Proximal Aortic Dilatation Research Articles

Comparison of aortic media changes in patients with bicuspid aortic valve stenosis versus bicuspid valve insufficiency and proximal aortic aneurysm

The aim of this study was to evaluate aortic media changes in bicuspid aortic valve (BAV) patients who underwent aortic valve replacement (AVR) and simultaneous replacement of the proximal aorta for BAV stenosis vs BAV insufficiency. Review of our institutional BAV database identified a subgroup of 79 consecutive BAV patients (mean age 52.3 ± 13 years, 81% men) with BAV stenosis or insufficiency and concomitant proximal aortic dilatation of ≥50 mm who underwent AVR and simultaneous replacement of proximal aorta from 1995 through 2005. All cases of BAV disease and concomitant ascending aortic dilatation of 40-50 mm underwent isolated AVR and therefore were excluded from this analysis. Proximal aortic media elastic fibre loss (EFL) was assessed (graded 0 to 3+) and compared between patients with BAV stenosis (Group I, n = 44) vs BAV insufficiency (Group II, n = 35). Follow-up (690 patient-years) was 100% complete and 9.1 ± 4.6 years long. Mean aortic media EFL was 1.3 ± 0.7 in Group I vs 2.5 ± 0.8 in Group II (P = 0.03). Moderate/severe EFL (i.e. defined as grade 2+/3+) was found in 13 patients (29%) in Group I vs 28 patients (80%) in Group II (P < 0.001). Logistic regression identified BAV insufficiency as the strongest predictor of moderate/severe EFL (OR 9.3; 95% CI 3.2-29.8, P < 0.001). Valve-related event-free survival was 64 ± 8% in Group I vs 93% ± 5% in Group II at 10 years postoperatively (P = 0.05). A total of 4 patients (5%, 3 from Group I and 1 from Group II) underwent redo aortic root surgery for prosthetic valve endocarditis during follow-up. Patients with BAV insufficiency and a proximal aorta of ≥50 mm have a significantly higher rate of moderate/severe EFL as compared to their counterparts with BAV stenosis.

EVAR Continues to Cost More than Open AAA Repair

eGFR and three patients required hemodialysis. Five patients had full recovery of renal function by discharge. In hospital, 30-day morbidity/ mortality were 25%/3% respectively. At a mean follow-up of 3 years, six patients had an eGFR significantly less than the preoperative value. Late interventions related to the AAA repair were required in eight patients. Indications included: wound complication (3), anastomotic aneurysm (2), incisional hernia (1), anastomotic graft stenosis (1), and proximal aortic dilatation (1). Overall 5-year intervention free survival was 61% and overall survival 79%. Intervention free survival was decreased by perioperative pneumonia (P 1⁄4.01) and enhanced by antiplatelet (P 1⁄4 .05) use whereas overall survival was decreased by COPD (P 1⁄4 .03) and perioperative pneumonia (P 1⁄4 .001). Conclusions: A quarter of patients requiring a suprarenal cross-clamp during open AAA repair experience renal dysfunction. Late graft related complications are few with preoperative and perioperative pulmonary function negatively impacting intervention-free and overall patient survival.

Patterns of Aortic Dilatation in Bicuspid Aortic Valve–Associated Aortopathy

Bicuspid aortic valves (BAVs) are associated with aortopathy. Recent studies suggest that aortic dilatation is more likely to be seen with left-right coronary cusp fusion (type I) compared with right-noncoronary cusp fusion (type II). The aim of this study was to investigate the association between BAV morphology and patterns of aortopathy. Aortic dimensions and BAV morphology were obtained retrospectively from archived cine loops of 581 consecutive patients with BAVs and 277 matched normal controls from the Vancouver General Hospital echocardiography database. Patient demographics and other echocardiographic parameters were extracted from the database. The study population was composed of 71% type I BAVs (415 patients) and 26% type II BAVs (149 patients). Aortic dilatation was present in 30% of the population. Type I BAV was associated with increased dimensions indexed to body surface area at the sinus of Valsalva compared with type II BAV. No difference in proximal ascending aortic dimension was seen between different BAV morphologies. The pattern of dilatation with type I BAV was more likely to be at the level of the annulus or sinus of Valsalva compared with type II BAV (62% vs 33%, P= .002). Type I BAV was an independent predictor of proximal aortic dilatation (odds ratio, 3.42; 95% confidence interval, 1.07-10.9). Type I BAV is associated with a greater likelihood of dilatation at the annulus and sinus of Valsalva. There is relative sparing of this region of the aorta in patients with type II BAVs. Individuals with different BAV morphologies may require different strategies of aortopathy surveillance.

