A modified David V: Technique and outcomes from a large single centre experience.

Success in Valve-Sparing Aortic Root Replacement: Judgment and Skill

Marfan syndrome (MFS) is a heritable disorder of the connective tissue with a prevalence of ≈1 in 3000 to 5000 individuals. The condition is inherited in an autosomal dominant manner with complete penetrance but demonstrates variable expression with significant intra- and interfamilial variation. Approximately 25% of patients do not have a family history and represent sporadic, new mutations for the condition. The cardinal features of MFS involve the cardiovascular, ocular, and skeletal systems. The most life-threatening complication of MFS is thoracic aortic aneurysms leading to aortic dissection, rupture, or both. This article focuses on medical and surgical treatment of aortic disease in patients with MFS and addresses the treatment of aortic disease in children and pregnant women with the condition. The most common cardiovascular complication in patients with MFS is progressive aortic root enlargement initially occurring at the sinuses of Valsalva. Ascending aortic aneurysm can precipitate acute type A aortic dissection, aortic rupture, aortic regurgitation (AR), or all 3, and these complications were the primary cause of death before the advent of successful preventive therapies. Treatment of the aorta consists of regular imaging to detect and quantify progression of aortic dilation, β-adrenergic receptor antagonist therapy, and prophylactic aortic repair when the dilation reaches a sufficient size to threaten dissection or cause AR. Before the era of open-heart surgery, the majority of patients with MFS died prematurely of rupture of the aorta, with an average life expectancy of 45 years.1 The success of current medical and surgical treatment of aortic disease in MFS has substantially improved the average life expectancy, extending it up to 70 years.2,3 Cardiovascular manifestations in MFS also include valvular disease involving the mitral valve, aortic valve, or both. Mitral valve prolapse is the most prevalent valvular abnormality, affecting 35% to 100% of patients.4 Mitral …

Isolated Bicuspid Aortic Valve Repair With Double Annuloplasty: How I Teach It

Discussion: Session 1—Ascending Aorta

Sparing aortic valve during combined aortic root replacement (ARR) and total aortic arch replacement (TAR) adds surgical complexity; however, the long-term outcomes are unknown. We examine the safety of aortic valve-sparing during these operations. To include patients who were potentially eligible for valve-sparing procedures, aortic stenosis, endocarditis, and previous aortic valve surgery were excluded, leaving 81 patients who underwent ARR and TAR between 2004 and 2021 at 2 major aortic centers. Overall, 34 underwent valve-sparing aortic root replacement (VSRR) and 47 underwent composite valve graft root replacement (CVG). The primary endpoint was uneventful recovery: a composite endpoint describing any patient discharged from the hospital without mortality or any postoperative complications including stroke, re-operation for bleeding, prolonged ventilation, or acute renal failure. Secondary endpoints were long-term survival and cardiovascular reintervention at 12years. VSRR was more frequently performed in younger patients with connective tissue disorder (P = 0.006) and less than moderate aortic insufficiency (P = 0.002). VSRR had longer cross-clamp time (243, [200-286] vs. 216, [181-250] minutes, P = 0.032). In-hospital mortality (VSRR: 5.9% vs CVG: 10.6%, P = 0.693) and uneventful recovery (VSRR: 47.1% vs CVG: 44.7%, P = 1.000) were not different. Multivariable Logistic regression showed that VSRR was not associated with the uneventful recovery (OR 1.165, 95% CI [0.356-3.814], P = 0.801). Twelve-year survival (VSRR: 80.8% [63.1-100.0%] vs. CVG: 66.3% [47.9-91.7%]; P = 0.320) and the incidence of reintervention (VSRR: 39.0% [19.0-59.0%] vs. CVG: 39.0% [16.0-61.0%], P = 0.820) were similar between groups. In appropriately selected patients requiring concomitant ARR and TAR, aortic valve-sparing operation may be performed safely.

