Natural History of Dilated Cardiomyopathy in Children

The long-term progression of idiopathic dilated cardiomyopathy (DCM) in pediatric patients compared with adult patients has not been previously characterized. In this study, we compared outcome and long-term progression of pediatric and adult DCM populations

Conflicting gender-related differences in the natural history of patients with Idiopathic Dilated Cardiomyopathy

Objective. To evaluated possible clinical and instrumental, natural history and prognostic divergences in women and men with idiopathic dilated cardiomyopathy (IDCM). Patients and Methods. From 1988 to 2012, we evaluated 803 consecutive patients with IDCM recorded in the Heart Muscle Disease Registry of Trieste (Italy). All patients had serial follow-up evaluations at 6, 12, and 24 months, and subsequently every two years, or more frequently if clinically indicated. Results. Two hundred and twenty-seven patients (28%) were female. At first evaluation women were significantly older (48 vs. 45 years old, p = 0.008); presented more frequently left bundle branch block at ECG (38% vs. 28%, p = 0.01), smaller left ventricular end-diastolic indexed volume at echocardiography (85 vs. 93 ml/m2, p <0.002) and more frequently moderate to severe mitral regurgitation at Doppler (43% vs. 33%, p = 0.015). No differences in NYHA class, medical treatment and device implantation rates were found. During a median of 108 months follow-up, women showed a significantly lower ten-year total mortality/heart transplantation (20% vs. 32% respectively, p = 0.001) and cardiovascular mortality rates (9% vs. 15%, p = 0.024) despite a less marked clinical and echocardiographic improvement. Conclusions. In our population of patients with IDCM, women showed a better long-term prognosis notwithstanding a presentation with a more advanced disease and a lower clinical-instrumental improvement on optimal medical therapy compared to men.&nbsp

Conflicting gender-related differences in the natural history of patients with idiopathic dilated cardiomyopathy

Objective: To evaluated possible clinical and instrumental, natural history and prognostic divergences in women and men with idiopathic dilated cardiomyopathy (IDCM). Patients and Methods: From 1988 to 2012, we evaluated 803 consecutive patients with IDCM recorded in the Heart Muscle Disease Registry of Trieste (Italy). All patients had serial follow-up evaluations at 6, 12, and 24 months, and subsequently every two years, or more frequently if clinically indicated. Results: Two hundred and twenty-seven patients (28%) were female. At first evaluation women were significantly older (48 vs. 45 years old, p = 0.008); presented more frequently left bundle branch block at ECG (38% vs. 28%, p = 0.01), smaller left ventricular end-diastolic indexed volume at echocardiography (85 vs. 93 ml/m2, p <0.002) and more frequently moderate to severe mitral regurgitation at Doppler (43% vs. 33%, p = 0.015). No differences in NYHA class, medical treatment and device implantation rates were found. During a median of 108 months follow-up, women showed a significantly lower ten-year total mortality/heart transplantation (20% vs. 32% respectively, p = 0.001) and cardiovascular mortality rates (9% vs. 15%, p = 0.024) despite a less marked clinical and echocardiographic improvement. Conclusion: In our population of patients with IDCM, women showed a better long-term prognosis notwithstanding a presentation with a more advanced disease and a lower clinical-instrumental improvement on optimal medical therapy compared to men

Long-term outcome of 'super-responder' patients to cardiac resynchronization therapy.

AIMS: To evaluate the long-term changes of clinical and echocardiographic parameters, the incidence of cardiac events and parameters associated with late cardiac events in 'super-responders' to cardiac resynchronization therapy (CRT) with [CRT defibrillator (CRT-D)] or without defibrillator back-up. METHODS AND RESULTS: In all consecutive patients treated with CRT in two Italian centres (Trieste and Udine) with left ventricular ejection fraction (LVEF) 640.35 at implantation (Timp) and LVEF > 0.50 1 and/or 2 years (Tnorm) after implantation, the long-term outcome and the evolution of echocardiographic parameters were assessed; factors associated with a higher risk of cardiac events, defined as hospitalization or death for heart failure (HF), sudden death, or CRT-D appropriate interventions, were also analysed. Among the 259 patients evaluated, 62 (24%) had LVEF 65 0.50 at Tnorm (n = 44 with at 1 year, n = 18 at 2 years). During a mean follow-up of 68 \ub1 30 months, one cardiac death (for HF) and eight cardiovascular events (two hospitalization for HF and six appropriate CRT-D interventions) occurred. At the last echo evaluation (Tfup) performed 51 \ub1 26 months after Timp, LVEF was 0.45 in four of them). At univariable analysis, only LV end-systolic volume evaluated at Tfup was associated with a higher risk of cardiac events during follow-up. CONCLUSION: In 'super-responders' to CRT long-term outcome is excellent. However, cardiac events, mainly CRT-D appropriate interventions, can occur despite the persistence of LVEF > 0.50. Early identification of these patients is still an unsolved issu