Augmentation index and aortic stiffness in bicuspid aortic valve patients with non-dilated proximal aortas

BackgroundWe compared aortic stiffness, aortic impedance and pressure from wave reflections in the setting of bicuspid aortic valve (BAV) to the tricuspid aortic valve (TAV) in the absence of proximal aortic dilation. We hypothesized BAV is associated with abnormal arterial stiffness.MethodsTen BAV subjects (47 ± 4 years, 6 male) and 13 TAV subjects (46 ± 4 years, 10 male) without significant aortic valve disease were prospectively recruited. Characteristic impedance (Zc) was derived from echocardiographic images and pulse wave Doppler of the left ventricular outflow tract. Applanation tonometry was performed to obtain pulse wave velocity (PWV) at several sites as measures of arterial stiffness and augmentation index (AIx) as a measure of wave reflection.ResultsThere were no significant differences between BAV and TAV subjects with regard to heart rate or blood pressure. Zc was similar between BAV and TAV subjects (p=0.25) as was carotid-femoral pulse wave velocity (cf-PWV) and carotid-radial PWV (cr-PWV) between BAV and TAV subjects (p=0.99). Carotid AIx was significantly higher in BAV patients compared with TAV patients (14.3 ± 4.18% versus -3.02 ± 3.96%, p=0.007).ConclusionsAortic stiffness and impedance is similar between subjects with BAV and TAV with normal aortic dimensions. The significantly higher carotid AIx in BAV, a proxy of increased pressure from wave reflections, may reflect abnormal vascular function distal to the aorta.

Reply to Dayan et al.

I would like to thank Dayan et al. for their insightful comments [1] on our recently published manuscript [2]. Moreover, Dayan et al. should be congratulated for their own contributions in the field of BAV-associated aortopathy [3, 4]. The above-discussed manuscript does not give definite answer to the question of predictors for proximal aortic dilatation after AVR surgery in BAV patients [2]. Our multivariate analysis was based on eight (5%) clinically relevant adverse aortic events, which inherently limit the number of variables, which may be reasonably included in the regression analysis. We did not include the variable ‘BAV configuration’ in our statistical analysis, as this morphological information was available retrospectively in only 55% of our study population (i.e. 84 of 152 patients). In contrast to the findings of Dayan et al. [4], we did not find any significant correlation between the body surface area (BSA), family history of BAV and the increased risk of adverse aortic events in our study. One possible explanation for this discrepancy may be the inclusion of BAV patients with an isolated/predominant aortic valve insufficiency (i.e. 17% of the study cohort) in the series of Dayan et al. [3]. This specific subgroup of BAV patients, which may be also defined as a ‘root phenotype’, is predominantly male (i.e. larger BSA) and have often a strong family history of BAV/aortopathy [5]. Recent data from the literature indicate significantly increased risk of late adverse aortic events in this specific cohort of BAV patients after isolated AVR [6]. There is emerging evidence that this phenotype of BAV disease may represent a predominantly congenital/ genetic form of BAV/aortopathy [5]. The inclusion of BAV patients with a ‘root phenotype’ in the study of Dayan et al. [4] may reasonably explain the progression of aortic root dimensions late after AVR surgery and the observed correlations with the BSA as well as with the family history of BAV/aortopathy. In contrast to that, we included only BAV patients with an isolated/predominant aortic valve stenosis [2]. Unfortunately, our study was not designed to specifically analyse the protective effects of long-term pharmacological treatment (i.e. effects of beta-blockers and statins). The vast majority of our patients (i.e. 95% of the study cohort) underwent long-term beta-blocker therapy after AVR, whereas only very few of them received statins. Therefore, we were not able to reasonably include these variables in our Cox regression model. Dayan et al. [4] found recently some positive effects of betablocker/statin use in the BAV patients in preventing aortic root dilatation between 6 and 8 years after an isolated AVR. However, such data should be interpreted with a caution. Only a small proportion of the initial study cohort received pharmacological treatment postoperatively (i.e. 11 (18%) patients with betablockers and 8 (13%) patients with statins postoperatively) [3]. Moreover, only 23 of 60 (38%) patients of the initial study cohort underwent an echocardiographical follow-up examination at 8 years post-AVR [4]. This would result in a very limited number of pharmacologically treated BAV patients at 8 years post-AVR. In summary, the protective effect of long-term betablocker/statin therapy in BAV-associated aortopathy is still unclear and should be addressed in future by means of largescale prospective studies.

