A Recurrent c.416C>T Variant in the B3GAT3 Gene in the Turkish Population: Report of Two Siblings and Expanding the Clinical Spectrum.

Introduction: Linkeropathies are a group of rare multi-systemic genetic disorders primarily affecting the skeletal and cardiac systems due to defects in the enzymes responsible for proteoglycan synthesis. Case Presentation: We present a case of two siblings with the B3GAT3 variant. The 14-year-old boy exhibited short stature, severe kyphoscoliosis, splenomegaly, and aortic root dilatation, along with several physical abnormalities including bifid uvula, blue sclera, limited elbow extension, and pectus carinatum. His 6-year-old sister also exhibited comparable yet less pronounced physical features. Clinical exome sequencing analysis revealed a homozygous c.416C>T variant in the B3GAT3 gene for the sister; the same variant was also present in the boy patient. The boy underwent preoperative halo-gravity traction for severe kyphoscoliosis, followed by posterior instrumentation and fusion surgery without complications. Discussion/Conclusion: B3GAT3-related linkeropathy syndrome is a rare disorder and we further expand the clinical spectrum with novel findings.

Aortic root replacement for bicuspid aortic valve dysfunction does not impair survival rates.

BACKGROUND: Patients with a bicuspid aortic valve carry an increased risk for developing an ascending aortic aneurysm due to intrinsic aortic wall alterations. A lower threshold for aortic surgery may therefore be considered in these patients, especially in those who require aortic valve surgery. This study aimed to compare the outcomes of an isolated aortic valve replacement with that of an aortic root replacement in bicuspid aortic valve patients with an indication for aortic valve surgery. METHODS: Patients were included in retrospect from a tertiary academic hospital. Included patients received an elective aortic valve (AVR) or a composite valve-graft conduit (both mechanical and biological) between 2006 and 2021 without any concomitant procedure. Mortality data were retrieved from a national database and comparisons, including survival analyses, were performed between both groups. RESULTS: A total of 132 isolated AVR and 149 aortic root replacements were included. Patients who received an isolated AVR were significantly older than the aortic root replacement group (62.9 vs. 57.7 year respectively, p < 0.001). Survival analyses showed a comparable long-term mortality between both groups (8.1% vs. 9.1%, p = 0.321). CONCLUSION: This study shows that performing an aortic root replacement with a composite valve-graft conduit in bicuspid aortic valve patients does not impair the survival outcomes. In the light of preventing potential future aortic complications within this patient group with a congenitally and structurally weakened aortic wall, a more aggressive approach towards the treatment of BAV aortopathy might be considered.

Early surgical outcomes in the composite-valve root replacement with bioprosthesis after surgery for acute type A aortic dissection.

OBJECTIVES: This study aimed to evaluate the surgical outcomes of composite-valve root replacement with bioprosthesis (b-CVRR) after acute type A aortic dissection (AAAD) repair. METHODS: We included 41 patients who underwent b-CVRR after surgery for AAAD from 2007 to 2022. We excluded seven patients with VSRR, three with mechanical valve use, one with mycotic aneurysm, and one with cardiopulmonary resuscitation. RESULTS: The overlapping surgical indications for b-CVRR were pseudoaneurysm in 21 patients (51.2%), severe AI in 19 (46.3%), re-dissection in 15 (36.6%), root dilatation (> 50 mm) in 12 (41.5%), and rupture in 3 (7.3%). The coronary artery reconstruction methods were bilateral Carrel patch in 13 patients (32%), Carrel patch with Piehler in 16 patients (38%), bilateral Piehler in six patients (15%), and Piehler with coronary artery bypass (CABG) in six patients (15%). Four patients who underwent the Carrel patch technique required additional intraoperative CABG. Three hospital mortality occurred (7.3%; myonephropathic metabolic syndrome, heart failure, bleeding from the thyroid carotid artery without injury). No thrombosis of the reconstructed site was observed with the Piehler procedure. CONCLUSION: Surgical outcomes for patients treated with b-CVRR after AAAD repair were acceptable. Intraoperative coronary artery events occurred due to stenosis caused by traction on the reconstructed coronary artery due to adhesions.

