P6505 Urinary desmosine, an elastin-specific degradation product is associated with maximum aortic root size and aortic z-scores in patients with bicuspid aortic valve

Z Iskandar, J T J Huang, I Mordi, L D Miller, C Chin, D Goudie, C Mcwilliam, J Berg, A Neagoie, C C Lang, A M Choy

Research output: Contribution to conferenceAbstract

Abstract

Background Bicuspid aortic valve (BAV) is the commonest congenital cardiac abnormality affecting up to 2% of the general population. BAV is highly diverse in phenotype however a common feature is its inherent risk of morbidity and mortality from aortic root dilatation and dissection. Aortic dilatation involves elastin degradation and desmosine is an amino acid cross-link that is released into the bloodstream and urine following elastin degradation. It is known from an earlier pilot study (DESMA) of desmosine in Marfan Syndrome (MFS) patients that plasma desmosine among patients with MFS is significantly elevated and correlates with aortic size however whether this same observation is seen in other forms of inherited aortopathies such as BAV is unclear. Objectives 1. To investigate whether patients with BAV have higher elastin degradation as indicated by plasma desmosine and urinary desmosine levels. 2. To explore the relationship between plasma and urinary desmosine levels with aortic root size in patients with BAV. Methods We measured urinary (ng/mg creatinine) and plasma desmosine (ng/mL) in 20 patients with BAV and healthy control subjects using liquid chromatography-tandem mass spectrometry (LC-MS/MS). Aortic root size and corresponding Z-scores were measured on echocardiogram. Correlation was analysed with Spearman's rank test. Results The patients with BAV were predominantly male (n=15, 75%) with a mean age of 50.5 years ± 17.6 [SD]. All the BAV patients had normal LV systolic function and none had prior aortic root surgery or were current smokers. Nine BAV patients had treated hypertension. Compared to controls, both plasma desmosine (0.30±0.10 vs 0.26±0.075 ng/mL, p=0.01) and urinary desmosine (15.9±4.6 vs 7.2±2.8 ng/mg creatinine, p<0.001) were significantly elevated in patients with BAV. Urinary and plasma desmosine (Figure 1) were also significantly correlated (r=0.55, p=0.01). There was a significant association between urinary desmosine and maximal aortic root size and Z-scores in the BAV cohort (Figure 2) compared to controls (p=0.02), however this was not seen with plasma desmosine.
Original languageEnglish
Pagesehz746.1095
DOIs
Publication statusPublished - 21 Oct 2019
EventESC Congress 2019: together with the World Congress of Cardiology - Paris expo Porte de Versailles - VIPARIS, Paris, France
Duration: 31 Aug 20194 Sep 2019
https://en.convention.parisinfo.com/paris-show-exhibition/165578/esc-congress-2019-european-society-of-cardiology

Conference

ConferenceESC Congress 2019
CountryFrance
CityParis
Period31/08/194/09/19
Internet address

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Desmosine
Elastin
Aortic Valve
Marfan Syndrome
Bicuspid Aortic Valve
Dilatation
Creatinine
Tandem Mass Spectrometry
Liquid Chromatography

Cite this

@conference{35ce0225e93c4c9ba07c45cc2df75592,
title = "P6505 Urinary desmosine, an elastin-specific degradation product is associated with maximum aortic root size and aortic z-scores in patients with bicuspid aortic valve",
abstract = "Background Bicuspid aortic valve (BAV) is the commonest congenital cardiac abnormality affecting up to 2{\%} of the general population. BAV is highly diverse in phenotype however a common feature is its inherent risk of morbidity and mortality from aortic root dilatation and dissection. Aortic dilatation involves elastin degradation and desmosine is an amino acid cross-link that is released into the bloodstream and urine following elastin degradation. It is known from an earlier pilot study (DESMA) of desmosine in Marfan Syndrome (MFS) patients that plasma desmosine among patients with MFS is significantly elevated and correlates with aortic size however whether this same observation is seen in other forms of inherited aortopathies such as BAV is unclear. Objectives 1. To investigate whether patients with BAV have higher elastin degradation as indicated by plasma desmosine and urinary desmosine levels. 2. To explore the relationship between plasma and urinary desmosine levels with aortic root size in patients with BAV. Methods We measured urinary (ng/mg creatinine) and plasma desmosine (ng/mL) in 20 patients with BAV and healthy control subjects using liquid chromatography-tandem mass spectrometry (LC-MS/MS). Aortic root size and corresponding Z-scores were measured on echocardiogram. Correlation was analysed with Spearman's rank test. Results The patients with BAV were predominantly male (n=15, 75{\%}) with a mean age of 50.5 years ± 17.6 [SD]. All the BAV patients had normal LV systolic function and none had prior aortic root surgery or were current smokers. Nine BAV patients had treated hypertension. Compared to controls, both plasma desmosine (0.30±0.10 vs 0.26±0.075 ng/mL, p=0.01) and urinary desmosine (15.9±4.6 vs 7.2±2.8 ng/mg creatinine, p<0.001) were significantly elevated in patients with BAV. Urinary and plasma desmosine (Figure 1) were also significantly correlated (r=0.55, p=0.01). There was a significant association between urinary desmosine and maximal aortic root size and Z-scores in the BAV cohort (Figure 2) compared to controls (p=0.02), however this was not seen with plasma desmosine.",
author = "Z Iskandar and Huang, {J T J} and I Mordi and Miller, {L D} and C Chin and D Goudie and C Mcwilliam and J Berg and A Neagoie and Lang, {C C} and Choy, {A M}",
year = "2019",
month = "10",
day = "21",
doi = "10.1093/eurheartj/ehz746.1095",
language = "English",
pages = "ehz746.1095",
note = "ESC Congress 2019 : together with the World Congress of Cardiology ; Conference date: 31-08-2019 Through 04-09-2019",
url = "https://en.convention.parisinfo.com/paris-show-exhibition/165578/esc-congress-2019-european-society-of-cardiology",

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P6505 Urinary desmosine, an elastin-specific degradation product is associated with maximum aortic root size and aortic z-scores in patients with bicuspid aortic valve. / Iskandar, Z; Huang, J T J; Mordi, I; Miller, L D; Chin, C; Goudie, D; Mcwilliam, C; Berg, J; Neagoie, A; Lang, C C; Choy, A M.

