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Tricuspid versus bicuspid patients with age-and gender-matched normal population—survival after 30 days for patients with bicuspid aortic valves (BAVs) is equivalent to the norm, while the tricuspid (TAV) patient has worse survival than the corresponding matched normal population.
Source publication
Objectives:
The aim of this study is to investigate the clinical characteristics and postoperative outcome of patients with a bicuspid aortic valve (BAV) suffering acute dissection in comparison with their tricuspid peers.
Methods:
Between 1995 and 2011, 460 consecutive patients underwent emergency repair for acute type A aortic dissection. In 3...
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Background
The optimal surgical approach for management of acute type A aortic dissection remains controversial. This study aimed to assess outcomes of reoperation after acute type A dissection repair to help guide decision making around index operative strategy.
Methods and Results
All aortic reoperations (n=129) at a single referral institution...
The Ross procedure is an excellent choice for younger patients in need of aortic valve replacement. While patients have benefited from superior survival rates associated with this procedure, complications related to aortic root dilatation and degeneration of the autograft may be encountered later in life. These challenges may be exacerbated in thos...
p> Aim: The study was designed to investigate predictors of effective valve-sparing ascending aortic replacement in patients with Stanford type A aortic dissection combined with aortic insufficiency and to analyze efficacy and safety of this kind of surgery.
Methods: From January 2010 to December 2015, 49 patients with Stanford type A aortic dissec...
Background:
This study aims to evaluate the results of late-onset type A aortic dissection following primary cardiac surgery and to compare the outcomes of patients with or without prior coronary artery bypass grafting.
Methods:
Between January 2005 and December 2015, data of 32 patients (16 males, 16 females; mean age 58.1±10.9 years; range, 45...
Objectives:
Bicuspid aortic valve (BAV)-associated aortopathy has been proposed to progress after isolated aortic valve replacement (AVR) surgery, which has been traditionally used as an argument against a TAVR procedure in this clinical subset. Still, more than half of BAV patients have a normal-sized proximal aorta at the time of AVR surgery. We...
Citations
... In particular, the root phenotype with aortic regurgitation has recently been associated with a higher risk of aortic dissection. Data from IRAD showed that among 3393 patients with aortic dissection, 113 (3.3%) had BAV, containing 93 patients (82.3%) with type A aortic dissection and 20 patients (17.7%) with type B aortic dissection [59]. ...
The aorta is the largest artery in the body, delivering oxygenated blood from the left ventricle to all organs. Dissection of the aorta is a lethal condition caused by a tear in the intimal layer of the aorta, followed by blood loss within the aortic wall and separation of the layers to full dissection. The aorta can be affected by a wide range of causes including acute conditions such as trauma and mechanical damage; and genetic conditions such as arterial hypertension, dyslipidaemia, and connective tissue disorders; all increasing the risk of dissection. Both rapid diagnostic recognition and advanced multidisciplinary treatment are critical in managing aortic dissection patients. The treatment depends on the severity and location of the dissection. Open surgical repair is the gold standard of treatment for dissections located to the proximal part of the aorta and the arch, while endovascular interventions are recommended for most distal or type B aortic dissections. In this review article, we examine the epidemiology, pathophysiology, contemporary diagnoses, and management of aortic dissection.
... When aortopathy occurs at the level of the aortic root, the sinuses of Valsalva are predominantly affected (59). There is evidence suggesting that dilatation of this segment is associated with more rapidly progressive aortopathy (60)(61)(62). In contrast, non-BAV aortic aneurysms, such as degenerative aneurysms, tend to begin in the mid ascending aorta and progress distally and proximally, while aneurysms secondary to connective tissue diseases are limited to the aortic root (59). ...
Bicuspid aortic valve (BAV), which affects up to 2% of the general population, results from the abnormal fusion of the cusps of the aortic valve. Patients with BAV are at a higher risk for developing aortic dilatation, a condition known as bicuspid aortopathy, which is associated with potentially life-threatening sequelae such as aortic dissection and aortic rupture. Although BAV biomechanics have been shown to contribute to aortopathy, their precise impact is yet to be delineated. Herein, we present the latest literature related to BAV biomechanics. We present the most recent definitions and classifications for BAV. We also summarize the current evidence pertaining to the mechanisms that drive bicuspid aortopathy. We highlight how aberrant flow patterns can contribute to the development of aortic dilatation. Finally, we discuss the role cardiac magnetic resonance imaging can have in assessing and managing patient with BAV and bicuspid aortopathy.