Management of Fallot's Uncorrected Tetralogy in Adulthood: A Narrative Review.

The majority of cyanotic congenital cardiac defects are caused by the tetralogy of Fallot. Some symptoms include a biventricular connection of the aortic root, right ventricular hypertrophy, blockage of the right ventricular outflow tract, and a ventricular septal defect. Our understanding of tetralogy of Fallot (TOF) has significantly advanced since it was first described in 1888, and early diagnosis has led to improved surgical management and increased life expectancy. Adults with unrepaired and repaired TOF present with a range of late complications, including heart failure, the need for re-interventions, and late arrhythmias. Right ventricular (RV) failure, often caused by chronic pulmonary regurgitation, is a significant cause of heart failure in patients with TOF. Current treatment options are limited, and mainstay surgical procedures such as pulmonary-valve replacement (PVR), trans-annular repair (TAR), or infundibular widening repair have not shown a significant reduction in preventing right ventricular (RV) failure or death. Here, we explain the mechanisms of RV failure in ToF, chronic pulmonary regurgitation, heart failure, and secondary polycythemia. HF management in untreated adults is discussed. The progression of the disease, as well as complications, are also discussed. The treatment plan and the need to investigate the best management approach for this unsolved problem are included. This review aims to fill the knowledge gaps and supply valuable information regarding mechanisms of RV failure, chronic pulmonary regurgitation, and secondary polycythemia. To summarize, a new combat strategy must be found to battle RVF, and a more profound vision of these mechanisms is required. If it is not corrected, it will be one of the future research lines that will contribute to designing more efficacious treatment techniques for adults with TOF.

Aortic Root Dilation and Genotype Associations in Phelan-McDermid Syndrome.

Phelan-McDermid syndrome (PMS) is a rare genetic neurodevelopmental disorder that results from the loss of one functional copy of the SHANK3 gene. While many clinical features of PMS are well-understood, there is currently limited literature on cardiovascular abnormalities in PMS. This report aims to evaluate the prevalence of aortic root dilation (ARD) among individuals with PMS and to understand if underlying genetic variation relates to risk for ARD. We present findings from 59 participants collected from a multisite observational study evaluating the phenotype and natural history of PMS. Individual echocardiographic and genetic reports were analyzed for aortic root measurements and genetic variant data, respectively. Our a priori hypothesis was that participants with chromosome 22 deletions with hg19 start coordinates on or before 49,900,000 (larger deletions) would have more instances of ARD than participants with deletion start coordinates after 49,900,000 (smaller deletions). Eight participants (14%) had ARD, and its presence was statistically significantly associated with large deletions (p = 0.047). Relatedly, participants with ARD had significantly more genes deleted on chromosome 22 than participants without ARD (p = 0.013). These results could aid in the identification of individuals with PMS who are at higher risk for ARD.

Complex Bentall Operation: Clinical Pearls to Standardize the Procedure.

BACKGROUND: A straightforward Bentall operation can be performed safely with low mortality, but some challenging cases require a more complex operation. In this manuscript, we will discuss the steps of the Bentall procedure. METHODS: We reviewed specific scenarios such as acute aortic dissection, native valve or prosthetic valve endocarditis, redo Bentall after aortic root replacement, calcified aortic root, and patients with prior coronary artery bypass grafting, mechanical aortic valve replacement, stentless aortic valve replacement, and prior extensive aortic arch repair with proximalization of neck vessels. RESULTS: Variety of techniques were reported regarding reconstruction of aortic annulus (e.g. Dacron graft is everted to create 5 to 6 crimps when sewing a bioprosthesis. The height of the skirt can be adjusted depending on tissue defect) and reimplantation of coronary buttons (Interposition of Dacron graft for coronary button reimplantation [original Cabrol technique], short interposition of Dacron graft is known as Piehler technique, and technique in redo Bentall after prior aortic root replacement). In patients with a history of coronary artery bypass grafting, direct reimplantation of previous vein graft patch to the Dacron graft or interposition of short Dacron graft were introduced. In addition, repair of coronary button in Type A dissection or calcified aortic root were also described. CONCLUSIONS: There are various techniques available in modified Bentall operation. Surgeons should be familiar with the setup, anatomy of aortic root and surrounding structures, ways to treat tissue defect and prepare coronary buttons, and the various bail-out procedures.

