Pulmonary artery root dilatation in Marfan syndrome: quantitative assessment of an unknown criterion

Marfan syndrome is a connective tissue disorder with manifestations in multiple organ systems.1 Dilatation of the main pulmonary artery is one of the established criteria for the diagnosis of Marfan syndrome. Normal values have been assessed for the pulmonary artery bifurcation but not for the pulmonary artery root .2 Since the pulmonary artery root consists basically of the same tissue as the ascending aorta, dilatation of the pulmonary artery might predominantly occur in the root similar to the dilatation process occurring in the ascending aorta in Marfan patients.3 The prevalence and prognosis of pulmonary artery dilatation in Marfan syndrome is still unknown. The aim of the present study was to assess main pulmonary artery dimensions both at the level of the pulmonary artery bifurcation and at the pulmonary artery root in normal subjects and in Marfan patients with and without aortic root replacement. Fifty consecutive Marfan patients (mean age 33 (10) years, 34 men, 16 women) underwent routine cardiac magnetic resonance (MR) imaging. Thirty five of these patients had previously undergone aortic root replacement. The control group consisted of 15 age and sex matched healthy subjects (mean age 28 (4) years, nine men, six women). Main pulmonary artery dimensions were assessed …