Aortic Valve-sparing Operations Research Articles

The usefulness of the genetic panel in the classification and refinement of diagnostic accuracy of Mexican patients with Marfan syndrome and other connective tissue disorders.

Marfan syndrome (MFS) is a multisystem genetic disorder with over 3000 mutations described in the fibrillin 1 (FBN1) gene. Like MFS, other connective tissue disorders also require a deeper understanding of the phenotype-genotype relationship due to the complexity of the clinical presentation, where diagnostic criteria often overlap. Our objective was to identify mutations in patients with connective tissue disorders using a genetic multipanel and to analyze the genotype-phenotype associations in a cohort of Mexican patients. We recruited 136 patients with MFS and related syndromes from the National Institute of Cardiology. Mutations were identified using next-generation sequencing (NGS). To examine the correlation between mutation severity and severe cardiovascular conditions, we focused on patients who had undergone Bentall-de Bono surgery or aortic valve repair. The genetic data obtained allowed us to reclassify the initial clinical diagnosis across various types of connective tissue disorders. The transforming growth factor beta receptor 2 (TGFBR2) rs79375991 mutation was found in 10 out of 16 (63%) Loeys-Dietz patients. We observed a high prevalence (65%) of more severe mutations, such as frameshift indels and stop codons, among patients requiring invasive treatments like aortic valve-sparing surgery, Bentall and de Bono procedures, or aortic valve replacement due to severe cardiovascular injury. Although our study did not achieve precise phenotype-genotype correlations, it underscores the importance of a multigenetic panel evaluation. This could pave the way for a more comprehensive diagnostic approach and inform medical and surgical treatment decision-making.

Functional interaction of aortic valve and ascending aorta in patients after valve-sparing procedures

Pressure recovery (PR) is essential part of the post stenotic fluid mechanics and depends on the ratio of EOA/AA, the effective aortic valve orifice area (EOA) and aortic cross-sectional area (AA). In patients with advanced ascending aortic aneurysm and mildly diseased aortic valves, the effect of AA on pressure recovery and corresponding functional aortic valve opening area (ELCO) was evaluated before and after valve-sparing surgery (Dacron graft implantation). 66 Patients with ascending aortic aneurysm (mean aortic diameter 57 +/− 10 mm) and aortic valve-sparing surgery (32 reimplantation technique (David), 34 remodeling technique (Yacoub)) were routinely investigated by Doppler echocardiography. Dacron graft with a diameter between 26 and 34 mm were implanted. EOA was significantly declined after surgery (3.4 +/− 0.8 vs. 2.6 +/− 0.9cm2; p < 0.001). Insertion of Dacron prosthesis resulted in a significant reduction of AA (26.7 +/− 10.2 vs. 6.8 +/− 1.1cm2; p < 0.001) with increased ratio of EOA/AA (0.14 +/− 0.05 vs. 0.40 +/− 0.1; p < 0.001) and pressure recovery index (PRI; 0.24 +/− 0.08 vs. 0.44 +/− 0.06; p < 0.0001). Despite reduction of EOA, ELCO (= EOA corrected for PR) increased from 4.0 +/− 1.1 to 5.0 +/− 3.1cm2 (p < 0.01) with reduction in transvalvular LV stroke work (1005 +/− 814 to 351 +/− 407 mmHg × ml, p < 0.001) after surgery. These effects were significantly better in patients with Yacoub technique than with the David operation. The hemodynamic findings demonstrate a valve-vessel interaction almost entirely caused by a marked reduction in the ascending AA with significant PR gain. The greater hemodynamic benefit of the Yacoub technique due to higher EOA values compared to the David technique was evident and may be of clinical relevance.

Aortic valve-sparing operations: my perspectives

Aortic valve-sparing operations: my perspectives

Cardiovascular Events After Aortic Root Repair in Patients With Marfan Syndrome

BackgroundThe usefulness of aortic valve sparing operations to treat aortic root aneurysm in patients with Marfan syndrome (MS) remains controversial. ObjectivesThe purpose of this study was to evaluate the occurrence of cardiovascular events in patients with MS who have undergone valve-preserving aortic root replacement. MethodsPatients with MS who had aortic valve sparing operations (reimplantation of the aortic valve or remodeling of the aortic root) from 1988 through 2019 were followed prospectively for a median of 14 years. Pertinent data from clinical, echocardiographic, computed tomography, and magnetic resonance images of the aorta were collected and analyzed. ResultsThere were 189 patients whose mean age was 36 years, and 67% were men. Ten patients presented with acute type A dissection and 29 had mitral regurgitation. There were 52 patients at risk at 20 years. Mortality rate at 20 years was 21.5% (95% CI: 14.7%-30.8%); advancing age and preoperative aortic dissections were associated with increased risk of death by multivariable analysis. At 20 years, the cumulative incidence of moderate or severe aortic insufficiency was 14.5% (95% CI: 9.5%-22.0%), reoperation on the aortic valve was 7.5% (95% CI: 3.9%-14.7%), and new distal aortic dissections was 19.9% (95% CI: 13.9%-28.5%). Remodeling of aortic root was associated with greater risk of developing aortic insufficiency and aortic valve reoperation than reimplantation of the aortic valve. ConclusionsAortic valve sparing operations provide stable aortic valve function and low rates of valve-related complications during the first 2 decades of follow-up but aortic dissections remain problematic in patients with MS.