Third sternotomy with modified Bentall/Konno procedure for recurrent multilevel left ventricular outflow tract obstruction in a 15-year-old.

This is a 15-year-old, 73.5 kg male who was born with congenital aortic valve stenosis. He underwent a transcatheter balloon aortic valvuloplasty in the neonatal period, followed by an open aortic valvotomy at 2 months of age. When he was 3 years old, he underwent aortic root replacement with a 15 mm aortic homograft. Recently, he presented with recurrent left ventricular outflow tract obstruction that appeared valvular and subvalvular in nature. We present our technique of repeat aortic root replacement and left ventricular outflow tract enlargement using modified Bentall combined with Konno-Rastan aortoventriculoplasty.

Correlation of aortic root dimensions and mortality in acute heart failure: A nationwide prospective cohort study.

Background: An association between increased aortic root dimensions (ARD) and elevated risk of cardiovascular mortality has been reported in the general population. However, evidence regarding the association between ARD and mortality in patients with acute heart failure (AHF) is limited. Methods: In a nationwide prospective cohort of the China Patient-Centered Evaluative Assessment of Cardiac Events Prospective Heart Failure Study, ARD was measured during diastole using echocardiography and indexed to body mass index (BMI). Cox proportional hazard models were used to validate the association between BMI-indexed ARD and mortality. Additionally, the relationship between BMI-indexed ARD and mortality was presented using restricted cubic spline in all populations, and both sexes. Results: A total of 2125 participants with ARD were included in the final analysis, among of 38.4 % were women, with a median age of 67 years. Over a median follow-up period of 54.4 (interquartile range: 30.1 to 59.7) months, 895 deaths occurred, with 750 attributed to cardiovascular causes and 145 to non-cardiovascular causes. Compared to the highest tertile group of BMI-indexed ARD, the lowest tertile group had a lower risk of cardiovascular mortality (hazard ratio [HR], 0.71; 95 % confidence interval [CI], 0.58 to 0.87; P < 0.001) and all-cause mortality (HR, 0.68; 95 % CI, 0.56 to 0.81; P < 0.001). Similarly, the middle tertile group also had a lower risk of cardiovascular mortality (HR, 0.78; 95 % CI, 0.65 to 0.93; P = 0.007) and all-cause mortality (HR, 0.75; 95 % CI, 0.63 to 0.89; P < 0.001). Considering the competing risks, the lowest BMI-indexed ARD groups showed a significant mortality risk of cardiovascular mortality in all populations, and both sexes. Moreover, the relationship between BMI-indexed ARD and mortality was linear in males, while a "J" shaped relationship was observed in females. Conclusion: Lower BMI-indexed ARD was associated with a decreased risk of all-cause and cardiovascular mortality than those with higher BMI-indexed ARD in AHF. Additionally, a discrepancy was observed between the sexes in the relationship between BMI-indexed ARD and mortality. These findings contribute to the prompt identification of potential mortality risks in patients with AHF.

Impact of the Grade and Jet-flow Direction of Residual Aortic Regurgitation after Valve-Sparing Root Replacement.

