Valve-sparing Repair Research Articles

Mid-Term Results of Pulmonary Valve-Sparing Repair for Tetralogy of Fallot With Pulmonary Stenosis.

The transannular patch remains the most common procedure performed for patients with Tetralogy of Fallot (TOF) with pulmonary stenosis. Pulmonary regurgitation has a negative impact on early and late outcomes. To address this issue pulmonary valve-sparing repair (PVSR) has been developed. Our study goal is to evaluate the mid-term outcomes (five years) of PVSR at our institution. The data were collected retrospectively from June 2014 to June 2022. A total of 390 patients had total repair of TOF. Among these, PVSR was performed in 154 (39.4%) patients. The mid-term outcomes on the status of the pulmonary valve gradient, degree of pulmonary regurgitation, reintervention rate, and mid-term survival after PVSR were investigated. The median age at time of TOF repair was 12 (interquartile range [IQR]: 8-48) months and the median weight was 7.9 (IQR: 3.1-49.5) kg. The mean preoperative right ventricular outflow tract (RVOT) gradient was 77 ± 19.6 mm Hg. All patients had a pulmonary valve Z score of more than -2.5. The post-repair mean RV/LV pressure ratio was 0.49 ± 0.12. There was no surgical mortality. The median follow-up was 3 years (6 months to 8 years). The reintervention rate on the pulmonary valve was 4/154 (2.6%) at five years. The freedom from reintervention and from developing moderate pulmonary valve regurgitation at 5 years was 95% (151/154) and 77% (119/154), respectively. Pulmonary valve-sparing repair gives good mid-term outcomes in a specific group of patients with TOF. Reintervention rates are very low and the peak gradient across the pulmonary valve came down in the majority of patients during mid-term follow up. An RVOT gradient more than 40mm Hg at discharge predicts a high risk of need for reintervention. We continue to monitor our patients for the long term outcome.

Burden of reintervention after tetralogy of Fallot repair: A joint pediatric and adult congenital experience over 30 years

ObjectiveThere is a high burden of reintervention after repair of tetralogy of Fallot (TOF). We compare procedural burden and late outcomes in valve-sparing repair (VSR) and transannular patch (TAP) cohorts over 30 years. MethodsPatients undergoing TOF repair (1990-2021, excluding complex TOF) were included in this study (n = 1239) with subsequent comparisons between TAP (n = 550) and VSR (n = 648) cohorts. Descriptive statistics, cumulative incidence frequencies, survival analysis, and propensity matching (n = 425) were used to analyze reintervention burden and survival. ResultsOverall survival of the cohort was 96.7% at 15 years and 95.6% at 25 years, with similar survival between TAP and VSR cohorts (P = .22). The TAP cohort had increased incidence of procedural burden at 25 years (TAP 69.8% vs VSR 37.2%; P < .001), with 34.6% undergoing ≥2 reinterventions. The TAP cohort had higher incidence of surgical pulmonary valve replacement at 15 years (TAP 20.7% vs VSR 7.6%; P < .001) and placement of pulmonary artery stents (TAP 20.2% vs VSR 4.9%; P < .001). By contrast, VSR had higher incidence of right ventricular outflow tract (RVOT) reoperation at 15 years (VSR 7.3% vs TAP 3.6%; P = .047). After propensity score matching there was no survival advantage between the VSR and TAP cohorts (Era 2), whereas the need for RVOT reoperation was not different between the 2 cohorts (P = .060). ConclusionsThe procedural burden remains high following TOF repair. TAP is associated with higher procedural burden in matched and nonmatched cohorts. VSR has increased risk of reoperation for RVOT obstruction only in nonmatched comparisons. Anatomical complexity and surgical repair strategy influence procedural burden following TOF repair.

Comparing clinical and echocardiographic outcomes following valve-sparing versus transannular patch repair of tetralogy of Fallot: a systematic review and meta-analysis.