... This is very important because unlike other studies, our study was comprised mainly of younger adults with normal or only mildly dilated aorta at baseline. Moreover, aortic dissection in BAV can have a distinctive entry tear pattern in the aortic root in addition to ascending aorta, emphasizing the need to monitor aortic root growth in this population [33]. In line with the current practice guidelines [34], our findings support close monitoring with serial imaging surveillance and follow-up for BAV patients with baseline enlarged aortic root, especially if ≥4.5 cm, regardless of their measured biomechanical status. ...
Background
Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Studies in aortopathies have shown a correlation between increased aortic stiffness and aortic dilation. We aimed to evaluate aortic stiffness measures as predictors of progressive aortic dilation by cardiac magnetic resonance (CMR) in BAV patients.
Methods
This is a retrospective study of 49 patients with BAV (median age 21.1 years at first CMR visit) with ≥2 CMR at the Wisconsin Adult Congenital Heart Disease Program (WAtCH). Circumferential aortic strain, distensibility, and β-stiffness index were obtained from CMR-derived aortic root cine imaging, and aortic dimensions were measured at aortic root and ascending aorta. A linear mixed-model and logistic regression were used to identify important predictors of progressive aortic dilation.
Results
Over a median of 3.8 years follow-up, the annual growth rates of aortic root and ascending aorta dimensions were 0.25 and 0.16 mm/year, respectively. Aortic strain and distensibility decreased while β-stiffness index increased with age. Aortic root strain and distensibility were associated with progressive dilation of the ascending aorta. Baseline aortic root diameter was an independent predictor of >1 mm/year growth rate of the aortic root (adjusted OR 1.34, 95 % CI 1.03–1.74, p = 0.028). Most patients (61 %) had coexisting coarctation of aorta. Despite the higher prevalence of hypertension in patients with aortic coarctation, hypertension or coarctation had no effect on baseline aorta dimensions, stiffness, or progressive aortic dilation.
Conclusion
Some CMR-derived aortic stiffness parameters correlated with progressive aortic dilation in BAV and should be further investigated in larger and older BAV cohorts.
... The analyzed group of AADA patients displayed a mortality rate of 28% in the current series, with no significant differences between the groups before and after matching. We have previously reported similar findings in these high-risk patients (18), as have several other large German centers with aortic expertise (19). As no difference in organ malperfusion [one of the major determinants of outcomes in AADA (15)] was present preoperatively between our two patient groups, no significant outcome differences could be detected. ...
... The pathological risk of the presence of a BAV has been more frequently investigated over the past decade, impacting guidelines for aortic replacement and shifting the absolute aortic diameter as indicator for aortic surgery between 45 and 50 mm for this specific patient group (5). Studies have demonstrated that every ninth AADA patient is a carrier of a BAV and that the dissection entry is more often located in the aortic root leading to a more extensive surgical repair (18). Apparently, no difference in incidence of rupture or dissection between BAV and TAV patients has been detected in the past (21). ...
Background:
Concomitant replacement of the aortic root and aortic valve is a widely used treatment strategy in elective patients with aortic valve stenosis and root aneurysm. It is also a strategy frequently employed in patients with acute aortic dissection type A (AADA), involving the aortic root. Although more patients have undergone valve sparing procedures over the past decades, the classic 'modified Bentall technique' remains a valid option, particularly for patients with a bicuspid aortic valve (BAV). We aimed to compare the results of elective and emergency modified Bentall procedures in patients with bicuspid and tricuspid aortic valves (TAVs).
Methods:
We retrospectively reviewed our database for patients undergoing either elective or emergency modified Bentall procedures between 2000 and 2018 and identified 827 elective cases (44% BAV) and 258 emergency cases (15% BAV). Analysis of intra- and postoperative outcomes and early mortality was performed. Due to inequality of the groups, a matching analysis was performed.
Results:
We found BAV patients to be significantly younger (elective: 58±18 vs. 65±14, P<0.001; emergency: 49±17 vs. 62±19, P<0.001) and healthier at time of surgery. In the AADA cohort, malperfusion rate was not different between bicuspid and tricuspid patients, however bicuspid AADA patients presented more often with an entry in the aortic root. After matching, procedure times and early outcomes did not differ between the groups, except for significantly higher rates of respiratory failure in elective TAV patients (10% vs. 5%, P=0.033). The 30-day mortality was 2% in elective cases and 22% in emergency AADA surgery. A subgroup analysis of elective patients with aortic diameter <55 mm also showed excellent outcomes.