OBJECTIVES: The current study aimed to investigate the impact of the grade and jet direction of residual aortic regurgitation (rAR) after valve-sparing root replacement (VSRR). METHODS: The study enrolled 248 adult patients who underwent VSRR from 1995 to 2021. The patients were divided into groups based on the postoperative rAR. Patients with rAR were further categorized per the rAR grade and jet direction. The primary endpoint was the development of aortic regurgitation ≥ moderate and/or the need for valve replacement during the follow-up, analyzed by a multivariable competing risk analysis. The secondary endpoints included the occurrence of rAR and overall survival. RESULTS: The median age of the patients was 36.5 years, and 79.8% were diagnosed with connective tissue disease. After VSRR, 146 patients did not present with rAR. However, 102 had rAR (77 with minimal central, 18 with minimal eccentric, and 7 with mild). The 5- and 8-year incidence rates of the primary endpoint were 14.6% and 17.9%, respectively. The rAR was a significant risk factor (P=0.001), and eccentricity and mild rAR seemed to play an important role. The risk factors of rAR included dilated root, preoperative moderate regurgitation, and redo sternotomy. The overall survival was influenced only by age. CONCLUSION: rAR after VSRR operation could be a risk factor for AR progression. Minimal central rAR generally has a tolerable clinical course. However, patients with even minimal eccentric AR may develop AR progression, so active surveillance and timely management might be required. Further, early VSRR can help reduce the rAR.

Hemodynamic Analysis in Aortic Dilatation after Arterial Switch Operation for Patients with Transposition of Great Arteries Using Computational Fluid Dynamics.

After an arterial switch operation for complete transposition of the great arteries, neo-aortic root dilatation occurs, with unclear hemodynamic effects. This study analyzes three groups (severe dilation, mild dilation, and normal) using computational fluid dynamics (CFD) on cardiac CT scans. Aortic arch angles in severe (median 72.3, range: 68.5-77.2) and mild dilation (76.6, 71.1-85.2) groups are significantly smaller than the normal group (97.3, 87.4-99.0). In the normal and mild dilatation groups, Wall Shear Stress (WSS) exhibits a consistent pattern: it is lowest at the aortic root, gradually increases until just before the bend in the aortic arch, peaks, and then subsequently decreases. However, severe dilation shows disrupted WSS patterns, notably lower in the distal ascending aorta, attributed to local recirculation. This unique WSS pattern observed in severely dilated patients, especially in the transverse aorta. CFD plays an essential role in comprehensively studying the pathophysiology underlying aortic dilation in this population.

Complete Aortic Valve Reconstruction with Autologous Pericardium: Analysis of Mid-Term Results of Single-Center Experience with AVNeo Procedure.

PURPOSE: Aortic valve neocuspidization (AVNeo) is a relatively recent advancement in surgical AV replacement. Data on its performance beyond the short term are limited. We assessed the mid-term outcomes in patients undergoing AVNeo, focusing on feasibility, perioperative details, and its role in AV pathology treatment. METHODS: Sixty-five consecutive patients underwent AVNeo between December 2016 and February 2018. Clinical data were prospectively collected and retrospectively analyzed. Tricuspid reconstruction with autologous pericardium was performed in all cases. Echocardiographic follow-up was conducted post-discharge, at 6 and 12 months, and annually thereafter. RESULTS: The mean age was 62.6 ± 18.7 years. AVNeo was feasible in all cases. Concomitant procedures were performed in 43 (66.2%) patients. Mean bypass and cross-clamp times were 119.2 ± 30.3 and 87.1 ± 22.9 minutes, respectively. Postoperative transvalvular hemodynamics was excellent. There was one (1.5%) in-hospital death. Follow-up (mean 66.72 ± 12.77 months) was complete in 58 patients (89.2%). There were no detected valve-related or thromboembolic events. Transvalvular hemodynamic parameters were stable during the observation period: peak pressure gradient at discharge and follow-up was 15.3 ± 4.6 mmHg and 15.01 ± 6.3 mmHg, respectively (ρ = 0.346). CONCLUSIONS: AVNeo demonstrated the feasibility and favorable mid-term outcomes. Studies with longer-term observation are warranted to evaluate its durability.

Nature of aortic annulus: Influence of annulus dynamic on the aortic valve hemodynamics.