Transannular patch (TAP) repair of tetralogy of Fallot (ToF)relieves right ventricular tract obstruction but may lead to pulmonary regurgitation. Valve-sparing (VS) procedures can avoid this situation, but there is a potential for residual pulmonary stenosis. Our goal was to evaluate clinical and echocardiographic outcomes of TAP and VS repair for ToF. A systematic search of the PubMed, Embase, Scopus, Cochrane Central Register of Controlled Trials and Web of Science databases was carried out to identify articles comparing conventional TAP repair and VS repair for ToF. Random-effects models were used to perform meta-analyses of the clinical and echocardiographic outcomes. Forty studies were included in this meta-analysis with data on 11723 participants (TAP: 6171; VS: 5045). Participants who underwent a VS procedure experienced a significantly lower cardiopulmonary bypass time [mean difference (MD): -14.97; 95% confidence interval (CI): -22.54, -7.41], shorter ventilation duration (MD: -15.33; 95% CI: -30.20, -0.46) and shorter lengths of both intensive care unit (ICU) (MD: -0.67; 95% CI: -1.29, -0.06) and hospital stays (MD: -2.30; 95% CI: [-4.08, -0.52). There was also a lower risk of mortality [risk ratio: 0.40; 95% CI: (0.27, 0.60) and pulmonary regurgitation [risk ratio: 0.35; 95% CI: (0.26, 0.46)] associated with the VS group. Most other clinical and echocardiographic outcomes were comparable in the 2 groups. This meta-analysis confirms the well-established increased risk of pulmonary insufficiency following TAP repair while also demonstrating that VS repairs are associated with several improved clinical outcomes. Continued research can identify the criteria for adopting a VS approach as opposed to a traditional TAP repair.

Impact of right ventriculotomy on cardiac function after pulmonary valve sparing repair of tetralogy of Fallot and double outlet right ventricle with pulmonary stenosis.

Pulmonary valve-sparing repair of tetralogy of Fallot and double outlet right ventricle with pulmonary stenosis has the advantage of reduced incidence of late pulmonary valve regurgitation and better-preserved cardiac function. However, a right ventriculotomy is sometimes necessary in order to adequately relieve subvalvular pulmonary stenosis. We aimed to compare postoperative cardiac function and patients' symptoms between pulmonary valve-sparing repair with and without right ventriculotomy. We retrospectively collected data from electronic medical records of Ramathibodi Hospital from 1st January 2013 to 31st October 2023. Patients diagnosed with tetralogy of Fallot and double outlet right ventricle with pulmonary stenosis who underwent pulmonary valve-sparing repair were included. Patients who underwent other types of repairs and whose medical record data were significantly missing were excluded. Demographic data, operative, and postoperative details were collected and reviewed. There were 49 patients included in our study with 10 patients undergoing pulmonary valve-sparing repair with and the other 39 without right ventriculotomy. Before-discharge echocardiographic parameters were generally similar between both groups (tricuspid annular plane systolic excursion = 0.9 versus 0.89 cm, P = 0.737; pressure gradient across pulmonary valve across pulmonary valve = 24 versus 19 mmHg, P = 0.275; left ventricular end-systolic volume index = 17.84 versus 19.19 ml/m2, P = 0.437; left ventricular end-diastolic volume index = 63.79 versus 61.13 ml/m2, P = 0.436). Patients' symptoms were also not statistically different. There was no early and late death up to the end date of our study. Right ventriculotomy in pulmonary valve-sparing repair did not result in worse postoperative cardiac function and symptoms. This suggested that the previously thought-to-be hazardous incision could be strongly considered if mandated.

Longitudinal ECG changes in tetralogy of Fallot and association with surgical repair.

