Interdisciplinary Management of a Perforated Aneurysmal Arteria Lusoria: A Case Report.

Background An aberrant right subclavian artery (RSA) or arteria lusoria is the most common congenital abnormality of the aortic arch with an incidence of 0.3 to 3.0%. Case Description We report a case of a perforated aneurysmal aberrant RSA, managed using a hybrid approach. Conclusion In emergency cases with acute bleeding, we recommend an endovascular approach to avoid the lethal sequel of arterial leakage. Whenever possible, the pulsatile blood flow to the right arm should be restored. Management should be tailored to the nature of the aneurysmal aberrant RSA, patient's comorbidities, and concomitant lesions.

Evidence of subannular and left ventricular morphological differences in patients with bicuspid versus tricuspid aortic valve stenosis: magnetic resonance imaging-based analysis.

Objectives: Prospective analysis of left ventricular (LV) morphological/functional parameters in patients with bicuspid versus tricuspid aortic valve (TAV) stenosis undergoing aortic valve replacement (AVR) surgery. Methods: A total of 190 consecutive patients with BAV ( n = 154) and TAV stenosis ( n = 36) (mean age 61 ± 8 years, 65% male) underwent AVR ± concomitant aortic surgery from January 2012 through May 2015. All patients underwent preoperative cardiac magnetic resonance imaging in order to evaluate: (i) left ventricular outflow tract (LVOT) dimensions, (ii) length of anterior mitral leaflet (AML), (iii) end-systolic and end-diastolic LV wall thickness, (iv) LV area, (v) LV end-systolic and end-diastolic diameters (LVESD, LVEDD), (vi) LV end-diastolic and end-systolic volumes (LVEDV, LVESV) and (vii) maximal diameter of aortic root. These parameters were compared between the two study groups. Results: The LVOT diameter was significantly larger in BAV patients (21.7 ± 3 mm in BAV vs 18.9 ± 3 mm in TAV, P < 0.001). Moreover, BAV patients had significantly longer AML (24 ± 3 mm in BAV vs 22 ± 4 mm in TAV, P = 0.009). LVEDV and LVESV were significantly larger in BAV patients (LVEDV: 164.9 ± 68.4 ml in BAV groups vs 126.5 ± 53.1 ml in TAV group, P = 0.037; LVESV: 82.1 ± 57.9 ml in BAV group vs 52.9 ± 25.7 ml in TAV group, P = 0.008). A strong linear correlation was found between LVOT diameter and aortic annulus diameter in BAV patients ( r = 0.7, P < 0.001), whereas significantly weaker correlation was observed in TAV patients ( r = 0.5, P = 0.006, z = 1.65, P = 0.04). Presence of BAV morphology was independently associated with larger LVOT diameters (OR 9.0, 95% CI 1.0-81.3, P = 0.04). Conclusions: We found relevant differences in LV morphological/functional parameters between BAV and TAV stenosis patients. Further investigations are warranted in order to determine the cause of these observed differences.

Morphologic and Functional Markers of Aortopathy in Patients With Bicuspid Aortic Valve Insufficiency Versus Stenosis.

BACKGROUND: Bicuspid aortic valve (BAV)-associated aortopathy is heterogeneous and still insufficiently defined. We prospectively analyzed the morphologic and functional variables of aortopathy in patients undergoing operations for BAV insufficiency (BAV-AI) vs stenosis (BAV-AS). METHODS: A total of 172 consecutive patients (71% male, 59 ± 10 years) underwent aortic valve replacement with or without proximal aortic operation for BAV-AS (n = 137), and BAV-AI (n = 35) from January 2012 through December 2014. All patients underwent preoperative cardiac magnetic resonance imaging to evaluate morphologic and functional variables of the aortic root. Magnetic resonance imaging data were used to guide sampling of aortic tissue intraoperatively (ie, from the area where flow jet impacts on the aortic wall [jet sample] and the opposite aortic wall [control sample]). Aortic wall lesions were graded based on the histologic sum score (range, 0 to 21). Expression and severity of aortopathy were quantified by means of proximal aortic phenotype, indexed aortic diameters, and a sum score. RESULTS: Cross-sectional aortic diameters were significantly larger in the BAV-AI group vs the BAV-AS group (47 ± 8 mm vs 41 ± 8 mm, p = 0.001). Moreover, root dilatation phenotype was more frequent in the BAV-AI group (27% vs 6%, p = 0.01) and was associated with a significantly larger aortic annulus diameter (32 ± 3 mm vs 27 ± 3 mm, p < 0.001). The histologic sum score was significantly different between the study groups (3.7 ± 2.6 BAV-AI vs 2.5 ± 1.4 BAV-AS, p = 0.03). Logistic regression revealed a significant association between BAV-AI and indexed aortic diameter exceeding 22 mm/m2 (odds ratio, 4.7; p = 0.007). CONCLUSIONS: Our study demonstrates that BAV functional phenotype correlates significantly with the expression and severity of bicuspid aortopathy.

