Congenital Aortic Stenosis Research Articles

Commentary: Sometimes less is more…but more seems more in treatment of neonates and infants with congenital aortic stenosis

Commentary: Sometimes less is more…but more seems more in treatment of neonates and infants with congenital aortic stenosis

P16 Concurrent sacroiliitis and infective endocarditis in a young male with congenital aortic stenosis: a diagnostic challenge and learning points

P16 Concurrent sacroiliitis and infective endocarditis in a young male with congenital aortic stenosis: a diagnostic challenge and learning points

Reduced Sox7 contributes to abnormal valve development resulting in congenital aortic stenosis

Abstract Background Abnormal valve development is the most common congenital heart malformation. During valve remodeling, abnormal changes in valve cell proliferation, apoptosis, and extracellular matrix synthesis affect valve development, resulting in malformations. The molecular mechanisms underlying pathophysiology of aortic stenos is remain unclear. Purpose We would like to explore how Sox7 is involved in aortic valve development. Methods and Results We proved Sox7 was mainly expressed in endocardial and mesenchymal cells of aortic valves by immunofluorescence staining determined by at different stages of mice (E10.5～8 weeks) and human embryos and RNA-seq of E9.5 mouse embryonic hearts. We constructed a conditional Sox7 floxed allele in endothelial cells using an endocardial specific Cre. Echocardiography data showed that Nfat-KO mice had increased blood velocity, pressure gradient across aortic valves, left ventricular mass and dilated aortic roots. Then, HE and Sirius red staining to examine histological changes in aortic valves of Nfat-KO mice were performed, and we observed a remarkable increase in the size and increased staining of fibrotic cells in aortic valves of KO mice. Next, we collected embryonic hearts of Sox7 CNC-specific knockout mice (WNT1-KO) and controls at E16.5. According to HE staining results and echocardiography analysis, there was no difference in aortic valves between WNT1-KO mice and controls. Meanwhile, we collected Nfat-KO mice and control embryos at E11.5 days. HE staining results showed that EndMT process was not impaired. Interestingly, leaflets and cusps of Nfat-KO mice showed significantly augmented proliferation levels, decreased apoptosis levels, and calcium deposition in fibrin layer. A significant decrease in MMP9 levels were observed in both mRNA and protein levels. Co-transfection of Sox7-expressing vector and MMP9 luciferase in MEEC suggested that Sox7 significantly activated MMP9. We used adenovirus to overexpress Sox7 in valvular interstitial cells. Compared with that in control group, the Sox7-overexpression group increased protein level of MMP9 and cleaved caspase-3, and increased Tunel-positive rate and lower Ki-67 positive rate. Finally, we detected expression of Sox7 protein in human fetal aortic valves derived from patients with aortic stenosis using immunofluorescence. The results demonstrated that the number of Sox7 positive mesenchymal cells in human fetal aortic valves of patients was significantly lower than that in control group. Conclusion We demonstrated that targeted inactivation of Sox7 in the endocardium of mice affects valve development and leads to aortic stenosis. Mechanistically, no influence of Sox7 on the EndMT process of the OFT cushion. Moreover, CNC-derived Sox7 was not involved in aortic valve development. Interestingly, we found that Sox7 regulated ECM remodeling through matrix MMP9 and Sox7 had an impact on apoptosis, proliferation, and calcification.

Prognostic Effects of Operation Age for Pediatric Patients with Supravalvar Aortic Stenosis.

The appropriate age for surgical repair of asymptomatic congenital supravalvular aortic stenosis (SVAS) is still unknown. The purpose of this research was to assess the safety and effectiveness of various operation ages when managing SVAS. Consecutive asymptomatic SVAS pediatric patients in the Beijing Fuwai and Yunnan Fuwai hospitals over a period of 18 years were retrospectively analyzed. Patients were classified as follows: age <2.0 years (y) (n = 84), 2.0-5.0 y (n = 72), and >5.0 y (n = 92). The primary safety endpoint was in-hospital death or extracorporeal membrane oxygenation (ECMO) needed. The primary effectiveness outcome was re-operation or restenosis during follow-up. To calculate the hazard ratios (HR), Cox regression with inverse probability of treatment weighted was utilized. At the time of surgery, the median age of the 248 patients that were included was 4 y (interquartile range (IQR): 1.8-6.5). For the primary safety outcome, 7 (8.3%) patients in the age <2.0 y group had in-hospital death or ECMO needed, while no patients in the age 2.0-5.0 y and age>5.0 y groups (p = 0.001). The median follow-up was 25.5 months (IQR: 7.0-59.0). Compared with the age 2.0-5.0 y group, the age <2.0 y group and age >5.0 y group had a higher risk of re-operation or restenosis (age <2.0 y, HR = 3.27, 95% CI 1.25-8.60; age >5.0 y, HR = 2.87, 95% CI 1.19-6.91). Asymptomatic children with SVAS without other cardiovascular abnormalities should be considered for delayed surgical intervention until 2 years of age, and then surgery should be conducted as soon as possible. Children with severe symptoms should undergo surgery immediately, regardless of age. ChiCTR2300067851, https://www.chictr.org.cn/showproj.html?proj=177491.

