P899Clinical, electrocardiographic, echocardiographic and cardiac magnetic resonance imaging follow-up in patients with non-compaction cardiomyopathy in isolation or in association with other diseases

Abstract

Background: Non-compaction cardiomyopathy (NCC) is a rare disease, which main clinical manifestations are heart failure, arrhythmias and embolic events. The evolution of these patients is poorly known. Thus, the aims of this study was to analyze the late follow-up of patients with isolated NCC or associated with other diseases, screening of their first and second degree relatives, which were followed at a university cardiology hospital, and evaluate possible relationships between clinical outcomes with morphological and functional cardiac exams variables. Method(s): Patients with NCC confirmed by echocardiographic (echo) and/or cardiac magnetic resonance imaging (MRI) criteria were selected. During their follow-up, their relatives were recruted to perform screening of the disease using echo. The following variables were included: death, cardiac transplantation, embolic events (stroke, pulmonary embolism), hospital admissions, complex ventricular arrhythmias, atrial fibrillation (AF), presence of late gadolinium enhancement in MRI, echo and MRI indices, cardiac device implantation. Children (under 12 years old) were analyzed separately. Result(s): Two hundred and fifteen patients with NCC were followed for 5.9+/-4.47 years, mean age of 36.6+/-17.6 years, 108 (50.2%) males. Patients were divided into 2 groups: 193 with isolated NCC (Group 1) and 22 with mixed NCC (associated with genetic and congenital diseases, Chagas disease and giant cell myocarditis; Group 2). Palpitation was the most frequent clinical symptom, present in 42,8% of them. The survival rate was lower in Group 2 patients (p<0.05), left ventricular (LV) ejection fraction (EF) less than 50% (p=0.004), increased LV enddiastolic diameter and volume (p=0.018 and 0.017, respectively), with LV diastolic dysfunction (p=0.049), with right ventricular systolic dysfunction (p=0.003), hospital admissions (p<0.001), embolic events (p=0.022), complex ventricular arrhythmias (p=0.010) and pulmonary hypertension (p<0.001). All patients with isolated NCC and AF presented LVEF less than 0.40, and between patients without AF, only 41.7% presented LVEF less than 0.40 (p=0.048). Twenty-three patients was children, followed for 4.41+/-4.91 years, mean age of 5.52+/-3.62 years, 12 (52.2%) males. The proportion of deaths or cardiac transplantation was 3 times higher than in the adult population (34.8%) and heart failure was the most common outcome. Cardiac arrhythmias were rare and the embolic events were not found in this group. The familiar screening diagnosed NCC in 36.7% of the patients. Conclusion(s): The clinical and cardiac imaging methods allows us to draw a profile of this population and to estimate the risk of complications, emphasizing the need for early diagnosis and treatment. In children, NCC used to develop more severe disease. Family screening is a very important tool, allowing the identification of patients with subclinical stage of the disease.