Conclusions:
After adjustment for preoperative inequalities, no differences in early mortality and outcomes were found between bicuspid and tricuspid patients receiving elective or emergency modified Bentall surgery.
... It is the most studied type in the literature, and authors agree to say that it is linked with a rapid progression of aortic dilatation compared with other BAV [17,24]. Moreover, it appears that this conformation is favourable to earlier valve dysfunction with significant valve regurgitation [9,25]. Opinions differ concerning the predominant location of aortic dilation; for Verma et al., the tubular ascending aorta is the privileged target of aneurysms [26], whereas it is the annulus and the sinus of Valsalva for Schaefer et al. [3]. ...
... It is a cascading effect, because abnormal processing of Fibrillin 1 by vascular SMC initiates their own detachment from the ECM, lead to the release of MMPs together with their tissue inhibitors (TIMPs). The resulting matrix disruption, elastin, and lamellar fragmentation lead to increased apoptosis of vascular SMC and separation of the media layer, adversely affecting the structural integrity and flexibility of the aorta [25]. It appears that TIMPs' expression controls the ECM metabolism. ...
Background—Bicuspid aortic valve (BAV) disease is the most prevalent congenital heart disease in the world. Knowledge about its subtypes origin, development, and evolution is poor despite the frequency and the potential gravity of this condition. Its prognosis mostly depends on the risk of aortic aneurysm development with an increased risk of aortic dissection. Aims—This review aims to describe this complex pathology in way to improve the bicuspid patients’ management. Study design—We reviewed the literature with MEDLINE and EMBASE databases using MeSH terms such as “bicuspid aortic valve”, “ascending aorta”, and “bicuspid classification”. Results—There are various classifications. They depend on the criteria chosen by the authors to differentiate subtypes. Those criteria can be the number and position of the raphes, the cusps, the commissures, or their arrangements regarding coronary ostia. Sievers’ classification is the reference. The phenotypic description of embryology revealed that all subtypes of BAV are the results of different embryological pathogenesis, and therefore, should be considered as distinct conditions. Their common development towards aortic dilatation is explained by the aortic media’s pathological histology with cystic medial necrosis. At the opposite, BAV seems to display a profound genetic heterogeneity with both sporadic and familial forms. BAV can be even isolated or combined with other congenital malformations. Conclusions—All those characteristics make this pathology a highly complex condition that needs further genetic, embryological, and hemodynamic explorations to complete its well described anatomy.
... In acute dissection, the initial entry site can occur in various locations in the thoracic aorta with distribution varying between studies [8,22,23]. In our published experience, we observed that aortic intimal tears by CT were located in the STJ in 29%, AscAo in 29%, sinuses in 18% and remainder in the arch [8]. ...
... Unfortunately, the study did not report rates for TAV patients independently. In contrast in another study of 400 patients that presented with acute type A dissection, location of initial intimal tear was much more frequent in the AscAo versus aortic root (88% vs 12%, respectively) [22], which remained similar in proportion when exclusively examining TAV patients. One study has found that regions of high wall stress overlapped with the intimal tear locations of their dissection patients [25]. ...
OBJECTIVES
Ascending thoracic aortic aneurysms (aTAAs) carry a risk of acute type A dissection. Elective repair guidelines are based on diameter, but complications often occur below diameter threshold. Biomechanically, dissection can occur when wall stress exceeds wall strength. Aneurysm wall stresses may better capture dissection risk. Our aim was to investigate patient-specific aTAA wall stresses associated with a tricuspid aortic valve (TAV) by anatomic region.
METHODS
Patients with aneurysm diameter ≥4.0 cm underwent computed tomography angiography. Aneurysm geometries were reconstructed and loaded to systemic pressure while taking prestress into account. Finite element analyses were conducted to obtain wall stress distributions. The 99th percentile longitudinal and circumferential stresses were determined at systole. Wall stresses between regions were compared using one-way analysis of variance with post hoc Tukey HSD for pairwise comparisons.
