Surgical Management of Congenital Aortic and Truncal Valve Disease: A Comprehensive Review.

Commentary: Ozaki valve reconstruction in children: Is it still a valve replacement?

Commentary: Standardized aortic valve repair in pediatric patients

Congenital aortic and truncal valve reconstruction using the Ozaki technique: Short-term clinical results

Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease

Congenital aortic valve (AoV) disease has limited treatment options in growing children. Different initial strategies, AoV repair/surgical valvuloplasty (SAV), AoV replacement (AVR), and Ross procedure were compared to elucidate the current best strategy. All paediatric patients undergoing different initial AoV surgeries from 1976 to 2024 were included. Factors including prior balloon dilation (balloon valvuloplasty [BAV]), valve morphology, and initial disease (stenosis/regurgitation/mixed) were analysed. Survival and incidence of AoV reoperation/≥moderate AoV insufficiency/stenosis were evaluated. A total of 323 patients underwent 142 SAV/33 AVR/137 Ross at median age/weight of 5.7 years [interquartile range, 0.5-12.6]/19.9 kg [6.9-48.8]. Surgical valvuloplasty group was the youngest (P < .01). Thirty-day mortality was 2.5% (8/323) without group differences (P = .15). Median follow-up was 9.6 years [2.7-17.8] with 10-/20-year survival rates of 90.4% [86.1-93.4]/87.1% [81.4-91.1] without group differences. Twenty-year cumulative incidences of AoV reoperations were higher after SAV: 78.4% [70.8-86.9] vs 24.6% [11.7-51.7] after AVR and 15% [6.8-33.1] after Ross, P < .01. Survivors with their native AoV at 20 years (n = 48, 14.8%) were younger at initial surgery (P < .01), had predominantly AoV stenosis (91.7%), and had fewer BAVs (P < .01). Cox regression demonstrated 7.8-fold higher mortality hazard after AVR (P < .01) and a lower reoperation hazard after AVR and Ross (HR 0.1 [95% CI, 0.1-0.5], P < .01 and HR 0.1 [95% CI, 0.1-0.3], P < .01) compared to SAV. All initial AoV surgeries brought excellent early/long-term survival. Aortic valve reoperations occur most frequently after SAV. In case of favourable AoV anatomy, early SAV can preserve the native valve. Ross procedure identified as safe and durable.

Background: Congenital aortic valve (AV) disease is viewed in 2% of the population and in 20 out of 1000 neonates are presented with bicuspid configuration. Treatment of the congenital AV diseases requires multiple interventions. The ultimate goal is to provide adequate close to physiological left ventricle outflow and minimization of regurgitation. The optimal operative management of AV disease in children and young adults remains controversial. AV neocuspidization with glutaraldehyde‐treated autologous pericardium may be a satisfactory alternative in pediatric cardiac surgery.Objectives: The data of the AVNeo procedure results enable a comparative analysis of various AV reconstructive approaches in the pediatric cohort. In this article, we present the comparative study of various techniques in pediatric AV surgery, including the immediate and midterm outcomes of AVNeo, commissurotomy with aortic leaflet augmentation using pericardial patch (“free style” technique), and Ross procedure.Study Design: We conducted a retrospective analysis of the early and midterm results of surgical AV disease treatment of 44 children in 5 cardiac surgery centers from 2014 to 2022. The patients were divided into 3 groups: group I: glutaraldehyde‐treated autologous pericardium AVNeo (n = 12), group II: “free style” technique (n = 10), and group III: Ross procedure (n = 22). We described the immediate and midterm outcomes. The study design was approved by the ethics committees of the participating centers.Results: A total of 44 children after surgical treatment of the AV disease in 5 cardiac surgery centers were included in the study and were grouped as group I: AVNeo (n = 12), group II: “free style” technique (n = 10), and group III: Ross procedure (n = 22). The median follow‐up period was 57 ± 23.8 months. In‐hospital mortality was 4.5% (n = 2). In the midterm follow‐up, the peak pressure gradient was 18.45 ± 4.63 mmHg for group I and 8.9 ± 1.6 mmHg for group III (p = 0.00001), respectively. The cumulative freedom from reoperations was 100%, 30%, and 95.2% for 40 months of follow‐up and 100%, 30%, and 83.4% for 70 months of follow‐up in I, II, and III groups, respectively. No cases of midterm mortality were detected in all groups.Conclusion: The AV neocuspidization with glutaraldehyde‐treated autologous pericardium has an enormous advantage in pediatric AV surgery, significantly surpassing the freedom from resurgery of repair procedures and having comparable results with the Ross procedure in the midterm follow‐up.

