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Background: The impact of aortic valve replacement (AVR) type on health-related quality of life (HRQOL) in adolescents and young adults is unclear, but may vary depending on need for anticoagulation or re-intervention. We sought to determine the differences in HRQOL following AVR with either the Ross procedure or mechanical AVR in this young population. Methods: Patients 14–35 years old and at least 1 year post-AVR were included. HRQOL was assessed using the Short Form-36 (SF-36). Valve-specific concerns regarding anticoagulation and reoperation were also assessed. Clinical outcome data were obtained by chart review. Results: A total of 51 patients were enrolled: 24 (47%) Ross and 27 (53%) mechanical AVR. Ross patients were younger at time of AVR (16 vs. 22 years, p < 0.01) and study enrollment (23.7 vs 29.5 years, p < 0.01). Median follow-up from AVR to study enrollment was similar (5.4 years for Ross vs. 5.6 years for mechanical, p = 0.62). At last follow-up, clinical outcomes including cardiac function, functional class, and aortic valve re-intervention rates were similar between groups, although mechanical valve patients had more bleeding events (p = 0.012). SF-36 scores were generally high for the entire cohort, with no significant difference between groups in any domain. Mechanical AVR patients reported more concern about frequency of blood draws (p < 0.01). Concern for reoperation was similar between both groups. Conclusion: Despite more bleeding events and concern about the frequency of blood draws, adolescents and young adults with mechanical AVR reported similarly high levels of HRQOL compared to those following Ross AVR. [ABSTRACT FROM AUTHOR]

The surgical options for significant aortic valve disease include either Ross procedure (RP) or aortic valve replacement (AVR). The exercise stress test is routinely performed in these patients to assess the objective functional capacity. This retrospective study was conducted to evaluate the differences and the longitudinal changes of exercise capacity in patients following the RP and AVR for aortic valve disease. This is an IRB approved retrospective study and included patients who had either RP or AVR performed for aortic valve disease and had at least one exercise stress test performed after the surgical procedure. Patients with other congenital heart disease, pacemaker or defibrillators, and those with inadequate data were excluded. Demographic data including age at surgery, type of surgery and type of aortic valve was collected. Data regarding treadmill cardiopulmonary exercise test (CPET) was also collected. A total of 47 patients met inclusion criteria and were equally represented in each group, i.e. RP [n = 23, 73.9% male, age at surgery 11.2 (4.5–15.9) years] vs. AVR [n = 24, 88% mechanical AVR, 60.9% male, age at surgery 15.1 (12.8–19.4) years]. There was a significant decline in predicted oxygen consumption (%VO2) at time of first post-operative CPET in patients after AVR compared to RP (79 vs. 88%, p = 0.048) over a similar accrued median interval follow-up (4.6 vs. 6.2 years, p = 0.2). The longitudinal follow-up analysis of following AVR (n = 11, 54.5% male, median inter-test duration of 5 years) showed significant decline in peak exercise capacity or VO2 (34.2 vs. 26.2 vs., p = 0.006). In contrast, after RP (n = 12 patients [58.3% male, median inter-test duration 7.1 of years], exercise capacity and other key parameters remained preserved. In this small sentinel study, we report a better initial exercise capacity among patients after RP compared to AVR over an intermediate follow-up. During longitudinal follow-up in a subset of patients, exercise capacity remained preserved amongst the RP group while it further declined in the AVR group. [ABSTRACT FROM AUTHOR]

The Ross procedure provides excellent long-term survival, hemodynamics, freedom from valve-related complications, and quality of life. However, its usage has decreased mainly because of concerns with technical complexity and long-term durability. In the last 2 decades, the factors associated with late failure after the Ross procedure have been clearly identified. As a result, the surgical technique has been modified in order to address these issues, specifically aiming at stabilizing the autograft root at its different levels: annulus, sinus of Valsalva, and sinotubular junction; as well as mitigating the risk of pulmonary homograft dysfunction. We have implemented this systematic approach over the last decade with demonstrated safety and effectiveness. Furthermore, by breaking down the operation into its smaller steps, we believe it makes it highly reproducible in the hands of dedicated aortic reconstructive surgeons. This article details our step-by-step, systematic, and tailored approach to the Ross procedure in patients with aortic stenosis or regurgitation. [ABSTRACT FROM AUTHOR]

Cryopreserved pulmonary homografts (PH) are the current gold standard for right ventricular outflow tract (RVOT) reconstruction in the Ross procedure. Unfortunately, their use is limited by a relatively scarce availability and high cost. Porcine stentless xenografts (SX) such as the Medtronic Freestyle SX are increasingly being used, although it is unclear whether the hemodynamic performance and the long‐term durability are satisfactory. The present systematic review followed the Preferred Reporting Items for Systematic reviews and Meta‐Analysis statement. The pooled treatment effects were calculated using a weighted DerSimonian‐Laird random‐effects model. We also evaluated the effect of time after RVOT reconstruction on valve gradients using meta‐regression. Six studies with a total of 156 patients met the inclusion criteria. The pooled estimates for the pooled follow‐up of 37 months were: 1.3% operative mortality, 94.8% overall survival, 7.5% structural valve deterioration, 5.2% reintervention, 73.3% asymptomatic, and 1.5% moderate or severe pulmonary insufficiency. Peak valve gradients were significantly correlated with time after RVOT, increasing during follow‐up. Three studies compared PH with SX, one concluded that the SX is an acceptable alternative for RVOT reconstruction, whereas two concluded that this valvular substitute had inferior performance. The Freestyle SX can be considered as an alternative to PH, although it might be associated with more reinterventions, higher peak valve pressure gradients, and pulmonary valve dysfunction. [ABSTRACT FROM AUTHOR]