RESULTS
Peak longitudinal wall stresses on aneurysms (n = 204) were 326 [standard deviation (SD): 61.7], 246 (SD: 63.4) and 195 (SD: 38.7) kPa in sinuses of Valsalva, sinotubular junction (STJ) and ascending aorta (AscAo), respectively, with significant differences between AscAo and both sinuses (P < 0.001) and STJ (P < 0.001). Peak circumferential wall stresses were 416 (SD: 85.1), 501 (SD: 119) and 340 (SD: 57.6) kPa for sinuses, STJ and AscAo, respectively, with significant differences between AscAo and both sinuses (P < 0.001) and STJ (P < 0.001).
CONCLUSIONS
Circumferential and longitudinal wall stresses were greater in the aortic root than AscAo on aneurysm patients with a TAV. Aneurysm wall stress magnitudes and distribution relative to respective regional wall strength could improve understanding of aortic regions at greater risk of dissection in a particular patient.
... TAA and subsequent aortic dissection is a feared complication in patients with BAV and associated with significant mortality [1,5]. Despite limited reports on the exact lifetime risk of AAS in patients with BAV, several reports suggest an eight-fold higher risk than their counterparts with trileaflet aortic valve (TAV) [6]. The management of patients with aortopathies associated with BAV include routine surveillance and identification of high risk subjects who will warrant prophylactic surgical aortic intervention [7]. ...
... Etz et al. conducted a retrospective analysis including 460 patients who underwent surgical repair for acute aortic dissection. Etz et al. showed that among patients with acute dissection, those with BAV were almost 15 years younger (46.7 versus 61.6 years) and had fewer comorbidities compared with those with TAV [6]. Similar findings were also reported in a smaller observational analysis inclusive of 100 patients with acute type A dissection [22]. ...
... In the Olmsted County study, patients with BAV underwent aortic surgeries at younger age than the general population [24]. Also, reports have suggested earlier and probably faster aortic dilatation among patients with BAV compared with TAV [6,25]. The lower incidence of traditional risk factors for AAS, including hypertension among those with BAV, is probably related to different disease pathology for AAS among patients with BAV. ...
IntroductionThere is paucity of data on the outcomes of hospitalization for bicuspid aortic valve (BAV)-related aortopathies.Methods
We queried the NIS database (2012–2016) for hospitalizations for elective thoracic aortic repair or acute aortic syndrome (AAS) among those with BAV versus trileaflet aortic valve (TAV).ResultsOur analysis yielded 38,010 hospitalizations for elective aortic repair, of whom 34.4% had BAV, as well as 81,875 hospitalizations for thoracic AAS, of whom 1.1% had BAV. Hospitalizations for BAV were younger and had fewer comorbidities compared with their TAV counterparts. The number of hospitalizations for BAV during the observational period was unchanged. After propensity matching, elective aortic repair for BAV was associated with lower mortality (0.5% versus 1.7%, odds ratio = 0.28; 95% CI 1.5–0.50, p < 0.001), use of mechanical circulatory support, acute stroke, and shorter length of hospital stay compared with TAV. After propensity matching, AAS among those with BAV had a greater incidence of bleeding events, blood transfusion, cardiac tamponade, ventricular arrhythmias, and a longer length of hospital stay compared with TAV. Among those with BAV, predictors of lower mortality if undergoing elective aortic repair included larger hospitals and teaching hospitals. Predictors of higher mortality in patients with AAS included heart failure, chronic kidney disease, and coronary artery disease.Conclusion
Data from a national database showed no change in the number of hospitalizations for BAV-related aortopathy, with relatively lower incidence of AAS. Compared with TAV, elective aortic repair for BAV is associated with lower mortality, while BAV-related AAS is associated with higher in-hospital complications.
... При ДАК частота расширения в середине восходящей аорты в 2 раза выше по сравнению с расширением аорты на Bicuspid Aortic Valve Двустворчатый аортальный клапан уровне синусов Вальсальвы, для синдрома Марфана характерно расширение на уровне корня аорты -синусов Вальсальвы [22,23]. При ДАК дилатация или аневризма на уровне синусов Вальсальвы является наиболее «злокачественной», быстро прогрессирующей аортопатией [24,25]. ...
