Outcomes after Ross procedure in adult patients: A meta-analysis and microsimulation.

OBJECTIVE: We conducted a meta-analysis to estimate the risk of adverse events, life expectancy, and event-free life expectancy after the Ross procedure in adults. METHODS: We searched databases for reports evaluating the Ross procedure in patients aged more than or equal to 16 years of age. A microsimulation model was used to evaluate age- and gender-specific life expectancy for patients undergoing the Ross procedure. RESULTS: Data were pooled from 63 articles totaling 19 155 patients from 20 countries. Perioperative mortality was 2.5% (95% confidence interval [CI]: 1.9-3.1; N = 9978). We found a mortality risk of 5.9% (95% CI: 4.8-7.2) at a mean follow-up of 7.2 years (N = 7573). The rate of perioperative clinically significant bleeding was 1.0% (95% CI: 0.1-3.0); re-exploration for bleeding 4.6% (95% CI: 3.1-6.3); postoperative clinically significant bleeding from 30 days until a mean of 7.1 years was 0.5% (95% CI: 0.2-1.0). At a mean of 6.9 years of follow-up, reintervention rate of any operated valve was 7.9% (95% CI: 5.7-10.3). The risk of valve thrombosis was 0.3% (95% CI: 0.2-0.5) at 7.6 years; peripheral embolism 0.3% (95% CI: 0.2-0.4) at 6.4 years; stroke 0.9% (95% CI: 0.7-1.2) at 6.5 years; and endocarditis 2.1% (95% CI: 1.6-2.6) at 8.0 years. Microsimulation reported a 40-year-old undergoing the Ross procedure to have a life expectancy of 35.4 years and event-free life expectancy of 26.6 years. CONCLUSIONS: Ross procedure in nonelderly adults is associated with low mortality and low risk of adverse events both at short- and long-term follow-up. The surgical community must prioritize a large, expertize-based randomized controlled trial to definitively address the risks and benefits of the Ross procedure compared to conventional aortic valve replacement.

Hybrid palliation to promote growth of left ventricle and left ventricular outflow tract.

OBJECTIVES: In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS: We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS: In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross-Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS: Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed.

Transpericardial resection of a mediastinal paraganglioma.

Paragangliomas are tumours originating from the autonomic nervous system and rarely occur in the chest. They may manifest through symptoms of excess catecholamine release or due to local compression, can be an incidental finding on a computed tomography/magnetic resonance imaging examination or can be found when screening patients with specific gene mutations. Surgical removal is indicated in case of symptoms, (imminent) compression of vital structures or to prevent progression to malignancy. Resection of a paraganglioma in the middle mediastinum can be challenging. The relationship of the tumour to vital structures and its blood supply determine the surgical access route. In this case report, a large paraganglioma situated in the middle mediastinum is resected. Because of the close relationship to vital structures and the presence of feeding arteries from the aortic arch, a transsternal transpericardial approach is chosen. After a median sternotomy, stepwise dissection between the aorta, superior caval vein and right pulmonary artery and the opening of the posterior pericardium gives access to the middle mediastinum and the area between the tracheal bifurcation and the left atrial roof. These steps can be done without cardiopulmonary bypass. After identification and division of the feeding aortic arch arteries, the highly vascularized tumour can be further dissected and removed.

Aortic root replacement with the stentless Freestyle bioprosthesis.

In this video tutorial, the technical details for the implantation of the Freestyle stentless bioprosthesis are outlined based on the case of a 76-year-old male patient with symptomatic stenosis of a bicuspid aortic valve and aortic root dilatation.

Left heart growth and biventricular repair after hybrid palliation.