Initial balloon versus surgical valvuloplasty in children with isolated congenital aortic stenosis: Influence on timing of aortic valve replacement

ObjectiveTo evaluate the influence of initial intervention on the long-term outcomes in congenital aortic stenosis. MethodsTwo hundred forty-three children underwent initial intervention between 1997 and 2022, by surgical valvuloplasty in 92 (32% neonates, 36% infants) and balloon valvuloplasty in 151 (27% neonates, 30% infants). Twenty-eight patients (11.5%) had associated mitral valve stenosis. Competing risk analysis for death, alive after initial intervention, or alive after aortic valve replacement (AVR) was performed and factors influencing survival or AVR examined. ResultsThere were 9 early deaths (3.7%). During a median follow-up of 13.5 years (range, 1.5-26.7), 98 patients had reintervention on the aortic valve (40.3%), whereas 145 had AVR (59.6%) at a median age of 14.0 years (interquartile range, 9.0-17.0), which was by Ross procedure in 130 (89.6%). Of the 12 late deaths, 3 were perioperative and 9 occurred as outpatients. There were no perioperative or late deaths after AVR. AVR occurred earlier in patients who had initial balloon (12.0 years [interquartile range, 5.0-14.5]) rather than surgical (18.5 years [interquartile range, 15.5-21.5]) valvuloplasty (P < .05). Actuarial survival in the cohort was 91.3% at 25 years, with no difference between the 2 initial interventions. Critical aortic stenosis, mitral stenosis, and initial intervention as a neonate were independent risk factors for worse survival. ConclusionsWe demonstrate excellent early and late survival in patients with congenital aortic stenosis after initial balloon or surgical valvuloplasty. Whilst children who had balloon valvuloplasty had AVR earlier than those who had initial surgical valvuloplasty, patient factors had a greater influence on survival than choice of initial intervention.

Method of Recording Reverse and Delayed Turbulent Blood Flow in an Obese Pediatric Patient with Congenital Aortic Stenosis

Method of Recording Reverse and Delayed Turbulent Blood Flow in an Obese Pediatric Patient with Congenital Aortic Stenosis

Pictorial rendition of author’s observations on balloon valvuloplasty/angioplasty procedures: Aortic stenosis

Balloon aortic valvuloplasty (BAV) effectively decreases peak pressure gradients through the aortic valve both at the time of the procedure and during follow-up. This paper presents the author&amp;rsquo;s observations on the utility of BAV in treating congenital aortic valve stenosis (AVS). Previous work by the author noted intermediate-term restenosis and late-onset aortic insufficiency (AI). Factors contributing to restenosis include age under 3 years and post-balloon residual aortic valve gradients exceeding 30 mmHg. Repeat balloon valvuloplasty has been found to address restenosis effectively. However, the onset of AI at late follow-up is a significant disadvantage of BAV. Despite this, BAV is currently considered a beneficial treatment option for managing AVS in children, adolescents, and young adults. In contrast, seniors with calcific AVS do not experience relief from obstruction with BAV and are candidates for aortic valve replacement through transcatheter methodology, a procedure not reviewed in this paper. BAV provides relief of obstruction across the aortic valve and functions as an alternative to surgical intervention. Consequently, BAV is considered a preferred choice in addressing aortic stenosis in the pediatric patient.

Long-Term Results of Percutaneous Balloon Aortic Valvuloplasty in Children With Aortic Stenosis: A Single-Center Experience.

