Concomitant Aortic Valve Disease Research Articles

Sex Differences in Outcomes of Adults with Repaired Coarctation of Aorta and Concomitant Aortic Valve Disease

BackgroundAortic valve disease is common in adults with coarctation of aorta. However, no systematic comparative analyses have been performed of the clinical course of aortic valve disease for male vs female patients in this population. The purpose of this study was to compare cardiac remodelling, onset of symptoms, and incidence of aortic valve replacement (AVR) for male vs female patients. MethodsA retrospective study was conducted of adults with repaired coarctation of aorta and ≥ moderate aortic stenosis and/or aortic regurgitation. Cardiac remodelling (left ventricular [LV], left atrial, right ventricular [RV], and right atrial structure and function) and symptomatic and/or functional class were determined at the baseline encounter. Development of new-onset symptoms and the incidence of AVR were ascertained for the period from baseline to last encounter. ResultsWe identified 214 patients (121 male [57%], 93 female [43%]). Although both groups had a similar aortic valve gradient, aortic valve area indexed to body surface area, aortic regurgitation severity, and functional status at baseline, female patients had more LV concentric hypertrophy and remodelling, left atrial hypertension and dysfunction, elevated RV systolic pressure, and RV systolic dysfunction. Of 151 patients without symptoms at baseline,102 (72%) developed symptoms. Female sex was independently associated with new-onset symptoms (adjusted hazard ratio 1.14, [95% confidence interval 1.05-1.23]). Of 214 patients, 191 (89%) underwent AVR. Female sex was not associated with AVR upon multivariable analysis. However, LV concentric hypertrophy and remodelling (both of which were more common in female patients) were associated with new-onset symptoms and AVR. ConclusionsFemale patients, compared to male patients, had more-advanced cardiac remodelling, and more-rapid onset of symptoms, but a similar risk of AVR.

Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm.

Bicuspid Aortic Valves (BAV) are associated with an increased incidence of thoracic aortic aneurysms (TAA). TAA are a common aortic pathology characterized by enlargement of the aortic root and/or ascending aorta, and may become life threatening when left untreated. Typically occurring as the sole pathology in a patient, TAA are largely asymptomatic. However, in some instances, they are accompanied by aortic valve (AV) diseases: either congenital BAV or acquired in the form of Aortic Insufficiency (AI) or aortic stenosis (AS). When TAA are associated with aortic valve disease, determining an accurate and predictable prognosis becomes especially challenging. Patients with AV disease and concomitant TAA lack a widely accepted diagnostic approach, one that integrates our knowledge on aortic valve pathophysiology and encompasses multi-modality imaging approaches. This review summarizes the most recent scientific knowledge regarding the association between AV diseases (BAV, AI, AS) and ascending aortopathies (dilatation, aneurysm, and dissection). We aimed to pinpoint the gaps in monitoring practices and prediction of disease progression in TAA patients with concomitant AV disease. We propose that a morphological and functional analysis of the AV with multi-modality imaging should be included in aortic surveillance programs. This strategy would allow for improved risk stratification of these patients, and possibly new AV phenotypic-specific guidelines with more vigilant surveillance and earlier prophylactic surgery to improve patient outcomes.

Left ventricular remodeling following aortic root and ascending aneurysm repair.

Adverse left ventricular remodeling due to a mismatch between stiffness of native aortic tissue and current polyester grafts may be under-recognized. This study was conducted to evaluate the impact of proximal aortic replacement on adverse remodeling of the left ventricle. All aortic root and ascending aortic aneurysm patients were identified (n = 2,001, 2006-2019). The study cohort consisted of a subset of patients (n = 98) with two or more electrocardiogram (ECG)-gated CT angiograms, but without concomitant aortic valve disease or bicuspid aortic valve, connective tissue disease, acute aortic syndrome or prior history of aortic repair or mitral valve surgery. LV myocardial mass was measured from CT data and indexed to body surface area (LVMI). The study cohort was divided into a surgery group (n = 47) and a control group; optimal medical therapy group (OMT, n = 51). The mean age was 60 ± 11 years (80% male). Beta-blocker use was significantly more frequent in the surgery group (89 vs. 57%, p < 0.001), whereas, all other antihypertensive drugs were more frequent in the OMT group. The average follow-up was 9.1 ± 4.0 months for the surgery group and 13.7 ± 6.3 months for the OMT group. Average LVMI at baseline was similar in both groups (p = 0.934). LVMI increased significantly in the surgery group compared to the OMT group (3.7 ± 4.1 vs. 0.6 ± 4.4 g/m2, p = 0.001). Surgery, baseline LVMI, age, and sex were found to be independent predictors of LVMI increased on multivariable analysis. Proximal aortic repair with stiff polyester grafts was associated with increased LV mass in the first-year post-operative and may promote long-term adverse cardiac remodeling. Further studies should be considered to evaluate the competing effects of aortic aneurysm related mortality against risks of long-term graft induced aortic stiffening and the potential implications on current size thresholds for intervention.