OBJECTIVES: We evaluated the outcomes of biventricular repair after initial hybrid palliation performed in small infants with various forms of left ventricle hypoplasia. METHODS: Between September 2010 and January 2020, a total of 27 patients had biventricular repair after hybrid palliation at a median age of 11 days. Indications for the hybrid approach included growth promotion of the left ventricle outflow tract and/or the aortic valve in 14 patients and that of the left ventricle in 13 patients. Seven reinterventions and 7 reoperations were performed during the interstage period. Significant growth of left ventricle parameters was noted during the median interstage period of 62 days. Sixteen subjects had aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients had the Ross-Konno procedure; 5 patients underwent the Yasui procedure; and 1 patient had unbalanced atrioventricular septal defect and aortic arch repair. RESULTS: Twenty-three patients (85.2%) are alive at a median follow-up of 3.3 years. Two and 3 patients died early and late after achieving biventricular circulation, respectively. There were 22 reinterventions and 15 reoperations after biventricular repair. CONCLUSIONS: Hybrid palliation can stimulate left heart growth in some patients with left ventricle hypoplasia. More patients may eventually achieve biventricular circulation than was initially thought. Additional interventions and operations are foreseeable. Despite ventricular rehabilitation, some patients with borderline left ventricles may develop restrictive physiology.

Biventricular repair after the hybrid Norwood procedure.

OBJECTIVES: We analysed the outcomes of patients undergoing biventricular repair (BVR) after an initial hybrid Norwood approach as a salvage procedure in extremely sick infants; or as the initial palliation in patients with uncertain feasibility of single-stage BVR due to severe left ventricular outflow tract obstruction; or as part of a left ventricle (LV) recruitment strategy in patients with borderline LVs. METHODS: Between September 2010 and July 2018, 26 patients underwent BVR after initial hybrid palliation at a median age of 13 days. The rationale for the hybrid approach was to promote the growth of the LV in 10 patients and that of the left ventricular outflow tract and/or aortic valve in 12 patients and to be a salvage procedure in 4 patients. Significant growth of the LV was noted during the interstage period, which had a median length of 65 days (P = 0.008). Fourteen patients underwent aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients underwent the Yasui procedure; 4 patients had the Ross-Konno procedure; 2 patients had an arterial switch operation; and 1 patient had truncus arteriosus repair. RESULTS: Twenty-two patients (84.6%) are alive at a median follow-up period of 1.8 (range 0.04-6.2) years. There were 2 early and 2 late deaths. Nineteen catheter-based reinterventions and 15 reoperations were performed after BVR. CONCLUSIONS: The hybrid Norwood procedure permits stabilization of critical infants. It allows for growth of left ventricular structures in some patients with borderline left hearts and in those with severe left ventricular outflow tract obstruction. More patients may eventually have BVR than was thought during the newborn period.

Reinterventions after freestyle stentless aortic valve replacement: an assessment of procedural risks.

OBJECTIVES: Repeat aortic valve interventions after previous stentless aortic valve replacement (AVR) are considered technically challenging with an increased perioperative risk, especially after full-root replacement. We analysed our experience with reinterventions after stentless AVR. METHODS: A total of 75 patients with previous AVR using a Freestyle stentless bioprosthesis (31 subcoronary, 15 root-inclusion and 29 full-root replacement) underwent reintervention in our centre from 1993 until December 2018. Periprocedural data were retrospectively collected from the department database and follow-up data were prospectively collected. RESULTS: Median age was 62 years (interquartile range 47-72 years). Indications for reintervention were structural valve deterioration (SVD) in 47, non-SVD in 13 and endocarditis in 15 patients. Urgent surgery was required in 24 (32%) patients. Reinterventions were surgical AVR in 16 (21%), root replacement in 51 (68%) and transcatheter AVR in 8 (11%) patients. Early mortality was 9.3% (n = 7), but decreased to zero in the past decade in 28 patients undergoing elective reoperation. Per indication, early mortality was 9% for SVD, 8% for non-SVD and 13% for endocarditis. Aortic root replacement had the lowest early mortality rate (6%), followed by surgical AVR (13%) and transcatheter AVR (25%, 2 patients with coronary artery obstruction). Pacemaker implantation rate was 7%. Overall survival rate at 10 years was 69% (95% confidence interval 53-81%). CONCLUSIONS: Repeat aortic valve interventions after stentless AVR carry an increased, but acceptable, early mortality risk. Transcatheter valve-in-valve procedures after stentless AVR require careful consideration of prosthesis leaflet position to prevent obstruction of the coronary arteries.