Congenital aortic stenosis is a common pathology in the childhood age group and its clinical spectrum varies between asymptomatic and severe heart failure. In our study, we planned to evaluate the long-term results of patients who underwent balloon aortic valvuloplasty (BAV) due to critical aortic valve stenosis in our clinic. Patients aged 0-18 years who underwent aortic balloon valvuloplasty due to aortic stenosis in our clinic between January 2002 and January 2022 were retrospectively evaluated. Among the 48 patients who underwent balloon valvuloplasty due to aortic stenosis, 13 (27%) were female, and 35 (73%) were male. The median age at the time of the procedure was 27.5 months (IQR: 4-96), the median weight was 9.9 kg (IQR: 5.40-29.50), and the median height was 79 cm (IQR: 54-133). The median follow-up duration was 93.5 months (IQR: 38-132). Angiographic assessments in all patients revealed a median left ventricular pressure of 160 mmHg (IQR: 140-200) and a median pressure gradient between the left ventricle and the aorta of 60 mmHg (IQR: 42-80). The median balloon diameter used was 10 mm (IQR: 8-12). Post-procedural measurements showed a median mean gradient of 30 mmHg (IQR: 20-35) between the left ventricle and the aorta. The procedure was successful in 45 (93.5%) patients. During follow-up, 11 patients required surgical intervention. Ross procedure was performed in five patients, homograft in five patients, and mechanical valve implantation in one patient. Risk factors for the need for surgical intervention were evaluated in detail. During the follow-up, the risk factor for intervention was determined to be aortic insufficiency. Aortic valve balloon valvuloplasty is a safe and successful treatment method for critical aortic stenosis. It should be the first choice of treatment option in suitable patients.

A case of Turner's syndrome, factor 5 Leiden, and fibrinogen deficiency associated with congenital aortic stenosis complicated by aortic aneurysm and aortic regurgitation

A case of Turner's syndrome, factor 5 Leiden, and fibrinogen deficiency associated with congenital aortic stenosis complicated by aortic aneurysm and aortic regurgitation

Balloon Valvuloplasty in Congenital Critical Aortic Valve Stenosis in Neonates and Infants: A Rescue Procedure for the Left Ventricle.

Congenital critical aortic valve stenosis (CAVS) is a life-threatening disease requiring urgent treatment. First-line therapy is still controversial. The aim of our study was (1) to analyze retrospectively the patients of our institution who underwent balloon aortic valvuloplasty (BAV) due to CAVS and (2) describe the techniques for improved feasibility of intervention using microcatheters and retrieval loops. Twelve patients underwent 23 BAVs: 1 BAV was performed in 3 patients, 2 BAVs were performed in 7 patients, and 3 BAVs were performed in 2 patients. The peak trans-valvular pressure gradient (Δp) and left ventricular shortening fraction (LVSF) improved significantly in the first two interventions. In the first BAV, Δp decreased from 73.7 ± 34.5 mmHg to 39.8 ± 11.9 mmHg (p = 0.003), and the LVSF improved from 22.3 ± 13.5% to 31.6 ± 10.2% (p = 0.001). In the second BAV, Δp decreased from 73.2 ± 33.3 mmHg to 35.0 ± 20.2 mmHg (p < 0.001), and the LVSF increased from 26.7 ± 9.6% to 33.3 ± 7.4% (p = 0.004). Cardiac surgery during the neonatal period was avoided for all children. The median time to valve surgery was 5.75 years. Few complications occurred, namely mild-to-moderate aortic regurgitation, one remediable air embolism, and one intimal injury to the ascending aorta. We conclude that BAV is a successful emergency treatment for CAVS, resulting in left ventricular relief, clinical stabilization, and a time gain until cardiac surgery.

Unravelling supravalvular aortic stenosis in a young patient: look beyond the valve.

A 16-year-old adolescent girl was referred to our tertiary care center for management of a failed aortic valve balloon dilatation procedure for congenital valvular aortic stenosis. The medical records revealed a residual peak-to-peak gradient of 80 mm Hg after 2 dilations with 16 x 6-mm Tyshak balloon (NuMed Inc.).

Imaging Modalities in the Diagnosis of Congenital Abdominal Aortic Stenosis

Ischemic episodes affecting the aorta are uncommon in pediatric populations but demand urgent attention due to their potential for grave outcomes if left untreated. This case underscores the critical role of imaging modalities, particularly CT scanning, in diagnosing aortic ischemia swiftly and accurately, thereby facilitating timely therapeutic interventions.