Aortic Valve Sparing Operations in the Presence of Aortic Cusp Prolapse

Aortic Valve Sparing Operations in the Presence of Aortic Cusp Prolapse

Multi-modality imaging evaluation and pre-surgical planning for aortic valve-sparing operations in patients with aortic root aneurysm

Multi-modality imaging evaluation and pre-surgical planning for aortic valve-sparing operations in patients with aortic root aneurysm

Multi-modality imaging evaluation and pre-surgical planning for aortic valve-sparing operations in patients with aortic root aneurysm.

Cardiac computed tomography (CT) and magnetic resonance (CMR) supplement echocardiography in the evaluation of those with aortic root and ascending aortic dilation, determining timing for intervention, guiding pre-surgical planning and post-operative surveillance. The dynamic, three-dimensional complexity of the aortic root and how it relates to the base of the left ventricle must be understood in any surgical approach addressing the aneurysmal aortic root. With improved imaging technology and the importance for proper patient counseling, it is no longer acceptable to enter the operating theater without a detailed blue print of what the problem is, and how best to address it. In addition, reliance on surgical expertise alone for intraoperative evaluation and decision making could be suboptimal due to the unloaded condition of the aortic root and the variance of experience of the surgeons to successfully repair the aortic valve. This is exemplified by the selective surgeons and centers who have the ability to tackle these aortic valve and root pathologies, compared to mitral valve repair techniques that have been codified and are generalizable. This review discusses a multimodality imaging approach in the patient with aortic root aneurysm, focusing on the precision added with pre-surgical CT assessment to guide aortic-valve sparing operations. This precision is afforded with a detailed understanding of the anatomy of the aortic root and underlying support, and its accurate evaluation by standard two- and three-dimensional imaging. Furthermore, we describe the evolving ability to predict the location of ventricular components of the atrioventricular conduction axis with further clinical imaging to personalize surgical strategies.

Functional and pathomorphological anatomy of the aortic valve and root for aortic valve sparing surgery in tricuspid and bicuspid aortic valves.

Functional and pathomorphological anatomy of the aortic valve and root for aortic valve sparing surgery in tricuspid and bicuspid aortic valves.

Echocardiography of the root. A practical approach for aortic valve-sparing surgery.

Echocardiography of the root. A practical approach for aortic valve-sparing surgery.

Reimplantation of the aortic valve in patients with tricuspid aortic valve: the Toronto General Hospital experience.

Aortic valve sparing operations were introduced three decades ago but controversy remains regarding its appropriateness, reproducibility and durability. This article describes the long-term outcomes of patients who had reimplantation of the aortic valve. All patients who had reimplantation of a tricuspid aortic valve at Toronto General Hospital from 1989 through 2019 were selected for this study. Patients were followed prospectively with periodical clinical assessments and imaging of the heart and aorta. Four hundred and four patients were identified. The median age was 48.0 [interquartile range (IQR), 35.0-59.0] years and 310 (76.7%) were men. There were 150 patients with Marfan syndrome, 20 with Loeys-Dietz syndrome and 33 with acute or chronic aortic dissections. The median follow-up was 11.7 (IQR, 6.8-17.1) years. There were 55 patients alive and without reoperation at 20 years. The cumulative mortality at 20 years was 26.7% [95% confidence interval (CI): 20.6-34.2%], the cumulative incidence of reoperation on the aortic valve was 7.0% (95% CI: 4.0-12.2%) and the development of moderate or severe aortic insufficiency was 11.8% (95% CI: 8.5-16.5%). We could not identify variables associated with reoperation on the aortic valve or with the development of aortic insufficiency. New distal aortic dissections were common in patients with associated genetic syndromes. Reimplantation of the aortic valve in patients with tricuspid aortic valve provides excellent aortic valve function during the first two decades of follow-up. Distal aortic dissections are relatively common in patients with associated genetic syndromes.