BackgroundIn response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital aortic and pulmonary valve disease....

BackgroundTo assess the current state of patient information and decision-making in congenital aortic and pulmonary valve disease, we conducted a survey among patients, parents and physicians.MethodsA questionnaire was sent by...

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

GATA5 and Endothelial Nitric Oxide Synthase Expression in the Ascending Aorta Is Related to Aortic Size and Valve Morphology

Introduction: Rheumatic Heart Disease (RHD) is a non suppurative sequelae of group A beta haemolytic streptococci, resulting from inadequately treated streptococcal sore throat or scarlet fever and leading to valvular heart disease. Rheumatic heart disease is a major cause of morbidity and mortality in younger population in developing countries. The present study was done at a tertiary care medical college hospital with the objective of establishing prevalence and involvement of different valve patterns by Echocardiography (ECHO). Aim: To analyse the valvular pattern of RHD over a period of four years in a tertiary care centre and highlight the importance of ECHO in the definitive diagnosis of RHD, and to know the continuing burden of RHD. Materials and Methods: This was a hospital based retrospective observational study conducted at Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India. A total of 518 cases of RHD were selected as a study population among the ECHO performed between January 2016 and January 2020 after an exclusion criterion of degenerative mitral and aortic valve disease, congenital aortic and mitral valve disease, myxomatous mitral valve disease, trivial and functional regurgitation. Analysis of valvular pattern was performed. Data analysis was done by tables, charts, percentages and ratio. Results: A total of 518 patients were diagnosed to have RHD by 2-Dimensional ECHO. Among them 276 (53%) were females and 242 (47%) were males. The average age was 41.9 years. The most common valve involved independently and in combined lesions was the mitral valve. Of the study population, 446 patients had Mitral Stenosis (MS) and 393 had Mitral Regurgitation (MR). Aortic Stenosis (AS) was found among 111 patients and 304 patients had Aortic Regurgitation (AR). Tricuspid Stenosis (TS) (organic) was found in seven cases. Multiple valves were involved in 204 cases. Among them 104 of the cases had MS, MR and AR, 69 cases had MS, MR, AS and AR, 21 cases had MS, AS and AR, seven cases had MR, AS and AR and three cases had MS, AS, AR and TS. Though aortic valve was involved in multi valvular lesions, significant AR (moderate and severe) was seen in 109 patients and significant AS (moderate and severe) was seen in 67 patients. Conclusion: RHD continues to be a major burden to population in developing countries. In the present study, various patterns of valvular involvement were noted. Drastic measures are to be taken primary and secondary prevention of RHD.

Congenital aortic valve stenosis (AVS) is one of the most common valve anomalies and accounts for 3%-6% of cardiac malformations. As congenital AVS is often progressive, many patients, both children and adults, require transcatheter or surgical intervention throughout their lives. While the mechanisms of degenerative aortic valve disease in the adult population are partially described, the pathophysiology of adult AVS is different from congenital AVS in children as epigenetic and environmental risk factors play a significant role in manifestations of aortic valve disease in adults. Despite increased understanding of genetic basis of congenital aortic valve disease such as bicuspid aortic valve, the etiology and underlying mechanisms of congenital AVS in infants and children remain unknown. Herein, we review the pathophysiology of congenitally stenotic aortic valves and their natural history and disease course along with current management strategies. With the rapid expansion of knowledge of genetic origins of congenital heart defects, we also summarize the literature on the genetic contributors to congenital AVS. Further, this increased molecular understanding has led to the expansion of animal models with congenital aortic valve anomalies. Finally, we discuss the potential to develop novel therapeutics for congenital AVS that expand on integration of these molecular and genetic advances.