Accurate imaging reports of the aortic valve indicate that the diameter of the aortic annulus changes regularly during a cardiac cycle. Most of these studies aim to demonstrate the proper method for estimating the aortic annulus diameter before performing TAVR surgery, revealing that the aortic annulus is dynamic and not constant throughout the cardiac cycle. This raises the question of how fixing the aortic annulus might affect valve function, which is a question that still needs to be addressed. Therefore, the present study seeks to address this question and elucidate the dynamic impact of the aortic annulus on aortic valve hemodynamics. Two computational models based on this hypothesis were created and solved, and then their results were compared. Both models are identical, except for the intrinsic dynamic nature of the aortic annulus. One model consists of the dynamic behavior, and the other simulates a fixed annulus, resembling the effect of a TAVR operation, SAVR, or any phenomenon that eliminates the dynamic nature of the annulus. Our research findings indicate that the dynamic nature of the annulus enhances blood flow (+2.7 %), increases mean velocity (+11.9) and kinetic energy density (+34 %), prolongs momentum retention during systole, stabilizes the flow jet at the end of systole, reduces the required pressure to keep the leaflets open (-40.9 % at 0.3s), and sustains ventricular pressure superiority (+9.4 %) over the aorta for a longer duration (+17.7 % of systole), a crucial factor in preventing backflow during aortic valve closure. Based on these results, more attention should be paid to the dynamic nature of the annulus.

Decellularized aortic homografts versus mechanical composite grafts for aortic root replacement.

OBJECTIVES: Mechanical composite valve grafts (MCVGs) are the first-line therapy for aortic root replacement in young adults. Decellularized aortic homografts (DAH) present a promising novel alternative due to their lower thrombogenicity. We aimed to compare both treatment options regarding survival and valve-related adverse events. METHODS: This study was designed as a single-centre retrospective cohort study including patients who underwent root replacement with MCVG or DAH between 2000 and 2022. Urgent or emergent procedures were excluded. RESULTS: The study cohort included 289 patients (MCVG n = 216, DAH n = 73) with a mean age of 48.5 ± 12 years (MCVG 49 ± 12 years vs DAH 47 ± 11 years; P = 0.23) and a median EuroScore II of 1.7% (1.2, 2.6). The 30-day mortality was 1% (n = 3). Cumulative survival at 3 years was 99% for DAH and 94% for MCVG, respectively (P = 0.15). Mean follow-up was 98.9 ± 72.7 months. Bleeding events (n = 14, 6.5%) and thromboembolism (n = 14, 6.5%) were only observed in the MCVG group (P = 0.19 and 0.09, respectively). Four cases (5%) of moderate structural valve deterioration occurred, all in the DAH group (P ≤ 0.001). The cumulative incidence of a composite end point of valve-related adverse events was significantly higher in the MCVG group (P = 0.0295). CONCLUSIONS: Aortic root replacement with MCVGs and decellularized aortic homografts showed low mortality in an elective setting. Patients in the homograft cohort demonstrated significantly higher freedom from valve-related adverse events. DAH present a promising treatment option for young patients requiring root replacement; however, data on long-term durability are needed.

Anomalous coronary artery masquerading as a root abscess: a case report.

Congenital coronary artery anomalies are rare and most often clinically benign. We present a case of a 67-year-old male with osteomyelitis and persistent bacteremia with an anomalous left coronary artery mimicking an aortic root abscess. A transesophageal echocardiogram revealed a hypoechoic potential space around the aortic root, highly suspicious for a root abscess. Urgent cardiac surgery was performed, revealing no infection but an anomalous coronary artery arising from the right coronary sinus. This case highlights the importance of considering atypical anatomy in the diagnosis of infectious cardiac processes. While this resemblance should not delay intervention for suspected abscesses, it emphasizes the need to be aware of congenital differences in imaging for patients with known anomalies or asymptomatic patients with unknown anatomy.

Abnormal coronary arteries are rare and are usually not dangerous. We present a case of a 67-year-old male with an infection in the bone and persistent findings of bacteria in the blood who had an abnormal coronary artery that mimicked an infected space. Ultrasound of the heart found thickening and a space around the aortic root, highly suspicious for an infection in the setting of bacteria in the blood. Urgent surgery was performed, revealing no signs of infection but abnormal coronary artery anatomy. This case highlights the importance of considering atypical anatomy in the diagnosis of infection around the heart. While this should not delay intervention, it emphasizes the need to be aware of differences in anatomy.