Aortopathy in Bicuspid Aortic Valve Stenosis with Fusion of Right-Left versus Right-Non-Coronary Cusps: Are These Different Diseases?

BACKGROUND: The study aim was to prospectively analyze the impact of cusp fusion pattern on aortopathy severity in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. METHODS: A total of 137 consecutive patients with BAV stenosis (93 males, 44 females; mean age 61 ± 9 years) underwent aortic valve replacement with or without concomitant aortic surgery between January 2012 and March 2015. All patients underwent preoperative cardiac magnetic resonance imaging (cMRI) in order to evaluate morphological/functional parameters of the aortic root. Fusion of the right-left coronary cusps was identified in 99 patients (72%) (R/L group), while the remaining 38 patients (28%) had right-noncoronary cusp fusion (R/N group). cMRI data were used to guide sampling of the proximal aorta during surgery. The expression/severity of aortopathy was quantified by means of proximal aortic phenotype, indexed aortic diameter, and a histological sum-score. RESULTS: There were no significant differences in proximal aortic diameter between the R/L and R/N groups. Moreover, there was no significant difference in the prevalence of distinct aortic phenotypes between the study subgroups. The histological sumscore was comparable between the R/L and R/N groups (2.9 ± 1.5 versus 2.5 ± 1.1, p = 0.2). The angle LV/aorta was significantly larger in the R/L group than in the R/N group (52.1 ± 10.6° versus 45.1 ± 8.5°, p = 0.001). Logistic regression analysis demonstrated the angle LV/aorta and the angle jet/aorta to be predictive of a mid-ascending phenotype. The cusp fusion pattern had no significant impact on the occurrence of aortic phenotype. CONCLUSIONS: The study results demonstrated that cusp fusion morphotype has no significant impact on the expression and severity of bicuspid aortopathy in patients undergoing surgery for BAV stenosis.

Anticoagulation After Biological Aortic Valve Replacement: Is There An Optimal Regimen?

BACKGROUND: The anticoagulation of biological heart valves remains a 'hot spot' of discussion in various domains due to the risk of developing valve thrombosis and arterial thromboembolism. The situation has always been controversial, especially during the early postoperative phase. The American College of Cardiology/ American Heart Association and European Society of Cardiology guidelines recommend the use of warfarin for the first three months after biological aortic valve replacement (BAVR), although the American College of Chest Physicians guidelines suggest that these recommendations are experience-based and that the risk/benefit is unclear. The aim of the present study was to compare the efficacy of aspirin and warfarin in patients after BAVR. METHODS: A total of 863 patients who underwent BAVR between 2008 and 2015 was allocated to two groups. Each group was managed with a specific anticoagulation regimen, with 430 patients receiving warfarin during the first three postoperative months, and 433 receiving aspirin. The major study end points were bleeding, cerebral ischemic events, and survival. RESULTS: In total, 10 and 15 postoperative cerebral ischemic events occurred between 24 h and three months after surgery in patients treated with aspirin and warfarin, respectively. After three months the incidence of cerebral ischemic events did not differ greatly between the two groups. The rate of major bleeding events and rates of stroke-free survival and overall survival were not statistically significant between the warfarin and aspirin groups. CONCLUSIONS: Plasma anticoagulation with warfarin during the early postoperative phase was shown statistically to be inferior to platelet aggregation inhibition by aspirin with regards to postoperative bleeding risk, cerebral ischemic events, and survival.

Functional Aortic Root Parameters and Expression of Aortopathy in Bicuspid Versus Tricuspid Aortic Valve Stenosis.