Root remodeling for aortic root dilatation.

Dilatation of the ascending aorta has been found to be the most frequent pathogenic mechanism in aortic regurgitation (1,2). Root remodeling was first proposed by Sarsam and Yacoub as an operation for patients with aortic regurgitation and aortic aneurysm (3), with the assumption that regurgitation was solely due to aortic dilatation. The initial results were good, demonstrating that aortic valve function could be improved or even normalized. The concept of root remodeling was then challenged by aortic valve reimplantation within a vascular graft, as proposed by David (4). A complementary (rather than competitive) role of these two procedures was hypothesized (5). Experimental data investigating cusp mobility after application of these two techniques showed essentially normal cusp motion after root remodeling, which was strikingly different from the abrupt opening and closing movements after valve reimplantation (6). Subsequent studies indicated that the initial results proved difficult to reproduce. The degree of preoperative aortic regurgitation (7) or root dilatation (8), or the presence of acute aortic dissection (9) were observed as risk factors for postoperative aortic regurgitation. None of the authors, however, proposed a plausible mechanism of valve failure and it remains unclear whether failure was induced by the operation per se or the actual conduct of the operation. We have previously found that cusp prolapse can be induced by root replacement, which was for our institution the most frequent reason for secondary valve failure (10). Concomitant repair of cusp prolapse with root remodeling proved to be safe, and it did not compromise the late results (11). Based on the analysis of our failures and investigations in normal cusps, we introduced the effective height concept into aortic valve repair and valve-preserving surgery (12). Intraoperative determination of the effective height of aortic cusps allowed for more objective assessment of cusp configuration and reproducible correction of cusp prolapse (12,13). Furthermore, we have recently showed that the guided combination of prolapse repair with root remodeling has led to significantly better long-term aortic valve function compared to the isolated root procedure (14). In comparison to valve reimplantation, root remodeling consistently takes less time to perform and is applicable in acute dissection as well as in the presence of the usual cardiovascular comorbidities seen in proximal aortic dilatation. Root remodeling thus has become our preferred approach to dilatation of the aortic root. In light of the frequent existence of cusp prolapse it has become an aortic valve repair procedure rather than an aortic operation.

Risk of late aortic events after an isolated aortic valve replacement for bicuspid aortic valve stenosis with concomitant ascending aortic dilation

The optimal surgical treatment of patients with bicuspid aortic valve (BAV) disease and ascending aortic aneurysm is controversial. The aim of this study was to evaluate the risk of late aortic events after an isolated aortic valve replacement (AVR) for BAV stenosis with concomitant mild-to-moderate proximal aortic dilation. A review of our institutional BAV database identified a subgroup of 153 consecutive BAV patients (mean age 54.2 ± 10.5 years, 73% men) with BAV stenosis and concomitant ascending aortic dilation of 40-50 mm who underwent an isolated AVR from 1995 to 2000. All cases of simultaneous aortic surgery (i.e. ascending aorta with a diameter of >50 mm) were excluded. The follow-up (1759 patient-years) was 100% complete. The mean follow-up was 11.5 ± 3.2 years. Adverse aortic events were defined as the need for proximal aortic surgery, the occurrence of aortic dissection/rupture or sudden death during the follow-up. Actuarial survival rates of our study population were 86 and 78% at 10 and 15 years, respectively. Ascending aortic surgery was required in five patients (3%) for progressive ascending aortic aneurysm. Freedom from aortic interventions at 10 and 15 years was 97 and 94%, respectively. No documented aortic dissection or rupture occurred. Freedom from adverse aortic events was 95% at 10 years and 93% at 15 years postoperatively. In a separate group of patients presenting with aortic insufficiency (i.e. root phenotype), freedom from adverse aortic events was significantly lower (88 and 70% at 10 and 15 years, P = 0.009). BAV patients with aortic valve stenosis and concomitant mild-to-moderate ascending aortic dilation are at a considerably low risk of adverse aortic events at 15 years after an isolated AVR. The BAV phenotype should be considered when determining the risk of subsequent adverse aortic events and the need for concomitant aortic replacement.