Congenital aortic valve disease manifests itself either in the course of its natural history or as a consequence of an intervention (balloon dilatation or surgery). In infancy, a congenital aortic valve presents with stenosis, in childhood and adolescence as slowly evolving regurgitation after primary intervention/surgery and in late adulthood, they re-emerge as stenosis due to natural degeneration and calcification of the fused leaflets. The surgical approach to a congenital aortic valve disease differs depending on whether it is a malformed or a normally laid down (tri-sinusoidal tricuspid) valve; it also differs depending on the type of deformity, dysfunction and valve tissue presenting at surgery. Acutely regurgitant aortic valve in a neonate or an infant after balloon dilatation of congenital aortic stenosis is an infrequently occurring difficult problem with few available options. This video presentation demonstrates a xenopericardial patch repair of the torn fused leaflet (fusion between the right and the noncoronary cusp) of a congenitally stenotic valve, followed by height augmentation of all the three leaflets. Because of the relative hypoplasia of the aortic annulus and the ascending aorta, the aortic root and proximal ascending aorta were enlarged by an oblong xenopericardial patch. The following text includes additional technical issues involved in congenital aortic valve repair. A brief summary of literature is presented.

In order to investigate the clinicopathological characteristics of aortic valve disease in children, all the native surgically excised aortic valves obtained between January 2003 and December 2005 were studied macroscopically and microscopically. The patients’ medical records were reviewed and the clinical information was extracted. According to preoperative echocardiography, intraopera- tive assessment, and postoperative pathology, combined with clinical symptoms and signs, aortic valve diseases were divided into three categories: aortic stenosis (AS), aortic insufficiency (AI), and aortic stenosis with insufficiency (AS-AI). The etiology was determined according to the macro- scopic, microscopic and clinical findings. The results showed that among 70 aortic valves, patient age ranged from 6 to 18 years, with a mean of 15.4 years, and there were 56 boys and 14 girls (male: fe- male=4:1). Forty-four children only had pure aortic valve disease, and the other 26 children had aor- tic valve disease associated with other heart valve diseases. There were 5 cases of AS (7.14%), 60 cases of AI (85.71%) and 5 cases of AS-AI (7.14%). The causes were congenital aortic valve mal- formation (32 cases, 45.71%), rheumatic disease (28 cases, 40%), infective endocarditis (7 cases, 10%), Marfan syndrome (2 cases, 2.86%), and undetermined (1 case, 1.43%). It was concluded that the common causes of aortic valve disease in order of frequency in children were congenital aortic valve malformation, rheumatic disease, infective endocarditis, and Marfan syndrome. AI was more common in children with aortic valve disease. Compared with adult patients, congenital bicuspid aor- tic valve in children was often AI. Histologically, the leaflets of congenital bicuspid aortic valve were mainly myxomatous, fibrosis and calcification less seen. AI was frequently found in rheumatic dis- ease, mostly associated with other heart valve diseases. Macroscopic and microscopic examinations together with clinical information, echocardiographic findings a

Introduction: Mixed aortic valve disease (MAVD) is defined by simultaneous occurrence of aortic stenosis (AS) and aortic regurgitation (AR). In our review, we focus on treatment options for nonelderly MAVD patients (age<55 years), who suffer from congenital aortic valve disease (unicuspid/bicuspid aortic valves). Areas covered: A systematic literature search was performed on PubMed and Embase databases using the following terms: mixed aortic valve disease, aortic stenosis/regurgitation, bicuspid/unicuspid aortic valve, mechanical/bioprosthetic aortic valve replacement, TAVR, Ross procedure. After preselection of title and abstracts, two authors (M.S. and E.G.) assessed the methodological quality of the full-text articles prior to final inclusion in the manuscript. Expert opinion: Currently, no ideal valvular substitutes are available in the treatment of nonelderly MAVD patients. Mechanical valves are associated with a reduced life expectancy due to a combination of prothesis-associated factors, mainly thrombotic and bleeding complications. Bioprostheses degenerate in the second decade and re-operations are inevitable, which also limit life expectancy. Long-term outcomes and durability of transcatheter aortic valve replacement are currently unknown. Finally, only Ross procedure is a therapeutic option with excellent long-term outcomes comparable to the healthy population. However, the Ross procedure has some important drawbacks and should therefore be only performed in expert centers and in well-selected patients.