Anomalous Aortic Origin of a Coronary Artery in Adults

Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in youth. However, its significance and optimal management in adults is poorly understood. Our objective is to characterize AAOCA in a large single-center adult cohort based on coronary anatomic variants and surgical management strategies. We reviewed imaging, clinic, and operative reports for 645 adults with an encounter diagnosis code of congenital coronary anomaly from July 2015 to July 2017. After excluding other congenital heart defects, we characterized 167 patients with AAOCAs by anatomic variant, symptoms at diagnosis, indication for advanced imaging, and if performed, surgical repair. To describe the anatomic variant, we classified the origin and course by following the atomization scheme developed by the Congenital Heart Surgeon's Society's AAOCA registry. Among adults with AAOCA, the anomalous origin involved the right coronary artery in 57% (96 of 167), left main coronary artery in 23% (39 of 167), left anterior descending in 2% (4 of 167), circumflex in 16% (26 of 167), and multiple coronaries in 1% (2 of 167). Anomalous right coronary arteries were diagnosed at an older median age than anomalous left main coronary arteries (55 vs 51 years, respectively; P= .026). Surgical repair of AAOCA occurred in 22% (36 of 167) of patients. Concomitant cardiac surgical procedures accompanied 36% (13 of 36) of them. No deaths occurred over a median follow-up of 2.5 years. Most patients in our single-center AAOCA registry were diagnosed in the presence of cardiac symptoms. Concomitant aortic valve disease and coronary atherosclerotic burden complicate both the evaluation and surgical approach to adult AAOCA repair.

Prognostic differences between atrial functional mitral regurgitation and ventricular functional mitral regurgitation

Abstract Introduction Atrial functional mitral regurgitation (A-FMR) has been under-recognized until recently as a cause of FMR, and the prognostic difference between A-FMR and ventricular FMR (V-FMR) has not been fully elucidated. As there has been different mechanisms of FMR suggested in A-FMR and V-FMR, we hypothesized that prognosis and prognostic predictors of A-FMR may differ from those of V-FMR. Purpose To investigate the prognosis and prognostic predictors of A-FMR in comparison with V-FMR. Methods Among 1312 consecutive patients with grade 3+ (moderate to severe) or 4+ (severe) MR, 378 consecutive FMR patients were identified by excluding patients with degenerative MR, previous cardiac surgery, or concomitant aortic valve disease and/or mitral stenosis. FMR with ejection fraction (EF) &amp;lt;40% or FMR due to regional wall motion abnormalities with leaflet tethering were classified as V-FMR (N=288), and FMR due to left atrial (LA) and/or annular dilatation with preserved or mid-range EF (≥40%) were classified as A-FMR (N=90). All-cause death and heart failure hospitalization were analyzed as cardiovascular (CV) events in this study. Surgical or percutaneous mitral valve intervention without CV events was handled as not reaching an endpoint and these cases were censored. Results A-FMR were significantly older (76 [69–82] vs. 70 [58–77] years), higher rates of female (64 vs. 35%) and atrial fibrillation (88 vs. 42%), and lower B-type natriuretic peptide (BNP) values (169 [101–318] vs. 447 [213–952] pg/ml) compared to V-FMR (all P&amp;lt;0.05). On echocardiography, LV end-diastolic and end-systolic dimensions (52 [48–57] vs. 64 [58–72] mm, 34 [31–37] vs. 55 [48–64] mm), respectively) were smaller, and EF (55 [50–60] vs. 28 [19–35] %) and LA volume (99 [73–137] vs. 73 [57–91] ml/m2) were larger in A-FMR (all P&amp;lt;0.05). Effective regurgitant orifice area (32 [26–40] vs. 31 [24–45] mm2) and regurgitant volume (50±15 vs. 52±16 ml) were similar (both n.s.). During a median follow up of 1407 days, 206 (54%) patients developed CV events. Kaplan-Meier analysis revealed that V-FMR had a significantly higher rates of CV events compared to A-FMR (Figure) with adjusted hazard ratio (HR) of 1.762 [1.168–2.660], P=0.007 after adjusted for variables including age, sex, New York Heart Association functional class, previous heart failure hospitalization, estimated glomerular filtration rate (eGFR) and BNP. Further, stepwise multivariate analysis showed that independent prognostic predictors of A-FMR were LA volume and eGFR, while those for V-FMR were LA volume, age, and LV end-systolic dimension. Conclusions A-FMR had relatively better prognosis compared to V-FMR, and there were different prognostic predictors between A-FMR and V-FMR. Our results suggest that different treatment strategies need to be considered between A-FMR and V-FMR. The Kaplan-Meier life table Funding Acknowledgement Type of funding source: None

The impact of moderate Aortic Valve Disease in patients undergoing MitraClip for severe MR.