This article discusses a study conducted by Geuens et al. on the current practices and opinions of cardiac surgeons in Europe and North America regarding aortic valve replacement (AVR) techniques in non-elderly patients. The study found that mechanical valves were the most frequently chosen substitute, but the popularity of biological valves increased with increasing patient age. The Ross procedure, a surgical technique, was employed in less than 10% of cases, and transcatheter AVR (TAVR) was performed in a similar percentage of cases. The study also highlighted that 23% of patients were not involved in the decision-making process regarding the selection of the valve substitute. The article emphasizes the ongoing debate surrounding the ideal aortic valve substitute for young and middle-aged adults and provides recommendations from different guidelines. It also discusses the potential benefits and concerns associated with different AVR techniques, such as the durability of mechanical valves, the feasibility of TAVR within surgical bioprostheses, and the underutilization of the Ross procedure. The article concludes by emphasizing the importance of informed patient involvement in the decision-making process and the need for a multidisciplinary approach to ensure the best long-term outcomes for patients. [Extracted from the article]

Aortic valve repair, Ross procedure, Aortic valve disease At the outset, it is important to recognize that a Ross series such as this is highly selected, the selection criteria are rarely reported, and results of the overall aortic valve practice containing the Ross patients are seldom provided. Keywords: Aortic valve repair; Ross procedure; Aortic valve disease EN Aortic valve repair Ross procedure Aortic valve disease 1 2 2 11/02/23 20231001 NES 231001 With recurrent interest in the Ross operation, Shih, Brinkman and co-authors analysed their series of 225 Ross procedures performed over the past 25 years [[1]]. [Extracted from the article]

Background: Treatment of aortic-valve disease in young patients still poses challenges. The Ross procedure offers several potential advantages that may translate to improved long-term outcomes.Objectives: This study reports long-term outcomes after the Ross procedure.Methods: Adult patients who were included in the Ross Registry between 1988 and 2018 were analyzed. Endpoints were overall survival, reintervention, and major adverse events at maximum follow-up. Multivariable regression analyses were performed to identify risk factors for survival and the need of Ross-related reintervention.Results: There were 2,444 adult patients with a mean age of 44.1 ± 11.7 years identified. Early mortality was 1.0%. Estimated survival after 25 years was 75.8% and did not statistically differ from the general population (p = 0.189). The risk for autograft reintervention was 0.69% per patient-year and 0.62% per patient-year for right-ventricular outflow tract (RVOT) reintervention. Larger aortic annulus diameter (hazard ratio [HR]: 1.12/mm; 95% confidence interval [CI]: 1.05 to 1.19/mm; p < 0.001) and pre-operative presence of pure aortic insufficiency (HR: 1.74; 95% CI: 1.13 to 2.68; p = 0.01) were independent predictors for autograft reintervention, whereas the use of a biological valve (HR: 8.09; 95% CI: 5.01 to 13.08; p < 0.001) and patient age (HR: 0.97 per year; 95% CI: 0.96 to 0.99; p = 0.001) were independent predictors for RVOT reintervention. Major bleeding, valve thrombosis, permanent stroke, and endocarditis occurred with an incidence of 0.15% per patient-year, 0.07% per patient-year, 0.13%, and 0.36% per patient-year, respectively.Conclusions: The Ross procedure provides excellent survival over a follow-up period of up to 25 years. The rates of reintervention, anticoagulation-related morbidity, and endocarditis were very low. This procedure should therefore be considered as a very suitable treatment option in young patients suffering from aortic-valve disease. (Long-Term Follow-up After the Autograft Aortic Valve Procedure [Ross Operation]; NCT00708409). [ABSTRACT FROM AUTHOR]

OBJECTIVES Open in new tab Download slide Open in new tab Download slide Pulmonary allografts (AG) are the gold standard for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure. However, there is limited availability of AG in some countries, and the use of alternative grafts for RVOT reconstruction remains controversial. This study aimed to compare the rates of freedom from RVOT graft dysfunction for AG and diepoxide-treated pericardial xenografts (DPXG). METHODS Between 1998 and 2015, 793 adult patients underwent the Ross procedure in our centre. Using propensity score matching, the clinical outcomes and echocardiographic results of AG and DPXG were compared. RESULTS Propensity score matching resulted in 2 groups (AG and DPXG) of 122 patients each. No difference was found in early mortality (2.5%) in both groups. The freedom from RVOT graft dysfunction curves were comparable between the AG and DPXG groups (P  = 0.186) and the 8-year rates of freedom from graft dysfunction were 91.8% and 82.2%, respectively. The survival rates at 8 years were 90.5% and 90.1%, and the rates of freedom from RVOT reintervention at 8 years were 100% and 96.8% for the AG and DPXG groups, respectively. At discharge and follow-up, transprosthetic gradients were significantly higher in the DPXG group. The rate of the RVOT gradient progression was also higher in the DPXG group than in the AG group (1.80 ± 0.06 vs 1.39 ± 0.04 mmHg/year, P  < 0.001). CONCLUSIONS There was no difference in freedom from RVOT graft dysfunction by 8 years when using AG and DPXG in adult Ross patients, nor in survival and freedom from RVOT conduit reintervention. Long-term results need further evaluation. [ABSTRACT FROM AUTHOR]

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) developing a risk score to predict adverse outcomes in patients with hypoplastic left heart syndrome using the national pediatric cardiology quality improvement collaborative, (2) outcomes of the Ross procedure in patients with a previous aortic valve surgery showing better outcomes in these patients compared to patients with the primary Ross procedure, (3) the initial experience with continuous flow ventricular assist devices in pediatric patients showing low mortality post implantation, (4) lower socioeconomic status is associated with worse long-term neurodevelopmental outcomes in patients with hypoplastic left heart syndrome, (5) QRS fragmentation is associated with higher incidence of appropriate shock after implantable cardioverter defibrillator implantation in repaired tetralogy of Fallot, (6) atrioventricular valve repair may have better outcomes if performed before the Fontan operation compared to after the Fontan operation. [ABSTRACT FROM AUTHOR]