ECG abnormalities have been linked to adverse changes in right ventricular (RV) morphology and poor clinical outcomes in repaired Tetralogy of Fallot (rTOF). Our aim was to describe how ECG changes progress in early and intermediate follow-up and whether types of surgical strategy at the time of primary repair affected these changes. We studied patients with rTOF born 2000-2018 operated at our institution. Seven time points in relation to primary repair, follow-up, and pulmonary valve replacement (PVR) were identified. Patients correct with valve sparing repair (VSR), trans-annular patch (TAP) including with a monocusp valve (TAP + M) and with at least 3 ECGs were included. PQ interval, QRS duration, dispersion, and fragmentation, QTc duration and dispersion, JTc as well as presence of a right bundle branch block (RBBB) were analyzed. Medical records were reviewed for demographic and surgical data. Two hundred nineteen patients with 882 ECGs were analyzed with a median follow-up time of 12.3 years (8.4, 17) with 41 (19%) needing PVR during the study period. QRS duration increased at time of primary repair to discharge from 66 msec (IQR 12) to 129 msec (IQR 27) (p < 0.0001) and at 1- and 6- year follow-up but showed only a modest and temporary decrease after PVR. QTc increased at the time of primary repair as well as prior to PVR. PQ interval showed a small increase at the time of primary repair, was at its highest prior to PVR and decreased with PVR. Type of surgical repair affected mainly QTc and JTc and was consistently longer in the TAP + M group until PVR. In VSR, QTc and JTc were prolonged initially compared to TAP but were similar after 1 year. After PVR, there were no differences in adverse ECG changes between surgical groups. PQ interval and QRS duration best correspond to the assumed volume load whereas the relationship with QTc and JTc is more complex, suggesting that these represent more complex remodeling of the myocardium. Before PVR, QTc and JTc are longer in the TAP + M group which may be due to a longer surgical incision.

Abstract 15187: Risk Factors for Type B Dissection in Marfan Syndrome: The Cornell Aortic Aneurysm Registry

Introduction/Background: With improvements in the medical and surgical management of patients with Marfan syndrome (MFS), type A dissections are decreasing. As a corollary, most MFS dissections are now type B, for which risk factors remain largely uncertain. Research Questions/Hypothesis: In this prospective and retrospective observational study, we explore determinants of type B dissection risk in a large single-center MFS registry through analysis of systematic clinical and imaging data over four decades. Goals/Aim: Determinants of type B dissection risk in MFS will inform appropriate prognostication, management, and treatment of this patient population. Methods/Approach: Demographic and anthropometric features, cardiovascular disease, and surgical history were compared in MFS patients with and without type B dissection. Results/Data: Of 335 MFS patients, 56 (17%) experienced a type B dissection (versus type A in 9%). Patients with type B dissection were more likely to have undergone elective aortic root replacement (ARR; 75 vs. 46%, p&lt;0.001). 55% of patients had type B dissection a mean of 14.1 years after ARR, whereas 45% experienced type B dissection before ARR. Valve replacement was more common than valve-sparing repair in those with post-operative type B dissection (27% vs. 12%, p=0.02), although this difference was eliminated after adjusting for duration of post-operative follow up. 50 (89%) patients were aware of their MFS diagnosis prior to type B dissection. Among those with pre-dissection imaging, the descending aorta was normal or minimally dilated in 87%. In multivariable analyses, patients with type B dissection were more likely to have undergone ARR, mitral valve surgery, had a type II dissection, and to have lived longer. Conclusions: In our contemporary cohort, type B dissections are more common than type A. The associations of type B dissection with ARR, mitral surgery, and type II dissection suggest a more severe phenotype in the setting of prolonged life expectancy.

Outcomes Following Balloon Pulmonary Valvuloplasty in Symptomatic Neonates With Tetralogy of Fallot-A CCRC Study.

