Aortic root dilatation and its complications are known to be the most important and life limiting features in patients with Marfan syndrome (MFS). Since monitoring of patients, preventive medical and surgical treatments are available nowadays, other MFS pathologies are becoming more relevant for the outcome of the disease. Main pulmonary artery (MPA) dilatation is a cardiac finding, which has not been fully investigated in children. Due to the similarities in tissue composition of the aortic and pulmonary root, MPA dilatation may cause complications and require treatment. In addition, it may be a predictor for severe connective tissue involvement. We retrospectively examined 135 pediatric patients with MFS. 8.1% showed MPA dilatation. MPA dilatation was associated with earlier occurrence of aortic dilatation, mitral valve prolapse, and systemic manifestations of MFS compared with patients without MPA dilatation (p < 0.05). The presence of MPA dilatation was also associated with a higher incidence of ectopia lentis (p < 0.05). Medical treatment was started earlier in MPA dilatation patients than in those without (p < 0.05). We conclude that MPA dilatation is a sign of more severe vascular and connective tissue involvement. Regular examination of the pulmonary artery is essential in MFS to avoid complications. As medical treatment of life threatening MFS events has improved, other features of MFS need to be investigated to improve quality of life.

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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.