Ventricular Outflow Tract Reintervention Research Articles

Ross procedure in neonates and infants: A valuable operation with defined limits

ObjectiveThe Ross procedure is an important tool that offers autologous tissue repair for severe left ventricular outflow tract (LVOT) pathology. Previous reports show that risk of mortality is highest among neonates and infants. We analyzed our institutional experience within this patient cohort to identify factors that most affect clinical outcome. MethodsA retrospective chart review identified all Ross operations in neonates and infants at our institution over 27 years. The entire study population was analyzed to determine risk factors for mortality and define outcomes for survival and reintervention. ResultsFifty-eight patients underwent a Ross operation at a median age of 63 (range, 9-156) days. Eighteen (31%) were neonates. Eleven (19%) patients died before hospital discharge. Multiple regression analysis of the entire cohort identified young age (hazard ratio [HR], 1.037; P = .0045), Shone complex (HR, 17.637; P = .009), and interrupted aortic arch with ventricular septal defect (HR, 16.01; P = .031) as independent predictors of in-hospital mortality. Receiver operating characteristic analysis (area under the curve, 0.752) indicated age younger than 84 days to be the inflection point at which mortality risk increases. Of the 47 survivors, there were 2 late deaths with a mean follow-up of 6.7 (range, 2.1-13.1) years. Three patients (6%) required LVOT reintervention at 3, 8, and 17.5 years, respectively, and 26 (55%) underwent right ventricular outflow tract reintervention at a median of 6 (range, 2.5-10.3) years. ConclusionsRoss procedure is effective in children less than one year of age with left sided obstructive disease isolated to the aortic valve and/or aortic arch. Patients less than 3 months of age with Shone or IAA/VSD are at higher risk for morbidity and mortality. Survivors experience excellent intermediate-term freedom from LVOT reintervention.

Evolving Solutions in the Surgical Treatment of Isolated Pulmonary Valve Stenosis

Background: Surgical treatment of isolated pulmonary valve stenosis in infants and children has evolved over the years, shifting from the original exclusive aim at lowering right ventricle pressure to the current concomitant focus on preserving pulmonary valve function. In our study, we sought to analyze the effect of such evolving philosophy on long-term results. Methods: All consecutive patients treated in our center between July 1983 and March 2019 were included. Patients were categorized into 2 groups based on the introduction into treatment practice of sparing valve techniques (1995). Actuarial survival, freedom from reintervention on the right outflow tract, transvalvular mean pressure gradient decrease, and pulmonary valve function at follow-up were analyzed. Results: One hundred twenty-three patients operated before (Group I, n=81) or since (Group II, n=42) 1995 were enrolled in the study. Mean age and weight were 3.0 ± 0.36 years and 16.6 ± 1.7kg, respectively. Early mortality occurred exclusively in 3 patients of Group 1. Transvalvular mean pressure gradient decreased in the entire patient population (from 63.28 ± 12.9mmHg to 16.46 ± 7.9mmHg). At a mean follow-up interval of 4.9 ± 33 years, freedom from death was comparable, but freedom from right ventricular outflow tract reintervention was significantly greater in Group II. Although the transvalvular gradient remained stable over time in non-reoperated survivors (mean value of 16.46 ± 7.9mmHg), pulmonary valve function on 2D-Echo showed severe incompetence in 2 patients of Group I and just mild to moderate incompetence in 20 patients of Group II, with a significantly negative effect of unsuccessful preoperative pulmonary balloon valvuloplasty (14/20 vs 6/20, p=0.025) in the latter. Conclusion: Current pulmonary valve sparing techniques are associated with better results, particularly in terms of freedom from re-interventions and pulmonary valve function at follow-up. Balloon valvuloplasty prior to surgery may worsen operative results, promoting pulmonary insufficiency and therefore should probably be avoided in all patients in whom anatomical characteristics predict failure of percutaneous therapy.

Comparison of staged repair versus single-stage complete repair for pulmonary atresia with ventricular septal defect: A systematic review and meta-analysis.