A germline chimeric KANK1-DMRT1 transcript derived from a complex structural variant is associated with a congenital heart defect segregating across five generations.

Structural variants (SVs) pose a challenge to detect and interpret, but their study provides novel biological insights and molecular diagnosis underlying rare diseases. The aim of this study was to resolve a 9p24 rearrangement segregating in a family through five generations with a congenital heart defect (congenital pulmonary and aortic valvular stenosis and pulmonary artery stenosis), by applying a combined genomic analysis. The analysis involved multiple techniques, including karyotype, chromosomal microarray analysis (CMA), FISH, genome sequencing (GS), RNA-seq, and optical genome mapping (OGM). A complex 9p24 SV was hinted at by CMA results, showing three interspersed duplicated segments. Combined GS and OGM analyses revealed that the 9p24 duplications constitute a complex SV, on which a set of breakpoints matches the boundaries of the CMA duplicated sequences. The proposed structure for this complex rearrangement implies three duplications associated with an inversion of ~ 2Mb region on chromosome 9 and a SINE element insertion at the more distal breakpoint. Interestingly, this genomic structure of rearrangement forms a chimeric transcript of the KANK1/DMRT1 loci, which was confirmed by both RNA-seq and Sanger sequencing on blood samples from 9p24 rearrangement carriers. Altogether with breakpoint amplification and FISH analysis, this combined approach allowed a deep characterization of this complex rearrangement. Although the genotype-phenotype correlation remains elusive from the molecular mechanism point of view, this study identified a large genomic rearrangement at 9p24 segregating with a familial congenital heart defect, revealing a genetic biomarker that was successfully applied for embryo selection, changing the reproductive perspective of affected individuals.

Long-term outcome after neonatal intervention for congenital critical aortic stenosis

BackgroundThis study explored long-term outcome and functional status of patients born with critical aortic stenosis (CAS) following neonatal surgical or catheter interventions. MethodsA 40-year retrospective review of all consecutive patients within a large, single-center referral unit who required neonatal (<30 days) intervention for CAS. Additional detailed evaluation of surviving patients >7 years age was performed, with clinical assessment, objective cardiopulmonary exercise testing and state-of-the-art characterization of myocardial function (advanced echocardiography and cardiac MRI). ResultsBetween 1970 and 2010, ninety-six neonates underwent CAS intervention (mean age 9 ± 7.5 days). Early death occurred in 19 (19.8%) and late death in 10 patients. Overall survival at 10 and 30 years was 70.1% and 68.5%, freedom from reintervention was 41.8% and 32.9% respectively.Among the 25 long-term survivors available for detailed assessment (median age 15.7 ± 6.4 years), 55% exhibited impaired peak oxygen uptake. Mean left ventricle (LV) ejection fraction was 65 ± 11.2%, with a mean LV end-diastolic volume z-score of 0.02 ± 1.4. Mean LV outflow tract Vmax was 2.3 ± 1.02 m/s. CAS patients had reduced LV longitudinal and increased radial strain (p = 0.003, p < 0.001 respectively). Five patients had severe LV diastolic dysfunction associated with endocardial fibroelastosis (EFE) (p = 0.0014). ConclusionDespite high early mortality rate, long-term survival of patients with CAS is reasonable at the expense of high reintervention rate. With successful intervention, there remained long-term clinical and subclinical LV myocardial impairment, of which EFE was one marker. Long-term follow-up of all CAS patients is crucial, involving detailed myocardial functional assessment to help elucidate physiology and optimise management.

Knockdown of ANGPTL2 promotes left ventricular systolic dysfunction by upregulation of NOX4 in mice.

