Publications (2)2.95 Total impact
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ABSTRACT: Background and aims: Cardiac amyloidosis (CA) is usually characterised by a poor outcome in the short-term; clinical and instrumental features are heterogeneous and could characterise subgroups with different prognoses. The aim of our study was to describe a subgroup of patients with CA showing an impressive favourable long-term survival. Methods: Out of 50 patients (males 65%, 63 ± 11 years) with an echocardiographic and bioptic diagnosis of CA observed from 1991 to 2009, we selected a subgroup of patients surviving more than 50 months from diagnosis (group 1). We described their features at enrolment and during follow-up, comparing them with patients surviving less than 12 months (group 2). Results: We found seven patients (14%) belonging to group 1 and 26 (52%) to group 2. Four out of seven long term survivors suffered from AL amyloidosis, in one case the underlying aetiology was a chronic inflammatory disease, while in two cases remained unknown. At enrolment, group 1 patients showed higher systolic blood pressure with respect to group 2 (140 ± 25 vs. 112 ± 18 mmHg, respectively, p=0.011), and a less thick interventricular septum (IVS) (IVS thickness > 15 mm in 29% vs. 69% of patients, p = 0.049). No patient of group 1 presented left ventricular restrictive filling pattern (0 vs. 31% in group 1 and 2 respectively, p = 0.035), atrial fibrillation (0 vs. 35%, p = 0.024), or progression towards a more severe disease during follow-up. Conclusions: A not negligible proportion of patients with CA can have a long-term survival. They showed a less severe disease at diagnosis, with substantial stability over time. Further studies on larger populations are necessary to understand the mechanisms underlying this more favourable natural history of the disease.
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ABSTRACT: AIM: Amyloidosis is a systemic disease, related to different underlying causes, with frequent cardiac involvement. Clinical evaluation, echocardiography and electrocardiography represent important noninvasive tools in identification of cardiac involvement. The aim of this study was to assess the clinical-laboratory features of a series of patients affected by cardiac amyloidosis in order to evaluate the risk of cardiac mortality. METHODS: We evaluated 48 patients (men 65%, mean age 63 ± 11 years) with biopsy-proven diagnosis of amyloidosis and heart involvement observed from 1991 to 2009. All patients underwent clinical-laboratory evaluation at baseline and were followed up. RESULTS: During a median follow-up of 9.5 months (first to third interquartile: 3-41.5 months), 24 patients (50%) died as a result of a cardiac cause. Survival free from cardiac death was 69, 50, 48 and 41% at 6, 12, 24 and 60 months from diagnosis, respectively. At multivariable Cox regression analysis, the presence of heart failure at enrolment [hazard ratio (HR) 4.67, 95% confidence interval (CI) 1.07-20.27, P = 0.04] and history of recent syncope (HR 3.97, 95% CI 1.28-12.34, P = 0.017) emerged as independent predictors of cardiac death. By using the equation derived from the multivariate analysis, individual survival probability at different times of follow-up was calculated. CONCLUSION: We confirm the particularly poor outcome of cardiac amyloidosis in the short term. A careful clinical evaluation emerges as the most important tool for the prognostic stratification and quantification of risk in patients with cardiac amyloidosis.
Università degli Studi di TriesteTrst, Friuli Venezia Giulia, Italy