The management of aortic valve disease in the pediatric population is complex and requires an individualized approach and opportune application of techniques focused on each individual patient's specific anatomy, pathology, and clinical presentation. Though some patients may require variations in the approach to management, the ultimate goal should be to perform a Ross procedure when aortic valve replacement is indicated. [ABSTRACT FROM AUTHOR]

Abstract Background Reoperations are required frequently after the Ross procedure in rheumatic patients. The use of transcatheter aortic valve implantation (TAVI) in those patients could decrease the risk of future open procedure; however, the outcome may be affected by the concomitant mitral valve disease, and subsequent mitral reoperation may distort the implanted aortic valve. Case presentation We present a female patient who had a beating mitral valve replacement after valve-in-valve TAVI in a patient with prior Ross procedure. Weaning from cardiopulmonary bypass was difficult, and the patient needed extra-cardiac membrane oxygenation (ECMO) and intra-aortic balloon pump because of right ventricular dysfunction. The right ventricular dysfunction could be due to the concomitant coronary artery disease or air embolism during the beating mitral valve surgery. Recovery was gradual, and the patient was discharged after 33 days. Pre-discharge echocardiography showed a maximum gradient of 9 mmHg on the aortic valve and mild paravalvular leak. Conclusions Mitral valve replacement in a patient with prior TAVI and the Ross procedure was feasible; it decreased the operative risk and did not distort the implanted aortic valve.

[Display omitted] [ABSTRACT FROM AUTHOR]

The Ross procedure has been considered in children as an optimal surgical procedure due to potential growth of the aortic annulus, lack of anticoagulation requirement, very low morbidity rate and excellent survival. Five-hundred-thirty-six (366 male, mean age 29.4 ± 11.1 years) underwent Ross procedure between 1990 and 2016 and had complete clinical and echocardiographic follow-up. Mean follow-up was 16.3 ± 4.9 years. Patients were divided in 2 groups according to age at surgery. Group 1 consisted of 320 (60%) patients less than 18 years old (223 male, mean age at surgery of 9.5 ± 5.6 years). Group 2 consisted of 216 (40%) patients older than 18 years of age (143 male, mean age at surgery of 26.3 ± 8.2 years). One-hundred-thirty (24%) patients had a redo procedure or surgery. Freedom from all re-operation and or percutaneous reintervention on either the aortic and pulmonary valves was 99% after 1 year, 94% after 5 years, 89% after 10 years, 83% after 15 years and 78% after 20 years. Freedom from redo surgery for AV 99% after 1 year, 94% after 5 years, 90% after 10 years, 81% after 15 years and 80% after 20 years. Freedom from redo surgery for PV was 100% after 1 year, 95% after 5 years, 89% after 10 years, 78% after 15 years and 76% after 20 years. The ideal candidate for Ross operation is a patient with congenital aetiology and an aortic root diameter ≤ 15 mm/m2. A pulmonary fresh preserved homograft seems to perform better on the long term. • The use of the Ross procedure represents <1% of all aortic valve replacements. • Freedom from all re-operation was 89% after 10 years and 83% after 15 years. • Freedom from redo surgery for AV was 90% after 10 years and 81% after 15 years. • Rheumatic valves and aortic root diameter > 15 mm/m2 have worse outcome. • A pulmonary fresh preserved homograft seems to perform better on the long term. [ABSTRACT FROM AUTHOR]

The Ross procedure represents a valid option for aortic valve replacement in young adults and was repeatedly shown to restore survival to that of the age- and sex-matched general population. However, its major drawback relies in the risk of pulmonary autograft (PA) dilation, negative histological remodeling and need for reoperation. Several techniques and materials to reinforce the PA have been proposed. They mainly include Dacron, personalized external aortic root support with a polyethylene terephthalate mesh system, autologous aortic tissue and bioresorbable materials. Synthetic materials, despite widely used in cardiac surgery, have significant biocompatibility issues with the PA and their interaction with this living structure translates into negative remodeling phenomena and disadvantageous biomechanical behaviors. Conversely, biomaterials with tailored degradable profiles might be able to reinforce while integrating with the PA and enhance its remodeling capabilities. The recent advancement in this field are here discussed. [ABSTRACT FROM AUTHOR]

Aortic valve replacement is the commonest cardiac surgical operation performed worldwide for infective endocarditis (IE). Long-term durability and avoidance of infection relapse are goals of the procedure. However, no detailed guidelines on prosthesis selection and surgical strategies guided by the comprehensive evaluation of the extension of the infection and its microbiological characteristics, clinical profile of the patient, and risk of infection recurrence are currently available. Conventional mechanical or stented xenografts are the preferred choice for localized aortic infection. However, in cases of complex IE with the involvement of the root or the aortomitral continuity, the use of homograft is suggested according to the surgeon and center experience. Homograft use should be counterbalanced against the risk of structural degeneration. Prosthetic bioroot or prosthetic valved conduit (mechanical and bioprosthetic) are also potentially suitable alternatives. Further development of preservation techniques enabling longer durability of allogenic substitutes is required. We evaluate the current evidence for the use of valve substitutes in aortic valve endocarditis and propose an evidence-based algorithm to guide the choice of therapy. We performed a systemic review to clarify the contemporary surgical management of aortic valve endocarditis. [ABSTRACT FROM AUTHOR]