Twenty-year experience with stentless biological aortic valve and root replacement: informing patients of risks and benefits.

OBJECTIVES: The aim of this study was to provide predictive data on the performance of the Freestyle stentless bioprosthesis that can be used to support and improve the shared decision-making process of prosthetic valve choice for aortic valve replacement. METHODS: Between 1993 and 2014, 604 patients received the Freestyle stentless bioprosthesis (143 subcoronary, 58 root inclusion and 403 full-root replacement). Perioperative data were collected retrospectively, and follow-up data were collected prospectively from 2015. Follow-up was 96% complete (median 4.3 years), with 114 (19%) patients having a follow-up period exceeding 10 years. A competing risks regression model was developed to predict the probability of mortality, structural valve deterioration (SVD) and reoperation for other causes than SVD. RESULTS: The median age of patients was 64 years, 91 (15%) patients had undergone previous aortic valve replacement and 351 (58%) underwent concomitant procedures. The 15-year probability of SVD, reoperation for other causes and death were 16.9%, 8.1% and 47.7%, respectively. Linearized occurrence rates for prosthesis endocarditis, thromboembolic events and bleeding were 0.5%, 0.9% and 0.1% per patient-year, respectively. The constructed predictive model, including age, renal function and implantation technique as significant covariates, had good to fair predictive performance up to 19 years. CONCLUSIONS: The Freestyle stentless bioprosthesis is an efficient prosthesis for aortic valve replacement or root replacement, with low incidences of SVD and valve-related events at long-term follow-up. The predictive model designed in this study can be used to fully inform patients about their expected individual trajectory after implantation of this prosthesis. This improves the shared decision-making process between patients and clinicians.

The Ross reimplantation technique.

Pulmonary autograft replacement of a diseased aortic valve (the Ross procedure) is effective in children, where growth is essential, and in young patients for whom a biological solution is preferred. Long-term outcomes are generally good. However eventual autograft dilatation may necessitate reoperation. In order to diminish the risk of autograft dilatation, several 'wrapping' techniques have been developed. Here, we present our technique of choice: the reimplantation of the pulmonary autograft in a vascular tube graft, scalloping the sinuses of Valsalva. This leaves no bulky tissue inside the vascular tube graft and makes autograft dilatation impossible.

Extensive infective endocarditis of the aortic root and the aortic-mitral continuity: a mitral valve sparing approach†.

OBJECTIVES: Severe cases of infective endocarditis (IE) of the aortic valve can cause aortic root destruction and affect the surrounding structures, including the aortic-mitral continuity, the anterior mitral valve leaflet and the roof of the left atrium. Reconstruction after resection of all infected tissue remains challenging. We describe our surgical approach and the mid-term results. METHODS: Between January 2004 and December 2015, 35 patients underwent surgery for extensive IE of the aortic valve with destruction of the aortic root, the aortic-mitral continuity and the mitral valve. Mean age was 60.4 ± 13.7; 26/35 (74%) patients had prosthetic valve endocarditis. Four patients were in critical preoperative state. Median EuroSCORE II was 18.0% [interquartile range (IQR) 11.0-26.7]. RESULTS: Aortic root replacement was performed in 32 (91%) patients. The remaining patients underwent aortic valve replacement. Reconstruction of the aortic-mitral continuity and the roof of the left atrium were performed using a folded pericardial patch. In 28 patients (80%), mitral valve repair was performed. Postoperative mechanical circulatory support, acute kidney failure and surgical re-exploration were seen in 5 (16%), 10 (31%) and 4 (13%) patients, respectively. Early survival rate was 77% (27 patients). During a median follow-up of 29.8 months (IQR 6.4-62.9), 7 (26%) patients required reintervention (3-42 months after surgery); 4 were due to mitral incompetence, early in our experience. CONCLUSIONS: Extensive IE of the aortic root with destruction of the surrounding tissues remains a complex disease with high morbidity and mortality rates. Our technique allows native mitral valve preservation but is technically challenging.

