Background: Cardiac amyloidosis (CA) is associated with typical morphological features on echocardiography, including concentric LV hypertrophy (LVH). Cardiac magnetic resonance (CMR) can accurately depict anatomy in different cardiomyopathies. Our aim was to describe the morphological features and remodelling patterns of CA with CMR, and establish their diagnostic accuracy, as well as the value of traditional diagnostic criteria derived from echocardiography and electrocardiography.
Methods: Consecutive patients referred for CMR for possible CA were retrospectively evaluated. The diagnosis of CA was established in the presence of a positive cardiac biopsy and/or a typical pattern of myocardial late gadolinium enhancement. Morphological parameters were obtained from standard cine sequences. The presence and distribution of LVH, relative wall thickness (RWT) and LV remodelling patterns were determined.
Results: 130 patients (92 males (70.8%), age 64±13 years) were included. CA was diagnosed in 51 (39.2%). Patients with CA had increased LV wall thickness and LV mass index. An LV remodelling pattern different from concentric LVH was found in 42% of patients with CA, and asymmetric LVH was noted in 68.6%. A model including RWT, asymmetric LVH, and LVMI showed diagnostic accuracy of 88%, sensitivity of 67% and specificity of 86% for CA detection. Traditional diagnostic criteria for CA showed high specificity but poor sensitivity.
Conclusions: Asymmetric LVH and remodelling patterns different from concentric LVH are common in CA. Increased LV mass index, increased RWT, and asymmetric LVH are independently associated with the diagnosis. Traditional diagnostic criteria show poor sensitivity.
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http://dx.doi.org/10.1136/heartjnl-2014-305710 | DOI Listing |
Arch Cardiol Mex
February 2024
Department of Cardiology, Cardiology Critical Care Unit, Hospital Universitari Arnau de Vilanova, Institut de Recerca Biomèdica, Lleida, Spain.
In the presence of the left ventricle hypertrophy (LVH), the differential diagnosis with hypertrophic cardiomyopathy (HCM) or some phenocopy must be always considered, which can be easily suspected when the hypertrophy is markedly asymmetric. However, when the hypertrophy is homogeneous, especially if the patient has concomitant hypertension, it may be a challenge to distinguish between hypertensive and HCM, although some clinical features may help us to suspect it. In addition, patients with HCM may present with exertional angina due to microcirculation involvement in the setting of the hypertrophy itself or dynamic obstruction in the left ventricular outflow tract, but in some cases, the presence of concomitant coronary artery disease must be suspected as the cause of angina, especially if the patient has an intermediate or high-risk probability of having ischemic heart disease.
View Article and Find Full Text PDFKidney Int Rep
October 2023
Michael Stern Laboratory for Polycystic Kidney Disease, Westmead Institute for Medical Research, The University of Sydney, Sydney, New South Wales, Australia.
Cardiovascular disease (CVD) is the major cause of mortality in autosomal dominant polycystic kidney disease (ADPKD) and contributes to significant burden of disease. The manifestations are varied, including left ventricular hypertrophy (LVH), intracranial aneurysms (ICAs), valvular heart disease, and cardiomyopathies; however, the most common presentation and a major modifiable risk factor is hypertension. The aim of this review is to detail the complex pathogenesis of hypertension and other extrarenal cardiac and vascular conditions in ADPKD drawing on preclinical, clinical, and epidemiological evidence.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
August 2023
Division of Cardiology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town 7505, South Africa.
J Assoc Physicians India
January 2023
All India Institute of Medical Sciences, Kalyani, West Bengal, India.
Introduction: To our knowledge this is the first & only case report in India wherein primary aldosteronism (adrenal adenoma) presented with cardiomyopathy (regressed post-surgery).
Materials: First reported case in India.
Result: Herein August 2018 IPGMER-SSKM-Kolkata 29-year female presented with 1-month exertional dyspnoea, occasional chest pain, sweating, fainting.
The renin-angiotensin-aldosterone system (RAAS) plays a vital role in cardiovascular homeostasis by regulating blood pressure, salt, and water balance. The kidneys produce renin which converts angiotensinogen to angiotensin-1 (AT-I) and angiotensin-converting enzyme (ACE) to angiotensin-II (AT-II). AT-II binds to receptors in the adrenal cortex to release aldosterone.
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