High surgical risk patients presenting with severe mitral valve regurgitation (MR) and concomitant aortic valve disease are frequently a challenge for the interdisciplinary heart team meeting. If open-heart surgery for severe MR is performed, aortic stenosis (AS) or regurgitation (AR) is corrected during the same procedure if at least moderate severity of AS or AR has been confirmed. In patients with prohibitive surgical risk, optimal management strategies in the light of available transcatheter interventions still needs to be established. In this retrospective single center study, we aimed to investigate the impact of coincident moderate aortic valve disease on the outcome of patients undergoing MitraClip for severe MR. In 286 MitraClip procedures performed in our institution, 21 patients (7,3%) were identified to suffer from concomitant moderate AS and 28 patients had moderate AR (9,8%). Patients with AS were found to have a higher incidence of >moderate MR following the procedure when compared to patients without aortic valve disease (14,3% vs. 8,9%, p = 0.001). No differences between the groups were found regarding a combined endpoint of all cause deaths and heart failure hospitalizations after 1 year follow up (no aortic-valve disease vs. moderate AS: 19% vs 18%; p = 0,881 and no aortic valve disease vs moderate AR: 19% vs. 25%; p = 0.477). However, mortality was significantly higher in patients with coincidental moderate AR (3.8% patients without aortic valve disease, 5% in patients with AS, 17,9% in patients with AR; p = 0.006). According to our analysis coincidental Aortic valve stenosis may be associated with worse technical results regarding residual MR after MitraClip. Although our results regarding a combined endpoint of all-cause mortality and heart failure hospitalizations within one year of follow up were comparable between the groups, patients with moderate AR had significantly higher mortality rates. Due to the limited number of patients, our study is only hypothesis generating. Larger trials are necessary to confirm our result.

Late onset of atrial fibrillation in patients undergoing mitral valve repair for type II dysfunction

BackgroundThere are few reports regarding factors related to late-onset of atrial fibrillation (Af) after mitral valve repair, which can compromise long-term outcome. We analyzed the risk factors for late Af after mitral valve repair. Methods and resultsBetween 2001 and 2015, 318 patients (214 males; median, 57.7 years) underwent mitral valve repair for degenerative mitral insufficiency (MI) without concomitant aortic valve disease in our institute. Patients with a history of paroxysmal or chronic Af preoperatively were excluded. Serial follow-up echocardiography was used to evaluate cardiac function. The follow-up rate was 99%. The mean follow-up period was 6.0±4.1 years. There was no early death and there were nine late deaths. A total of 29 (9.1%) patients developed late Af. Freedom from late Af was 94.0% at 5 years and 82.9% at 10 years. Small ring annuloplasty, left atrial diameter, and pressure half time, which were measured at follow-up echocardiography, were considered as significant (p<0.05) risk factors for late Af. Patients who had late Af developed recurrent MI more frequently than those who did not (p<0.05). ConclusionsRemodeling of the atrium caused by functional mitral stenosis is important for late Af, as well as recurrent MI. To prevent these problems, avoiding selection of a small ring at primary mitral repair for type II dysfunction and durable repair are required.

Midterm Outcomes and Quality of Life of Aortic Root Replacement: Mechanical vs Biological Conduits

Background Aortic root replacement is a complex operation for severe aortic root pathology such as aneurysms and dissections with concomitant aortic valve disease. Biological and mechanical valve conduits are available. Methods Early and midterm results were analyzed in patients undergoing aortic root replacement. From January 1, 1998, to May 31, 2007, 144 patients underwent aortic root replacement (Bentall procedures) with either a mechanical (n = 51) or a biological (n = 93) valve conduit. Cox proportional hazard analysis was used to determine whether valve type was an independent predictor of all-cause mortality, and analysis of covariance was used to compare general and disease-specific health-related quality-of-life scores. Results Operative mortality was 2.1%. Median follow-up time was 40 months; 1- and 5-year survival rates for the mechanical group were 96.0% and 89.0%, respectively, vs 93.0% and 84.0% for the biological group. Valve type was not predictive of all-cause mortality, and valve-related complications were not significantly different between groups. At follow-up, 31.5% of patients in the biological group were on anticoagulant. General and disease-specific health-related quality-of-life scores were not significantly different between groups. Conclusions Aortic root replacement with either mechanical or biological valved conduits is a safe procedure. Morbidity, mortality, and adverse quality of life were not associated with the type of valve conduit. Further studies are required to assess long-term durability of biological valve conduits used for aortic root replacement.