Long-Term Follow-Up After the Ross Procedure: A Single Center 22-Year Experience.

BACKGROUND: The aim of this study was to analyze long-term outcomes after the Ross procedure, focusing on autograft function and risk of reoperation in time. METHODS: Between February 1994 and February 2016, 154 patients underwent the Ross (n = 105) and Ross-Konno (n = 49) procedure at our institution and were included in this study. Data were collected retrospectively from patients' medical records or through telephone contact. Competing risks analyses were performed to determine incidences of death and reoperation. A multistate model was constructed to provide insights in the clinical trajectory after operation. RESULTS: Median age was 12 years, 74% were pediatric patients, and 66% had previous surgical procedures. There were 8 (5%) early deaths, 6 of whom underwent the Ross-Konno procedure, and 10 (7%) late deaths. Survival rates at 15 and 20 years were 86% in the total cohort and 91% in the isolated Ross subgroup. Linearized occurrence rates of endocarditis and valve thrombosis, thromboembolism, and bleeding events combined were 0.30% per patient-year and 0.15% per patient-year, respectively. Cumulative incidences of all-cause reoperation at 15 and 20 years were 35.2% and 45.3%, respectively. Twenty-six patients needed autograft reoperation, 20 due to dilatation. Cumulative incidences of autograft reoperation at 15 and 20 years were 20.1% and 31.1%, respectively. At latest echocardiogram, 4 patients had moderate aortic regurgitation and none had stenosis. CONCLUSIONS: The Ross procedure can be performed safely in young patients with low number of valve-related events. Autograft function remains stable in the first decade after operation, but autograft dilatation in the second decade necessitates reintervention.

Twenty-year experience with the Ross-Konno procedure.

OBJECTIVES: The Ross-Konno procedure is a last resort for patients with complex multilevel left ventricular outflow tract obstruction (LVOTO) often having other cardiovascular anomalies. It is typically preceded by multiple surgeries. Literature is scarce on long-term follow-up series. Therefore, we have reviewed our 20-year experience with this procedure in order to provide insights in patients' outcomes and to optimize patient selection. METHODS: Between January 1995 and December 2014, 48 patients underwent the Ross-Konno procedure. The median age at operation was 12.8 months (range, 11 days to 31 years). Twenty-two (46%) patients were under 1 year of age. Forty-four (92%) patients had undergone a total of 82 previous procedures. Eleven (23%) patients had concomitant surgery, predominantly mitral valve (n = 5) and aortic arch surgery (n = 5). RESULTS: The median follow-up time was 4.3 years (range, 0-20 years). There were 6 (12.5%) early deaths and 4 (8.3%) late deaths. Estimated overall survival at 5, 10 and 15 years was 83, 79 and 70%, respectively. Poor LV function was a risk factor for early mortality (odds ratio = 9.5; 95% confidence interval = 1.4-63.7; P = 0.020). Twelve patients required a total of 29 procedures in 17 reoperations. Five patients required reoperation for autograft failure at a median of 14 years (range, 5-15 years) postoperatively. Estimated freedom from all causes of reoperation at 5, 10 and 15 years was 82, 55 and 30%, respectively. All patients had complete and durable relief of LVOTO. At latest follow-up, 5 patients had a sinus of Valsalva Z-score of 5 or greater. One patient had Grade II autograft insufficiency. CONCLUSIONS: The Ross-Konno procedure is a durable solution for multilevel LVOTO in a highly complex patient population with high incidence of previous procedures. High early mortality rates in patients with impaired left ventricular function emphasize the importance of patient selection. Freedom from reoperation shows a continuous attrition rate. Reoperation for autograft failure may occur late after the Ross-Konno procedure.