Bicuspid aortic valve refers to common (0.5-2% of the population) congenital heart defects that are asymptomatic throughout life, with valve dysfunction and/or aortopathy (pathia- from Greek pathos disease), manifested by expansion, aneurysm or dissection of the vessel. The pathogenesis of the formation of a bicuspid valve is unknown, a genetic component is noted, since the defect develops as sporadic, familial, in combination with other congenital heart defects and with hereditary connective tissue disorders. Morphogenetic studies suggest that different phenotypes of bicuspid aortic valve can be considered as etiologically different diseases, with valve dysfunction or valve dysfunction and aortopathy. Aortic lesion is characterized by phenotypic heterogeneity due to genetic or hemodynamic features. Researchers are discussing the relationship between the phenotype of the bicuspid aortic valve and aortopathy to predict the course of the disease and select the optimal surgical treatment technique. Diagnosis of heart disease is based on the results of an echocardiographic study, magnetic resonance imaging. Surgical treatment is performed for significant hemodynamic disturbances resulting from insufficiency or stenosis of the aortic valve, in cases of infective endocarditis, the risk of which is high, with aneurysm or aortic dissection.
... Notably, when the phenotypic heterogeneity of BAV aortopathy was accounted for in analyses, studies suggested a greater risk of dissection with the root phenotype dilatation compared to the more frequent ascending phenotype [116] and the "hinge point" of the risk of dissection at presentation was at 50 mm for the root and 53 mm for the ascending tract in a large study from the Cleveland Clinic [117]. Several reports consistently found significantly greater diameters at the time of acute dissection in BAV than in TAV patients [118,119,120]: this finding, apparently in contrast with the evidence of greater risk of dissection in BAV subjects, moreover at a younger mean age, remains unexplained and yet it stresses the idea that the risk of dissection in general is only in part related to the size of the aorta [121]. ...
... This observation supports the notion that BAV-associated aortopathy predominately affects the proximal aorta. [11][12][13] Turbulent blood flow patterns due to BAV morphology with abnormal helix formation and increased wall shear stress have been discussed as possible underlying flow architecture-related pathomechanisms of aortic dilation in BAV patients. 14-17 Aortic diameters exceeding a 5.0 cm threshold may aggravate the effects of blood flow alterations and amplify structural aortic wall degeneration. ...
... Norton and Yang even suggested classifying BAV patients with AR and root aneurysm as 'malignant' BAV, with a potential genetic link justifying an aggressive surgical approach. 30 A recent analysis 31 compared 72 BAV and 1068 tricuspid aortic valves (TAV) patients with AADA at two centers over a 15-year time period, confirming the results of previous studies 11,12 : BAV patients were younger, had lower incidence of cardiovascular risk factors and larger proximal aortic diameters, and more often required aortic root replacement during their AADA operation. 31 Interestingly, the risk of aortic arch involvement in BAV patients with AADA is even lower than that of TAV patients. ...
... Furthermore, in contrast to Marfan patients, BAV patients are not at increased risk for recurrent aortic dissection post-repair. 33,34 A study by Etz et al. demonstrated a long-term survival benefit for BAV patients compared to their TAV peers post-AADA surgery, 11 suggesting that they may be cured by proximal aortic repair restoring their longevity to that of a normal age-and sex-matched population. ...
Patients with a bicuspid aortic valve (BAV) frequently develop aneurysms of the aortic root and tubular ascending aorta. Aneurysms of the aortic arch, in the absence of concomitant aortopathies, are much less common. According to the 2018 American Association of Thoracic Surgery consensus guidelines on BAV-related aortopathy, prophylactic surgical aortic repair / replacement is recommended starting at a maximum aortic diameter of 50 mm in patients with risk factors. Concomitant aortic surgery is also recommended at an aortic diameter of 45 mm in those patients with other indications for cardiac surgery (most commonly aortic valve procedures). The ultimate goal of prophylactic aortic surgery is the prevention of aortic catastrophes, e.g. aortic rupture or acute aortic dissection, which are associated with high morbidity and mortality. The surgical technique used – in elective and emergency cases - depends on the involvement and nature of the aortic valve disease, as well as the extent of aortic aneurysm formation. The current review focusses on the surgical management of the aortic root, tubular ascending aorta, and proximal aortic arch in BAV patients. Despite the abovementioned recommendations, many BAV patients develop acute aortic syndromes below the recommended aortic diameter thresholds. Further research is therefore required in order to identify high-risk BAV subgroups that would benefit from earlier surgical repair.