Background: The Ross procedure in children is performed either as a primary operation, or a secondary operation after initial aortic valve surgery.Objectives: The study aimed to determine whether the outcomes of primary and secondary Ross procedure are similar.Methods: All patients who underwent Ross procedure between 1995 and 2018 were included in the study. Outcomes were compared between those who had primary Ross procedure and those who had secondary Ross procedure, after aortic valve surgery. Propensity score matching for baseline characteristics and risk factors for death and reoperation was performed.Results: Of 140 Ross procedures, 51.4% (n = 72 of 140) were primary operations, while 48.6% (n = 68 of 140) were secondary operations. Patients undergoing primary Ross procedure tended to be older (median age 8.6 years vs. 7.0 years; p = 0.10) and have a higher weight (28.9 kg vs. 19.4 kg; p = 0.07). There were no significant differences in survival or freedom from reoperation in the unmatched cohort. Propensity score matching resulted in 50 well-matched pairs. In the matched cohort, survival at 10 years was 90.0% (95% confidence interval [CI]: 77.5% to 95.7%) in the primary Ross group, compared with 96.8% (95% CI: 79.2% to 99.5%) in the secondary Ross group (p = 0.04). Freedom from autograft reoperation at 10 years was 82% (95% CI: 64.1% to 91.5%) in the primary Ross group, compared with 97.0% (95% CI: 80.4% to 99.6%) in the secondary Ross group (p = 0.03).Conclusions: Secondary Ross procedure performed after initial aortic valve surgery achieves superior long-term survival and freedom from autograft reoperation compared with primary Ross procedure. A strategy of initial aortic valve repair followed by delayed Ross procedure may provide better long-term survival and freedom from autograft reoperation. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Surgical treatment of infective endocarditis (IE) remains a challenge. The Ross procedure offers the benefit of a living substitute in the aortic position but it is a more complex operation which may lead to increased operative risk. The aim of this study was to assess the safety and late outcomes of the Ross procedure for the treatment of active IE. METHODS From 2000 to 2019, a total of 31 consecutive patients underwent a Ross procedure to treat active IE (mean age 43 ± 12 years, 84% male). All patients were followed up prospectively. Four patients (13%) were intravenous (IV) drug users and 6 patients (19%) had prosthetic IE. The most common infective organism was Streptococcus (58%). Median follow-up was 3.5 (0.9–4.5) years and 100% complete. RESULTS There were no in-hospital deaths. One patient suffered a postoperative stroke (3%) and 1 patient (3%) required reintervention for bleeding. Three patients had a new occurrence endocarditis: 2 patients were limited to the pulmonary homograft and successfully managed with IV antibiotics, whereas 1 IV drug user patient developed concomitant autograft and homograft endocarditis. Overall, cumulative incidence of IE recurrence was 13 ± 8% at 8 years. The cumulative incidence for autograft endocarditis was 5 ± 4% at 8 years. Two patients (6%) died during follow-up, both from drug overdoses. At 8 years, actuarial survival was 88 ± 8%. CONCLUSIONS In selected patients with IE, the Ross procedure is a safe and reasonable alternative with good mid-term outcomes. Freedom from recurrent infection on the pulmonary autograft is excellent, labelporting the notion that a living valve in the aortic position provides good resistance to infection. Nevertheless, in IV drug user patients, pulmonary homograft endocarditis remains a challenge. Continued follow-up is needed to ascertain the long-term benefits of this approach. [ABSTRACT FROM AUTHOR]

To examine the role and outcomes of all interventions for aortic stenosis in children, with focus on freedom from reintervention and the aim to minimise prosthetic aortic valve replacement (pAVR) during childhood. Retrospective analysis of 194 consecutive children who underwent any aortic valve intervention for a biventricular repair strategy at a single institution between 1995 and 2017. Data were obtained from hospital records and follow-up was 100% complete. Over a 22-year period, 194 children underwent total 313 aortic valve procedures: Primary interventions were surgical valvotomy (SV)/surgical repair (SR) in 94 (48.5%), balloon valvuloplasty (BV) in 60 (30.9%), pAVR in 8 (4.1%) and Ross/Ross-Konno procedure in 32 (16.5%). Median age at first intervention was 1.1 years (interquartile range [IQR] 0.1-9.4) and varied with type of intervention: SV/SR were most common in neonates (33, 75%) and infants (35, 68%), whilst BV was most frequent in older children (42, 42%). Operative survival was 99% (2 early deaths, both in neonates with critical aortic stenosis and poor left ventricular function) and 15-year survival was 95%. A Ross procedure was performed in 79 (40.7%) patients over the 15-year study period, 1 of whom required late pAVR for autograft failure. Freedom from any reintervention after SV/SR and BV was 41% and 40% at 10 years, compared to 90% at 10 years with the Ross procedure (P < 0.001). Among neonatal SV/SR and BV, 98% required reintervention during childhood with no difference between groups. Valve morphology did not influence freedom from ultimate valve replacement. In patients who went on to have a Ross procedure, median time from initial intervention to Ross was 2.8 years (IQR 0.1-11.9) in neonates and 6.0 years (IQR 3.1-7.5) in all other age groups. Overall freedom from pAVR was 97% at 10 years and was similar in the SV/SR and BV groups. A strategy of simple valve repair and primary Ross procedure provides excellent survival and good freedom from pAVR. However, reintervention rates after simple interventions for congenital AS are high, especially in younger age groups. The Ross procedure offers the best freedom from reintervention of any technique and wider use of primary Ross in younger age groups should be considered. [ABSTRACT FROM AUTHOR]

Patients after the Ross procedure are at risk for right (RV) and left ventricular (LV) dysfunction due to neo-aortic and pulmonary dysfunction. While neo-aortic root dilatation has been related to LV dysfunction, the potential contributions of aortic stiffness and ventricular interactions have not been evaluated. Patients status post Ross procedure up to age 18 years with cardiac magnetic resonance (CMR) exam from 2007 to 2018 were retrospectively reviewed. Aortic pulse wave velocity (PWV) was calculated from phase contrast and angiogram images. RV and LV peak global longitudinal (GLS) and circumferential strain (GCS) were measured using tissue tracking software. Multivariable regression was performed for variables associated with parameters of LV function. In 58 patients (median age 20.5 years at CMR exam), male gender, longer time since Ross procedure, aortic root dilatation, and lower RV ejection fraction (EF) were associated with decreased LV EF. There was no association with LV late gadolinium enhancement or neo-aortic or conduit regurgitation. LV GCS and GLS also correlated with RV GCS, RV GLS and PWV. In multivariable analysis, the relation of RV and LV systolic function, but not aortic measurements, remained significant. In conclusion, in long-term follow-up after pediatric Ross procedure, RV function rather than aortic root size or aortic stiffness most closely relates to LV function. Ventricular interactions may impact decision-making on timing of conduit intervention, which could differ from established criteria in populations with only aortic or pulmonary valve disease. Further study is warranted to evaluate possible association with clinical outcome. [ABSTRACT FROM AUTHOR]