Background: Angiopoietin-like 2 (ANGPTL2) is a pro-inflammatory and pro-oxidant circulating protein that predicts and promotes chronic inflammatory diseases such as atherosclerosis in humans. Transgenic murine models demonstrated the deleterious role of ANGPTL2 in vascular diseases, while deletion of ANGPTL2 was protective. The nature of its role in cardiac tissues is, however, less clear. Indeed, in adult mice knocked down (KD) for ANGPTL2, we recently reported a mild left ventricular (LV) dysfunction originating from a congenital aortic valve stenosis, demonstrating that ANGPTL2 is essential to cardiac development and function. Hypothesis: Because we originally demonstrated that the KD of ANGPTL2 protected vascular endothelial function via an upregulation of arterial NOX4, promoting the beneficial production of dilatory H2O2, we tested the hypothesis that increased cardiac NOX4 could negatively affect cardiac redox and remodeling and contribute to LV dysfunction observed in adult Angptl2-KD mice. Methods and results: Cardiac expression and activity of NOX4 were higher in KD mice, promoting higher levels of cardiac H2O2 when compared to wild-type (WT) mice. Immunofluorescence showed that ANGPTL2 and NOX4 were co-expressed in cardiac cells from WT mice and both proteins co-immunoprecipitated in HEK293 cells, suggesting that ANGPTL2 and NOX4 physically interact. Pressure overload induced by transverse aortic constriction surgery (TAC) promoted LV systolic dysfunction in WT mice but did not further exacerbate the dysfunction in KD mice. Importantly, the severity of LV systolic dysfunction in KD mice (TAC and control SHAM) correlated with cardiac Nox4 expression. Injection of an adeno-associated virus (AAV9) delivering shRNA targeting cardiac Nox4 expression fully reversed LV systolic dysfunction in KD-SHAM mice, demonstrating the causal role of NOX4 in cardiac dysfunction in KD mice. Targeting cardiac Nox4 expression in KD mice also induced an antioxidant response characterized by increased expression of NRF2/KEAP1 and catalase. Conclusion: Together, these data reveal that the absence of ANGPTL2 induces an upregulation of cardiac NOX4 that contributes to oxidative stress and LV dysfunction. By interacting and repressing cardiac NOX4, ANGPTL2 could play a new beneficial role in the maintenance of cardiac redox homeostasis and function.

Long-term Outcomes After Surgical Intervention for Congenital Supravalvar Aortic Stenosis in Children

BackgroundPrimary supravalvar aortic stenosis (SVAS) is a rare congenital cardiovascular condition that can coexist with Williams-Beuren syndrome, coronary artery involvement, aortic coarctation, and pulmonary artery stenosis. SVAS repair can be achieved with low perioperative mortality, but long-term survival remains less well understood. We used the Pediatric Cardiac Care Consortium, a multicenter United States-based registry for pediatric cardiac operations, to assess long-term outcomes after SVAS repair. MethodsWe used Kaplan-Meier plots and Cox proportional hazards regression to examine factors associated with postdischarge deaths. These included sex, age-group, weight z-score, coexisting conditions (Williams-Beuren syndrome, coronary artery involvement, coarctation, and pulmonary artery stenosis), surgical techniques, and era, defined as early (1982-1995) or late (1996-2003). Survival was assessed by matching with the National Death Index through 2021. ResultsOf 333 patients who met inclusion criteria, 313 (94.0%) survived to discharge and 188 (60.1%) had identifiers for National Death Index matching. Over a median follow-up of 25.2 years (interquartile range, 21.1-29.4 years), 17 deaths occurred. The 30-year survival after discharge from SVAS repair was 88.7% (95% CI, 82.9%-94.8%). Infantile surgery and non-Williams-Beuren syndrome were associated with decreased 30-year survival. From the various repairs, the 2-sinus technique had better outcomes compared with all other types, except the 3-sinus technique (nonsignificant difference). Adjusted analysis revealed infantile age and type of repair as associated with postdischarge probability of death. ConclusionsThese data demonstrate favorable long-term outcomes after SVAS repair, except for the infantile group that was associated with more diffuse arteriopathy. As techniques continue to evolve, future studies are warranted to investigate their long-term outcomes.

Effectiveness and Safety of Different Patch Materials for Supravalvar Aortic Stenosis (Middle-Term Outcomes).

