title: 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.
abstract: 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.
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. s.sheikhzadeh@uke.de.
authors.forename: Sara
authors.initials: S
authors.lastname: Sheikhzadeh
authors.affiliation: Centre for Medical Genetics, University Hospital Ghent, Ghent, Belgium. julie.debacker@ugent.be.
authors.forename: Julie
authors.initials: J
authors.lastname: De Backer
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. palatum28@yahoo.de.
authors.forename: Neda Rahimian
authors.initials: NR
authors.lastname: Gorgan
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. m.berner@uke.de.
authors.forename: Meike
authors.initials: M
authors.lastname: Rybczynski
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. m.hillebrand@uke.de.
authors.forename: Mathias
authors.initials: M
authors.lastname: Hillebrand
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. s.blankenberg@uke.de.
authors.forename: Helke
authors.initials: H
authors.lastname: Schüler
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. al.bernhardt@uke.de.
authors.forename: Alexander M
authors.initials: AM
authors.lastname: Bernhardt
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. koschyk@uke.de.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Koschyk
authors.affiliation: Department of Diagnostic and Interventional Radiology, University Hospital Eppendorf, Hamburg, Germany. p.bannas@uke.de.
authors.forename: Peter
authors.initials: P
authors.lastname: Bannas
authors.affiliation: Institute of Human Genetics, Hannover Medical School, Hannover, Germany. keyser.britta@mh-hannover.de.
authors.forename: Britta
authors.initials: B
authors.lastname: Keyser
authors.affiliation: Medizinische Klinik II / Kardiologie, Angiologie, am Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Germany. Kai.Mortensen@uk-sh.de.
authors.forename: Kai
authors.initials: K
authors.lastname: Mortensen
authors.affiliation: Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Münster, Germany. robert.radke@ukmuenster.de.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Radke
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. mir@uke.de.
authors.forename: Thomas S
authors.initials: TS
authors.lastname: Mir
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. t.koelbel@uke.de.
authors.forename: Tilo
authors.initials: T
authors.lastname: Kölbel
authors.affiliation: Institute of Medical Genetics, Charité Universitätsmedizin Berlin, Berlin, Germany. peter.robinson@charite.de.
authors.forename: Peter N
authors.initials: PN
authors.lastname: Robinson
authors.affiliation: Institute of Human Genetics, Hannover Medical School, Hannover, Germany. Schmidtke.Joerg@mh-hannover.de.
authors.forename: Jörg
authors.initials: J
authors.lastname: Schmidtke
authors.affiliation: Department of Medical Biometry and Epidemiology, University Hospital Eppendorf, Hamburg, Germany. berger@uke.de.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Berger
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. s.blankenberg@uke.de.
authors.forename: Stefan
authors.initials: S
authors.lastname: Blankenberg
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany. kodolitsch@uke.de.
authors.forename: Yskert
authors.initials: Y
authors.lastname: von Kodolitsch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001014:Aortic Aneurysm / Q000000981:diagnostic imaging* / Q000503:physiopathology; D003430:Cross-Sectional Studies; D004452:Echocardiography / Q000592:standards; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008875:Middle Aged; D011651:Pulmonary Artery / Q000000981:diagnostic imaging* / Q000503:physiopathology; D012016:Reference Values; D012189:Retrospective Studies; D014664:Vasodilation; D055815:Young Adult