BACKGROUND: The correlation between bicuspid aortic valve (BAV) disease and aortopathy is not fully defined. OBJECTIVES: This study aimed to prospectively analyze the correlation between functional parameters of the aortic root and expression of aortopathy in patients undergoing surgery for BAV versus tricuspid aortic valve (TAV) stenosis. METHODS: From January 1, 2012 through December 31, 2014, 190 consecutive patients (63 ± 8 years, 67% male) underwent aortic valve replacement ± proximal aortic surgery for BAV stenosis (n = 137, BAV group) and TAV stenosis (n = 53, TAV group). All patients underwent pre-operative cardiac magnetic resonance imaging to evaluate morphological/functional parameters of the aortic root. Aortic tissue was sampled during surgery on the basis of the location of eccentric blood flow contact with the aortic wall, as determined by cardiac magnetic resonance (i.e., jet sample and control sample). Aortic wall lesions were graded using a histological sum score (0 to 21). RESULTS: The largest cross-sectional aortic diameters were at the mid-ascending level in both groups and were larger in BAV patients (40.2 ± 7.2 mm vs. 36.6 ± 3.3 mm, respectively, p < 0.001). The histological sum score was 2.9 ± 1.4 in the BAV group versus 3.4 ± 2.6 in the TAV group (p = 0.4). The correlation was linear and comparable between the maximum indexed aortic diameter and the angle between the left ventricular outflow axis and aortic root (left ventricle/aorta angle) in both groups (BAV group: r = 0.6, p < 0.001 vs. TAV group r = 0.45, p = 0.03, z = 1.26, p = 0.2). Logistic regression identified the left ventricle/aorta angle as an indicator of indexed aortic diameter >22 mm/m(2) (odds ratio: 1.2; p < 0.001). CONCLUSIONS: Comparable correlation patterns between functional aortic root parameters and expression of aortopathy are found in patients with BAV versus TAV stenosis.

Are normal-sized ascending aortas at risk of late aortic events after aortic valve replacement for bicuspid aortic valve disease?

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 aimed to analyse the long-term risk of adverse aortic events after isolated conventional AVR surgery for BAV and normal-sized proximal aorta. METHODS: A total of 200 consecutive patients (mean age 56 ± 13 years, 73% men) with BAV disease and ascending aortic diameter of <40 mm underwent isolated conventional AVR from 1995 through 2008 and were identified from our institutional BAV database. Long-term follow-up data (a total of 1532 patient-years) were obtained for all hospital survivors. Composite adverse aortic/cardiovascular events were defined as the need for redo aortic surgery, the occurrence of aortic dissection/rupture, or sudden cardiac death during follow-up. RESULTS: A total of 25 (13%) patients died after a mean follow-up of 8.5 ± 5 years post-AVR surgery, which resulted in an overall survival rate of 75 ± 6 and 87 ± 7% at 15 years postoperatively, for BAV-aortic stenosis and BAV-aortic regurgitation, respectively (P = 0.6). An adverse aortic event occurred in 1 (0.5%) study patient, while 5 (2.5%) further patients suffered sudden cardiac death. Only 1 patient required redo aortic surgery. No documented aortic dissection/rupture occurred. Redo AVR surgery due to endocarditis or structural valve degeneration was performed in 6 (3%) patients. The rate of freedom from composite adverse events was 92 ± 5 and 95 ± 4% at 15 years, for the AS and AR subgroups, respectively (P = 0.7). CONCLUSIONS: BAV patients with aortic valve dysfunction and normal-sized ascending aorta are at considerably low risk of late adverse aortic events after isolated AVR.

The fate of mild-to-moderate proximal aortic dilatation after isolated aortic valve replacement for bicuspid aortic valve stenosis: a magnetic resonance imaging follow-up study†.

