, Weston, FL, USA.

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Cardiac Sarcoidosis: A Comprehensive Clinical Review.

Rev Cardiovasc Med

February 2024

Division of Non-Invasive Cardiology, Department of Medicine, University of Szeged, 6720 Szeged, Hungary.

Article Synopsis
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Article Synopsis
  • Cardiac amyloidosis (CA) is caused by the buildup of abnormal proteins in the heart muscle, leading to a severe form of heart disease called restrictive cardiomyopathy.
  • The two main types of CA are AL, linked to abnormal immunoglobulin light chains from plasma cells, and ATTR, which involves misfolded transthyretin proteins that can be either hereditary or age-related.
  • Recent advancements in diagnostic imaging techniques and the development of new therapies have improved the detection and treatment of CA, emphasizing the importance of early diagnosis for better patient outcomes.
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Cardiac amyloidosis is an infiltrative disease characterized by the extracellular deposition of misfolded protein in the myocardium, leading to increased stiffness and an eventual restrictive cardiomyopathy. The slow onset of symptoms and overlap with other cardiomyopathies make prompt diagnosis a challenge. Clinicians should be alerted and include amyloidosis in their differential diagnosis, particularly in patients with heart failure with preserved ejection fraction, unexplained left ventricle hypertrophy, particularly in those shown intolerance to previous antihypertensive medication, and early orthopedic manifestations of the disease such as carpal tunnel syndrome and spinal stenosis.

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Purpose Of Review: Cardiac amyloidosis is a condition marked by the misfolding of precursor proteins into insoluble amyloid fibrils, leading to restrictive cardiomyopathy and heart failure symptoms. This review discusses advancements in nuclear imaging techniques that enhance the diagnosis and guide the management of cardiac amyloidosis, addressing the critical need for early and accurate detection in clinical practice.

Recent Findings: Recent studies and guidelines emphasizes the pivotal role of nuclear imaging techniques in diagnosing cardiac amyloidosis.

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Left ventricular thrombus (LVT) has historically been reported as a complication of acute left ventricular (LV) myocardial infarction. It is most commonly observed in cases of LV systolic dysfunction attributed to ischemic or nonischemic etiologies. Conversely, the occurrence of LVT in normal LV systolic function is an exceptionally rare presentation and is predominantly associated with conditions such as hypereosinophilic syndrome (HES), cardiac amyloidosis, left ventricular noncompaction, hypertrophic cardiomyopathy (HCM), hypercoagulability states, immune-mediated disorders, and malignancies.

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Radionuclide Imaging of Cardiac Amyloidosis: An Update and Future Aspects.

Semin Nucl Med

September 2024

Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, MA; Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, MA; CV Imaging Program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, MA. Electronic address:

Cardiac amyloidosis (CA) is caused by the misfolding, accumulation and aggregation of proteins into large fibrils in the extracellular compartment of the myocardium, leading to restrictive cardiomyopathy, heart failure and death. The major forms are transthyretin (ATTR) CA and light-chain (AL) CA, based on the respective precursor protein. Each of them requires early diagnosis for a timely treatment initiation that will improve patient outcomes.

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