To evaluate comparative outcomes of the repair of pulmonary atresia with ventricular septal defect (PAVSD), done as staged repair (SR) or single-stage complete repair (CR). A systematic online search was conducted using the following databases: PubMed, Scopus, Cochrane database, The Virtual Health Library, Clinical trials.gov, and ScienceDirect for studies comparing SR versus CR for treatment of PAVSD. Total mortality rate, operative and early post-operative mortality rate, post-operative ventilation duration, duration of post-operative intensive care unit (ICU) stay, need for post-operative extra-corporeal membrane oxygenation (ECMO) support rate, transcatheter re-intervention rate, unplanned re-operation rate, freedom from right ventricular outflow tract (RVOT) re-intervention, and length of hospital stay (LOS) were the evaluated outcome parameters. Four comparative studies reporting a total of 264 patients who underwent SR (167 patients) or CR (97 patients) were included. Total mortality was higher in the SR group compared to the CR group (odds ratio (OR) 2.58, P = 0.03). Two groups were comparable regarding operative and early post-operative mortality (OR 1.37, P = 0.62), post-operative ventilation duration (mean difference (MD) - 1.58, P = 0.43), need for post-operative ECMO support rate (OR 4.72, P = 0.16), transcatheter re-intervention rate (OR 0.60, P = 0.53), unplanned re-operation rate (OR 0.73, P = 0.57), and LOS (MD - 3.39, P = 0.45). Higher rate of freedom from RVOT re-intervention was observed in the SR group (OR 4.16, P = 0.00001). Single-staged early CR of PAVSD significantly reduced total mortality rate as compared with SR. However, there is life-long risk of frequent need for RVOT re-interventions. Future high-quality randomised studies with robust methodological qualities are encouraged to evaluate the optimal timing and technique for repair of PAVSD, by analysing more outcomes in large patient groups and multi-centre studies.

Multicenter Study of Endocarditis After Transcatheter Pulmonary Valve Replacement

BackgroundEndocarditis has emerged as one of the most impactful adverse events after transcatheter pulmonary valve replacement (TPVR), but there is limited information about risk factors for and outcomes of this complication. ObjectivesThe purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort. MethodsThe authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes. ResultsInvestigators submitted data for 2,476 patients who underwent TPVR between July 2005 and March 2020 and were followed for 8,475 patient-years. In total, 182 patients were diagnosed with endocarditis a median of 2.7 years after TPVR, for a cumulative incidence of 9.5% (95% CI: 7.9%-11.1%) at 5 years and 16.9% (95% CI: 14.2%-19.8%) at 8 years (accounting for competing risks: death, heart transplant, and explant) and an annualized incidence of 2.2 per 100 patient-years. Staphylococcus aureus and Viridans group Streptococcus species together accounted for 56% of cases. Multivariable analysis confirmed that younger age, a previous history of endocarditis, and a higher residual gradient were risk factors for endocarditis, but transcatheter pulmonary valve type was not. Overall, right ventricular outflow tract (RVOT) reintervention was less often to treat endocarditis than for other reasons, but valve explant was more often caused by endocarditis. Endocarditis was severe in 44% of patients, and 12 patients (6.6%) died, nearly all of whom were infected with Staphylococcus aureus. ConclusionsThe incidence of endocarditis in this multicenter registry was constant over time and consistent with prior smaller studies. The findings of this study, along with ongoing efforts to understand and mitigate risk, will be critical to improve the lifetime management of patients with heart disease involving the RVOT. Although endocarditis can be a serious adverse outcome, TPVR remains an important tool in the management of RVOT dysfunction.

Reconstruction of right ventricular outflow tract stenosis and right ventricular failure after Ross procedure \u2013 comprehensive assessment of adult congenital heart disease with four-dimensional imaging: a case report

BackgroundRe-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.Case summaryA 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.DiscussionA 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.

Right Ventricular Outflow Tract Reintervention in the Transcatheter Era: Outcomes and Cost Analysis.

