Severe aortic wall degeneration after aortic wrapping

Author(s):  
Tristan Ehrlich ◽  
Jan M Federspiel ◽  
Rainer M Bohle ◽  
Hans-Joachim Schäfers

Abstract We report a case in whom aortic wrapping had been performed for aortic dilatation. Ten years later, further progression of aortic root diameter was documented (root size of 66 mm). We performed complete replacement of the proximal aorta. Macroscopically and microscopically, there was extreme degeneration and thinning of the aorta under the graft.

2017 ◽  
Vol 25 (3) ◽  
pp. 192-198
Author(s):  
Pablo Straneo ◽  
Gabriel Parma ◽  
Natalia Lluberas ◽  
Alvaro Marichal ◽  
Gerardo Soca ◽  
...  

Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m−2, p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = −0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Torres Sanabria ◽  
E Garcia Izquierdo Jaen ◽  
V Monivas Palomero ◽  
A Forteza Gil ◽  
S Navarro Rico ◽  
...  

Abstract Background Elastic properties of the thoracic aorta in patients with Marfan Syndrome (MS) have already been evaluated with classic echocardiographic parameters. In the latest years the use of Speckle-Tracking (STE) ecocardiography has been widely extended. Our aim is to describe and provide new parameters of aortic deformation measured by STE in patients with MS. Methods 95 unoperated adult patients with MS and 32 healthy controls were prospectively enrolled. We measured classic parameters of the aortic root using 2D echocardiography. We calculated the posterior aortic wall systolic excursion at the sinuses of Valsalva and ascending aorta using M Mode in TDI colour; with ST 2D ecocardiography we measured the aortic strain at the sinuses of Valsalva (SV) and the anterior and posterior aortic wall displacement at the SV. Aortic distensibility was calculated using the formula: 1000 * (Ds − Dd)/Dd * 1/(Ps − Pd) in mmHg–1 (Ds: systolic and Dd: diastolic diameters, Ps systolic and Pd diastolic blood pressure). Aortic stiffness index was calculated as Ln((Ps/Pd)/(Ds-Dd)/Dd)). Results As shown in the table bellow, patients with MS had lower aortic strain, aortic anterior and posterior wall displacement and impaired aortic distensibility and stiffness index compared to healthy controls. We found a strong negative linear correlation between aortic root diameter at the SV and aortic root strain (r=−0.56, figure 1). Results of statistical analysis MS (n=95) Controls (n=32) p Age (years) 32.84±12.35 32.41±7.98 0.85 Aortic root diameter at the sinuses of Valsalva (mm) 38.82±5.35 30.92±3.65 <0.001 Aortic root strain (%) 4.66±2.45 9.19±2.49 <0.001 Anterior aortic wall displacement STE (mm) 10.39±3.64 13.10±2.26 <0.001 Posterior aortic wall displacement STE (mm) 9.02±2.87 11.04±1.82 <0.001 Aortic distensibility 0.98±0.46 1.37±0.72 0.01 Aortic stiffness index 3.74±0.43 3.47±0.51 0.01 MS = Marfan Syndrome; STE = Speckle Tracking Ecocardiography. Figure 1. Dispersion plot Conclusions Our results suggest that aortic deformation and displacement obtained by STE echocardiography is impaired in MS, showing a reduced distensibility and an increased stiffness of the aortic wall, with a strong negative correlation between aortic root dilation and aortic strain. All these parameters may be useful as additional tools for the diagnosis and follow-up of Marfan patients, and could be useful to to improve the echocardiographic evaluation of the aortic root.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Z Iskandar ◽  
J T J Huang ◽  
I Mordi ◽  
L D Miller ◽  
C Chin ◽  
...  

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. Conclusions Urinary and plasma desmosine levels are significantly higher in patients with BAV compared to controls. Urinary desmosine is also significantly associated with maximal aortic root size, reflecting higher elastin degradation. This suggests a potential use of desmosine as a biomarker to monitor disease progression in patients with BAV. Acknowledgement/Funding Anonymous Trust


1976 ◽  
Vol 17 (4) ◽  
pp. 465-470 ◽  
Author(s):  
Kotaro FURUKAWA ◽  
Junichi YOSHIKAWA ◽  
Kumeo TANAKA ◽  
Chujiro TANAKA ◽  
Seiki KAWAI ◽  
...  