Complete repair (CR) can be delayed in neonates with symptomatic tetralogy of Fallot (sTOF) using surgical or transcatheter palliation to relieve cyanosis. Balloon pulmonary valvuloplasty (BPV) is an established treatment for pulmonary valve stenosis; however, its effectiveness in palliating neonates with sTOF has not been well investigated. A retrospective chart review between 2005 and 2017 on neonates with sTOF who underwent initial BPV from 9 participating centers of the Congenital Cardiac Research Collaborative was performed. Primary outcome was CR at >30 days after BPV without interval reintervention (RI). In total, 47 neonates with sTOF underwent BPV, of whom 27 (57%) underwent CR at >30 days after BPV without RI. The median time to CR was 151 days (106-210). RI before CR occurred in 17 patients (36%): surgical shunt (n = 7), outflow tract stenting (n = 6), patent ductus arteriosus stenting (n = 2), and surgical outflow patch (n = 2). Valve-sparing repair at CR was performed in 6 patients (13%) after initial BPV. RI or CR ≤30 days from BPV was associated with smaller infundibular diastolic diameter (P = .004). An infundibular diastolic diameter of <3.4 mm demonstrated 75% sensitivity and 67% specificity to predict early CR or RI. BPV can be an effective palliative therapy in select neonates with sTOF to delay CR. A smaller diastolic infundibulum diameter is a predictor of RI or early CR, and valve-sparing repair is uncommon, making patient selection and alternative palliative methods key when considering BPV palliation in neonates with sTOF.

Prenatal diagnosis, management, and outcomes of fetuses with tetralogy of Fallot in China after prenatal counseling: a prospective cohort study.

The study aimed to monitor fetuses with tetralogy of Fallot (TOF) after prenatal counseling and how it influenced the decision of parents to terminate the pregnancy. Fetuses with isolated TOF diagnosed between January 2019 and December 2021 were prospectively enrolled. The follow-up period extended until termination or 6 months after the operation. Of the 1,026 fetuses diagnosed with cardiac defects, 129 were identified to have isolated TOF and completed the follow-up. A total of 55 (42.6%) fetuses were terminated, with larger maternal age (odds ratio: 0.893, 95% confidence interval: 0.806-0.989, P = 0.031) as the protective factor. The maternal anxiety score, gestational weeks, and pulmonary-to-aortic-diameter ratio lost significance in multivariate analysis. Subjectively, the two most common reasons for terminating the pregnancy were worries about the prognosis (41.8%) and concerns about the possible suffering of the unborn child (18.2%). The prenatal diagnosis was accurate in 73 of the 74 (98.6%) live births. Out of the 64 live births that underwent surgical repair in our center, 57 (89.1%) received primary repair, with a median age of 104 days, and 49 (76.6%) underwent valve-sparing repair. No perioperative death occurred. Termination for fetuses with TOF remains common in China. Live births with TOF can be safely and effectively managed.

Outcomes of aortic root replacement in patients with Marfan syndrome: the role of valve-sparing and valve-replacing approaches.

Marfan syndrome (MFS) is a heritable thoracic aortic disease with pervasive cardiovascular effects, including commonly, a dilated aortic root. Traditionally, the root is replaced using a mechanical composite valve graft (CVG); however, this valve-replacing (VR) approach necessitates a lifelong regimen of anticoagulation with a potential for late bleeding complications. In time, valve-sparing (VS) approaches were developed. Today, several options for aortic root replacement (ARR) exist; each has advantages and disadvantages that helps inform choice. The Aortic Valve Operative Outcomes in Marfan Patients (AVOMP) is a multi-center international registry to analyze clinical outcomes of ARR in MFS patients using either VR or VS techniques to better elucidate choice. We summarize outcomes of AVOMP and present our own experience. We performed 223 consecutive elective ARR [1991-2023] in patients with MFS; 15 such repairs were included in AVOMP. Repairs included 113 (51%) using a mechanical CVG, 62 (28%) using a VS approach, and 48 (22%) using a bioprosthetic root. Many patients underwent aortic arch repair (30% to 54% by type). The median patient age was 38 [29-52] years. In comparing VS and VR groups, patients were similar in age and rates of major comorbidities and symptoms. Patients with VR repair had a more complex aortic history. The rate of redo sternotomy was 24% (n=54). Operative death was uncommon [4% overall (10/223); ranging from 2% to 8% by type], and stroke was rare [1/223 (<1%)]. Late survival and reoperation differed by operative approach; survival was improved in patients who underwent VS repair. We found that repair in patients with MFS undergoing ARR resulted in low operative risk. Our late results were similar to those of AVOMP in that patients undergoing VS repair tended to experience greater rates of valvular-structural deterioration, although this did not appear to impact survival.

