Clinical presentation and echocardiographic characteristics in women with peripartum cardiomyopathy: insights from the Italian multicentre registry

Abstract Background Peripartum cardiomyopathy (PPCM) is a rare but potentially life-threatening form of heart failure (HF) occurring toward the end of pregnancy or in the months following delivery. Variable clinical presentations and progression have been reported worldwide. Purpose We conducted a national, multicenter, observational registry to characterize clinical, echocardiographic and circulating multi-omics features of PPCM patients in Italy, and report here results of clinical and echocardiographic evaluations. This study was founded by the European Union. Methods Women with history of PPCM or newly diagnosed cases were considered eligible. Inclusion criteria were (i) age ≥18 years; (ii) development of HF signs or symptoms in the last month of pregnancy or within 5 months of delivery, (iii) absence of an identifiable cause of HF; (iv) left ventricle ejection fraction (LVEF) ≤45 % (v) absence of recognizable heart disease before the last month of pregnancy. Data included clinical details, echocardiographic parameters, and blood samples to perform multi-omic profiling. Results Twenty-eight women with confirmed PPCM diagnosis were enrolled. The mean age at clinical onset was 33.9 ± 5.1 years. Six patients (21.4%) experienced hypertensive disorders of pregnancy and 14.3% pre-eclampsia. Overweight was observed in 25% of patients, and few of them (11%) had severe obesity. Two thirds of women presented with severe symptoms (NYHA class III/IV) within the first month after delivery, and 67% had an initial LVEF ≤35%. In fewer cases (25%) the clinical presentation consisted of an arrhythmic phenotype. A persistent severe LV dysfunction (LVEF35%) was reported in 14% of patients, that required cardioverter-defibrillator implantation. Despite the initial severity, 50% of patients showed complete recovery of systolic function over an average follow-up of 11 ± 19 months, with no reported deaths. At transthoracic echocardiography, when categorized according to the subsequent LV systolic function, women with persisting LV dysfunction had more dilated LV (end-diastolic diameter 61.2±8.1 mm in LVEF35% group vs 51.8±6.9 mm in 35%EF p="0.017), left" Conclusions PPCM presents with heterogeneous clinical and imaging profiles. Though initial presentation is often severe, outcomes are favorable in many cases. Clinical and echocardiographic factors related to LVEF recovery are still unknown. These findings emphasize the need for increased awareness and further investigation, especially regarding underlying mechanisms and longitudinal outcomes through integrated multi-omics approaches./EF