A novel risk score to predict survival in advanced heart failure due to cardiac amyloidosis

ConclusionsRisk stratification for cardiac amyloidosis with the newly developed “HeiRisk” score may be superior to other staging systems for patients with advanced heart failure due to amyloid cardiomyopathy.
Source: Clinical Research in Cardiology - Category: Cardiology Source Type: research

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Authors: Nehashi T, Oikawa M, Amami K, Kanno Y, Yokokawa T, Misaka T, Yamada S, Kunii H, Nakazato K, Ishida T, Takeishi Y Abstract Hereditary ATTR amyloid cardiomyopathy is defined as the intramyocardial deposition of amyloid fibrils derived from the mutation of transthyretin (TTR). A 51-year-old man was referred to our hospital for congestive heart failure. He and his family had no past history of heart diseases. Echocardiography showed remarkable left ventricular hypertrophy and reduced ejection fraction. Endomyocardial biopsy specimens presented positive staining of Congo-Red and transthyretin. A genetic test sh...
Source: International Heart Journal - Category: Cardiology Tags: Int Heart J Source Type: research
CONCLUSIONS: Risk stratification for cardiac amyloidosis with the newly developed "HeiRisk" score may be superior to other staging systems for patients with advanced heart failure due to amyloid cardiomyopathy. PMID: 31630214 [PubMed - as supplied by publisher]
Source: Clin Med Res - Category: Research Authors: Tags: Clin Res Cardiol Source Type: research
Conclusions In clinical practice, pimobendan seems to have beneficial effects in heart failure management for improving physical activities and the quality of life in patients with transthyretin cardiac amyloidosis. PMID: 31588087 [PubMed - as supplied by publisher]
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Cardiac involvement is associated with poor prognosis in immunoglobulin light-chain amyloidosis (AL amyloidosis), with a median survival of 6 months without heart transplantation (HT) from the onset of symptomatic heart failure. Although HT is a promising treatment option for patients with amyloid cardiomyopathy (ACM), consensus on its indication is not fully established because of limited experience. Method: We reviewed our prospective AL amyloidosis database to identify all patients who received HT for their ACM.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Source Type: research
AbstractTransthyretin cardiac amyloidosis (ATTR ‐CA) demonstrates progressive, potentially fatal, and infiltrative cardiomyopathy caused by extracellular deposition of transthyretin‐derived insoluble amyloid fibrils in the myocardium. Two distinct types of transthyretin (wild type or variant) become unstable, and misfolding forms aggregate, r esulting in amyloid fibrils. ATTR‐CA, which has previously been underrecognized and considered to be rare, has been increasingly recognized as a cause of heart failure with preserved ejection fraction among elderly persons. With the advanced technology, the diagnostic tools have...
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Review Source Type: research
This article will review ATTR cardiac amyloidosis to provide physicians with the tools they need to establish a definitive diagnosis when there is a clinical suspicion of amyloidosis and provide the most appropriate care. Expert commentary: Increased awareness and improved diagnostic techniques will lead to earlier diagnosis and a greater understanding of the clinical presentation. The anticipated increases in the prevalence of this disease due to increased clinical awareness will require, and in-part, facilitate the development of new therapies to manage this patient population. PMID: 31478389 [PubMed - as supplied by publisher]
Source: Expert Review of Cardiovascular Therapy - Category: Cardiology Tags: Expert Rev Cardiovasc Ther Source Type: research
Rationale: Cardiac amyloidosis, considered for the last years to be a rare disease, is one of the determinants of HFpEF. The non-specific clinical presentation and the difficulties related to endomyocardial biopsy have made cardiac amyloidosis an underdiagnosed clinical entity. Improvement of non-invasive diagnostic techniques and the development of new therapies increased clinical awareness for this form of restrictive cardiomyopathy. We here summarize echocardiography and 99mTc-HDP scintigraphy findings in 6 cases of cardiac amyloidosis and review the literature data of this progressive and fatal cardiomyopathy. Patie...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
ConclusionsWhen recognized, neurogenic orthostatic hypotension can be treated. Discontinuation of potentially aggravating medications, patient education and non-pharmacologic approaches should be applied first. Droxidopa (Northera®), a synthetic norepinephrine precursor, has shown efficacy in controlled trials of neurogenic orthostatic hypotension in patients with hereditary TTR amyloidosis and is now approved in the US and Asia. Although they may be useful to ameliorate autonomic dysfunction in hereditary TTR amyloidosis, the impact of disease-modifying treatments on neurogenic orthostatic hypotension is still uninvestigated.
Source: Clinical Autonomic Research - Category: Research Source Type: research
Publication date: October 2019Source: American Heart Journal, Volume 216Author(s): Peter Czobor, Yun-Yi Hung, David Baer, Dana McGlothlin, Dana Weisshaar, Jonathan ZaroffBackgroundLight Chain (AL) and transthyretin (ATTR) amyloidosis are the most common forms of amyloid cardiomyopathy. Population based studies describing the epidemiology and clinical features of amyloid cardiomyopathy are often based in tertiary medical centers and thus may be limited by referral bias.Methods and ResultsWe performed a cohort study of 198 patients diagnosed and treated in the Kaiser Permanente Northern California health care system who had ...
Source: American Heart Journal - Category: Cardiology Source Type: research
ConclusionThis retrospective study provides an estimate of the number of patients diagnosed with ATTR-CM in a large in-hospital database in Japan over a period of 9  years. Improving awareness of disease prevalence may improve diagnosis and treatment.FundingPfizer.
Source: Cardiology and Therapy - Category: Cardiology Source Type: research
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