Natural history of familial dilated cardiomyopathy: the heart muscle disease registry of trieste

Lenarda, Andrea Di; Carniel, Elisa; Sabbadini, Gastone; Driussi, Mauro; Rakar, Serena; Chiara, Cinzia Di; Zanchi, Cristiana; Sinagra, Gianfranco; Mestroni, Luisa

doi:10.1016/s0735-1097(02)80658-1

Cited by 4 publications

(5 citation statements)

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“…The 5-year mortality of our patients was about 50%, which is consistent with the 5-year survival of 50% to 70% reported in more recent studies on the prognosis of non-ischemic DCM [29][30][31]. The explicit prevalence of fatal pulmonary embolisms in RV-DCM patients is an important feature of RV-DCM outcomes.…”

Section: Fig 1 Causes Of Death In Dcm Patientssupporting

confidence: 90%

Clinical Profile and Prognosis of Patients with Right Ventricular Dilated Cardiomyopathy: Results of a Prospective Study

Akhmatov¹,

Abdullayev²,

Марданов³

2015

Int J Biomed

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The aim of our study was to investigate the clinical prevalence of dilated cardiomyopathy (DCM) with predominantly failure of the right-side heart (right ventricular DCM, RV-DCM), and features of the clinical course and prognosis of the disease compared to DCM with biventricular heart failure (BV-HF).The study design suggests a prospective observation of 300 patients with idiopathic DCM between 2000 and 2012. Herewith, we followed the criteria of the WHO/ISFC task force (1995) on the definition and classification of cardiomyopathies. All patients underwent a comprehensive examination. Two groups were formed for further comparative analysis. Group 1 included 22 patients (mean age 42.9±14.3 years, male/female 5/17) with RV-DCM. Group 2 included 38 patients (mean age 43.6±13.8, male/female 29/9) with DCM and BV-HF. The groups were matched for age, NYHA class II-III, and disease duration. According to our aim, we studied 5-year survival prognosis and analyzed the incidence and causes of deaths, as well as the occurrence of nonfatal complications of the disease. Medical therapy for DCM patients was performed according to the CHF therapy guidelines (ACC/ AHA 2001.The results of our investigations during many years of research have shown that the clinical incidence of RV-DCM was 7.3% among all forms of DCM. The study of life prognosis in patients with 2 forms of DCM showed that 5-year mortality of patients was about 50%. Fatal pulmonary embolism was a leading cause (50%) in RV-DCM patients.

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Section: Fig 1 Causes Of Death In Dcm Patientssupporting

confidence: 90%

Clinical Profile and Prognosis of Patients with Right Ventricular Dilated Cardiomyopathy: Results of a Prospective Study

Akhmatov¹,

Abdullayev²,

Марданов³

2015

Int J Biomed

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“…The diagnosis of DCM was based on WHO criteria at the Cardiovascular Department, Azienda Ospedaliera-Universitaria of Trieste [14]. …”

Section: Methodsmentioning

confidence: 99%

“…From April 2007 to February 2008, 312 subjects underwent screening: 104 adults with DCM (59 males, 45 females, median age 52 years, range 28-61), 44 first-degree relatives of these patients (27 males, 17 females, median age 44 years, range 18-55), 63 diseased controls (43 males, 20 females, median age 59 years, 39-72) and 101 apparently healthy nurses and residents working in cardiology or paediatric departments (60 males, 41 females, median age 40 years, range 27-50). The diagnosis of DCM was based on WHO criteria at the Cardiovascular Department, Azienda Ospedaliera-Universitaria of Trieste [ 14 ].…”

Section: Methodsmentioning

confidence: 99%

Speeding up coeliac disease diagnosis in cardiological settings

Chicco

Taddio

Sinagra³

et al. 2010

aoms

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IntroductionHigh prevalence of coeliac disease (CD) has been reported among patients with idiopathic dilated cardiomyopathy (DCM). We evaluated the feasibility and diagnostic accuracy of screening for CD by rapid test of anti-transglutaminase antibodies in the cardiology outpatients’ clinic.Material and methodsWe screened the blood samples of 104 patients with DCM, 44 of their first-degree relatives, 63 diseased controls and 101 healthy controls for the presence of anti-transglutaminase antibodies in a drop of whole blood using a rapid assay. This test was compared to the enzyme-linked immunosorbent assay and the anti-endomysium antibody test.ResultsOur rapid test was positive in three (2.9%) DCM patients, in one (2%) relative and in one (1%) healthy control. These subjects were positive at both control assays. Two DCM patients had iron-deficient anaemia. The healthy relative was asymptomatic, while the healthy control experienced extreme asthenia. The relative refused intestinal biopsy, while the others showed histological evidence of CD. During the gluten-free diet, the patient with the worst left ventricular ejection fraction (LVEF) underwent heart transplant, and LVEF values improved in the other two. Anaemia and tiredness resolved in all patients.ConclusionEarly detection of CD in a cardiological setting allows prompt treatment with a gluten-free diet of gluten-dependent complaints with potential benefits for the course of DCM.

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“…The natural history of DCM has dramatically improved in the last 20 years as a result of the introduction in clinical practice of beta-blockers, ACE inhibitors, and mineralocorticoid receptor antagonists which showed not only a reduction in mortality and morbidity but also significant improvements in terms of LV reverse remodeling (LVRR) [27,28,97,98]. Therefore, studies on the prognostic role of echocardiography should be contextualized in their historical phase of conception, keeping in mind that during the last three decades, the gradual optimization of medical therapy has paralleled a significant improvement in survival [99].…”

Section: Prognostic Role Of Echocardiographic Data In Dcmmentioning

confidence: 99%