Echocardiography of the aortic root: a practical approach for aortic valve-sparing surgery.

Owing to its excellent long-term results, aortic valve-sparing surgery is increasingly used in patients with aortic regurgitation and/or ascending aortic aneurysm. Moreover, in patients with a bicuspid valve who meet the criteria for replacement of the aortic sinuses or aortic regurgitation surgery, valve-sparing surgery may be considered if performed at a comprehensive valve center (Class 2b indication in both the American and European guidelines). Reconstructive valve surgery aims at restoring a normal aortic valve function and a normal aortic root shape as well. Echocardiography plays a central role in defining abnormal valve morphologies, in quantifying aortic regurgitation and mechanisms, and in appreciating tissue valve quality and surgical results. Therefore, despite the emergence of other tomographic techniques, 2D and 3D echocardiography represents the cornerstone for patient selection and prediction of the probability of a reliable repair. The present review focuses on echocardiographic evaluation to detect aortic valve and aortic root abnormalities, to quantify aortic valve regurgitation, to predict aortic valve reparability, and to assess immediate postoperative results in the operating room. Echocardiographic predictors of successful valve and root repair are presented in a practical way.

Functional and pathomorphological anatomy of the aortic valve and root for aortic valve sparing surgery in tricuspid and bicuspid aortic valves.

The aortic valve (AV) is a three-dimensional structure, with leaflets that are suspended within the functional aortic annulus (FAA). These structures (AV and FAA) are therefore intrinsically connected and disease of just one component can independently lead to AV dysfunction. Hence, AV dysfunction can occur in the setting of entirely normal valve leaflets. However, as these structures are functionally inter-connected, disease of one component can lead to abnormalities of the other over time. Thus, AV dysfunction is often multifactorial. Valve-sparing root procedures require an in-depth understanding of these inter-relationships, and herein we are providing a detailed account of some of the most pertinent anatomical relationships.

Reply: Reimplantation should be the gold standard to treat the regurgitant bicuspid aortic valve.

Reply: Reimplantation should be the gold standard to treat the regurgitant bicuspid aortic valve.

Systematic review and meta-analysis of aortic valve-sparing surgery versus replacement surgery in ascending aortic aneurysms and dissection in patients with Marfan syndrome and other genetic connective tissue disorders.

BackgroundAortic aneurysm and dissection are important causes of morbimortality in patients with Marfan syndrome (MFS) and other connective tissue diseases that affect the cardiovascular tissues. Timely intervention through different surgical techniques improves the prognosis. Both sparing and replacement-type interventions of the aortic valve are used, but selection depends on the condition of the patient at the time of diagnosis, the patient’s emergency condition, surgeon preference and hospital resources. Previous meta-analyses have suggested an advantage with the use of sparing-type interventions, but this finding must be updated and extended to patients with other connective tissue disorders. The objetive of this study is to evaluate the outcomes of valve-sparing root replacement versus aortic root replacement procedures in patients with MFS and similar connective tissue diseases that present with aortic aneurysm or dissection.MethodsA systematic review of cohort studies that evaluated sparing-type (preserving, remodeling, reimplantation, Yacoub, David or Florida Sleeve) or replacement-type (repair, Bentall, Button-Bentall, composite valve graft or Cabrol) procedures in patients with Marfan, Loeys-Dietz, Beals-Hecht or Ehlers-Danlos syndromes was done. Studies were retrieved from the SCOPUS, MEDLINE, CINAHL, EMBASE and LILACS electronic databases up to January 2020 without language restrictions. Only studies that directly compared sparing- versus replacement-type procedures were included in the meta-analysis.ResultsA total of 33 studies (n=1,807 subjects) reported sparing-type surgical interventions and 26 studies (n=2,218 subjects) reported replacement-type surgical interventions. Pooled rates of endocarditis, thromboembolism and aneurysm were higher in replacement-type surgical intervention studies. Sixteen studies were included in the meta-analysis. Sparing-type interventions were associated with a reduced risk of endocarditis (RR =0.13, 95% CI: 0.03–0.61); however, replacement-type interventions favored freedom from valve reoperation (RR =2.39, 95% CI: 1.24–4.60). All studies were at low risk of bias.ConclusionsThe choice of the best surgical technique is dependent on the type of disease (MFS or other connective tissue diseases) as well as the accompanying aortic and cardiovascular damage, since these key factors are heterogeneous. Although the results of this meta-analysis tend to show some advantages for one type of surgical intervention over the other and viceversa, the surgeon can only make the best decision during the surgical act.

Valve-sparing aortic root replacement in adult patients with congenital heart disease.