Surgical pulmonary valve insertion (SPVI) for re-entry right ventricular outflow tract intervention (RVOTI) remains an established and reproducible approach. Fast-track in patients undergoing RVOTI of the comprehensive valve program targets early ICU and hospital discharge (Hd). Feasibility study for outcome and cost analysis was undertaken. Between January 2015 and December 2016, 34 patients underwent re-entry RVOTI. Seventeen had SPVI and 17 transcatheter PVI (TPVI). Surgical perioperative fast-track protocol was used. Echocardiographic evaluation preoperatively (TTE-1), after RVOTI (TTE-2), at hospital discharge (TTE-3), and follow-up (TTE-4) were obtained. Cost Analysis included procedural and hospital costs. Mean follow-up period was 11.3 ± 6.9months. All patients were extubated prior to ICU arrival. Mean age was 8.5 ± 7.8 for SPVI [vs 28.5 ± 8.6years for TPVI] (p < 0.05). There was no hospital mortality or 30-day readmission for SPVI (versus 1 for TPVI).Mean hospital length of stay (LOS) was 4.1 ± 1.1days for SPVI [vs 1.1 ± 0.7days for TPVI] (p < 0.05). Number of prior sternal re-entry had no influence on outcome. RV systolic pressure referenced to LVSP (rRVSP, %) and diastolic dimension (RVEDDi, z score) showed sustainable improvement (TTE-2, TTE-3, TTE-4) in both groups compared to TTE-1 (p < 0.05). Mean total hospital cost was $5475.86 ± 2503.91 lower after SPVI (p=0.09), 21.7% procedural cost reduction. Patients undergoing RVOTI can be safely stratified, based on a customized concept, towards SPVI or TPVI. Standardized strategy can advocate a fast-track path. SPVI is associated with comparable mid-term outcomes to TPVI although SPVI is delivered in younger patients. Despite longer LOS SPVI is associated with reduced hospital cost. Multisite studies might help determine suitability for each strategy on cost containment/quality of life basis.

Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results

Tetralogy of Fallot with absent pulmonary valve syndrome (ToF-APVS) is a rare variant of tetralogy, associated with severe pulmonary valve regurgitation and aneurysmal dilation of the pulmonary arteries (PAs). Reported outcomes after surgical corrections are limited to single center, older series and might not reflect the current outcome. We aim to use data from a national registry to evaluate short- and long-term outcomes after surgical repair of ToF-APVS, to serve for counselling and planning. All children undergoing ToF-APVS repair in the UK between 2002 and 2013 were included. Survival and freedom from reintervention were estimated using the Kaplan-Meier method, and univariable analysis was done using the Weibull regression model. A total of 98 children, 45% male, 10% with DiGeorge syndrome, median age of 213 days (1 day to 13 years) were included. Mortality at 30 days was 3.3%, higher for neonates (6.7% vs 2.7%, P = 0.4) and those on preoperative mechanical ventilatory support (16.7% vs 1.3%, P = 0.04). Survival was 92.1% and freedom from pulmonary valve or conduit replacement (PVR) 73.2% at 10 years. Neonates had worse survival (hazard ratio [HR] 6.2, P = 0.02), freedom from PVR (HR 4.5, P = 0.01), freedom from PAs arterioplasty (HR 6.6, P = 0.001), and overall freedom from any reintervention (HR 5.3, P < 0.001). Low weight at repair was associated with worse freedom from PVR (P = 0.02) and from PAs arterioplasty (P = 0.009), preoperative ventilatory support with increased mortality (P = 0.009), the presence of DiGeorge syndrome was associated with worse freedom from PVR (HR 4, P = 0.02). Surgical repair of ToF-APVS can be performed with low early and late mortality, with improving results even in those with preoperative mechanical ventilatory support. The need for right ventricular outflow tract reintervention, including on the PAs, is an expected issue in the long term for the majority of patients.

The Ross Procedure: A Systematic Review, Meta-Analysis, and Microsimulation.