Background: Re-intervention after Ross procedure into the right ventricular outflow tract might be needed in patients in the long term. However, right ventricular outflow tract re-intervention indications are still unclear. Comprehensive assessment of total hemodynamics is needed. A 42-year-old Japanese woman was referred to our hospital for moderately severe pulmonary regurgitation and severe tricuspid regurgitation after a Ross-Konno procedure. Thirteen years after surgery, she developed atrial fibrillation and atrial flutter and complained of dyspnea. Electrophysiological studies showed re-entry circuit around the low voltage area of the lateral wall on the right atrium. Four-dimensional flow magnetic resonance imaging revealed moderate pulmonary regurgitation, severe tricuspid regurgitation, and a dilated right ventricle. Flow energy loss in right ventricle calculated from four-dimensional flow magnetic resonance imaging was five times higher than in normal controls, suggesting an overload of the right-sided heart system. Her left ventricular ejection fraction was almost preserved. Moreover, the total left interventricular pressure difference, which shows diastolic function, revealed that her sucking force in left ventricle was preserved. After the comprehensive assessments, we performed right ventricular outflow tract reconstruction, tricuspid valve annuloplasty, and right-side Maze procedure. A permanent pacemaker with a single atrial lead was implanted 14 days postoperatively. She was discharged 27 days postoperatively. Echocardiography performed 3 months later showed that the size of the dilated right ventricle had significantly reduced.Discussion: A four-dimensional imaging tool can be useful in the decision of re-operation in patients with complex adult congenital heart disease. The optimal timing of surgery should be considered comprehensively. [ABSTRACT FROM AUTHOR]

Background and Aims: There is still no optimal strategy for aortic valve repair in children. To assess the safety and efficacy of an original surgical technique: Partial-Ross procedure.Methods: Eight pediatric patients with bicuspid aortic valve underwent the Partial-Ross procedure.Results: The patients were doing well at a longest duration of 58-month follow-up visit. There was no case with aortic stenosis and three cases with mild to moderate aortic regurgitation, which had no significant clinical impact.Conclusion: Partial-Ross procedure could be used as an alternative for aortic valve repair in children. [ABSTRACT FROM AUTHOR]

Background: In addition to being associated with aortopathy, a bicuspid aortic valve (BAV) has been posed to be a risk factor for the dilation of the pulmonary autograft in the aortic position. The aim of this study is to assess the association between the subtype of native aortic valve leaflet fusion (right and noncoronary leaflets [R/N] vs right and left leaflets [R/L]) and autograft dilation and valve dysfunction after the Ross procedure.Methods: We performed a retrospective review of 43 patients with BAV who underwent a Ross procedure in our center from 1993 to 2013. Serial transthoracic echocardiography was used to measure changes in autograft and ascending aortic diameter over time. The aortic diameter was measured at four levels, and Z values were computed. Aortic dilation was defined as a Z value greater than 3.Results: The mean age at the time of the Ross procedure was 13.5 ± 9.2 years. R/L was the most prevalent native aortic valve subtype (R/L, n = 26, 61% vs R/N, n = 17, 39%). PreRoss procedure, aortic dilation was more frequent in patients with R/N fusion (P = .02), whereas the initial aortic valve gradient and grade of aortic insufficiency (AI) did not differ between the subgroups. At follow-up, (mean = 9.6 ± 4.3 years) dilation of the autograft and ascending aorta was seen more often in patients with R/N leaflet fusion (P = .03). Conversely, the prevalence of more than moderate AI was significantly higher in patients with R/L leaflet fusion (P = .03). There was no significant difference between groups among numbers of late reintervention on the aortic valve or root (P = .75); however the type of intervention varied by morphologic subtype. Patients with R/L fusion underwent more aortic valve replacements (AVRs) while patients with R/N fusion underwent more valve-sparing aortic root replacements.Conclusions: After Ross procedure, both groups of patients were likely to have a combination of dilation of the aortic root and the tubular portion of the ascending aorta at follow-up. Patients with R/L fusion were more likely to have a prevalence of root dilation, while patients with R/N fusion were more likely to have tubular ascending aorta dilation. The R/L phenotype is associated with a slightly more rapid dilation at follow-up and is more likely to have postoperative autograft insufficiency. This information may serve to guide patient and procedure selection for AVR. [ABSTRACT FROM AUTHOR]

Primary aortic insufficiency (AI) is a risk factor for autograft reintervention in adults undergoing the Ross procedure. We sought to examine the influence of preoperative AI on autograft durability in children and adolescents. From 1993 to 2020, 125 consecutive patients between ages 1 and 18 underwent a Ross procedure. The autograft was implanted using a full-root technique in 123 (98.4%) and included in a polyethelene terephthalate graft in 2 (1.6%). Patients with aortic stenosis (aortic stenosis group) (n = 85) were retrospectively compared with those with AI or mixed disease (AI group) (n = 40). Median length of follow-up was 8.2 years (interquartile range, 3.3-15.4 years). The primary end point was the incidence of severe AI or autograft reintervention. Secondary end points included changes in autograft dimensions analyzed using mixed-effect models. The incidence of severe AI or autograft reintervention was 39.0% ± 13.0% in the AI group and 8.8% ± 4.4% in the aortic stenosis group at 15 years (P =.02). Annulus z scores increased in both aortic stenosis and AI groups over time (P <.001). However, the annulus dilated at a faster rate in the AI group (absolute difference, 3.8 ± 2.0 vs 2.5 ± 1.7; P =.03). Sinus of Valsalva z scores increased in both groups as well (P <.001), but at similar rates over time (P =.11). Children and adolescents with AI undergoing the Ross procedure have higher rates of autograft failure. Patients with preoperative AI have more pronounced dilatation at the annulus. Akin to adults, a surgical aortic annulus stabilization technique that modulates growth is needed in children. [Display omitted] [ABSTRACT FROM AUTHOR]

Quadricuspid pulmonary valve (QPV) is a rare congenital cardiac malformation which is notable for its asymptomatic behavior. With only a few hundred cases reported in literature, its clinical significance is not exactly known, although many authors have placed its implications on the Ross procedure. We report a case of QPV which was detected on routine cardiac screening.

