The main pulmonary artery in adults: a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome

Orphanet Journal of Rare Diseases

Yskert von Kodolitsch⁶  Stefan Blankenberg⁶  Jürgen Berger⁴  Jörg Schmidtke³  Peter N Robinson⁸  Tilo Kölbel⁶  Thomas S Mir⁶  Robert M Radke⁵  Kai Mortensen²  Britta Keyser³  Peter Bannas⁷  Dietmar Koschyk⁶  Alexander M Bernhardt⁶  Helke Schüler⁶  Mathias Hillebrand⁶  Meike Rybczynski⁶  Neda Rahimian Gorgan⁶  Julie De Backer¹  Sara Sheikhzadeh⁶ 

[1] Centre for Medical Genetics, University Hospital Ghent, Ghent, Belgium;Medizinische Klinik II / Kardiologie, Angiologie, am Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Germany;Institute of Human Genetics, Hannover Medical School, Hannover, Germany;Department of Medical Biometry and Epidemiology, University Hospital Eppendorf, Hamburg, Germany;Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Münster, Germany;Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, Hamburg, 20246, Germany;Department of Diagnostic and Interventional Radiology, University Hospital Eppendorf, Hamburg, Germany;Institute of Medical Genetics, Charité Universitätsmedizin Berlin, Berlin, Germany

关键词: Reference values;    Echocardiography;    FBN1;    Marfan syndrome;    Pulmonary artery;   

Others  :  1138697
DOI  :  10.1186/s13023-014-0203-8

 received in 2014-07-09, accepted in 2014-11-26,  发布年份 2014

【 摘 要 】

Background

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. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS).

Methods

We 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.

Results

MPA 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.

Conclusions

We 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.

【 授权许可】

   
2014 Sheikhzadeh et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

Figure 3.

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