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,

A Simple Score to Assess Mortality Risk in Patients Waiting for Coronary Artery Bypass Grafting

Independent risk factors for death in patients waiting for elective coronary artery bypass surgery have previously been identified. A prioritization where these factors are considered may potentially reduce waiting list mortality. A simple score based on the risk factors was constructed and validated. A scoring system based on risk factors in 5,864 consecutive patients operated from 1995 to 1999 was constructed. The following factors were included in the score: unstable angina (3 points [p]), left main stenosis (2p), concomitant aortic valve disease (2p), operative risk (0-2p), left ventricular ejection fraction (0-2p), and male gender (1p). The score was retrospectively validated in 5,167 new patients operated from 1999 to 2003. Based on the sum of risk score points, the patients were divided into three risk groups: low risk (0-2p), intermediate risk (3-5p) and high risk (> or = 6p). The risk groups were related to waiting list mortality and clinical priority (imperative, urgent, and routine). Median waiting time was 33 days. Forty-two patients (0.8%) died while waiting for surgery (5.2 deaths/100 waiting years). Of the patients, 2,406 (47%) were low risk, 1,990 (38%) intermediate risk, and 771 (15%) high risk. Mortality incidence in the high-risk group was fivefold higher than in the intermediate group and 25-fold higher than in the low-risk group (32, 7, and 1.3 deaths/100 waiting years, respectively, p < 0.001 between all groups). Twenty-three percent of the patients in the high-risk group had not been given imperative clinical priority. The score system identifies patients with increased risk of death while waiting for coronary artery bypass grafting. The score may be used to facilitate and improve the prioritization process.

Mortality on the waiting list for coronary artery bypass grafting: incidence and risk factors

Background Insufficient capacity for coronary artery bypass grafting results in waiting times before operation, prioritization of patients and, ultimately, death on the waiting list. We aimed to calculate waiting list mortality and to identify risk factors for death on the waiting list. Methods The study included 5,864 consecutive patients accepted for elective coronary artery bypass grafting (78% male; mean age, 66 ± 9 years). The patients were categorized at acceptance into three priority groups: imperative (39%), urgent (36%), or routine (25%). Waiting list mortality was calculated and compared between groups, and risk factors were identified by Poisson regression. Results Median waiting time for the whole population was 55 days. Seventy-seven patients (1.3%) died, corresponding to a mortality rate of 5.8 deaths per 100 patient-years. The mortality rate per 100 patient-years was highest for those in the imperative group, 15.1 deaths, compared with 5.3 deaths in the urgent group and 3.2 in the routine group ( p < 0.001). Independent risk factors were male sex ( p = 0.032), Cleveland Clinic risk score ( p = 0.005), impaired left ventricular ejection fraction ( p = 0.007), unstable angina pectoris ( p = 0.001), concomitant aortic valve disease ( p = 0.002), priority group ( p < 0.001), and time after acceptance ( p = 0.019). The mortality risk increased with time after acceptance by 11% a month. Conclusions Long waiting lists for coronary artery bypass grafting are associated with considerable mortality. The risk of death increases significantly with waiting time. Sex, unstable angina, perioperative risk, impaired left ventricular function, and concomitant aortic valve disease are independent risk factors and should be considered at triage.