Temporal changes in the surgical management of patients with tetralogy of Fallot in Denmark: a nationwide cohort study.

To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977-1991), intermediate (1992-2006) and late (2007-2021). The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7-30.5). There was a temporal trend towards less shunt palliation (-0.3% per year, 95% CI -0.05 to -0.1). Median age at intracardiac repair was 2.9 years (1.8-5.0), 0.8 years (0.5-1.3) and 0.5 years (0.4-0.7) (P < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (-0.7% per year, 95% CI -0.5 to -1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5-1.0). Survival at 10 years was 79% (64-76), 90% (87-93) and 95% (92-98) (P < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1-6), 12% (8-16) and 21% (13-29) (P < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair.

Technique of Valve-Sparing Repair With Skeletonization of the Pulmonary Annulus for Tetralogy of Fallot

Pulmonary valve dysfunction after tetralogy of Fallot (TOF) repair increases morbidity and mortality in the long term. Several pulmonary valve‐sparing techniques have been introduced to prevent such adverse sequelae. In this article we describe our valve-sparing technique for TOF repair that primarily consists of skeletonization of the anterior part of the pulmonary annulus and gentle dilation to preserve the pulmonary valve and annulus function. Pulmonary valve dysfunction after tetralogy of Fallot (TOF) repair increases morbidity and mortality in the long term. Several pulmonary valve‐sparing techniques have been introduced to prevent such adverse sequelae. In this article we describe our valve-sparing technique for TOF repair that primarily consists of skeletonization of the anterior part of the pulmonary annulus and gentle dilation to preserve the pulmonary valve and annulus function.

Fate of the Right Ventricular Outflow Tract Following Valve-Sparing Repair of Tetralogy of Fallot

Valve-sparing repair (VSR) of tetralogy of Fallot (TOF) tends to result in higher residual right ventricular outflow tract (RVOT) gradients. We evaluated the progression and clinical implications of RVOT gradients following VSR of TOF. Demographic, clinical, and operative data were retrospectively collected from consecutive TOF patients who underwent VSR at our institution between 01/2010 and 06/2021. RVOT gradient, pulmonary valve annulus (PVA) diameter and Boston Z-scores were recorded from serial echocardiograms. Data are presented as median and interquartile range or number and percentage. A total of 156 children (boys 92, 59%) underwent VSR at 6.5 (4.9-8.4) months of age and 6.6 kg (5.6- 7.7) weight. There was 1 (0.6%) operative mortality. The remaining 155 patients were followed for 69.4 months (4-106.2). RVOT gradient was 2.4m/s (1.7-2.9) at discharge. It transiently increased, then declined and stabilized during follow-up. PVA Z-score was -1.7 (-3.1 to 0.5) at discharge and 'grew' to -0.8 (-1.7 to 0.4) at last follow-up. Freedom from RVOT re-intervention was 97%, 94% and 91% at 1, 5 and 10-year follow-up. Among 67 (43%) patients with PVA Z-score < -2, a similar RVOT gradient pattern was observed and freedom from RVOT re-intervention was 97%, 95% and 95% at 1, 5 and 8-year follow-up. Following VSR of TOF, RVOT gradients transiently increase and then fall as PVA growth catches up, resulting in durable intermediate outcomes. Patients with PVA Z-score < -2 demonstrated a similar pattern of hemodynamics in the RVOT and excellent freedom from reintervention.