Learning three-dimensional aortic root assessment based on sparse annotations.

Purpose: Analyzing the anatomy of the aorta and left ventricular outflow tract (LVOT) is crucial for risk assessment and planning of transcatheter aortic valve implantation (TAVI). A comprehensive analysis of the aortic root and LVOT requires the extraction of the patient-individual anatomy via segmentation. Deep learning has shown good performance on various segmentation tasks. If this is formulated as a supervised problem, large amounts of annotated data are required for training. Therefore, minimizing the annotation complexity is desirable. Approach: We propose two-dimensional (2D) cross-sectional annotation and point cloud-based surface reconstruction to train a fully automatic 3D segmentation network for the aortic root and the LVOT. Our sparse annotation scheme enables easy and fast training data generation for tubular structures such as the aortic root. From the segmentation results, we derive clinically relevant parameters for TAVI planning. Results: The proposed 2D cross-sectional annotation results in high inter-observer agreement [Dice similarity coefficient (DSC): 0.94]. The segmentation model achieves a DSC of 0.90 and an average surface distance of 0.96 mm. Our approach achieves an aortic annulus maximum diameter difference between prediction and annotation of 0.45 mm (inter-observer variance: 0.25 mm). Conclusions: The presented approach facilitates reproducible annotations. The annotations allow for training accurate segmentation models of the aortic root and LVOT. The segmentation results facilitate reproducible and quantifiable measurements for TAVI planning.

Aortic valve performance after remodelling versus reimplantation in a propensity-matched comparison.

OBJECTIVES: Both aortic root remodelling and aortic valve (AV) reimplantation have been used for valve-sparing root replacement in patients with aortic root aneurysm with or without aortic regurgitation. There is no clear evidence to support one technique over the another. This study aimed to compare remodelling with basal ring annuloplasty versus reimplantation on a multicentre level with the use of propensity-score matching. METHODS: This was a retrospective international multicentre study of patients undergoing remodelling or reimplantation between 2010 and 2021. Twenty-three preoperative covariates (including root dimensions and valve characteristics) were used for propensity-score matching. Perioperative outcomes were analysed along with longer-term freedom from AV reoperation/reintervention and other major valve-related events. RESULTS: Throughout the study period, 297 patients underwent remodelling and 281 had reimplantation. Using propensity-score matching, 112 pairs were selected and further compared. We did not find a statistically significant difference in perioperative outcomes between the matched groups. Patients after remodelling had significantly higher reintervention risk than after reimplantation over the median follow-up of 6 years (P = 0.016). The remodelling technique (P = 0.02), need for decalcification (P = 0.03) and degree of immediate postoperative AV regurgitation (P < 0.001) were defined as independent risk factors for later AV reintervention. After exclusion of patients with worse than mild AV regurgitation immediately after repair, both techniques functioned comparably (P = 0.089). CONCLUSIONS: AV reimplantation was associated with better valve function in longer-term postoperatively than remodelling. If optimal immediate repair outcome was achieved, both techniques provided comparable AV function.

The aortic root, do we see the 'hole' picture?

The aortic root is the segment of the aorta between the left ventricular outflow tract and the sinotubular junction of the ascending aorta, and, on one level, is merely a tube, with a valve at its base, dynamic structures below it, and notable for having the life-limiting coronary arteries originate within its sinuses. However, we propose that the perception of the aortic root has been historically grossly over-simplified by virtue of a bias towards its internal aspect, in terms of coronary ostia and subvalvar relationships through the fibrous skeleton and in so-doing a myocardial component on the external aspect has all but been ignored. This myocardial mass, a component of the left ventricular free wall, is sometimes termed the 'left ostial process' but appears to be rarely, if ever, considered by anatomists, cardiologists, and surgeons alike. By virtue of its direct continuity to the aortic root and proximal left coronary artery, it may have unique roles and, at the very least, deserves greater recognition and investigation. Herein, we propose that it could play a crucial role in cardiac embryology including coronary dominance, and may afford a physiological advantage, to the extent that it may have been selected for in evolutionary terms.

