Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Tailored Approach.

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.

Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Supported Ross.

The Ross procedure is an excellent option for aortic valve replacement resulting in outstanding hemodynamic performance and the ability to avoid systemic anticoagulation. The long-term durability of the autograft is generally good but concerns for later aortic root dilation with ensuing neoaortic insufficiency have prompted efforts to stabilize the autograft, root, sinuses and Sino-tubular junction in order to delay or entirely avoid late reinterventions on the neoaortic root. We have employed an inclusion technique, supporting the Auto-graft in a Terumo Gelweave™ Valsalva graft. We performed a retrospective study of all 129 patients undergoing the Ross procedure from 1992 to 2019 at Children's Wisconsin. Fifty-one underwent the supported Ross (SR) and 78 underwent unsupported Ross (UR). Structured clinical data was collected and echocardiograms were reviewed. Median follow-up was 4.9 years (up to 22.6 years) for UR patients and 3.6 years (up to 11.4 years) for SR patients. In order to provide a fair comparison, we sub -analyzed patients aged 10 to 18 years who underwent the Ross procedure, 16 who underwent the UR and 18 patients who underwent the SR. Change in aortic annulus diameter (P = 0.002), aortic sinus diameter (P = 0.001) change in left ventricular function (P = 0.039) and change in aortic insufficiency (P = 0.008) were all worse in UR. The SR is simple, reproducible, and predictable. It seems to prevent change in annulus diameter, sinus diameter and to reduce late neoaortic insufficiency. Longer follow-up with a larger group of patients is required to draw definitive conclusions.

Surgical Heritage: You Had to Be There, Ross: The Comeback Kid.

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.

The Ross Procedure in Adolescence and Beyond: Are There Still Contraindications?

The Ross procedure is an excellent operation to treat children and adults with aortic valve disease. Compared to prosthetic aortic valve replacement, it provides important clinical benefits in terms of survival, hemodynamics, freedom from valve-related complications, and durability, especially in women of childbearing age. However, the Ross procedure is a longer and technically more challenging operation. As a result, the choice of procedure should be driven by patient anatomy and clinical characteristics. This highlights the importance of concentrating care in Ross reference centers where surgical expertise and experience are present to ensure patient safety and long-term effectiveness of the operation. This manuscript reviews the major and relative contraindications to the Ross procedure.

New insights into the hemodynamics of pulmonary homograft patients under stress echocardiography: The contribution of pressure recovery.

BACKGROUND: The importance of pulmonary artery pressure recovery (PR) in patients with Ross procedures in whom a homograft substitutes the resected pulmonary valve, is unknown. The aim of the study was to evaluate the occurrence and extent of PR in the pulmonary artery in 65 asymptomatic patients with pulmonary homograft after Ross surgery during rest and exercise. METHODS: Stress echocardiography was performed in 65 pulmonary homograft patients and 31 controls with native pulmonary valves up to 75 W. Right ventricular systolic pressure (RVSP), transvalvular flow, mean pressure gradient (Pmean ), valve resistance, and RV stroke work were determined in the exercise (max. 75 W) and recovery phases in increments of 25 W each. RESULTS: Pulmonary homografts demonstrated significantly elevated Pmean compared to controls at all stages. When considering pressure recovery (absolute and relative PR at rest 3.8 ± 1.8 mm Hg, 42.6 ± 7.2%, respectively) and transvalvular energy loss (EL; at rest 4.5 ± 4.3 mm Hg) the homograft hemodynamics reached the level of controls. In a subgroup of patients with tricuspid regurgitation, resting RVSP was the same in homograft patients and controls (21.3 ± 6.1 vs. 20.4 ± 6.3, p = .62), despite significant different Pmax values. CONCLUSIONS: Ross patients with pulmonary homograft showed systematically increased hemodynamic parameters compared to normal pulmonary valves. These differences were abolished when PR was considered for homograft patients. The equality of RVSP values at rest in both groups shows non-invasive evidence for PR in the pulmonary system after homograft implantation. Therefore, PR appears to be an important measure in calculating the actual hemodynamics in pulmonary homografts.

Quality of life after Ross procedure in children.