Relation of Bicuspid Aortic Valve Morphology to the Dilatation Pattern of the Proximal Aorta: Focus on the Transvalvular Flow

Whether the dilatation of proximal aorta in patients with bicuspid aortic valve is secondary to hemodynamic effects related to the abnormal aortic valve or a primary manifestation of the genetic disorder remains controversial. We discuss in this paper the recent data on the BAV function and transvalvular flow patterns in relation with the dilatation type of the proximal aorta. Different morphological forms of bicuspid aortic valve in relation with the specific transvalvular blood flow patterns are focus of the first paragraph of this paper. In the second part of this paper we present the pathogenetic insight into the different clinically observed phenotypes of bicuspid aortic valve disease (i.e., association of proximal aortic shapes with the specific cusp fusion patterns), based on the data from recent rheological studies.

Echocardiographic and Tissue Doppler Imaging Alterations Associated with Spontaneous Canine Systemic Hypertension

Feline systemic arterial hypertension (SHT) is associated with a wide spectrum of left ventricular (LV) geometric patterns as well as diastolic, and to a lesser extent, systolic myocardial dysfunction. However, little is known about SHT-related cardiac changes in dogs. SHT in dogs is responsible for morphological and functional cardiac alterations. Thirty dogs with spontaneous untreated SHT and 28 age- and body weight-matched healthy dogs as controls. Prospective observational study. Conventional echocardiography and 2-dimensional color tissue Doppler imaging were performed in SHT dogs by trained observers and compared with controls. Forty-seven percent of SHT dogs (14/30) had diffuse concentric hypertrophy. None had left atrial dilatation and 10/30 (33%) had aortic insufficiency (AoI) associated with proximal aortic dilatation. Longitudinal diastolic left ventricular free wall (LVFW) motion was altered in all SHT dogs at the base (early to late diastolic wave ratio, E/A = 0.5 ± 0.1 versus 1.3 ± 0.3 for controls, P < .0001) and the apex (E/A = 1.6 ± 1.7 versus 3.9 ± 3.1, P < .05). Longitudinal motion of the interventricular septum at the base (E/A = 0.7 ± 0.4 versus 1.1 ± 0.1, P < .01) and radial LVFW motion in the subendocardium (E/A = 0.9 ± 0.5 versus 1.6 ± 0.3, P < .01) were also altered in dogs with SHT. Longitudinal LVFW systolic velocities and gradients were also significantly decreased (P < .05) in SHT dogs. As in SHT in cats, SHT in dogs is associated with myocardial dysfunction independently of the presence of myocardial hypertrophy. However, unlike feline SHT, it results in a homogeneous LV geometric pattern with a relatively high prevalence of AoI.

Dilatation of the ascending aorta and serum alpha 1-antitrypsin level in patients with bicuspid aortic valve