To support decision-making in aortic valve replacement in children and adults, we provide a comprehensive overview of outcome after the Ross procedure. A systematic search was conducted for publications reporting clinical outcome after the Ross procedure, published between January 1, 2000, and November 22, 2017. Reported event rates and time-to-event data were pooled and entered into a microsimulation model to calculate life expectancy and lifetime event risk. Ninety-nine publications were included (13 129 patients; total follow-up: 93 408 patient-years, pooled mean follow-up: 7.9±5.3 years). Pooled mean age at surgery was 9.4±5.5 years for children and 41.9±11.4 for adults. For children and adults, respectively, pooled early mortality risk was 4.19% (95% CI, 3.21-5.46) and 2.01% (95% CI, 1.44-2.82), late mortality rate was 0.54%/y (95% CI, 0.42-0.70) and 0.59%/y (95% CI, 0.46-0.76), autograft reintervention 1.28%/y (95% CI, 0.99-1.66) and 0.83%/y (95% CI, 0.68-1.01), and right ventricular outflow tract reintervention 1.97%/y (95% CI, 1.64-2.36) and 0.47%/y (95% CI, 0.37-0.59). Pooled thromboembolism and bleeding rates were low and comparable to the general population. Lifetime risks of autograft and right ventricular outflow tract reintervention were, respectively, 94% and 100% for children and 49% and 19% for a 45-year-old. Estimated life expectancy after surgery was 59 years for children (general population: 64 years) and 30 years for a 45 years old (general population: 31 years). Through excellent survival and avoidance of the burden of anticoagulation, the Ross procedure provides a unique opportunity for patients whose preferences do not align with the outcome provided by mechanical valve replacement and for growing children who also benefit from autograft diameter increase along with somatic growth. On the downside, almost all pediatric and many adult Ross patients will require a reintervention in their lifetime.

Long-term performance of pulmonary homografts after the Ross procedure: experience up to 25 years.

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. 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. 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). Pulmonary homografts provide very satisfying long-term results after the Ross procedure. Differences in long-term performance are related to undersizing and young age.

Right ventricular outflow tract reintervention after primary tetralogy of Fallot repair in neonates and young infants

ObjectiveTo assess the outcomes following primary tetralogy of Fallot (TOF) repair in neonates and young infants with pulmonary stenosis (PS) and pulmonary atresia and compare differences in reintervention on the right ventricular outflow tract (RVOT) among those undergoing valve sparing repair (VSR), transannular RVOT patch (TAP), and right ventricle–to–pulmonary artery (RV-PA) conduit surgeries. MethodsData were collected retrospectively in 101 patients who underwent TOF repair over a 10-year period between January 2005 and September 2015. The primary endpoint was reintervention on the RVOT, defined as a surgical procedure or cardiac catheterization–based RVOT reintervention. ResultsForty-three patients had TOF/PS, of whom 24 (56%) underwent TAP and 19 (44%) underwent VSR. Fifty-eight patients had TOF/PA, 14 (24%) underwent TAP and 44 (76%) underwent RV-PA conduit repair. Overall patient mortality was 2.9% (3 of 101). Thirty-three patients underwent surgical reintervention, and 52 underwent catheterization-based reintervention. Patients with TOF/PA who underwent RV-PA conduit repair had a higher surgical reintervention rate than those who underwent TAP (45% vs 21%). Patients with TOF/PSs undergoing VSR with a lower median birth weight (2.5 kg vs 3.7 kg) required more surgical reintervention. ConclusionsNeonatal TOF repair can be performed with low mortality but frequent RVOT reinterventions. Surgical reintervention is earlier and the rate is higher among patients with TOF/PA undergoing RV-PA conduit repair compared with those undergoing TAP. Although there were no overall differences in RVOT reintervention rate between patients with TOF/PS undergoing VSR and those undergoing TAP, a lower birth weight in the patients undergoing VSR is associated with a higher surgical reintervention rate.

Relationships Among Conduit Type, Pre-Stenting, and Outcomes in Patients Undergoing Transcatheter Pulmonary Valve Replacement in the Prospective North American and European Melody Valve Trials