While it is the main viable option in the growing child and young adult, the Ross procedure has expanded its applicability to older patients, for whom long-term results are equivalent, if not superior, to prosthetic aortic valve replacement. Strategies aiming at mitigating long-term autograft failure from root enlargement and valve regurgitation have led some to advocate for root reinforcement with prosthetic graft material. On the contrary, we will discuss herein the rationale for a tailored approach to the Ross procedure; this strategy is aimed at maintaining the natural physiology and interplay between the various autograft components. Several technical maneuvers, including careful matching of aortic and autograft annuli and sino-tubular junction as well as external support by autologous aortic tissue maintain these physiologic relationships and the viability of the autograft, and could translate in a lower need for late reintervention because of dilation and/or valve regurgitation., Competing Interests: Declaration of competing interest The authors report no potential conflict of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Half a century after the first pulmonary autograft operation (Ross operation), performed in 1967 by Donald Ross in central London, there is a very strong conviction that the Ross operation is the best available valve substitute today, not only for children, but also for younger and older adults. The Ross operation has stimulated a lot of science to do with tissue-engineering and biology of heart valves, which is a promising avenue for the future. For one of us (M.Y.), it has certainly been a privilege to be associated with the comeback of the Ross operation., Competing Interests: Declaration of competing interest None., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

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. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The Ross procedure in patients with aortic regurgitation (AR) has been associated with increased autograft dilatation and late reintervention. The aim of this study was to evaluate the impact of a tailored approach aimed at mitigating that risk on early changes in autograft root dimensions following the Ross procedure in patients with AR. METHODS From 2011 to 2018, 241 consecutive patients underwent a Ross procedure with >1 year of follow-up [46 (7) years]. Aortic root dimensions were prospectively measured on serial echocardiograms. Patients with aortic stenosis group (n  = 171; 71%) were compared to those with AR or mixed aortic disease (AR group) (n  = 70; 29%). Mean length of follow-up was 29 ± 11 months (100% complete). Changes in aortic dimensions were analysed using mixed-effect models. RESULTS At 4 years, mean indexed diameters of the annulus, sinuses of Valsalva and the sinotubular junction in the AR group were 12.3 (0.2) mm/m2, 20.0 (0.4) mm/m2 and 16.3 (0.9) mm/m2, respectively, vs 11.9 (0.2), 18.4 (0.3) and 15.5 (0.5) in the aortic stenosis group. Overall, there were no significant differences in the rates of autograft annulus, sinuses of Valsalva and sinotubular junction dimension changes between the aortic stenosis and AR groups up to 4 years after surgery (P  = 0.55, P  = 0.12, P  = 0.59 and P  = 0.48, respectively). CONCLUSIONS Use of a tailored surgical approach, combined with a strict blood pressure control, appears to mitigate clinically significant early dilatation of the autograft root following a Ross procedure in patients with AR. Further follow-up is needed to determine if this will translate into a lower incidence of long-term reintervention. [ABSTRACT FROM AUTHOR]

Introduction: Although the Ross procedure offers several advantages over standard prosthetic AVR, its use remains limited. The risk of pulmonary autograft dilatation requiring reintervention remains one of the main concerns. Consequently, multiple techniques have been developed in attempt to mitigate this complication. Areas covered: This article reviews the incidence of pulmonary autograft dilatation, its risk factors and pathophysiology. The techniques of external pulmonary autograft support are discussed along with their respective advantages and limitations. Finally, future areas of research and developments are examined. Expert opinion: The risk of autograft dilatation is mainly prevalent in patients with aortic regurgitation and a dilated aortic annulus. In these selected patients, an external support may prevent dilatation of the autograft. However, any permanent support potentially restricts autograft root motion, mitigating some of the advantages associated with the Ross procedure. A bioresorbable matrix that could support the root during its initial adaptative phase could alleviate this problem. In our opinion, aggressive blood pressure control during the first postoperative year along with annular and sino-tubular junction support in selected patients provides optimal stability of autograft root dimensions while preserving root dynamics. Serial imaging and clinical follow-up are necessary to define the role of these various strategies. [ABSTRACT FROM AUTHOR]

Aortic valve (AV) disease in pediatric patients requires a complex decision process that has an impact on decades of life. The aim of this review is to summarize the current evidence surrounding AV interventions in this patient population. In neonates with critical aortic stenosis, the relative merit of surgical vs balloon valvuloplasty is debated and practices vary depending on centers' experience with little comparative literature. In children and adolescents, AV repair has regained interest in the last decades with encouraging early and mid-term results. The Ross procedure represents the best AV replacement option as it offers growth potential, excellent hemodynamics, low rates of endocarditis, and thromboembolism without the risks of anticoagulation. Based on contemporary literature, we propose a management algorithm for children AV disease. [ABSTRACT FROM AUTHOR]