Predictors of aortic dilatation are not well described in patients with bicuspid aortic valves (BAV). This study sought to examine the relationship between proximal aortic dilatation and matrix metalloproteinase-9 (MMP-9) and alpha 1-antitrypsin (α1AT) levels in patients with BAV. All patients underwent echocardiography using a standard protocol, and aortic measurements were taken in end-diastole. We studied 82 patients with BAV and categorized them into two groups according to aortic dimensions corrected for body surface area and age. The plasma level of α1AT was routinely determined using a BN ProSpec analyzer (Siemens Healthcare Diagnostics, Marburg, Germany), and that of MMP-9 were determined by ELISA (RayBiotech Inc. Norcross, GA, USA). Statistical analysis was performed using the Statistical Package for Social Sciences (SPSS; SPSS Inc., Chicago, IL, USA) software for Windows version 12. This study included patients with BAV with no or mild valvular impairment. There were no significant differences between groups in terms of gender, body surface area, associated hypertension, diabetes mellitus, hyperlipidemia, or smoking. The mean ascending aortic diameter was 4.38±0.5mm in group 1 and 3.34±0.35mm in group 2 (p<0.001). Plasma concentration of α1AT in patients with ascending aortic dilatation was significantly lower than that in the non-dilated group (1.32±0.27 and 1.49±0.25g/l, respectively; p=0.005). However, no significant difference was found in the MMP-9 level between the two groups (336.49±233.11 and 336.39±268.072pg/ml, respectively; p=0.96). We observed a significantly negative correlation between ascending aortic diameter and α1AT level (r=-0.300, p=0.006) and a positive correlation between ascending aortic diameter and age (r=0.413, p<0.001). No significant correlation was found between plasma MMP-9 concentration and ascending aortic diameter (r=-0.008, p=0.94). A multiple linear regression analysis was performed, including age, α1AT level, MMP-9 level, and left ventricular diastolic diameter. In this analysis, α1AT level and age were the independent predictors of aortic dilatation (p=0.03 and p=0.02, respectively).

Morphological and Mechanical Changes in Juxtarenal Aortic Segment and Aneurysm Before and After Open Surgical Repair of Abdominal Aortic Aneurysms

Objective: The aim of study was to assess how the ultrastructure of the wall of aortic aneurysms, sac and neck influences aortic wall distensibility and proximal dilatation 2 years after open repair. Methods: Biopsies for electron microscopy were taken from aneurysmal sac and neck of 30 patients. Patients were assessed by computed tomography (CT) and ultrasound for aneurysm diameter and distensibility (M-mode ultrasonography). Results: Postoperative CT of the aortic stump distinguished two groups. Group I (n = 11) with little enlargement, median 1 mm (1–3 mm) and group II (n = 19) with significant aortic enlargement, median 5.2 mm (4–12 mm). In group II, changes in elastic fibres in the aneurysm neck were comparable to, but as extreme as in the aneurysm sac. For group I, the distensibility of the aneurysmal sac was significantly lower than in the neck or at the renal arteries. For group II, the distensibility in both the neck and sac was significantly lower than at the juxtarenal segment (p = 0.01). The biopsies of group II patients showed the extensive degeneration of normal architecture, which was associated with altered wall distensibility in both the aneurysmal neck and sac. Conclusions: Disorganisation and destruction of normal aortic architecture at the ultrastructural level are associated with decreasing aortic distensibility. Low aortic neck distensibility is associated with proximal aortic dilatation at 2 years postoperatively.

Morphological and Mechanical Changes in Juxtarenal Aortic Segment and Aneurysm Before and After Open Surgical Repair of Abdominal Aortic Aneurysms

Objective The aim of study was to assess how the ultrastructure of the wall of aortic aneurysms, sac and neck influences aortic wall distensibility and proximal dilatation 2 years after open repair. Methods Biopsies for electron microscopy were taken from aneurysmal sac and neck of 30 patients. Patients were assessed by computed tomography (CT) and ultrasound for aneurysm diameter and distensibility (M-mode ultrasonography). Results Postoperative CT of the aortic stump distinguished two groups. Group I ( n = 11) with little enlargement, median 1 mm (1–3 mm) and group II ( n = 19) with significant aortic enlargement, median 5.2 mm (4–12 mm). In group II, changes in elastic fibres in the aneurysm neck were comparable to, but as extreme as in the aneurysm sac. For group I, the distensibility of the aneurysmal sac was significantly lower than in the neck or at the renal arteries. For group II, the distensibility in both the neck and sac was significantly lower than at the juxtarenal segment ( p = 0.01). The biopsies of group II patients showed the extensive degeneration of normal architecture, which was associated with altered wall distensibility in both the aneurysmal neck and sac. Conclusions Disorganisation and destruction of normal aortic architecture at the ultrastructural level are associated with decreasing aortic distensibility. Low aortic neck distensibility is associated with proximal aortic dilatation at 2 years postoperatively.