ObjectivesThis study sought to evaluate the incidence of and risk factors for conduit and stent-related outcomes following transcatheter pulmonary valve replacement (TPVR). BackgroundStent fracture (SF) and right ventricular outflow tract (RVOT) reintervention are among the most important adverse outcomes after TPVR using the Melody valve (Medtronic, Minneapolis, Minnesota). The conduit environment and conduit preparation practices vary among patients who undergo TPVR. MethodsData from 3 prospective Melody valve multicenter studies were pooled and analyzed. All patients who had successful implant of a Melody valve that was present at hospital discharge comprised the study cohort; patients who had TPVR into a stentless conduit comprised the analysis cohort. SF was diagnosed using protocol-specified or clinical fluoroscopy or radiography, and classified as major or minor. ResultsOf 358 patients who underwent catheterization with intent to perform TPVR, 309 were discharged with the Melody valve in place (study cohort) of which 251 patients had TPVR into a stentless conduit (analysis cohort). Median follow-up was 5 years. New pre-stents were placed in 68% of patients with a stentless conduit, and 22% received multiple pre-stents. At 3 years, freedom from any SF and major SF was 74 ± 3% and 85 ± 2%, respectively, and freedom from RVOT reintervention was 85 ± 2%. New pre-stents were associated with longer freedom from SF and RVOT reintervention than was no pre-stent. ConclusionsRisks of SF and reintervention after TPVR with a Melody valve were reduced by implantation of pre-stents, which has become standard practice. This study supports pre-stenting as an important component of TPVR therapy.

Outcomes of Reintervention on the Autograft After Ross Procedure

After a Ross procedure, a small subset of patients requires reintervention for autograft dilatation or valve insufficiency. We sought to determine the indications, nature, and outcomes of autograft reinterventions in the left ventricular outflow tract after a Ross procedure. We retrospectively reviewed the charts of 316 consecutive patients, age 4 days to 70 years, who underwent a Ross procedure at our institution. Of these, 47 patients (15%) required autograft reintervention during a median follow-up of 8.2 years. Forty-seven patients, median age 26 years (interquartile range [IQR], 14.4 to 44.8 years), 32 (68%) men, required autograft reintervention a median 5.5 years (IQR, 1.8 to 9.4 years) after a Ross procedure. In 47% (22of 47 patients), the autograft valve could be salvaged (15 valve-sparing aortic replacements, 7 valve repairs). The remaining 53% underwent replacement of the root (10 mechanical, 5 homograft) or valve alone (7 mechanical, 3 bioprosthetic). Twenty patients presented without autograft root dilatation 1 year (IQR, 0.5 to 2.9 years) after Ross. Fifteen of them (75%) required valve replacement. The 27 patients who demonstrated root dilatation presented 6.9 years (IQR, 4.5 to 9.7 years; p < 0.01 compared with the nondilated group) after Ross, and 17 (63%) of these valves could be spared (p= 0.01). There was no surgical mortality and 5 (11%) major morbidity events. Patients were followed up for 4.9 years (IQR, 2.1 to 7 years) after left ventricular outflow tract reintervention. For patients whose autograft valve could be spared, 3-, 5-, and 8-year freedom from valve replacement was 92%, 86%, and 86%, respectively. At last follow-up, only 1 patient had greater than mild aortic insufficiency, and all but 1 had normal ejection fraction. Autograft reintervention after a Ross appears to follow a bimodal distribution. Patients with primary autograft leaflet problems tend to present early without root dilatation and frequently require valve replacement. The autograft valve can be salvaged in the majority of patients who present later with root dilatation. Valve-sparing procedures are durable in the intermediate term and can be accomplished with low morbidity.

Ross Procedure in Neonates and Infants: A European Multicenter Experience

Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability. A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013. Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts. Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding.

Long-Term Survival and Reintervention After the Ross Procedure Across the Pediatric Age Spectrum

There are limited data regarding long-term outcomes after the Ross procedure in children. We evaluated mortality and reintervention in a large pediatric cohort. A retrospective analysis of all patients aged younger than 18 years who underwent the Ross procedure at our institution (1991 to 2013) was conducted. Kaplan-Meier curves and Cox proportion hazard models were used to evaluate long-term outcomes and associated risk factors. Included were 240 consecutive patients undergoing a Ross/Ross-Konno procedure: 18% infants, 48% children, and 33% adolescents. Infants were more likely tohave complex left heart disease (p= 0.005). Overall survival to hospital discharge was 96%; infants had the highest mortality (18%). Long-term survival status was known for 99.6% (median follow-up, 10.7 years). Overall 15-year survival was 87% (lowest in infants, 72%; p=0.003). Reintervention status was known in 87%.Overall 15-year freedom from any left ventricular outflow tract reintervention was 59%; 85% still had their autograft valve at the latest follow-up. Left ventricular outflow tract reintervention was uncommon in infants (n= 2). Overall 15-year freedom from right ventricular outflow tract reintervention was 53%, and was lower in infants (19%) than in children (51%) and adolescents (76%; p < 0.0001). Outcomes after the Ross procedure in children vary by age. Infants more commonly have complex left heart disease and experience higher mortality but have excellent long-term autograft durability. Children and adolescents have higher rates of left ventricular outflow tract reintervention, whereas infants areathighest risk of right ventricular outflow tract reintervention.