Aortic Root Thromboembolism and Associated Acute Myocardial Infarction in Patients With Contemporary Durable LVADs.

Patients with HeartMate 3 left ventricular assist devices may develop aortic root thrombus, yet its prevalence and associated risks are unknown. We present 2 patients who developed aortic root thromboembolism and acute coronary occlusions. We additionally present heart transplantation as viable treatment for thromboembolic disease and refractory right ventricular failure.

The proximal extension of acute type A aortic dissection is associated with ascending aortic wall degeneration.

Background: Aortic root involvement during acute type A aortic dissection (ATAAD) may depend on ascending aortic wall degeneration. Surgical decision-making for extended resection of the aortic root is clinically made without histopathology. The aim of the study was to investigate whether the degree of degeneration of the ascending aortic wall found in patients with ATAAD is associated with the aortic root involvement. Methods: Collectively, 141 consecutive patients undergoing ATAAD surgery at Tampere University Heart Hospital were investigated. The ascending aortic wall resected in surgery was processed for 11 different variables that describe medial and adventitial degeneration. In addition, atherosclerosis and inflammation were separately evaluated. Patients undergoing aortic root replacement were compared with those with supracoronary reconstruction of the ascending aorta with/without aortic valve surgery (root-sparing surgery) during a mean 4.9-year follow-up. Results: Aortic root replacement together with the ascending aortic replacement was performed in 39% of the patients (n=55). The mean age for all patients was 65 years [standard deviation (SD 13)]. Many patients with aortic root replacement had moderate to severe aortic valve regurgitation (85.5%). Most of the patients with aortic root-sparing surgery included a supracoronary tube prosthesis (89.5%), while nine patients also had aortic valve replacement. The degree of mucoid extracellular matrix accumulation was more prominent in patients with aortic root replacement compared to patients with root-sparing surgery (2.1 SD 0.4 vs. 1.9 SD 0.4, P=0.04, respectively). During follow-up, there were 52 deaths among patients (log rank P=0.79). Conclusions: Histopathology of the ascending aorta during ATAAD reveals distinctive aortic wall degeneration in patients with aortic root involvement vs. not. The degree of mucoid extracellular matrix accumulation assessed postoperatively is associated with the choice of surgical procedure in many patients.

Impact of the Aortomitral Positional Anatomy on Atrioventricular Conduction Disorder Following Mitral Valve Surgery.

BACKGROUND: Variations in the aortomitral positional anatomy, including aortic root rotation appear to be related to variations in the location of the conduction system, including the bundle of His. However, little is known about their clinical significance. METHODS AND RESULTS: This study included 147 patients with normal ECGs who underwent mitral valve surgery. The aortomitral anatomy was classified using preoperative 3-dimensional transesophageal echocardiography, and postoperative conduction disorders, including atrioventricular block and bundle branch block, were analyzed. Variations classified as aortomitral appearance were designated as having a center appearance (85.7%, n=126/147) or lateral appearance (14.3%, n=21/147) on the basis of whether the aortic root was located at the center or was shifted to the left fibrous trigone side. Subsequently, those with a center appearance, aortic root rotation was classified as having a center rotation (83.3% [n=105/126]), in which the commissure of the left and noncoronary aortic leaflet was located at the center, lateral rotation (14.3% [n=18/126]), rotated to the left trigone side, or medial rotation (2.4% [n=3/126]), rotated to the right. The incidence of 3-month persistent new-onset conduction disorder was higher in the lateral appearance than the center appearance group (21.1% versus 5.0%; P=0.031) and higher in the lateral rotation than in the center or medial rotation groups (29.4% versus 1.0% versus 0.0%, respectively; P<0.001). CONCLUSIONS: Aortomitral variations can be classified using 3-dimensional transesophageal echocardiography. Lateral appearance and lateral rotation are risk factors for conduction disorders in mitral valve surgery.