Abstract 2247: Patent Foramen Ovale And Marfan Syndrome: A New Association
Marfan syndrome (MS) is the most common inherited disorder of connective tissue which affects multiple organ systems. Cardiovascular involvement in MS includes dilatation or dissection of the aortic root, mitral (MVP) and tricuspid valve prolapse (TVP), pulmonary artery dilatation and mitral subvalvular calcification. Patent foramen ovale (PFO) has been associated with aortic root dilatation and MVP, but there are no data about incidence of PFO in MS.
Aim: to assess the incidence of PFO in MS and its association with other cardiovascular manifestations in these patients.
Methods: From 2004 to 2008 101 patients (mean age 31 ± 10.6 years), previously diagnosed of MS in basis of “Ghent nosology”, were examined in the outpatient MS unit. All patients underwent a transthoracic echocardiographic examination. Aortic root dilatation was defined according to body surface area and age. For PFO study, imaging was performed in the apical four chamber view, with injection of 10 ml of agitated saline at rest and with Valsalva manoeuvre. The existence of PFO was determined by the presence of bubbles in the left heart within five cardiac cycles.
Results: The spectrum of echocardiographic findings included: aortic root dilatation (69%, mean diameter 39 ± 7, range 27 to 56 mm), MVP (62%), PFO (61%), aortic dissection (7%), TVP (24%), pulmonary dilatation (9%), mitral subvalvular calcification (4%), and atrial septum aneurysm (ASA) (12%). We did not find association between the presence of PFO and aortic root dilatation or MVP. Aortic root diameter was not significantly different between patients with or without PFO (38 ± 6 vs 40 ± 7 mm). Only ASA was associated with PFO (p = 0.003).
Conclusions: Among the echocardiographic findings in MS, aortic dilatation resulted to be the most frequent, followed by MVP and PFO. Incidence of PFO in MS was significantly higher than in general population. In MS, PFO was not associated with aortic dilatation or MVP. So we suggest PFO could be part of the spectrum of disorders of connective tissue associated with MS.