OBJECTIVES: The treatment of mild-to-moderate aortic dilatation at the time of aortic valve replacement (AVR) in patients with bicuspid aortic valve (BAV) disease is still controversial. We aimed to estimate late progression of aortopathy in patients who underwent isolated AVR for BAV stenosis with concomitant proximal aortic dilatation of ≥40 mm. METHODS: The review of our institutional BAV database (n = 510) revealed a subgroup of 96 consecutive BAV patients (57 ± 10 years, 78% male) with BAV stenosis and concomitant ascending aortic dilatation of ≥40 mm [i.e. as defined by magnetic resonance imaging (MRI)/computed tomography (CT)], who underwent isolated AVR from 1995 to 2006. Aortic diameters were quantified by preoperative MRI/CT in all patients (i.e. all cases without MRI/CT were excluded). Moreover, we excluded all cases of simultaneous aortic surgery. MRI/CT follow-up (855 patient-years) was obtained in 83 (87%) patients. Study end-points were progression of proximal aortic diameters (mm/patient-year) and prevalence of aortic events (sudden death, aortic dissection and aortic surgery). RESULTS: MRI/CT follow-up (mean 10.3 ± 3.8 years post-AVR) revealed no significant progress of maximal cross-sectional aortic diameters (i.e. 46.4 ± 4.4 mm pre-AVR vs 46.9 ± 4.6 mm post-AVR, P = 0.1). Aortic diameters were identical in 54 (65%) patients. The mean progression rate of maximal cross-sectional aortic diameter was 0.09 mm/patient-year for the whole study cohort, whereas diameter increase of ≥5 mm was revealed in 1 (1%) patient. No aortic dissection occurred. Five (5%) patients underwent redo aortic surgery for aneurysm, whereas 3 of them had aortic diameters identical to pre-AVR. CONCLUSIONS: Mild-to-moderate ascending aortic dilatation remains stable in most BAV patients who underwent isolated AVR surgery for aortic valve stenosis at least 10-year post-AVR.

Aortopathy in patients with bicuspid aortic valve stenosis: role of aortic root functional parameters.

OBJECTIVES: We prospectively examined functional characteristics of the aortic root and transvalvular haemodynamic flow in order to define factors associated with the severity of aortopathy in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. METHODS: A total of 103 consecutive patients with BAV stenosis (mean age 61 ± 9 years, 66% male) underwent aortic valve replacement ± concomitant aortic surgery from January 2012 through March 2014. All patients underwent preoperative cardiac magnetic resonance imaging (MRI) in order to evaluate the systolic transvalvular flow and the following functional parameters: (i) angulation between the left ventricular outflow axis and the aortic root, (ii) geometrical orientation of residual aortic valve orifice and (iii) BAV cusp fusion pattern. MRI data were used to guide sampling of the ascending aorta during surgery [i.e. jet-sample from the area where the flow-jet impacts on the aortic wall and control sample from the opposite aortic wall (obtained from the aortotomy site)]. Aortopathy was quantified by means of a histological sum-score (0 to 21+) in each sample. RESULTS: A significant correlation was found between histological sum-score in the jet-sample and the angle between the LV outflow axis and the aortic root (r = 0.6, P = 0.007). Moreover, there was a linear correlation between proximal aortic diameter and the angle between systolic flow-jet and ascending aortic wall (r = 0.5, P = 0.006). Logistic regression identified the angle between the LV outflow axis and the aortic root (OR 1.1, P = 0.04) and the angle between the flow-jet and the aortic wall (OR 1.2, P = 0.001) as independent predictors of an indexed proximal aortic diameter ≥22 mm/m(2). CONCLUSIONS: Functional parameters of the aortic root may be used to predict the severity of aortopathy in patients with BAV stenosis, and may be useful in predicting future risk of aortic disease in such patients.

Long-Term Recovery of Reduced Left Ventricular Ejection Fraction after Aortic Valve Replacement in Patients with Bicuspid Aortic Valve Disease.

Background Long-term prognosis of patients with bicuspid aortic valve (BAV) disease and poor left ventricular ejection fraction (LVEF) who underwent aortic valve replacement (AVR) is unknown. We aimed to analyze the recovery of LVEF and incidence of adverse events after AVR in patients with BAV and poor LVEF. Materials and Methods A total of 90 consecutive BAV patients (mean age 57 ± 10 years, 89% male) with baseline LVEF ≤40% underwent an isolated AVR between January 1, 1995, and June 30, 2008, and served as our study population. Follow-up data (800 patient-years) were obtained for all 90 hospital survivors. A subgroup of patients who underwent AVR for BAV stenosis (Group aortic stenosis [AS], n = 70) was compared with those who underwent AVR for BAV regurgitation (Group aortic regurgitation [AR], n = 20). Primary end point was the recovery of LVEF in AS Group versus AR Group. Secondary end points were survival and freedom from adverse cardiac events (i.e., cardiac-related death and need for reinterventions due to persisting heart failure). Results There was a significant increase in LVEF (mean follow-up 9.0 ± 5 years) in AS versus AR Group (i.e., 32 ± 7% [baseline] and 53 ± 9% [follow-up], p < 0.001 in AS Group vs. 33 ± 7% [baseline] and 38 ± 13% [follow-up], p = 0.07 in AR Group). Recovery rate of LVEF was significantly higher in AS Group versus AR Group (i.e., 2.8 percentage points (pp)/year vs. 0.7 pp/year, respectively). In Group AS, 86% of patients were responders, whereas in Group AR, only 30% (p < 0.001). The subjects in Group AR did not show a difference between baseline and follow-up left ventricular end-diastolic diameter (LVEDD) (baseline 61 ± 12 vs. follow-up 58 ± 8, p = 0.813), whereas in Group AS, there was a significant difference of LVEDD (baseline 56 ± 7 vs. follow-up 54 ± 6 mm, p = 0.019). Ten-year survival was 76 ± 6.5% in AS Group versus 78 ± 11% in AR Group (p = 0.3). Prevalence of late adverse cardiac events was 7% in AS Group versus 40% in AR Group (p = 0.03). Conclusion The recovery of reduced LVEF after AVR surgery is significantly impaired in patients with BAV regurgitation as compared with BAV stenosis.