Aortic dissection and rupture in a 16-year-old girl with Turner syndrome following previous progression of aortic dilation

Aortic dissection occurs in Turner syndrome with substantially higher frequency in comparison to the general population, and its prevention is one of the main aims of cardiologic follow-up. Findings of cystic medial necrosis in the aortic wall and a high prevalence of aortic dilation suggest that a form of aortopathy exists in Turner syndrome. However, little is known about natural development of aortic dilation prior to dissection. We present a 16-year-old girl with Turner syndrome with a bicuspid aortic valve, aortic stenosis, and dilation of ascending aorta, who underwent annual echocardiographic examinations from early childhood. Significant progressions of proximal aortic dilation occurred twice at the age of 10 and 15 years. Thereafter, another rapid progression was observed during 8 months and within 3 weeks preceding dissection. Acute aortic dissection was diagnosed while the girl was waiting for elective surgery. She was successfully operated. Frequent estimations of aortic diameter in Turner patients with abnormal findings may help to anticipate this life-threatening event. Additionally, we learned that rapid progression of aortic dilation should lead to immediate surgery to prevent more risky urgent intervention following the dissection.

Endothelial Function, Carotid–Femoral Stiffness, and Plasma Matrix Metalloproteinase-2 in Men With Bicuspid Aortic Valve and Dilated Aorta

This study sought to examine the relationship between proximal aortic dilation and systemic vascular function in men with bicuspid aortic valve (BAV). Proximal aortic dilation in subjects with BAV is associated with structural and functional abnormalities in the ascending aorta. We studied 32 men (median age 31 years [range 28 to 32 years]) with nonstenotic BAV categorized into 2 subgroups according to proximal ascending aorta dimensions (nondilated <or=35 mm and dilated >or=40 mm, respectively). Sixteen healthy men were studied as control subjects. Flow-mediated dilation in response to hyperemia (a marker of endothelial dysfunction) and carotid-femoral pulse wave velocity (an index of aortic stiffness) were assessed, and peripheral blood was sampled for matrix metalloproteinases (MMP-2 and -9) and their tissue inhibitors (TIMP-1 and -2), respectively. Cardiac chamber and aortic dimensions were assessed by echocardiography and cardiac magnetic resonance imaging, respectively. Despite the similar severity of aortic stenosis, left ventricular mass, and function, men with dilated aortas had blunted brachial flow-mediated vasodilation to hyperemia (5% [interquartile range (IQR) 4% to 6%] vs. 8% [IQR 7% to 9%] change, p = 0.001), higher carotid-femoral pulse wave velocity (9.3 cm/s [IQR 9 to 10 cm/s] vs. 7 cm/s [IQR 6.9 to 7.4 cm/s], p = 0.001), and significantly higher plasma levels of MMP-2 (1,523 [IQR 1,460 to 1,674] vs. 1,036 [IQR 962 to 1,167], p = 0.001) compared with men with BAV and nondilated aorta. Values for MMP-9, TIMP-1 and -2 levels, and nitroglycerin-induced (endothelium-independent) vasodilation were similar in all 3 groups. Young men with BAV and dilated proximal aortas manifest systemic endothelial dysfunction, increased carotid-femoral pulse wave velocity, and higher plasma levels of MMP-2. These observations could introduce new targets for screening and perhaps for therapeutic intervention.

The surgical pathology of large vessel disease

The common disorders that affect large vessels are atherosclerosis, idiopathic proximal aortic dilatation (annulo-aortic ectasia), and cranial arteritis. Although an increasing number of patients undergo vascular surgery, especially carotid endarterectomy, we receive only about 250 vascular biopsies annually. Most are diagnosed easily. The most difficult diagnostic areas are the assessment of inflammatory infiltrates in aortic biopsies, the accurate identification of healed or healing cranial arteritis and the differentiation of Buerger's disease from atherosclerosis.

