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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

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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. / Sheikhzadeh, Sara; De Backer, Julie; Gorgan, Neda Rahimian; Rybczynski, Meike; Hillebrand, Mathias; Schüler, Helke; Bernhardt, Alexander M; Koschyk, Dietmar; Bannas, Peter; Keyser, Britta; Mortensen, Kai; Radke, Robert M; Mir, Thomas S; Kölbel, Tilo; Robinson, Peter N; Schmidtke, Jörg; Berger, Juergen; Blankenberg, Stefan; Kodolitsch, Yskert.

In: ORPHANET J RARE DIS, Vol. 9, No. 1, 10.12.2014, p. 203.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Sheikhzadeh, S, De Backer, J, Gorgan, NR, Rybczynski, M, Hillebrand, M, Schüler, H, Bernhardt, AM, Koschyk, D, Bannas, P, Keyser, B, Mortensen, K, Radke, RM, Mir, TS, Kölbel, T, Robinson, PN, Schmidtke, J, Berger, J, Blankenberg, S & Kodolitsch, Y 2014, '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 J RARE DIS, vol. 9, no. 1, pp. 203. https://doi.org/10.1186/s13023-014-0203-8

APA

Sheikhzadeh, S., De Backer, J., Gorgan, N. R., Rybczynski, M., Hillebrand, M., Schüler, H., Bernhardt, A. M., Koschyk, D., Bannas, P., Keyser, B., Mortensen, K., Radke, R. M., Mir, T. S., Kölbel, T., Robinson, P. N., Schmidtke, J., Berger, J., Blankenberg, S., & Kodolitsch, Y. (2014). 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 J RARE DIS, 9(1), 203. https://doi.org/10.1186/s13023-014-0203-8

Vancouver

Sheikhzadeh S, De Backer J, Gorgan NR, Rybczynski M, Hillebrand M, Schüler H et al. 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 J RARE DIS. 2014 Dec 10;9(1):203. https://doi.org/10.1186/s13023-014-0203-8

Bibtex

@article{ce9ed1b97c0a4d5697146102071cc140,
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 = "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.",
author = "Sara Sheikhzadeh and {De Backer}, Julie and Gorgan, {Neda Rahimian} and Meike Rybczynski and Mathias Hillebrand and Helke Sch{\"u}ler and Bernhardt, {Alexander M} and Dietmar Koschyk and Peter Bannas and Britta Keyser and Kai Mortensen and Radke, {Robert M} and Mir, {Thomas S} and Tilo K{\"o}lbel and Robinson, {Peter N} and J{\"o}rg Schmidtke and Juergen Berger and Stefan Blankenberg and Yskert Kodolitsch",
year = "2014",
month = dec,
day = "10",
doi = "10.1186/s13023-014-0203-8",
language = "English",
volume = "9",
pages = "203",
journal = "ORPHANET J RARE DIS",
issn = "1750-1172",
publisher = "BioMed Central Ltd.",
number = "1",

}

RIS

TY - JOUR

T1 - 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

AU - Sheikhzadeh, Sara

AU - De Backer, Julie

AU - Gorgan, Neda Rahimian

AU - Rybczynski, Meike

AU - Hillebrand, Mathias

AU - Schüler, Helke

AU - Bernhardt, Alexander M

AU - Koschyk, Dietmar

AU - Bannas, Peter

AU - Keyser, Britta

AU - Mortensen, Kai

AU - Radke, Robert M

AU - Mir, Thomas S

AU - Kölbel, Tilo

AU - Robinson, Peter N

AU - Schmidtke, Jörg

AU - Berger, Juergen

AU - Blankenberg, Stefan

AU - Kodolitsch, Yskert

PY - 2014/12/10

Y1 - 2014/12/10

N2 - 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.

AB - 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.

U2 - 10.1186/s13023-014-0203-8

DO - 10.1186/s13023-014-0203-8

M3 - SCORING: Journal article

C2 - 25491897

VL - 9

SP - 203

JO - ORPHANET J RARE DIS

JF - ORPHANET J RARE DIS

SN - 1750-1172

IS - 1

ER -