Abstract
BackgroundEchocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS).MethodsWe enrolled 123 normal adults (72 men, 52 women aged 42 ± 14 years) and 98 patients with MFS (42 men, 56 women aged 39 ± 14 years) in a retrospective cross-sectional observational controlled study in four tertiary care centers. We defined outcome measures including upper normal limits of MPA-d and MPA-r as 95 quantile of normal persons, MPA dilatation as diameters > upper normal limits, MPA aneurysm as diameters >4 cm, and indication for surgery as MPA diameters >6 cm.ResultsMPA diameters revealed normal distribution without correlation to age, sex, body weight, body height, body mass index and body surface area. The upper normal limit was 2.6 cm (95% confidence interval (CI) =2.44-2.76 cm) for MPA-d, and 1.05 (95% CI = .86–1.24) for MPA-r. MPA dilatation presented in 6 normal persons (4.9%) and in 68 MFS patients (69.4%; P < .001), MPA aneurysm presented only in MFS (15 patients; 15.3%; P < .001), and no patient required surgery. Mean MPA-r were increased in MFS (P < .001), but ratios >1.05 were equally frequent in 7 normal persons (5%) and in 8 MFS patients (10.5%; P = .161). MPA-r related to aortic root diameters (P = .042), reduced left ventricular ejection fraction (P = .006), and increased pulmonary artery systolic pressures (P = .040). No clinical manifestations of MFS and no FBN1 mutation characteristics related to MPA diameters.ConclusionsWe established 2.6 cm for MPA-d and 1.05 for MPA-r as upper normal limits. MFS exhibits a high prevalence of MPA dilatation and aneurysm. However, patients may require MPA surgery only in scarce circumstances, most likely because formation of marked MPA aneurysm may require LV dysfunction and increased PASP.
Highlights
Echocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults
Some case reports highlight that MPA dilatation may lead to severe complications in Marfan syndrome (MFS) [10], and current magnetic resonance imaging (MRI) and computed tomography (CT) studies suggest that pathology of the MPA is frequent in MFS [3,4,5,11,12]
Indicate that pulmonary hypertension (PH) is required as an additional factor to manifest formation of marked MPA aneurysm in MFS
Summary
Echocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. The old Ghent nosology considered dilatation of the main pulmonary artery (MPA) as a diagnostic criterion of Marfan syndrome (MFS) [1] but data on the prevalence of MPA dilatation or aneurysm remained both scarce and conflicting in MFS [2,3,4,5]. Cardiovascular criteria of MFS are usually assessed with transthoracic echocardiography, but in adults upper normal limits of diameters of the MPA (MPA-d) remain to be defined on echocardiography [9]. There is no current data on the echocardiographic prevalence of MPA dilatation in MFS patients, and for this reason the revised Ghent nosology removed MPA dilatation from the list of diagnostic criteria [9]. Echocardiographic examination of the MPA may be important in the evaluation of adults with MFS to further elucidate the diagnostic and prognostic implications of MPA dilatation
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