Dilatation of Pulmonary Artery Correlates with Severity of Marfan Phenotype in Childhood

Abstract

Introduction: Marfan syndrome (MFS) is an inherited connective tissue disorder with multifaceted phenotype especially in childhood. Due to risk of dilatation of sinus of valsalvae (SV) and aortic dissection frequent echocardiograpic follow-up is indispensible for MFS patients. Another symptom of MFS is dilatation of pulmonary artery (PA). Measurement of diameter of PA is easy executable using echocardiography and MRI. This study aims to demonstrate the correlation of dilatation of PA with other organ manifestations in MFS in childhood to evaluate its usefulness for estimation of severity of Marfan phenotype.