Incidence of branch pulmonary artery stenosis requiring intervention based on surgical repair type for tetralogy of Fallot

BackgroundTetralogy of Fallot is a common form of cyanotic congenital heart disease. The right ventricular outflow tract obstruction and pulmonary stenosis are typically repaired using either a transannular patch or valve sparing repair. Following repair, one recognized complication is branch pulmonary artery stenosis. ObjectiveThis study sought to evaluate if there was a higher incidence of branch pulmonary artery stenosis requiring intervention in patients with tetralogy of Fallot who underwent transannular patch repair as compared to valve sparing repair. MethodsIn this retrospective study, 62 pediatric patients with tetralogy of Fallot underwent surgical repair at our institution between January 1, 2006 and August 2, 2016. Data, including echocardiographic measurements, surgical repair details, and post-operative follow up was recorded. Statistical analysis was performed, including chi-square test, Student t-test, multiple logistic regression, and Kaplan-Meier analysis. ResultsMedian length of follow up was 7.3 years (IQR 4.7–9.1). Of the 62 patients, 8 (13 %) developed branch pulmonary artery stenosis requiring intervention. A significantly higher incidence was seen in the transannular patch group (n = 7, 37 %) compared to the valve sparing repair patients (n = 1, 2 %) (p = 0.0002). Most patients required intervention within one year following repair. The effect of repair type persisted after controlling for multiple potential confounding variables, including Blalock-Taussig shunt placement and patch augmentation of the branch pulmonary arteries. ConclusionThere is a significantly higher incidence of branch pulmonary artery stenosis requiring intervention in patients with classic tetralogy of Fallot who underwent transannular patch repair as compared to valve sparing repair.

Outcome of humanitarian patients with late complete repair of tetralogy of Fallot: A 13-year long single-center experience

BackgroundSurgical repair of tetralogy of Fallot is usually performed between 3 and 6 months of age with pulmonary valve-sparing repair promoted for the best long-term result. Through a humanitarian program from developing countries, late complete surgical repair of tetralogy of Fallot has been performed at our institution for many years. MethodsRetrospective analysis of pre- and perioperative data, as well as 30-days outcome of patients older than one year with a confirmed diagnosis of tetralogy of Fallot who had a complete surgical repair between 2005 and 2018 at our institution. ResultsOne hundred sixty-five patients were included with a median age of 4.5 years [3.0–6.3], median weight of 13.5 kg [10.9 to 16.5], median transcutaneous oximetry of 78% [70 to 85] and median pulmonary valve annulus Z-score of −1.8 [-3.4 to −0.8]. There was no early surgical mortality. By multivariate analysis, only severe right ventricular hypertrophy, severe right ventricle outflow tract obstruction, and hypoplasia of the main pulmonary artery were independent predictors of failure to preserve the pulmonary annulus at surgical repair. ConclusionsLate complete surgical repair of tetralogy of Fallot has low mortality and morbidity even when pulmonary valve-sparing repair cannot be successfully performed. The preservation of the pulmonary valve function is significantly associated with shorter ventilation time, ICU and hospital lengths of stay. In the analyzed group of patients, a pulmonary valve-sparing repair cannot be predicted exclusively based on the dimension of the pulmonary valve annulus.

How to Manage the Pulmonary Valve During Repair of Tetralogy of Fallot

Evolution of Pulmonary Valve Management During Repair of Tetralogy of Fallot: A 14-year ExperienceThe Annals of Thoracic SurgeryPreviewThe optimal repair strategy for tetralogy of Fallot remains controversial. This report presents a 14-year evolution of management of the pulmonary valve (PV) from transannular patch to valve-sparing repair to neovalve creation using living right atrial appendage tissue. Full-Text PDF

Evolution of Pulmonary Valve Management During Repair of Tetralogy of Fallot: A 14-year Experience