Stentless bioprostheses: a versatile and durable solution in extensive aortic valve endocarditis.

OBJECTIVES: Infective endocarditis of the aortic valve with local aortic root destruction requires radical resection of infected tissues and subsequent reconstruction of periannular structures and the left ventricular outflow tract (LVOT). Homografts or stentless bioprostheses are recommended for use in this specific patient group. The Freestyle stentless bioprosthesis is a porcine aortic root prosthesis, which approaches the surgical versatility of the homograft, but has the advantage of ready availability and uniform quality. We assessed clinical and echocardiographic outcome following the use of this prosthesis in extensive aortic valve endocarditis. METHODS: Between June 2000 and December 2014, 55 Freestyle prostheses were implanted for aortic valve endocarditis involving the root in 54 patients (74% male). The mean age at operation was 61 ± 13 years. The mean EuroSCORE II was 20.1 ± 13.5. Twenty-nine (54%) patients had prosthetic valve endocarditis. The median follow-up time after surgery was 3.5 years, ranging from 0 to 15 years. RESULTS: Early and late mortality were 11% (6 patients) and 14% (7 patients), respectively. Estimated overall survival at 1 and 5 years was 83 and 70%, respectively. There was no survival difference between patients with native or prosthetic valve endocarditis. One patient underwent reoperation for recurrent endocarditis 2.3 years after the initial procedure. No other prosthesis failure occurred. At a median follow-up of 3.3 years, mean gradient over the prosthesis was 4.3 ± 2.3 mmHg. No patient had more than mild aortic regurgitation. CONCLUSIONS: The Freestyle stentless bioprosthesis is a valuable option to reconstruct the LVOT after debridement in extensive aortic valve endocarditis. It is readily available with a versatility and clinical outcome comparable with that of homografts. Although early mortality remains considerable in this high-risk group of patients, late survival is good with low rates of recurrence of endocarditis, immediate local control and good haemodynamic performance on echocardiography.

More than 25 years of experience in managing pulmonary atresia with intact ventricular septum.

BACKGROUND: Management of pulmonary atresia with intact ventricular septum (PAIVS) is challenging and depends on the individual patient's morphologic characteristics. We evaluated outcomes of 25 years of experience in morphologically driven management of PAIVS. METHODS: Between January 1985 and December 2011, 60 patients were treated for PAIVS. Patients were retrospectively categorized into mild (n=27), moderate (n=18), and severe (n=15) right ventricular (RV) hypoplasia subgroups based on morphologic characteristics at first presentation. Tricuspid valve (TV) annulus z score increases were analyzed in patients who underwent biventricular repair and in patients who underwent 1.5-ventricular repair. Median follow-up time was 9.8 years (range, 0-25 years). One patient was lost to follow-up. RESULTS: Fifty-nine (98.3%) patients underwent operation. There were 7 early postprocedure deaths and 1 late death. Estimated overall survival was 86.5% at 10 years after initial admission for the total group, and 96.3%, 77.8%, and 79.4% for the subgroups, respectively. In the respective subgroups, biventricular repair was achieved in 24 (88.9%), 3 (16.7%), and 0 patients. In the patients who underwent biventricular repair, there was a significant increase of the TV annulus z score. At latest follow-up, mean New York Heart Association (NYHA) class was 1.3 for the total group and 1.1, 1.2, and 1.5 for the patients who underwent biventricular, 1.5-ventricular, and univentricular repair, respectively. CONCLUSIONS: Our individualized management approach to PAIVS showed good early and long-term results. Early decision on the management goal, determined by individual morphologic characteristics at presentation, is of paramount importance. In specific patients, however, taking into account RV development at a later point in time may improve outcome.