Long-term results of the Ross procedure in a population-based follow-up.

The purpose of this study was to evaluate the long-term outcomes of the Ross procedure in a nationwide follow-up. This retrospective study involved all children treated with the Ross procedure in Finland between 1994 and 2009. The clinical records were reviewed for demographic and anatomical characteristics, Ross operation data, surgical history and status at the latest follow-up. The median follow-up time was 11.5 (range 2.4-19.2) years. Fifty-one patients underwent either the Ross (n = 37) or the Ross-Konno (n = 14) procedure at a median age of 4.8 (range 0.02-16.3) years, including 13 infants (<1 year of age). The indication for the Ross procedure was aortic valve stenosis, regurgitation or both, which was observed in 29, 24 and 47% of patients, respectively. The early mortality (before hospital discharge) rate was 10% (31% in infants) and the late mortality rate 6% (15% in infants). Higher mortality was discovered in patients treated with the Ross-Konno procedure (P = 0.001). The most common cause for reintervention was pulmonary homograft stenosis. The rate of freedom from right ventricular outflow tract reintervention was 98% at 5 years, 83% at 10 years and 59% at 15 years. The rate of freedom from autograft reintervention was 98% at 5 and 10 years, and 81% at 15 years. At the latest follow-up visit, mild-to-moderate aortic root dilatation was reported in 52% of patients, and 4 patients had undergone autograft-related reinterventions. Trivial autograft valve regurgitation was commonly seen, but only 1 patient developed severe autograft regurgitation requiring mechanical valve replacement 15.9 years after the Ross operation. The most common reason for reintervention after the Ross procedure in children is homograft stenosis. Aortic root dilatation and autograft valve regurgitation are relatively common but rarely lead to reinterventions before adulthood. Intraoperative complications and complex cardiac anatomy are associated with high mortality in infants undergoing the Ross-Konno procedure. In our centre, the Ross procedure has provided good long-term results in this challenging group of paediatric patients.

Late functional outcomes after repair of tetralogy of Fallot with atrioventricular septal defect: A double case-match control study

We sought to elucidate late functional outcomes of the right ventricular outflow tract and atrioventricular valves after repair of tetralogy of Fallot with atrioventricular septal defect. From 1990 to 2010, the data from 41 patients who underwent repair were retrospectively reviewed. The median age at repair was 22.4 months (interquartile range, 10.8-41.6 months). Of the 41 patients, 13 (32%) had received previous palliations. The preoperative anatomic differences, physiologic differences, and long-term functional outcomes were determined using a double case-matched control with isolated tetralogy of Fallot and isolated atrioventricular septal defect. The right ventricular outflow tract was reconstructed, with the pulmonary valve preserved in 23 patients (56%). There were 3 early deaths and no late deaths. Survival was 92.1% at 15 years. During a median follow-up period of 5.9 years (interquartile range, 0.14-13.7 years), 29 reinterventions were performed in 13 (32%) patients. Freedom from all reintervention at 15 years was 52.8%. Of the 29 procedures, 12 (41%) were related to the right ventricular outflow tract. Freedom from right ventricular outflow tract-related reintervention was greater in patients who had their pulmonary valve preserved (95% vs 70% at 10 years; P=.046). Reinterventions were performed for the atrioventricular valve in 2 (5%) patients and subaortic stenosis in 1 (2%) patient. Freedom from right ventricular outflow tract-related reintervention (tetralogy of Fallot with atrioventricular septal defect, 88.6% vs tetralogy of Fallot alone, 83.9% at 5 years; P=.809) and atrioventricular valve/left ventricular outflow tract-related reintervention (tetralogy of Fallot with atrioventricular septal defect, 95.2% vs atrioventricular septal defect alone, 86.0% at 5 years; P=.332) were comparable between the tetralogy of Fallot with atrioventricular septal defect and matched control groups. Late survival and atrioventricular valve function after repair of tetralogy of Fallot with atrioventricular septal defect were excellent. Pulmonary valve preservation and avoidance of an artificial conduit were associated with greater freedom from right ventricular outflow tract reintervention. In the current era, the surgically modified history of tetralogy of Fallot with atrioventricular septal defect is not significantly different from that of isolated tetralogy of Fallot or isolated atrioventricular septal defect.

