P390 CARDIAC INVOLVEMENT IN A VERY RARE CASE OF CUTIS LAXA

Abstract

Abstract The "Cutis Laxa" is a rare pathology of the connective tissue characterized by redundant skin and diffuse manifestations are identified by other organs (mainly heart and lungs). The low elasticity of the tissue is believed to be attributable to an anomalous elastin formation. Cutis Laxa is usually a genetic disease, but acquired forms have also been described. The case we describe is that of a 34 –year –old young woman with a diagnosis of Cutis Laxa acquired from the age of 15, complicated by a serious impairment at the pulmonary level in the form of marked emphysema and severe asthmatic framework. It came to our observation following the PEA type cardiovascular arrest episode during tracheostomy procedure, promptly resolved by resuscitation maneuvers according to the Als protocol. The echocardiogram was performed by the only belowocostal window by which a 4 –chamber apical projection was viewed, highlighting an anomalous position of the cardiac Hague (lower and media). The left ventricle appeared to be normal endocavity dimensions and parietal thicknesses with preserved indexes of systo–diastolic functionality in the presence of SIV discline; The right ventricle was instead slightly dilated, hypertrophic, with reduced Chinese of the free wall (nappese 18 mm), the tricuspid regurgitation was moderate with indirect signs of increased lung pressures. The echocardiographic appearance was therefore compatible with a chronic pulmonary heart frame, secondary to progressive pulmonary enfisema, and described in literature as the most fearful complication of Cuis Laxa syndrome, associated with an inauspicious prognosis and premature death.