To determine the effectiveness and safety of different patch materials in the treatment of pediatric patients with congenital supravalvular aortic stenosis (SVAS). 218 consecutive SVAS patients (age 14 years) who underwent surgery from Beijing Fuwai and Yunnan Fuwai hospital between 2002 and 2020 were included. Patients were divided into the pericardium patch group (133 (61.0%)), modified patch group (43 (19.7%)) and artificial patch group (42 (19.3%)). The primary safety endpoint was patch-related adverse complications (post-operation patch hemorrhage or aortic sinus aneurysm at 2-year follow-up). The primary effectiveness outcome was the re-operation or restenosis at 2-year follow-up. Multivariable cox regression was used to obtain the hazard ratio (HR). The median age at operation was 43.5 months (IQR 24.0-73.0). Only three patients had patch-related adverse complications, and no difference existed among the three groups (p = 0.763). After a median follow-up of 24.0 months (IQR 6.0-48.0), patients with a pericardium patch had a lower re-operation or restenosis rate compared with the other two groups (pericardium patch vs modified patch, HR = 0.30, 95% CI 0.12-0.77; pericardium patch vs artificial patch, HR = 0.33, 95% CI 0.13-0.82), even in the main subgroup and sensitivity analysis. In pediatric patients, the safety of autologous pericardium patch is acceptable, along with lower rates of middle-term re-operation or restenosis. http://www.chictr.org.cn, number: ChiCTR2300067851.

Bicuspidization Using the Open-Sleeve Technique for Congenital Aortic Stenosis during Infancy

Bicuspidization Using the Open-Sleeve Technique for Congenital Aortic Stenosis during Infancy

The Role of Speckle-Tracking Echocardiography in Predicting Mortality and Morbidity in Patients With Congenital Heart Disease: A Systematic Review and Meta-analysis

Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease (CHD) is not well described. We conducted a systematic review to evaluate the prognostic value of STE in patients with CHD. The EMBASE, Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to January 2023 for terms related to all CHD, STE, and prognosis. Meta-analysis of association of right ventricle and left ventricle strain (RV Sl and LV Sl, respectively) with major adverse cardiovascular events (MACEs) was performed in atrial switch transposition of the great arteries (asTGA)/congenitally corrected TGA (ccTGA), tetralogy of Fallot (ToF), and congenital aortic stenosis (cAS)/bicuspid aortic valve (BAV). P-value combination analysis was additionally performed for all CHD groups. A total of 33 studies (30 cohorts, n=8,619 patients, children, and adults) were included. Meta-analysis showed the following parameters as being associated with MACE: RV Sl in asTGA/ccTGA (hazard ratio [HR]=1.1/%; CI, [1.03; 1.18]), RV Sl and LV Sl in ToF (HR=1.14/%; CI, [1.03; 1.26] and HR=1.14/%; CI, [1.08; 1.2], respectively), and LV Sl in cAS/BAV (HR=1.19/%; CI, [1.15; 1.23]). The RV Sl and strain rate were associated with outcomes also in single ventricle/hypoplastic left heart syndrome (at all palliation stages except before Norwood stage 1) and LV Sl in Ebstein's anomaly. This systematic review and meta-analysis showed that biventricular strain and strain rate were associated with outcomes in a variety of CHD, highlighting the need for updated recommendations on the use of STE in the current guidelines, specific to disease types.

The mid-term outcomes of aortic root replacement after surgical repair for CHD.

The purpose of this study is to assess the mid-term outcomes of aortic root replacement after repair of CHDs. This is a single-institutional retrospective, cohort study with consecutive patients undergoing aortic root replacement after surgical repair of CHDs between 1999 and 2022. Operative indications included aortic root dilatation with/without aortic insufficiency, sinus of Valsalva rupture, or aortic dissection involving the root. Forty-four patients (36 male and 8 female) were enrolled. Mean age at the root replacement was 36.6 ± 11.9 years. The most frequent primary diagnosis was congenital aortic stenosis (n = 10) and ventricular septal defect (n = 10). Mean time from the surgical repair to aortic root replacement was 26.6 ± 13.0 years. Operative indications were aortic root enlargement with or without aortic valve aetiology (n = 40), sinus of Valsalva rupture (n = 2), and aortic dissection (n = 2). Forty-two patients underwent valve-replacing aortic root replacement, and two patients underwent valve-sparing, with 40 concomitant procedures. The median follow-up was 3.5 (1.3-7.6) years. There were one early and five late mortalities and five cardiovascular-related reoperations. Actuarial survival at 5-10 years after root replacement was 81.0 ± 6.6%. The cumulative incidence of cardiovascular-related reoperation and aortic root or valve-related reoperation at 5 years after root replacement was 11.9% and 5.6%, respectively. The early and mid-term outcomes of aortic root replacement for patients with a history of repair of CHDs were favourable in terms of survival and aortic root or valve-related reoperation.