BackgroundThe optimal repair strategy for tetralogy of Fallot remains controversial. This report presents a 14-year evolution of management of the pulmonary valve (PV) from transannular patch to valve-sparing repair to neovalve creation using living right atrial appendage tissue. MethodsA retrospective review of 172 consecutive patients undergoing complete repair for TOF between January 2007 and June 2021 was performed. Clinical and follow-up data were analyzed by repair group. Neopulmonary valve (NPV) creation using right atrial appendage tissue was introduced in 2019. Failure of valve-sparing repair was defined as needing reintervention for recurrent right ventricular outflow tract obstruction (RVOTO). ResultsMedian age and weight at repair were 4.9 months and 6 kg, respectively. Median preoperative PV size and z-score were 6.4 mm (5.2-8.3 mm) and −3.2 (−4.1 to −2.1), respectively. Patients who underwent valve-sparing repair had larger PV size and z-score compared with patients who underwent transannular patch procedures (8 mm vs 5.6 mm; −2.1 vs −3.2; both P < .001). There were no hospital mortalities. Overall follow-up was 44 months. At last follow-up, 10% of patients who underwent valve-sparing repair had repeat intervention for recurrent RVOTO. Patients who had failed valve-sparing repair had significantly lower PV z-scores (−2.6 vs −1.9; P = .01). An NPV was used in 8 patients with a median PV z-score of −4 (−4.7 to −3.9). At 6 months, 6 patients (75%) had mild or trivial pulmonary insufficiency after NPV placement. ConclusionsRepair of tetralogy of Fallot is a safe operation with excellent outcomes. Valve-sparing repair avoids right ventricular dilation but may fail for RVOTO at a PV z-score <−2. NPV creation offers an alternative option in patients with a small PV.

Long-Term Results after Fallot Repair.

The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after initial repair of tetralogy of Fallot (TOF). The cohort of 306 patients treated between 1980 and 2017 was divided into anatomical subgroups according to the diagnosis of TOF-pulmonary stenosis, TOF-pulmonary atresia and TOF-double outlet right ventricle. Patients were treated with transannular patch (TAP), valve sparing repair (VSR), or conduits from the right ventricle to the pulmonary arteries (RVPA conduits). There were 21 deaths (6.9%), 14 being hospital deaths (4.6%) after primary correction and four deaths (1.3%) occurred after PVR. One patient died after a non-cardiac operation (0.3%). There were two late deaths (0.7%). During the past 12 years no early mortality has been observed. Ninety-one patients (30.4%) received PVR after a median of 12.1 ± 7.0 years with an early mortality of 4.4% (n = 4) and no late mortality. A significant difference in freedom from reoperation after TAP, VSR, and RVPA-conduits could be identified. Multivariate analysis displayed transannular repair (p = 0.016), primary palliation (p <0.001), the presence of major aortopulmonary collateral arteries (MAPCA; p = 0.023), and pulmonary valve Z-scores < - 4.0 (p = 0.040) as significant risk factors for PVR. TOF repair has a beneficial long-term prognosis with low morbidity and mortality. Pulmonary valve Z-scores < - 4.0, transannular repair, and presence of MAPCAs are associated with earlier PVR. Non-VSRs and TOF-pulmonary atresia lead to earlier reoperation but have no negative impact on survival.

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair.

Although valve-sparing repair remains ideal for patients with tetralogy of Fallot, the durability of valve-sparing repair and which patients may have been better served with a transannular patch remain unclear. Retrospective review was performed of tetralogy of Fallot operations at our institution from January 2008 to December 2018. Standard demographic data were collected, including echocardiographic parameters, operative details, and clinical outcomes. Statistical analysis was performed comparing the transannular patch and valve-sparing repair groups. Sixty-seven patients underwent tetralogy of Fallot repair with a median age of 4.5 (3.2-6.0) months and weight of 5.8 (5.2, 6.7) kg. Seventeen (25%) patients underwent transannular patch repair and 50 (75%) patients underwent valve-sparing repair. There was no difference in age or weight between patients who underwent a transannular patch repair and those who underwent a valve-sparing repair. At last follow-up (median 42 months), there was a trend of a higher peak pulmonary valve/right ventricular outflow tract gradient (P = .06) in the valve-sparing group, but no difference in the pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3 ± 1.0 on predischarge echocardiogram of to -1.2 ± 1.6 on last follow-up, with the peak gradient on predischarge 23 (0-37) mm Hg remaining stable on last follow-up at 18 (0-29) mm Hg. There was one reoperation: pulmonary valve replacement six years after a transannular patch. Obtaining a postrepair pulmonary valve z-score of -2 yields satisfactory, stable valve-sparing repair with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from reintervention during follow-up.