Aortic events after isolated aortic valve replacement for bicuspid aortic valve root phenotype: echocardiographic follow-up study.

OBJECTIVES: Various forms of bicuspid aortic valve (BAV) aortopathy exist, and the optimal treatment for the different subgroups is insufficiently defined. We aimed to analyse the risk of adverse aortic events after aortic valve replacement (AVR) for BAV insufficiency and concomitant mild-to-moderate dilatation of the aortic root (i.e. BAV root phenotype). METHODS: A total of 56 consecutive patients (mean age 47 ± 11 years, 95% men) with BAV insufficiency and a root diameter of 40-50 mm underwent AVR surgery from 1995 to 2008. All patients, as identified from our institutional BAV database, had a dilated aortic annulus (i.e. defined as valve prosthesis size ≥27 mm) without aortic stenosis (i.e. mean gradient ≤20 mmHg). Patients who underwent concomitant aortic surgery were excluded. Follow-up (622 patient-years) including echocardiography data was available for all patients. Aortic events were defined as the need for proximal aortic surgery, the occurrence of aortic dissection/rupture, echocardiographic evidence of increasing aortic root diameter/occurrence of late paravalvular leakage or sudden death during follow-up. RESULTS: Actuarial survival was 90% at 10 years and 78% at 15 years. Adverse aortic events occurred in 19 (34%) study patients. Redo aortic surgery was performed in 6 patients (11%), 2 of which were for aortic dissection. Four patients (7%) suffered sudden cardiac death. Moreover, follow-up echocardiography revealed a significant, progressive enlargement of the aortic root diameter in 7 (13%) patients and occurrence of late de novo paravalvular leakage in 2 (3%) patients. The resultant freedom from aortic events was 81% at 10 years and 51% at 15 years. CONCLUSIONS: Patients with a BAV root phenotype are at significant risk of aortic events after isolated AVR. Simultaneous root/ascending aortic surgery should be strongly considered in such patients.

Correlation between systolic transvalvular flow and proximal aortic wall changes in bicuspid aortic valve stenosis.

OBJECTIVES: The purpose of this study was to analyse the correlation between preoperative systolic transvalvular flow patterns and proximal aortic wall lesions in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. METHODS: A total of 48 consecutive patients with BAV stenosis (mean age 58 ± 9 years, 65% male) underwent aortic valve replacement (AVR) ± proximal aortic surgery from January 2012 through February 2013. Preoperative cardiac phase-contrast cine magnetic resonance imaging (MRI) assessment was performed in all patients in order to detect the area of maximal flow-induced stress in the proximal aorta. Based on these MRI data, two aortic wall samples (i.e. area of the maximal stress (jet sample) and the opposite aortic wall (control sample)) were collected during AVR surgery. Aortic wall changes were graded based on a summation of seven histological criteria (each scored from 0 to 3). Histological sum score (0-21) was separately calculated and compared between the two aortic samples (i.e. jet sample vs control sample). RESULTS: An eccentric transvalvular flow jet hitting the proximal aortic wall could be identified in all 48 (100%) patients. The mean histological sum score was significantly higher in the jet sample vs control sample areas of the aorta (i.e. 4.1 ± 1.8 vs 2.2 ± 1.5, respectively) (P = 0.02). None of the patients had a higher sum score value in the control sample. CONCLUSIONS: Our study demonstrates a strong correlation between the systolic pattern of the transvalvular flow jet and asymmetric proximal aortic wall changes in patients undergoing AVR for BAV stenosis.

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

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.