Objectives We report the mid-term outcomes of valve-sparing aortic root replacement (VSRR) in a cohort including patients with bicuspid aortic valve (BAV), connective tissue disorder (CTD), aortic dissection (AD), and congenital heart disease (CHD). Design. From 2005 to 2017, 174 patients underwent VSRR with the reimplantation technique. The mean age was 46 ± 14 years. The mean follow-up time was 4.8 ± 2.8 years. The indication for operation was aortic aneurysm for 127 (73%), aortic insufficiency (AI) for 38 (22%), and AD for 9 patients (5%). Preoperatively, 53 patients (31%) had ≥ moderate AI. BAV, CTD (Marfan or Loyes-Dietz), previous Ross procedure, or CHD was present in 57 (33%), 28 (16%), 7 (4%) and 12 patients (7%), respectively. Concomitant aortic valve repair was performed for 103 patients (59%). Results. Thirty-day mortality was zero. Four patients underwent aortic valve replacement (AVR) during follow-up. Kaplan–Meier estimates for survival, freedom from AVR, and freedom from ≥ moderate AI or reoperation were 96, 98, and 97% at 5 years. There was no difference in survival, freedom from AVR, or freedom from ≥ moderate AI or reoperation in patients with and without BAV, CTD, leaflet repair, or preoperative ≥ moderate AI. In Cox regression analysis, BAV, CTD, aortic valve repair, preoperative ≥ moderate AI, or aortic dimension were not risk factors for reoperation or valve dysfunction. Conclusions. Mid-term outcomes of VSRR for patients with diverse indications in terms of survival, reoperation rate, and valve dysfunction rate were excellent in a center with a limited annual volume of VSSR.

Valve-sparing aortic root replacement (VSARR) is an alternative to valve-replacing aortic root replacement (VRARR) with valved-conduits based on recent guidelines for clinical practice. This study investigated outcomes of these two procedures in patients with nonstenotic valves. Between January 7, 2007 and June 30, 2019, 475 patients with aortic root aneurysm without aortic stenosis underwent VSARR (151) or VRARR (324) techniques. Propensity score-matching (PSM) was used to alleviate confounding. Endpoints were 30-day mortality, 8-year survival and reoperation, aortic regurgitation, and valve gradients. PSM created 69 pairs of patients with a mean age 52 ± 13 years (10.1% Marfan syndrome, 34.8% bicuspid aortic valve). There was no statistically significant difference in major perioperative morbidity or 30-day mortality (0% VSARR vs.1.4% VRARR; p = 0.316). Overall survival was significantly higher (p = 0.025) in the VSARR group versus the VRARR group (8-year estimates 100% vs. 88.9%, respectively), while freedom from valve reoperation was similar (p = 0.97, 8-year estimates 90.9% vs. 96.7%, respectively). Freedom from > moderate-severe AR was not significantly different (p = 0.08, 8-year estimates 90.0% VSARR group vs. 100% VRARR), but mean valve gradients at last follow-up were better in the VSARR group (5.9 vs. 13.2 mmHg, p < 0.001). VSARR is a safe operation in patients with aortic root aneurysm and nonstenotic aortic valves in the hands of experienced surgeons. Freedom from reoperation is similar and the mode of aortic valve failure differs between the two groups.

Mid and long-term data regarding the durability of bicuspid aortic valve sparing root replacement is not completely understood. In this study, our institutional experience is reviewed regarding this procedure with special attention to the operative technique. From 2004 to 2019, 1241 patients underwent aortic root replacement and of these 79 patients underwent bicuspid aortic valve sparing root replacement. Early and late outcomes along with echocardiographic data were reviewed. Univariate analysis was used to identify risk factors for mortality. Median age at operation was 43 (inner quartile range: 34.5-50.5) years, with 17% (14) being female. Median follow up time was 4 (inner quartile range: 1-8) years with 359 years of total follow up. Early mortality was 2.5% (2). Overall 10-year freedom from aortic valve intervention was 95.6%. Risk factor analysis did not demonstrate preoperative root diameter, aortic diameter, or aortic insufficiency to be risk factors for mortality. Bicuspid aortic valve sparing root replacement can be performed safely in the setting of aortic root pathology and valve insufficiency. With appropriate selection and excellent surgical technique, long-term durable valve function is observed without the need for repeat valve intervention.