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Tetralogy of Fallot With Atrioventricular Septal Defect: Surgical Strategies for Repair and Midterm Outcome of Pulmonary Valve-Sparing Approach

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.

Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence

The Ross procedure is used to treat aortic valve disease in children. The advantages include autograft growth, long-term durability, and avoidance of anticoagulation. Long-term follow-up of the Ross procedure in infancy is limited. We sought to characterize the long-term outcomes of infants undergoing the Ross procedure. We performed a retrospective review of all patients who underwent a Ross operation at 18 months of age or younger at New-York Presbyterian and Cardiothoracic Center of Monaco from 1991 to 2010. The clinical, catheterization, and surgical records were reviewed. The most recent follow-up information, including echocardiogram and electrocardiogram, was obtained and analyzed. A total of 34 patients underwent a Ross procedure at a median age of 6 months (range, 4 days to 18.4 months). All had congenital aortic stenosis. All but 1 patient had undergone previous surgical or catheter-based interventions. The median follow-up was 10.6 years (range, 1.4-20.4 years). There were 4 early deaths and 1 late transplant. The freedom from right ventricular outflow tract reintervention was 85% at 5 years and 64% at 10years. The freedom from autograft reintervention was 95.5% at 10 years. In 20 subjects, late follow-up echocardiograms showed a significant difference between the mean early and late Z scores of the autograft annulus (0.8 vs 2.4, P=.03), sinus (0.8 vs 2.8, P=.002), and sinotubular junction (1.2 vs 2.7, P=.04). Mild or less aortic insufficiency occurred in 17 subjects. None had significant aortic stenosis. The long-term outcomes of the Ross procedure in infants and toddlers are favorable despite moderate dilatation of the autograft. Reintervention at the right ventricular outflow tract is common.

Pulmonary Valve Replacement Function in Adolescents: A Comparison of Bioprosthetic Valves and Homograft Conduits

Data comparing function and reintervention rates of various pulmonary valve replacements in adolescents are sparse. New transcatheter therapies-including transcatheter pulmonary valves-have been developed, and their utilization could be justified by these data. We performed a retrospective review that included baseline and operative data with cross-sectional follow-up on adolescents aged 10 to 21 years who underwent pulmonary valve replacement (n=254). We compared homograft conduits (n=84) with bioprosthetic pulmonary valves (n=170) in terms of freedom from valve dysfunction--right ventricular outflow tract obstruction greater than 50 mm Hg, or moderate or greater pulmonary regurgitation--and reintervention. Median follow-up duration was 4.4 years and was longer in the homograft cohort. Freedom from valvar dysfunction was 72%±4% at 5 years and 48%±8% at 10 years. Freedom from right ventricular outflow tract reintervention was 90%±3% at 5 years, 67%±5% at 10 years, and 47%±8% at 15 years. No differences existed for dysfunction or reintervention between the homograft and bioprosthetic valve cohorts. Shorter freedom from dysfunction was associated with younger age, smaller implanted valve Z-score, valve replacement after prior homograft, and higher preoperative right ventricular outflow tract (RVOT) gradient. Shorter freedom from reintervention was associated with younger age, smaller implanted valve Z-score, and more recent valve implant. Adolescents represent an important age group as an increasing number are undergoing pulmonary valve replacement, and they may be particularly impacted by new transcatheter therapies. This study provides baseline comparative data for current surgical options that should help inform medical decision making as longer term data become available for transcatheter pulmonary valve replacement and other new technologies.