Aortic root dilatation is frequently observed in patients with congenital heart defects (CHD), but has received little attention in terms of developing a best practice approach for treatment. In this study, we analysed our experience with aortic valve-sparing root replacement in patients following previous operations to repair CHD. In this study, we included 7 patients with a history of previous surgery for CHD who underwent aortic valve-sparing operations. The underlying initial defects were tetralogy of Fallot (n = 3), transposition of great arteries (n = 2), coarctation of the aorta (n = 1), and pulmonary atresia with ventricle septum defect (n = 1). The patients' age ranged from 20 to 40 years (mean age 31 ± 6 years). David reimplantation was performed in 6 patients and a Yacoub remodelling procedure was performed in 1 patient. Four patients underwent simultaneous pulmonary valve replacement. The mean interval between the corrective procedure for CHD and the aortic valve-sparing surgery was 26 ± 3 years. There was no operative or late mortality. The patient with transposition of great arteries following an arterial switch operation was re-operated 25 months after the valve-sparing procedure due to severe aortic regurgitation. In all other patients, the aortic valve regurgitation was mild or negligible at the latest follow-up (mean 8.7 years, range 2.1-15.1 years). Valve-sparing aortic root replacement resulted in good aortic valve function during the first decade of observation in 6 of 7 patients. This approach can offer a viable alternative to root replacement with mechanical or biological prostheses in selected patients following CHD repair.

Normative Aortic Valvar Measurements in Adults Using Cardiac Computed Tomography - A Potential Guide to Further Sophisticate Aortic Valve-Sparing Surgery.

A thorough understanding of the anatomy of the aortic valve is necessary for aortic valve-sparing surgery. Normal valvar dimensions and their relationships in the living heart, however, have yet to be fully investigated in a 3-dimensional fashion.Methods and Results:In total, 123 consecutive patients (66±12 years, Men 63%) who underwent coronary computed tomographic angiography were enrolled. Mid-diastolic morphology of the aortic roots, including height of the interleaflet triangles, geometric height, free margin length of each leaflet, effective height, and coaptation length were measured using multiplanar reconstruction images. Average height of the interleaflet triangle, geometric height, free margin length, effective height, and the coaptation length were 17.3±1.8, 14.7±1.3, 32.6±3.6, 8.6±1.4, and 3.2±0.8 mm, respectively. The right coronary aortic leaflet displayed the longest free margin length and shortest geometric height. Geometric height, free margin length, and effective height showed positive correlations with aortic root dimensions. Coaptation length, however, remained constant regardless of aortic root dimensions. Diversities, as well as characteristic relationships among each value involving the aortic root, were identified using living-heart datasets. The aortic leaflets demonstrated compensatory elongation along with aortic root dilatation to maintain constant coaptation length. These measurements will serve as the standard value for revealing the underlying mechanism of aortic regurgitation to plan optimal aortic valve-sparing surgery.

Multimodality Imaging of the Anatomy of the Aortic Root.

The aortic root has long been considered an inert unidirectional conduit between the left ventricle and the ascending aorta. In the classical definition, the aortic valve leaflets (similar to what is perceived for the atrioventricular valves) have also been considered inactive structures, and their motion was thought to be entirely passive—just driven by the fluctuations of ventricular–aortic gradients. It was not until the advent of aortic valve–sparing surgery and of transcatheter aortic valve implantation that the interest on the anatomy of the aortic root again took momentum. These new procedures require a systematic and thorough analysis of the fine anatomical details of the components of the so-called aortic valve apparatus. Although holding and dissecting cadaveric heart specimens remains an excellent method to appreciate the complex “three-dimensional” nature of the aortic root, nowadays, echocardiography, computed tomography, and cardiac magnetic resonance provide excellent images of cardiac anatomy both in two- and three-dimensional format. Indeed, modern imaging techniques depict the aortic root as it is properly situated within the thorax in an attitudinally correct cardiac orientation, showing a sort of “dynamic anatomy”, which admirably joins structure and function. Finally, they are extensively used before, during, and after percutaneous structural heart disease interventions. This review focuses on the anatomy of the aortic root as revealed by non-invasive imaging techniques.

Late results of aortic valve repair for isolated severe aortic regurgitation.

