P987Apparent and persistent healing in spite of severe clinical presentation in idiopathic dilated cardiomyopathy: long-term analysis of a subgroup of patients

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Background: Some patients with Idiopathic Dilated Cardiomyopathy (IDCM), have an initially severe clinical presentation, and present an apparent healing under treatment. Currently, there are no studies focused on this patient subgroup.Aim: To assess the clinical characteristics, natural history and long-term prognosis of this particular IDCM patient population.Methods: 361 consecutive patients with IDCM were enrolled in our Heart Muscle Disease Registry from 1988 to 1997 (males 73%; age 44±14 years; NYHA functional class III-IV 23%; Left Ventricular Ejection Fraction (LVEF) 30±10%, duration of heart failure (HF) at diagnosis 14±25 months). The subgroup of patients characterized by a LVEF at enrolment ≤35% and a subsequent normalization (LVEF≥50%) after 24 (9-36) months on tailored treatment (apparently "healed" IDCM patients) was defined and assessed about clinical and instrumental features during long-term follow-up.Results: The "healed" IDCM patient subgroup counted 22 (6%) cases of our study population (mean age of 42±9 years, males 77%). At enrolment, they had a worse presentation of the disease (NYHA III-IV functional class 27%, LVEF 28±6%, LV end-diastolic volume (EDV) 214±90 ml, significant mitral regurgitation (MR) 46%), but also a shorter history of HF (6±8 months)(p<0.05 vs remaining IDCM patients). Conversely, at 6, 24, 48 and 120 months of follow-up, none of the "healed" IDCM patients was in NYHA III-IV class (p<0.05 within the first six months), the mean LVEF was 48, 55, 51 and 46%, respectively (p<0.05 between 0-6 and 6-24 months), significant MR was present in 15, 0, 0 and 18% of all cases, respectively (p<0.05 between 0-6 months), and mean LVEDV was 154, 128, 128, and 136 ml, respectively (p<0.05 between 0-6 and 6-24 months).Conclusions: In our population with IDCM, some patients (6%) show an impressive improvement leading to normalization of LVEF at mid-term follow-up in tailored medical treatment. This subgroup of patients was characterized by a brief HF history, despite the initial severity of clinical presentation at enrolment. The near-normalization of the main echo-Doppler parameters of ventricular function was achieved and stably maintained during long-term follow-up, suggesting that in those patients the disease could approach a healing stability. The possible influence of genetic background on this favourable evolution has to be investigated in future research.

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