Invited commentary

Invited commentary

Diagnosis and differential therapy of mitral stenosis

Clinical symptoms and diagnostic findings in patients with mitral stenosis are usually determined by the extent of the stenosis. Compared to a normal mitral valve area (MVA) of > 4 cm2, MVA in patients with severe mitral stenosis is usually reduced to < 1.5 cm2. In older patients symptoms are frequently influenced by concomitant diseases (e.g. atrial fibrillation, arterial hypertension or lung disease). An important diagnostic element besides anamnesis, auscultation, ECG and chest X-ray is echocardiography, which is required in order to measure non-invasively and reliably the mitral valve gradient (MVG), the MVA and morphologic changes to the valves, as well as concomitant valvular disease, ventricular functions and, where appropriate, left-atrial thrombi. In addition to the surgical treatment of patients with severe mitral stenosis, which has been an established procedure for 50 years, percutaneous balloon mitral valvuloplasty (MVP) has recently established itself as an alternative option. At the current time, the Inoue technique seems to display the most advantages. Following transseptal puncture, the Inoue balloon is guided transvenously into the left atrium and then into the left ventricle using a special support wire. The balloon is short and soft. Its special unfolding character enables it to be placed securely in the mitral valve without any risk of ventricular perforation (Figure 1). As with surgical commissurotomy, balloon valvuloplasty leads to a separation of fused commissures. This results in a significant reduction of MVG, accompanied by an increase in the MVA (Figure 2). The results and success of MVP are influenced by the morphology of the valves and the changes to the subvalvular apparatus. In randomized studies, the results of surgical commissurotomy were comparable with those of balloon mitral valvulotomy. In our hospital, an increase in MVA from 1.0 to 1.8 cm2 could be achieved in 899 patients (mean age 56 +/- 3 years). In younger patients with less significantly changed valves, the results were correspondingly more favorable than in older patients (Figure 3). Provided valve morphology is suitable, a relapse following previous surgical commissurotomy is not a contraindication for MVP. The MVP complication rate is very low in skilled hands: mortality is below 1%; mitral insufficiency occurs in 3 to 10% of interventions; we observed a severe mitral insufficiency in 5% of our patient group. Thromboembolic complications may be prevented after exclusion of atrial thrombi by transesophageal echocardiography. The occurrence of a hemodynamically significant atrial septum defect is a very rare event. The mid-term results (5 to 10 years) and the low restenosis rate following MVP in patients with suitable valves are comparable with those of surgical commissurotomy. In older patients with considerably changed, calcified and fibrotic valves, restenosis may be expected within 1 to 5 years. In these patients MVP represents no more than a palliative intervention in order to prolong the point of surgery, for example in patients where a concomitant aortic valve disease in itself is not yet an indication for surgery. Special indications are to be found in young patients with severe mitral stenosis yet few symptoms, in pregnant females and in emergency situations, as well as in patients with Grade II mitral stenosis with intermittent atrial fibrillation. Catheter therapy is much less invasive than surgery. In case of failure the patient still has the option of surgical therapy. Patients with morphologically significantly altered valves usually receive a valve replacement since an unsuccessful reconstruction would lead to a second operation within a very short time interval. Contraindications for MVP are thrombi in the left atrium, a previously existing > Grade II mitral regurgitation and marked, degenerative destruction of the subvalvular apparatus or extensive calcification of the valves. MVP thus represents a significant addi

Repair of nonsevere rheumatic aortic valve disease during other valvular procedures: Is it safe?

Objective: To investigate the long-term performance of aortic valve repair, we analyzed the results obtained in a 22-year period in patients who underwent repair of nonsevere rheumatic aortic valve disease during other valvular procedures. Methods: Fifty-three patients (mean 40 ± 11.6 years of age) with predominant rheumatic mitral valve disease had concomitant aortic valve disease in association with serious tricuspid valve disease in 25 of them. Preoperatively, aortic valve disease was considered moderate in 47.2% of the patients and mild in 52.8%. All patients underwent reparative techniques of the aortic valve (free edge unrolling, 44; subcommissural annuloplasty, 40; commissurotomy, 36) at the time of mitral or mitrotricuspid valve surgery. The completeness of follow-up during the closing interval was 100%, with a mean follow-up of 18.8 years (range 8 to 22.5 years). Results: Hospital mortality rate was 7.5%. Of 49 surviving patients, 26 (53.1%) died during late follow-up. The actuarial survival curve including hospital mortality was 35.4% ± 8.7% at 22 years. For patients who underwent mitral and aortic valve surgery, the actuarial survival curve at 22 years was 32.3% ± 13%, whereas for patients who had a triple-valve operation the survival was 37.0% ± 10.1% ( p = 0.07). Twenty-five patients underwent an aortic prosthetic valve replacement. Actuarial free from aortic structural deterioration and valve-related complications at 22 years was 25.3% ± 9.3% and 12.7% ± 4.8%, respectively. Conclusions: Long-term functional results of reparative procedures of nonsevere aortic valve disease in patients with predominant rheumatic mitral valve disease have been inadequate at 22 years of follow-up. According to these data, conservative operations for rheumatic aortic valve disease do not seem appropriate. (J Thorac Cardiovasc Surg 1998;115:1130-5)