1978 ◽  
Vol 12 ◽  
pp. 381-381
Author(s):  
Lily George ◽  
James W Mathewson ◽  
Anthony A Demaria ◽  
Dean T Mason ◽  
Eli Gold

PEDIATRICS ◽  
1996 ◽  
Vol 98 (6) ◽  
pp. 1149-1153
Author(s):  
Ernest Z. Phillipos ◽  
Murray A. Robertson ◽  
Paul J. Byrne

Objectives. To assess the efficacy of Doppler echocardiography (DE) in the quantification of patent ductus arteriosus (PDA) shunt volume and to correlate PDA shunt volume with clinical outcome in infants with hyaline membrane disease. Methods. Ninety-eight DE studies were performed in 30 preterm ventilated infants with hyaline membrane disease within the first 24 hours of age and then at 48-hour intervals to a maximum of three studies while ventilated with a final study after extubation. Right and left ventricular outputs (QRV and QLV, respectively) and PDA flow were calculated using cross-sectional area and flow velocity integrals. Left atrial-to-aortic root diameter measurements were also taken. Clinical outcomes were correlated with the shunt fraction (QLV/QRV). Results. QLV/QRV demonstrated a linear relationship with the left atrial-to-aortic root diameter ratio (n = 92; r = .79). In the absence of a PDA (n = 33 studies), QRV versus QLV demonstrated a linear relationship (r = .88). In the presence of a PDA (n = 64 studies) the mean QLV (334 ± 133 ml/kg per minute) was significantly greater than the mean QRV (237 ± 84 ml/kg per minute). There was a linear relationship between QLV - QRV (PDA shunt volume) and PDA flow (n = 60; r = .84). In studies with exclusive left-to-right shunting at the PDA (n = 48), the mean QLV - QRV (112 ± 83 ml/kg per minute) was significantly higher than in those with bidirectional shunting (n = 16; mean QLV - QRV = 50 ± 27 ml/kg per minute). Two infants with severe intraventricular hemorrhage (IVH grade 3) and two infants with periventricular leukomalacia (PVL) had significantly higher QLV/QRV (2.09 ± 0.36 and 1.67 ± 0.02 respectively) than those with no IVH (n = 6; QLV/QRV = 1.31 ± 0.18) or those with IVH grades 1 and 2 (n = 8; QLV/QRV = 1.48 ± 0.27). There was no difference in QLV/QRV in infants with or without bronchopulmonary dysplasia retinopathy of prematurity. Necrotizing enterocolitis did not develop in any of the 30 infants. Conclusion. PDA shunt volume can be quantified by DE. Larger studies are needed to correlate clinical outcome with QLV/QRV.


Author(s):  
Ramachandran S. Vasan ◽  
Rebecca J. Song ◽  
Vanessa Xanthakis ◽  
Gary F. Mitchell

Higher central pulse pressure is associated with higher carotid-femoral pulse wave velocity (CFPWV) and an increased risk of cardiovascular disease (CVD). A smaller aortic root diameter (AoR) is associated with higher central pulse pressure. We hypothesized that the combination of a smaller AoR and higher CFPWV is associated with increased CVD risk (relative to a larger AoR and lower CFPWV). We tested this hypothesis in the community-based Framingham Study (N=1970, mean age 60 years, 57% women). We created sex-specific longitudinal echocardiographic AoR trajectories over 2 decades, categorizing participants into smaller versus larger AoR groups. We cross-classified participants based on their AoR trajectory and CFPWV (dichotomized at the sex-specific median). We used Cox regression to relate the cross-classified groups to CVD incidence on follow-up (median 17 years): lower CFPWV, larger AoR (referent group; 6.4/1000 person-years); lower CFPWV, smaller AoR (6.9/1000 person-years); higher CFPWV, larger AoR (23.1/1000 person-years); and higher CFPWV, smaller AoR (21.9/1000 person-years). In sex-pooled analyses, groups with higher CFPWV were associated with a multivariable-adjusted 1.8-fold risk of CVD ( P <0.01) regardless of AoR size. We observed effect modification by sex ( P for sex×AoR-CFPWV group interaction 0.04). In men, the group with smaller AoR and higher CFPWV was associated with a 2.5- to 2.8-fold risk of CVD ( P <0.001). In women, the group with larger AoR and higher CFPWV experienced a statistically nonsignificant 70% to 80% higher CVD risk. Our observations indicate that the prognostic significance of a smaller versus larger AoR varies in men versus women. Additional studies are warranted to confirm our findings.


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