Metabolic Adaptations to Interrupted Glycosaminoglycan Recycling

Lysosomal storage diseases (LSD) are metabolic disorders characterized by accumulation of undegraded material. The mucopolysaccharidoses (MPS) are LSDs defined by the storage of glycosaminoglycans. Previously, we hypothesized that cells affected with LSD have increased energy expenditure for biosynthesis because of deficiencies of raw materials sequestered within the lysosome. Thus, LSDs can be characterized as diseases of deficiency as well as overabundance (lysosomal storage). In this study, metabolite analysis identified deficiencies in simple sugars, nucleotides, and lipids in the livers of MPSI mice. In contrast, most amino acids, amino acid derivatives, dipeptides, and urea were elevated. These data suggest that protein catabolism, perhaps because of increased autophagy, is at least partially fulfilling intermediary metabolism. Thus, maintaining glycosaminoglycan synthesis in the absence of recycled precursors results in major shifts in the energy utilization of the cells. A high fat diet increased simple sugars and some fats and lowered the apparent protein catabolism. Interestingly, autophagy, which is increased in several LSDs, is responsive to dietary intervention and is reduced in MPSVII and MPSI mice fed a high fat diet. Although long term dietary treatment improved body weight in MPSVII mice, it failed to improve life span or retinal function. In addition, the ventricular hypertrophy and proximal aorta dilation observed in MPSVII mice were unchanged by a high fat, simple sugar diet. As the mechanism of this energy imbalance is better understood, a more targeted nutrient approach may yet prove beneficial as an adjunct therapy to traditional approaches.

Ross Operation in the Adult: Long-Term Outcomes After Root Replacement and Inclusion Techniques

Dilatation of the pulmonary autograft is a major concern after root replacement for the Ross operation. The inclusion technique would avoid this drawback, but few data are available on the long-term results of this technique. We retrospectively analyze long-term results of both techniques. Of 218 patients undergoing the Ross operation between 1991 and 2006, 148 (68%) had root replacement and 70 (32%) underwent the inclusion technique. The mean age of the patients was 40 +/- 10 years (range, 16 to 64). Mean follow-up was 94 +/- 44 months (range, 13 to 196). Echocardiographic controls were available in 197 patients. Proximal aorta dilatation was defined as diameter > 40 mm. In the root and inclusion groups, 10-year overall survival was 94% +/- 4% and 97% +/- 4%, respectively. Freedom from autograft reoperation was 81% +/- 10% and 84% +/- 13%, respectively. Main cause of reoperation was autograft dilatation in the root group (13 of 16) and valve prolapse in the inclusion group (5 of 6). Freedom from proximal aorta dilatation was 57% +/- 12% and 80% +/- 15%, respectively. In the root group, dilatations (n = 48) affected systematically the autograft sinuses or sinotubular junction, whereas in the inclusion group, dilatations (n = 10) affected principally the ascending aorta (8 of 10). Freedom from severe autograft regurgitation was 86% +/- 9% and 83% +/- 13%, respectively. Root technique, follow-up length, and preoperative aortic valve regurgitation were predictors of proximal aorta dilatation. In the long term, both techniques showed excellent survival and similar rates of autograft failure. For root replacement, autograft dilatation was the main cause of failure. For the inclusion technique, the autograft, but not the ascending aorta, was protected against dilatation and autograft valve prolapse was the main cause of failure.

No Difference in Medium-Term Outcome Between Zenith and Talent Stent-Grafts in Endovascular Aneurysm Repair

Many devices are available for endovascular aneurysm repair (EVAR). Our aim was to analyze morphological effects of the Zenith and Talent systems. Patients included underwent EVAR from June 1999 to June 2005 using a Zenith or Talent stent-graft, with computed tomography follow-up. Aortic dimensions over time and clinical outcome were analyzed. Twenty-nine patients with Zenith stent-grafts and 33 with Talent devices were included. Mean preoperative age was similar (75.5+/-6.0 years vs 74.2+/-6.7 years; P=.29). Preoperative neck length was longer in the Zenith group (29.9+/-15.2 mm vs 25.5 +/- 10.8 mm; P=.10), and stent-graft oversizing was greater in the Talent patients (20.2%+/-7.9% vs 23.0% +/- 11.3%). There was proximal aortic dilatation and aneurysm sac shrinkage in each group. Complication rates were comparable, with 83% of both groups free from 10-mm migration. Although device designs differ, there is no difference in clinical outcome between Zenith and Talent stent-grafts. Migration rates were not influenced by suprarenal fixation.