Making a Case for Late Repair of Tetralogy of Fallot

The history of management of tetralogy of Fallot illustrates the arc of progress in congenital heart surgery. From Blalock and Thomas to Lillehei and Kirklin, operations transitioned from palliation alone to palliation followed by staged repair in childhood. In the 1970s Barratt-Boyes and Castaneda pioneered primary repair in infancy. Subsequently there was a gradual evolution from staged repair toward single-stage repair at a progressively younger age, a phenomenon that mirrored the course of the specialty. 1 Barron D.J. Tetralogy of Fallot: controversies in early management. World J Pediatr Congenit Heart Surg. 2013; 4: 186-191 Crossref PubMed Scopus (28) Google Scholar As experience grew, middle infancy was recommended as the optimal timing for elective repair. 1 Barron D.J. Tetralogy of Fallot: controversies in early management. World J Pediatr Congenit Heart Surg. 2013; 4: 186-191 Crossref PubMed Scopus (28) Google Scholar ,2 Van Arsdell G.S. Maharaj G.S. Tom J. et al. What is the optimal age for repair of tetralogy of Fallot?. Circulation. 2000; 102: III123-III129 PubMed Google Scholar Over a decade ago The Society of Thoracic Surgeons Database revealed that primary repair in infants under 1 year of age had become the dominant strategy in North America. 3 Al Habib H.F. Jacobs J.P. Mavroudis C. et al. Contemporary patterns of management of tetralogy of Fallot: data from The Society of Thoracic Surgeons Database. Ann Thorac Surg. 2010; 90: 813-820 Abstract Full Text Full Text PDF PubMed Scopus (170) Google Scholar Most patients underwent repair in the first year of life, with the majority during the first half of infancy. Although not significant, the lowest mortality for primary repair (0.6%) was observed in the most prevalent age category (3-6 months). Palliative shunting had decreased to around 10% and was mostly confined to neonates. More recently a large meta-analysis corroborated this shift toward repair in younger patients with a corollary decline in palliative staging; notably it also showed that increased mortality was associated with older patient age and prior palliation. 4 Romeo J.L.R. Etnel J.R.G. Takkenberg J.J.M. et al. Outcome after surgical repair of tetralogy of Fallot: a systematic review and meta-analysis. J Thorac Cardiovasc Surg. 2020; 159: 220-236 Abstract Full Text Full Text PDF Scopus (13) Google Scholar Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional OutcomesThe Annals of Thoracic SurgeryVol. 113Issue 5PreviewThis study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot on pulmonary valve annulus growth, the rate of valve-sparing repair at the time of intracardiac repair, and long-term functional outcomes. Full-Text PDF

Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes

BackgroundThis study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot on pulmonary valve annulus growth, the rate of valve-sparing repair at the time of intracardiac repair, and long-term functional outcomes. MethodsThis retrospective study involved 330 patients with tetralogy of Fallot who underwent intracardiac repair between 1991 and 2019 and included 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0 ± 7.3 years. We compared the data of patients who underwent BTS and patients who did not undergo BTS before intracardiac repair. ResultsThe median age before BTS was 71 days (range, 28 to 199) and the median body weight was 4.3 kg (range, 3.3 to 6.8 kg). There were no inhospital or interstage deaths after BTS. The pulmonary valve annulus Z scores of patients with BTS revealed significant growth after BTS (from −4.2 ± 1.8 to −3.0 ± 1.7, P < .001). Valve-sparing repair was eventually performed in 207 patients (63%), including 26 (46%) who underwent staged repair. The overall freedom from pulmonary regurgitation-related reintervention was 99.7%, 99.1%, and 95.8% at 1, 5, and 20 years, respectively. ConclusionsA staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. Blalock-Taussig shunt may have contributed to the avoidance of primary transannular patch repair and facilitated pulmonary valve annulus growth; therefore, approximately half of the symptomatic neonates and infants were recruited for valve-sparing repair. Staged repair may have led to functionally reliable delayed transannular patch repair, thereby resulting in fewer surgical reinterventions.