The objectives were to analyze the long-term outcomes of tricuspid aortic valve repair for isolated severe aortic regurgitation and the impact of different annuloplasty techniques. The study cohort consists of 127 consecutive patients who received aortic valve repair for isolated severe aortic regurgitation in the tricuspid aortic valve between 1996 and 2019 in our institution. Exclusion criteria were aorta dilatation (≥45mm), connective tissue disease, active endocarditis, type A dissection, and rheumatic disease. Mean age of patients was 55.6±16years, and 80% were male. Median follow-up was 6.4years. Time-to-event analysis was performed, as well as risk of death, reoperation, and aortic regurgitation recurrence. Cusp repair was performed in 117 patients (92%), and annuloplasty was performed in 126 patients (99%) with Cabrol stitch (73%), reimplantation technique (19.7%), or ring annuloplasty (6.3%). There was no hospital mortality. At 10 and 14years, overall survival was 81%±5% and 71%±6%, respectively, and freedom from reoperation was 80%±5% and 73%±6%, respectively. Age and left coronary cusp repair were independent predictors of reoperation. Freedom from recurrent severe aortic regurgitation (>2+) was 73%±5% and 66%±7% at 10 and 12years, respectively. Age, left ventricular end-diastolic diameter, and patch repair were independent predictors of recurrent aortic regurgitation. Type of annuloplasty had no impact on survival or reoperation. Aortic valve repair for isolated severe aortic regurgitation in the tricuspid aortic valve is a safe procedure, and durability at 14years is acceptable. In this study, the annuloplasty technique did not influence repair durability as was found in bicuspid aortic valve repair or aortic valve-sparing surgery. Severity of cusp pathology seems to be the main determinant of repair durability.

Improved Outcomes Following Reimplantation of the Aortic Valve Compared to Remodeling in Marfan Syndrome: A Propensity-matched Study

Objective: We sought to compare perioperative and late outcomes of patients with Marfan syndrome undergoing aortic valve sparing (AVS) root replacement using the reimplantation versus the remodeling technique. Methods: From 1988 to 2018, 194 patients with Marfan syndrome underwent AVS procedures in a single center. Of these, 24 who had remodeling AVS were matched to 44 who had reimplantation (1:2 matching). After matching, baseline characteristics were well balanced including: age, sex, NYHA III/IV, moderate or severe AI, acute aortic dissection, and year of operation. Outcomes included perioperative mortality and complications, as well as late survival, freedom from reoperation on the aortic valve, and probability of developing moderate or severe AI. Results: There was no in-hospital mortality, stroke, sepsis, myocardial infarction, or need for permanent pacemaker. Re-exploration was slightly lower in reimplantation patients (n=1 2% vs n=3 12% p=0.04) ICU length of stay was 2±1 day and similar between groups. Median follow up was 17.5 years (IQR: 11.8 - 20.4). Survival at 20 years was not different following remodeling vs reimplantation (71% vs 80% p=0.36). However, 20-year moderate to severe aortic insufficiency and reoperation were higher in remodeling patients (31% vs 16% p=0.01; and 18% vs 0% p=0.01). Conclusions: AVS operations have excellent long term survival and durability in Marfan syndrome. Reimplantation was associated with less early re-exploration and lower risk of late aortic insufficiency and reintervention at 20 years.

Superior Long-term Survival After Aortic Valve-sparing Root Surgery in Patients with Connective Tissue Disorder

Objective: Most patients with connective tissue disorder present with aortic root dilatation that may require surgical intervention. In many specialized centres, aortic valve-sparing root surgeries have become the preferred surgical procedure to treat aortic root aneurysm in patients with connective tissue disorder. Methods: A total of 313 patients underwent aortic valve-sparing root surgery at our institution between 1997 and August 2019. Out of these, 83 patients had a connective tissue disorder and were included into further analysis. Follow-up protocol included clinical interview using a structured questionnaire and long-term echocardiographic follow-up. Time-to-event analyses were calculated using the Kaplan-Meier method. Primary endpoint was actual freedom from aortic valve (AV) re-intervention, secondary endpoints were survival and freedom from major adverse cardiac and cerebrovascular events (MACCE). Results: Mean age of the study cohort was 35.4 ± 12.4 years and 60% were male. Marfan syndrome was present in 95.2% and Loeys-Dietz syndrome in 4.8% of the patients. Reimplantation technique of the aortic valve was performed in 72 and remodeling technique of the aortic root in 11 patients. In-hospital and 30-day mortality was 0%. Mean follow-up was 69.6 ± 51.1 (range: 2 - 185 months). Overall survival was 92.1%, 77.5%, and 77.5% at 5, 10 and 15 years. Freedom from AV re-intervention at 5, 10 and 15 years was 92.8%, 78.5 and 78.5%, respectively. No further MACCE (e.g. endocarditis, stroke) occurred in this study cohort during follow-up. Conclusions: In specialized heart centers, aortic valve-sparing root surgery is a safe procedure with very low in-hospital mortality and favorable long-term results in patients with connective tissue disorder.