Aortic Root Replacement with a Composite Valved Conduit

Currently there are several operative techniques in use aroundtheworldtoaddressaorticrootpathologicproblems. These include valve-sparing aortic root replacement, stentless porcine aortic root replacement, pulmonary autograft root replacement, and composite valved graft conduits (CVG) housed with either a mechanical or in some cases a bioprosthetic valve. The CVG root replacement is the most time tested of all options and was first described by Bentall and DeBono in 1968. 1 Since then there have been many published series that have extended out beyond 20 years that demonstrate the efficacy of this approach. The indications for aortic root replacement include aneurysmal disease, aortic dissection when the primary tear occurs in the aortic root or with concomitant aortic valve disease or aneurysmal disease, and invasive aortic valve endocarditis that involves the aortic root. Most commonly aortic root replacement is performed for aneurysmal disease that stems from a degenerative process or is secondary to a genetic abnormality that predisposes to proximal aortic dilation as in Marfan syndrome, Loeys‐Dietz syndrome, Ehlers‐Danlos syndrome vascular-type (type IV), familial aortic aneurysm or dissection syndromes, and aneurysm associated with bicuspid aortic valve. Abnormal aortic root and ascending aortic dimensions have been determined based on patient age and body size. Accrual of large amounts of echocardiographic and crosssectional imaging data from normal individuals has allowed the development of regression formulas and nomograms to define abnormal aortic dilation. For example, an individual 18 to 40 years old will have an average aortic diameter at the sinus level equal to 0.97 (1.12 BSA [m 2 ]) (cm). The upper limit of normal is 2.1 cm/m 2 for the sinus segment in most adults. The aorta is pathologically dilated if its diameter exceedsthenormforagivenageandbodysurfacearea(BSA). Itisdefinedasananeurysmifitsdiameteris50%greaterthan the norm. There remains some debate as to what the indication for operative intervention should be based on maximal orthogonal diameter alone without consideration of other clinical factors like aortic valve pathologic complications. Clearly, there are cohorts of patients with aortic root and ascending aortic aneurysms that are at higher risk of an aortic catastrophe (dissection, disruption, or sudden death) than others, like patients with a bona fide connective tissue disorder or family history of aortic dissection. Moreover, surgeon experience with aortic root replacement operations varies widely. Highvolumecentershavetendedtogeneratethebestresults,

Replacement of the Proximal Aorta Adds No Further Risk to Aortic Valve Procedures

Aortic valve pathology is often associated with proximal aortic dilatation. Even after valve surgery, the proximal aorta can continue to dilate and thus be at risk for rupture, dissection, or later aortic replacement. We hypothesized that the addition of proximal aortic intervention adds no further risk to aortic valve surgery, which may avoid subsequent proximal aortic procedures or catastrophes. Between 1996 and 2004, 430 aortic valve interventions alone and 146 aortic valves with proximal aortic replacements were identified in elective adult patients. The age in the valve-alone patients (68.8 years) was slightly higher than the valve-plus-aorta group (valve/aorta, 60.5 years; p < 0.01), but comorbidities were similar between groups. We compared groups based on hospital mortality and incidence of complications. The 30-day mortality was similar between groups (valve-alone, 3.8% versus valve/aorta, 2.7%; p = 0.5), as were rates for bleeding and operative revision (valve-alone, 6.7% versus valve/aorta, 9.5%; p = 0.5). Pulmonary (valve-alone, 23.0% versus valve/aorta, 11.6%) and renal complications (valve-alone, 8.2% versus valve/aorta, 2.7%) were higher in the valve-alone group (p = 0.02). Logistic regression demonstrated no additional risk of death, neurologic, or cardiac event with replacement of the proximal aorta. Proximal aortic replacement adds no risk to the patient beyond the aortic valve intervention alone. These findings suggest proximal aortic replacement is safe for patients undergoing valve operations. Patients with a moderately enlarged proximal aorta that may dilate further should also be considered for aortic replacement at the time of valve procedures.