Valve-sparing root replacement in patients with bicuspid versus tricuspid aortic valves

Valve-Sparing Root Replacement in Bicuspid and Tricuspid Aortic Valves: Long-Term Outcomes Into the Second Decade.

Reported outcomes in patients with bicuspid aortic valves (BAVs) undergoing valve-sparing aortic root replacement (VSRR) are scarce. This study aims to evaluate outcomes in patients with BAV using the reimplantation (David) technique. Consecutive adult patients with BAV, aortic root aneurysm, and/or aortic valve insufficiency (AI) undergoing VSRR (reimplantation) were included from 5 centers experienced in reimplantation procedures. Patients were subcategorized into 2 groups with different primary indications for operation: (1) aneurysm, and (2) isolated AI. Exclusion criteria included acute aortic dissection, endocarditis, and valvular-stenosis. In total, 498 patients were included. Mean age was 45.4 years (±11.8 years); median follow-up was 5.4 years (interquartile range, 2.3-8.7 years). Group 1 included aneurysm (n = 144) and group 2 included AI (n = 354). There was 1 in-hospital death. Survival (overall) was 93.4% (95% confidence interval [CI], 92-97%) at 10 years, with no difference between groups (P = .93) observed. Freedom from reintervention at 1 year was 99.1% (95% CI, 99%-100%), at 5 years 95.4% (95% CI, 93%-97%), and at 10 years 89.2% (95% CI, 86%-93%) for patients with aneurysm 100% at 1 year and 95.4% (95% CI, 92%-98%) at 10 years; and for AI 98.9% (95% CI, 98%-99%) at 1 year and 86.4% (95% CI, 83%-91%) at 10 years. Cusp fenestrations (P = .01), prolapse (P = .04), and isolated AI (0.03) were associated with greater hazard of reintervention. This multicenter study shows excellent results after VSRR reimplantation procedure in patients with BAV and aortic aneurysm and/or valve insufficiency. Isolated AI and cusp abnormalities are associated with greater reintervention rates and may reflect more advanced leaflet disease. The reimplantation technique, when performed in expert centers, appears to be an excellent treatment strategy in BAV.

Left ventricular outflow tract obstructions (LVOTOs) encompass a series of stenotic lesions starting in the anatomic left ventricular outflow tract (LVOT) and stretching to the descending portion of the aortic arch (Figure 1). Obstruction may be subvalvar, valvar, or supravalvar. These obstructions to forward flow may present alone or in concert, as in the frequent association of a bicuspid aortic valve with coarctation of the aorta. All of these lesions impose increased afterload on the left ventricle and, if severe and untreated, result in hypertrophy and eventual dilatation and failure of the left ventricle. LVOTOs are congenital in the vast majority of individuals younger than 50 years in the United States; some variants of subaortic obstruction are the exception. It is imperative to consider all patients with LVOTO at a high risk for developing infective endocarditis, and one should always institute appropriate measures for prophylaxis. The present article is intended as a contemporary review of the causes, manifestations, treatments, and outcomes of LVOTO; it will not address LVOTO in the pediatric population or genetic hypertrophic cardiomyopathy but will focus strictly on congenital malformations in the adult. Figure 1. Artist’s rendering of the LVOTO lesions in sequence as viewed from a superolateral orientation. A, Gradient echo cardiac MR image as viewed from the frontal projection demonstrating flow acceleration at a site of supravalvar aortic stenosis (white arrow) in a patient with Williams syndrome. The black arrow identifies the level of the unrestricted aortic valve. B, Classic radiological signs of coarctation of the aorta: rib notching (white arrows) as seen on a posteroanterior chest x-ray in a patient with coarctation of the aorta. The rib notching is caused by erosion of the inferior rib margins by dilated pulsatile posterior intercostals collateral arteries. The black arrow points to the Figure 3 silhouette that …