2019. ehz746.1095 Abstract from ESC Congress 2019, Paris, France.

Research output: Contribution to conferenceAbstract

TY - CONF

T1 - P6505 Urinary desmosine, an elastin-specific degradation product is associated with maximum aortic root size and aortic z-scores in patients with bicuspid aortic valve

AU - Iskandar, Z

AU - Huang, J T J

AU - Mordi, I

AU - Miller, L D

AU - Chin, C

AU - Goudie, D

AU - Mcwilliam, C

AU - Berg, J

AU - Neagoie, A

AU - Lang, C C

AU - Choy, A M

PY - 2019/10/21

Y1 - 2019/10/21

N2 - Background Bicuspid aortic valve (BAV) is the commonest congenital cardiac abnormality affecting up to 2% of the general population. BAV is highly diverse in phenotype however a common feature is its inherent risk of morbidity and mortality from aortic root dilatation and dissection. Aortic dilatation involves elastin degradation and desmosine is an amino acid cross-link that is released into the bloodstream and urine following elastin degradation. It is known from an earlier pilot study (DESMA) of desmosine in Marfan Syndrome (MFS) patients that plasma desmosine among patients with MFS is significantly elevated and correlates with aortic size however whether this same observation is seen in other forms of inherited aortopathies such as BAV is unclear. Objectives 1. To investigate whether patients with BAV have higher elastin degradation as indicated by plasma desmosine and urinary desmosine levels. 2. To explore the relationship between plasma and urinary desmosine levels with aortic root size in patients with BAV. Methods We measured urinary (ng/mg creatinine) and plasma desmosine (ng/mL) in 20 patients with BAV and healthy control subjects using liquid chromatography-tandem mass spectrometry (LC-MS/MS). Aortic root size and corresponding Z-scores were measured on echocardiogram. Correlation was analysed with Spearman's rank test. Results The patients with BAV were predominantly male (n=15, 75%) with a mean age of 50.5 years ± 17.6 [SD]. All the BAV patients had normal LV systolic function and none had prior aortic root surgery or were current smokers. Nine BAV patients had treated hypertension. Compared to controls, both plasma desmosine (0.30±0.10 vs 0.26±0.075 ng/mL, p=0.01) and urinary desmosine (15.9±4.6 vs 7.2±2.8 ng/mg creatinine, p<0.001) were significantly elevated in patients with BAV. Urinary and plasma desmosine (Figure 1) were also significantly correlated (r=0.55, p=0.01). There was a significant association between urinary desmosine and maximal aortic root size and Z-scores in the BAV cohort (Figure 2) compared to controls (p=0.02), however this was not seen with plasma desmosine.

AB - Background Bicuspid aortic valve (BAV) is the commonest congenital cardiac abnormality affecting up to 2% of the general population. BAV is highly diverse in phenotype however a common feature is its inherent risk of morbidity and mortality from aortic root dilatation and dissection. Aortic dilatation involves elastin degradation and desmosine is an amino acid cross-link that is released into the bloodstream and urine following elastin degradation. It is known from an earlier pilot study (DESMA) of desmosine in Marfan Syndrome (MFS) patients that plasma desmosine among patients with MFS is significantly elevated and correlates with aortic size however whether this same observation is seen in other forms of inherited aortopathies such as BAV is unclear. Objectives 1. To investigate whether patients with BAV have higher elastin degradation as indicated by plasma desmosine and urinary desmosine levels. 2. To explore the relationship between plasma and urinary desmosine levels with aortic root size in patients with BAV. Methods We measured urinary (ng/mg creatinine) and plasma desmosine (ng/mL) in 20 patients with BAV and healthy control subjects using liquid chromatography-tandem mass spectrometry (LC-MS/MS). Aortic root size and corresponding Z-scores were measured on echocardiogram. Correlation was analysed with Spearman's rank test. Results The patients with BAV were predominantly male (n=15, 75%) with a mean age of 50.5 years ± 17.6 [SD]. All the BAV patients had normal LV systolic function and none had prior aortic root surgery or were current smokers. Nine BAV patients had treated hypertension. Compared to controls, both plasma desmosine (0.30±0.10 vs 0.26±0.075 ng/mL, p=0.01) and urinary desmosine (15.9±4.6 vs 7.2±2.8 ng/mg creatinine, p<0.001) were significantly elevated in patients with BAV. Urinary and plasma desmosine (Figure 1) were also significantly correlated (r=0.55, p=0.01). There was a significant association between urinary desmosine and maximal aortic root size and Z-scores in the BAV cohort (Figure 2) compared to controls (p=0.02), however this was not seen with plasma desmosine.

U2 - 10.1093/eurheartj/ehz746.1095

DO - 10.1093/eurheartj/ehz746.1095

M3 - Abstract

SP - ehz746.1095

ER -