View large Download slide View large Download slide OBJECTIVES Since 1967, the Ross procedure has been performed to treat aortic valve disease using homografts for pulmonary valve replacement. The decellularized Matrix P® prosthesis was developed to overcome (some) limitations of homografts. Until now, the long-term outcome data have been unavailable. METHODS Between 2002 and 2010, the Ross procedures using the Matrix P prosthesis were performed in 492 adult patients (mean age 57.2 ± 10.6 years, range 21–73 years) at our institution. Patient data were prospectively collected and analysed (3617.3 patient-years, mean follow-up 7.7  ±  4.3 years). Completeness of follow-up at 1, 5 and 10 years was 98.4%, 94.5% and 91.0%, respectively. RESULTS Hospital mortality was 3.9% (n   = 19). During follow-up, 121 patients died resulting in a survival rate at 5, 10 and 12.5 years of 82.8  ±  1.7%, 70.4  ±  2.3% and 62.4  ±  2.9%, respectively. Echocardiography revealed a high incidence of relevant dysfunction of the Matrix P prosthesis and subsequent right ventricular failure. Primary reoperation/reintervention was necessary for 150 Matrix P and 48 autografts. Freedom from pulmonary valve reoperation at 5, 10 and 12.5 years was 76.2  ±  2.1%, 58.6  ±  2.9% and 53.4  ±  3.4%, respectively. The autograft function and the left ventricular function showed similar results as previously reported with a freedom from autograft reoperation at 5, 10 and 12.5 years of 91.8  ±  1.4%, 86.1  ±  2.0% and 86.1  ±  2.0%, respectively. CONCLUSIONS The Matrix P prosthesis used for the right ventricular outflow tract reconstruction in the Ross procedure showed unfavourable long-term echocardiographic results with a high rate of reoperation/reintervention for structural pulmonary valve failure. As a consequence, long-term survival of this patient cohort was impaired. Based on these findings, the use of the Matrix P prosthesis for pulmonary valve replacement for Ross procedures in adults should not be recommended. [ABSTRACT FROM AUTHOR]

View large Download slide View large Download slide OBJECTIVES The aim of this study was to evaluate the long-term durability and function of pulmonary homografts used for right ventricular outflow tract reconstruction in the Ross procedure at a single centre with 25 years of experience. METHODS The study included 274 patients (212 male patients and 62 female patients; age 3–59 years), who underwent the Ross procedure between 1991 and 2014. Homograft-related complications and reinterventions were assessed. Homograft haemodynamic function was determined using transthoracic echocardiography undertaken by a single cardiologist. RESULTS The all-cause 30-day mortality was 1.1% (3 patients), and there were 17 late deaths. One death was associated with a homograft-related complication. During the observation period (median 13.3 years; 3327.5 cumulative patient-years), 21 patients (7.7%) underwent at least 1 right ventricular outflow tract reintervention. Freedom from homograft reintervention was 95.6%, 90.4% and 87.5% at 10, 15 and 20 years, respectively. Paediatric patients had a significant lower rate of freedom from reintervention (log-rank P  <   0.001). Remarkably, all patients who underwent reintervention were male (log-rank P  =   0.009). Female patients received homografts with a significantly higher (P  < 0.001) indexed diameter than male patients, which might be causally related to absent reinterventions in women. The linearized rate of homograft endocarditis was 0.2% per patient-year. At the latest echocardiography (median follow-up time 14.7 years; 164 patients), the peak transhomograft pressure gradient was <40 mmHg in 150 patients (91.5%), and homograft incompetence was none or trivial in 111 patients (67.7%), mild in 49 patients (29.9%) and moderate in 3 patients (1.8%). In 1 patient (0.6%), it was not possible to determine the degree of incompetence. Younger patient age (P  <   0.001), a smaller homograft diameter (P  =   0.014) and an increase in the body surface area during the follow-up time (P  =   0.006) were significantly correlated with a higher peak transhomograft pressure gradient. Men had a significantly higher peak transhomograft pressure gradient than women (P  = 0.018). CONCLUSIONS Pulmonary homografts provide very satisfying long-term results after the Ross procedure. Differences in long-term performance are related to undersizing and young age. [ABSTRACT FROM AUTHOR]

Progressive dilatation of the pulmonary autograft is one of the greatest concerns after the Ross procedure. Increased stress in the arterial wall may cause changes in the elastic properties of the pulmonary autograft, and thus lead to pathological dilatation. The present study aimed to investigate the changes in the autograft diameter and stiffness during follow-up after the Ross procedure. A total of ten patients underwent the Ross procedure at our institution between 2003 and 2011. Echocardiography was used to measure the diameters of the pulmonary autograft at the level of the annulus, sinus of Valsalva, and sinotubular junction. The stiffness index was calculated from the angiographic data, and compared with that of 16 age-matched control children. The diameters of the pulmonary autograft increased throughout the follow-up period, particularly at the level of the sinus of Valsalva and at the sinotubular junction. The aortic root was stiffer in Ross patients compared with control children (7.9 ± 1.8 vs. 3.9 ± 0.7 immediately postoperatively, p < 0.01; 10.1 ± 2.8 vs. 4.2 ± 1.4 at 5 years postoperatively, p < 0.01). Although no significant relationship was found between the stiffness index and the autograft diameter, the stiffness index tended to increase over time. Dilatation of the pulmonary autograft was accompanied by progressive change in aortic stiffness. Longer follow-up is warranted to clarify the impact of this change in aortic stiffness on autograft failure. [ABSTRACT FROM AUTHOR]

Abstract Objective The study objective was to examine the long-term results of the Ross procedure in a cohort of patients followed prospectively for more than 2 decades. Methods From 1990 to 2004, 212 consecutive patients with a median age (interquartile range) of 34 years (28-41) underwent the Ross procedure; 82% had congenital aortic valve disease. The technique of aortic root replacement was used in one half of the patients. Patients have been followed prospectively for a median (interquartile range) of 18.0 (14.6-21.2) years. Valve function was assessed by echocardiography. Results Cumulative mortality at 20 years was 10.8% (95% confidence interval, 6.5-17.8). Thirty patients required Ross-related reoperations and 3 for coronary artery disease. The cumulative probability of Ross-related reoperations at 20 years was 16.8% (95% confidence interval, 11.3-24.5), on the pulmonary autograft was 11.5% (95% confidence interval, 7.2-18.0), and on the pulmonary homograft was 8.2% (4.6-14.7). The implantation technique was not associated with the cumulative incidence of reoperations on the pulmonary autograft. The development of moderate or severe aortic insufficiency and pulmonary homograft dysfunction increased with time. At 20 years, the probability of aortic insufficiency was 13% (95% confidence interval, 8.0-20.3) and of pulmonary homograft dysfunction was 19.7% (95% confidence interval, 13.9-27.2). Preoperative aortic insufficiency was associated with increased odds of postoperative aortic insufficiency. Conclusions The long-term results of the Ross procedure are excellent regardless of the implantation technique, but there is a progressive deterioration of function of both semilunar valves. [ABSTRACT FROM AUTHOR]