Valve-sparing surgery in ascending aortic and root aneurysms combined with aortic regurgitation (AR) is a relevant and actively developing trend. The ways to improve these operations are the development of new devices to simplify and standardize valve-sparing procedures using animal models and testing in experiment. Objective. To review experimental methods of aortic root aneurysm and AR modeling, as well as devices simplifying aortic valve-sparing root replacement, including a new device for positioning of aortic valve (AV) cusps during valve-sparing root replacement with AV reimplantation (David procedure), which we have developed. Materials and methods. The components of the developed device were manufactured by three-dimensional printing with preliminary modeling in the parametric environment of computer-aided design with the open source code FreeCAD 0.20.1. The possibility of David procedure on the isolated swine aortic root in the experiment using the new device was evaluated. Results. During the experiments we performed aortic root replacement with AV reimplantation on the isolated porcine aortic root. We noted that the developed device provides good exposure of the surgical correction zone and reduces the probability of damage to the aortic root structures, especially to the AV cusps, in the process of reimplantation. The device eliminates the necessity to use assistants during the main stage of intervention, which reduces the probability of operator-associated complications. Conclusion. Standardization and simplification of reimplantation of AV cusps into the vascular graft during aortic root replacement with the help of the device developed by us is achieved by performing trial positioning of AV cusps and hydraulic tests at different positions of the cusps, which simplifies the search for the optimal point of coaptation and allows to keep the found best position of the cusps until their anchoring inside the graft. The directions of further research are improvement of the design of the developed device, experimental studies with evaluation of the result of valve-sparing surgery under conditions of pulsating fluid flow, testing of the technology on large laboratory animals.

1. Talha Niaz, MBBS* 2. Jonathan N. Johnson, MD*,† 3. Frank Cetta, MD*,† 4. Timothy M. Olson, MD*,† 5. Donald J. Hagler, MD*,† 1. *Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, and 2. †Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN * Abbreviations: AHA : : American Heart Association BAV : : bicuspid aortic valve TTE : : transthoracic echocardiography Bicuspid aortic valve is the most common congenital heart defect in children, adolescents, and adults. Primary care providers play an important role in screening, referral, and follow-up of these patients and should be aware of the family screening guidelines, sports participation recommendations, and periodic follow-up requirements for adequate surveillance of the complications that arise from bicuspid aortic valve. After reading this article, readers should be able to: 1. Describe the epidemiology and anatomy of bicuspid aortic valve (BAV). 2. Understand the clinical presentation and diagnosis of BAV in infants, children, and adolescents. 3. Identify the various complications of BAV disease. 4. Discuss the management and follow-up requirements for BAV. 5. Analyze the family screening and sports participation guidelines for patients with BAV. Bicuspid aortic valve (BAV) is the most common congenital heart defect in children, adolescents, and adults. (1) It is a heterogeneous disease that affects both the aortic valve and the aorta. It can lead to many complications, including aortic valve stenosis, regurgitation, or endocarditis. (2)(3) It also can lead to dilation of the aorta, predisposing individuals to a significantly higher risk of aortic aneurysm and dissection. (4) Although most individuals with BAV present with these long-term complications during adulthood, a considerable number of patients may also present during childhood and adolescence with early-onset disease; that may require interventions in up to 12% to 15% of the patients. (5)(6) Therefore, patients with BAV require lifelong follow-up and surveillance. BAV has multiple implications in terms of sports participation and family screening, making it an important subject for primary care providers. This article reviews the anatomy, genetics, presentation, diagnosis, …

Surgical treatment of bicuspid aortic valve disease: Knowledge gaps and research perspectives