INTRODUCTION: Health-related quality of life in children who have undergone the Ross procedure has not been well characterised. The aim of this study was to characterise health-related quality of life in this cohort and compare to children with other CHD. METHOD: In this cross sectional, single-centre study, health-related quality of life was assessed in patients who underwent a non-neonatal Ross procedure using the Pediatric Quality of Life Inventory. Ross cohort scores were compared with healthy norms, patients with CHD requiring no surgical intervention or had curative surgery (Severity 2, S2) and patients who were surgically repaired with ≥1 surgical procedure and with significant residual lesion or need for additional surgery (Severity 3, S3). Associations between Pediatric Quality of Life Inventory score and patient factors were also examined. RESULTS: 68 patients completed surveys. Nearly one-sixth of patients had overall scores below the cut-off for at-risk status for impaired health-related quality of life. There was no difference in overall health-related quality of life score between the Ross cohort and healthy children (p = 0.56) and S2 cohort (p = 0.97). Health-related quality of life was significantly higher in the Ross cohort compared to S3 cohort (p = 0.02). This difference was driven by a higher psychosocial health-related quality of life in the Ross cohort as compared to S3 cohort (p = 0.007). Anxiety scores were significantly worse in the Ross cohort compared to both S2 (p = 0.001) and S3 (p = 0.0017), respectively. CONCLUSION: Children who have undergone a Ross procedure report health-related quality of life equivalent to CHD not requiring therapy and superior to CHD with residual lesions. Despite these reassuring results, providers should be aware of potential anxiety among Ross patients.

The Ross Procedure in Children: Defining the Optimal Age.

BACKGROUND: It has been proposed that delaying the Ross procedure to later in childhood, allowing autograft stabilisation and placement of a larger pulmonary conduit, may improve outcomes. However, the effect of age at the time of Ross procedure on outcomes remains unclear. METHODS: All patients who underwent the Ross procedure between 1995 and 2018 were included in the study. Patients were divided into four groups: infants, age 1 to 5 years, age 5 to 10 years and age 10 to 18 years. RESULTS: A total of 140 patients underwent the Ross procedure in the study period. Early mortality was 23.3% (7/30) for infants compared to 0% for older children (p<0.001). Survival at 15 years was significantly lower in infants (76.3%±9.9%), compared to children aged 1 to 5 years (90.9%±20.1%), 5 to 10 years (94%±13.3%), and 10 to 18 years (86.7%±10.0%), p=0.01. Freedom from autograft reoperation at 15 years was significantly lower in infants (58.4%±16.2%), compared to children aged 1 to 5 years (77.1%±14.9%), 5 to 10 years (84.2%±6.0%) and 10 to 18 years (87.8%±9.0%), p=0.01. Overall freedom from reoperation at 15 years was 13.0%±6.0% for infants, 24.2%±9.0% for children aged 1 to 5 years, 46.7%±15.8% for children aged 5 to 10 years, and 78.4%±10.4%, p<0.001. CONCLUSIONS: The Ross procedure performed after 10 years of age appears to be associated with improved freedom from reoperation, primarily due to a reduction in reoperation on the pulmonary conduit.

Biological Surgical Options in Young Patients for the Treatment of Severe Aortic Stenosis: Is the Jury Still Out? A Review.

Aortic interventions remain the most effective treatment for severe aortic stenosis. In the recent years, advances in bioprosthetics and newer data have reduced the cut-off age for the use of bioprosthetic valves in younger patients, but the debate on whether to favor mechanical valves in younger patients remains a constant, especially with the undesired effects and considerations of anticoagulation therapy with vitamin K antagonists in this age group. Other options like the Ross procedure are gaining traction, despite still being undervalued and necessitating expertise centers. Hemodynamic considerations and durability of these options are important to consider, especially in this age group. Regardless of the choice of the prosthesis, patient informed consent is paramount since the decision affects the lifetime management of their initial condition, and expectations given must remain realistic.

Surgical Management of Complex Aortic Valve Disease in Young Adults: Repair, Replacement, and Future Alternatives.