In the sixties, Dr Donald Ross designed a surgical solution for young patients with aortic valve disease by using the patients' own pulmonary valve. The Ross procedure is the only aortic valve replacement technique that can restore long-term survival and preserve quality of life. The main failure mode of the Ross procedure is wall dilatation, potentially leading to valve regurgitation and leakage. Dilatation occurs due to the inability of the pulmonary autograft to adapt to the sudden increase in loading when exposing to aortic pressures. Previous experimental data has shown that a permanent external support wrapped around the artery can prevent the acute dilatation of the arterial wall. However, the textile support leads to stress-shielding phenomena due to the loss of mechanical wall compliance. We present a pragmatic and modular computational framework of arterial growth and remodeling predicting the long-term outcomes of cardiovascular tissue adaptation, with and without textile wrapping. The model integrates mean, systolic and diastolic pressures and assumes the resulting wall stresses to drive the biological remodeling rules. Rather than a single mean pressure or stress deviation from the homeostatic state, we demonstrate that only pulsatile stresses can predict available experimental results. Therefore, we suggest that a biodegradable external support could induce benign remodeling in the Ross procedure. Indeed, a biodegradable textile wrapped around the autograft fulfills the trade-off between prevention of acute dilatation on the one hand and recovery of arterial wall compliance on the other hand. After further validation, the computational framework can set the basis for the development of an actual biodegradable external support for the Ross procedure with optimized polymer mechanical properties and degradation behavior. [Display omitted] • Wall pulsatility is a driver of growth and remodeling in arterial tissue. • An optimized external support promotes benign adaptation in the Ross procedure. • Textile mechanical properties will prevent acute dilatation. • Polymer biodegradability can restore arterial wall compliance. [ABSTRACT FROM AUTHOR]

Aortic valve repair and the Ross procedure are widely used in children; however, it is unclear which provides the best outcomes. Patients who underwent primary aortic valve surgery from 1980 to 2018 were included. Propensity score matching was performed to adjust for baseline differences. Of 415 children, 82.7% (343/415) underwent repair and 17.3% (72/415) underwent the Ross procedure. At 15 years, survival was higher for aortic valve repair (93.9% ± 1.8% vs 80.9% ± 6.4%, P =.04); freedom from reoperation (45.7% ± 4.9% vs 48.5% ± 9.0%, P =.29) did not differ, and freedom from aortic valve reoperation was higher in the Ross procedure group (45.7% ± 4.9% vs 70.7% ± 8.0%, P <.001). When analyzed by quality of repair, acceptable repair provided the highest survival (P =.01). Acceptable repair and the Ross procedure had similar freedom from reoperation at 15 years, whereas suboptimal repair performed worse (acceptable: 54.9% ± 6.7%; Ross procedure: 48.5% ± 9.0%; suboptimal: 27.0% ± 7.7%, P <.001). Acceptable repair and the Ross procedure had similar freedom from aortic valve reoperation at 15 years, whereas suboptimal repair showed worse results (acceptable: 54.9 ± 6.7; Ross procedure: 70.7% ± 8.0%; suboptimal: 27.0% ± 7.7%, P <.001). Propensity score matching paired 66 patients who underwent the Ross procedure with 198 patients who underwent repair. At 15 years, repair was associated with better survival (98.0% ± 1.2% vs 78.5% ± 7.2%, P =.03), whereas freedom from reoperation was similar (42.6% ± 7.6% vs 50.7% ± 9.8%, P =.50). However, the Ross procedure was associated with higher freedom from aortic valve reoperation (42.6% ± 7.6% vs 72.3% ± 8.5%, P =.002). Primary aortic valve repair was associated with better survival than the Ross procedure, whereas overall freedom from reoperation was similar. When an acceptable intraoperative result was achieved, outcomes of repair were favorable. However, when the intraoperative result of repair was suboptimal, the Ross procedure showed better results. [Display omitted] [ABSTRACT FROM AUTHOR]

Background The pulmonary autograft is the ideal aortic valve substitute in young patients with aortic valve disease. Performing Ross procedure in patients with rheumatic heart disease is controversial as the autograft might be susceptible to recurrent rheumatic activity and early autograft dysfunction. The aim of the study is to evaluate the durability of the pulmonary autograft in series of patients with rheumatic aortic valve disease. This is a retrospective study enrolling patients who were admitted for Ross procedure and had a preoperative diagnosis of rheumatic heart disease from October 2009 to July 2021. Preoperative, intraoperative and postoperative data were collected from hospital records. Follow-up transthoracic echocardiogram was used to assess the autograft function, and Kaplan–Meier Curve was plotted to evaluate freedom from autograft dysfunction.Results The study included 28 patients who had Ross procedure with root replacement technique. The primary valve derangement is aortic regurgitation, and concomitant mitral disease requiring surgery was found in 32% of the patients. No operative mortality was recorded, and only one patient had late death throughout the follow-up period. Follow-up was complete in 75% of the cohort and the median follow-up was 4.75 years (range from 2 to 11.9 years). Freedom from any autograft dysfunction was 94.7% at 10 years. None of the patients required reintervention for the pulmonary autograft. Three patients had a total of 4 reinterventions; 2 for the homograft and 2 for the mitral valve.Conclusions Ross procedure remains a good option for replacing the aortic valve in young patients with rheumatic heart disease in the light of the good durability of the pulmonary autograft and the superior survival. Although longer follow-up duration and bigger sample size is needed to draw more significant results, the excellent initial outcomes guided by experienced hands should promote the use of Ross procedure in such cohort of patients.