The ideal aortic valve substitute in young adults remains unknown. Prosthetic valves are associated with a suboptimal survival and carry a significant risk of valve-related complications in young patients, mainly reinterventions with tissue valves and, thromboembolic events and major bleeding with mechanical prostheses. The Ross procedure is the only substitute that restores a survival curve similar to that of a matched general population, and permits a normal life without functional limitations. Though the risk of reintervention is the Achilles' heel of this procedure, it is very low in patients with aortic stenosis and can be mitigated in patients with aortic regurgitation by tailored surgical techniques. Finally, the Ozaki procedure and the transcatheter aortic valve implantation are seen by many as future alternatives but lack evidence and long-term follow-up in this specific patient population.

Bicuspid Aortic Valves: an Up-to-Date Review on Genetics, Natural History, and Management.

PURPOSE OF REVIEW: Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. It has a wide spectrum of clinical manifestations including aortic regurgitation (AR), aortic stenosis, and an associated aortopathy with a small but increased risk of aortic dissection. This review describes current knowledge of BAV, from anatomy and genetics to a discussion of multifaceted strategies utilized in the management of this unique patient population. This review will also highlight critical knowledge gaps in areas of basic and clinical research to enhance further understanding of this clinical entity. RECENT FINDINGS: The current knowledge regarding pathophysiologic mechanisms, screening, and surveillance guidelines for BAV and the associated aortopathy is discussed. We also discuss current management techniques for aortic valve repair versus replacement, indications for aortic surgery (root or ascending aorta), and the emergence of the Ross procedure as a viable management option not only in children, but also in adolescents and adults. The varied clinical phenotype of the BAV, resulting in its specific complex hemodynamic interactions, renders it an entity which is separate and distinct from the tricuspid aortic valve pathologies. While various aortic histopathologic and protein alterations in BAV patients have been described, it remains unclear if these changes are causal or the result of hemodynamic alterations imposed by sheer stress on the intrinsically dysfunctional BAV. Medical management for patients with BAV with AS, AI, or dilated aortic roots/ascending aortas remains challenging and needs further investigation. More than 50% of patients with BAV will undergo AVR during their lifetime, and more than 25% of patients with BAV undergo aortic surgery performed for dilation of the aortic root or ascending aorta, often concurrently with AVR. The search for the ultimate genetic or epigenetic cause of the different bicuspid phenotypes will ultimately be facilitated by the next-generation sequencing tools that allow for study of large populations at low cost. Improvements in diagnostic and stratification criteria to accurately risk assess BAV patients are critical to this process.

Rescue ross procedure and mitral valve repair on a low birth weight preterm neonate.

Although mid- and long-term outcomes after the Ross procedure for aortic valve disease have been increasingly improving over the years, this is still a rather challenging operation in neonates and small children. This is particularly true for patients with associated congenital heart defects and critical clinical conditions. Herein we describe the application of this procedure as a rescue operation in emergency circumstances in a low-birth-weight neonate with severe aortic stenosis, aortic regurgitation and mitral regurgitation after a previous aortic coartectomy.

Ross procedure for acute infective endocarditis.

Surgical treatment of infective endocarditis remains a challenge, with concerns of optimal prosthesis selection and risks of recurrent infection remaining paramount. The pulmonary autograft has unique features which may make it the ideal aortic valve substitute, especially in infectious endocarditis. We describe strategic considerations and technical details in performing a Ross procedure in a young patient with acute aortic valve endocarditis.

Right ventricular outflow tract reconstruction with Medtronic Freestyle valve in the Ross procedure: A systematic review with meta-analysis.

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.

The Ross Procedure in Children: The Gold Standard?

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.

Longitudinal Changes in Exercise Capacity in Patients Who Underwent Ross Procedure and Mechanical Aortic Valve Replacement: Does the Type of Surgery Matter?

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.

From Other Journals: A Review of Recent Articles in Pediatric Cardiology.

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.

Impact of Valve Type (Ross vs. Mechanical) on Health-Related Quality of Life in Children and Young Adults with Surgical Aortic Valve Replacement.

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.

Partial Ross procedure for aortic valve repair in children with bicuspid aortic valve: An original surgical technique.

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.

Does the pattern of bicuspid aortic valve leaflet fusion determine the success of the Ross procedure?

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.

Neo-aortic Root Dilatation, Aortic Stiffness, and Ventricular interactions in Long-Term Follow-Up After the Ross Procedure in Childhood.

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.