Fallot Repair Research Articles

Pulmonary Artery Angioplasty (Main Pulmonary Artery Rotation Flap Technique) in Tetralogy of Fallot Patients With Acute-Angle Branch Pulmonary Artery Stenosis

Despite excellent outcomes of tetralogy of Fallot (TOF) repair, reinterventions for recurrent or residual stenosis of branch pulmonary arteries are still required in many patients. The development of these lesions may be related to 2 inherent characteristic morphologies of TOF: Pulmonary ductal coarctation and acute angulation of branch pulmonary artery, especially the left pulmonary artery. I developed a main pulmonary artery rotation flap angioplasty technique to deal with acute-angle branch pulmonary artery stenosis without using any patch. Since 1998, 68 TOF patients have undergone this technique. During a median follow-up of 13.5 years, 20 (29%) patients underwent catheter or surgical-based intervention for branch pulmonary stenosis. Overall freedom from any intervention was 87%, 76%, 70%, and 59% at 1-year, 5-year, 10-year, and 20-year periods, respectively. I believe that this technique is reproducible and provides excellent intermediate and long-term outcomes in TOF patients with complicated branch pulmonary artery stenosis. Despite excellent outcomes of tetralogy of Fallot (TOF) repair, reinterventions for recurrent or residual stenosis of branch pulmonary arteries are still required in many patients. The development of these lesions may be related to 2 inherent characteristic morphologies of TOF: Pulmonary ductal coarctation and acute angulation of branch pulmonary artery, especially the left pulmonary artery. I developed a main pulmonary artery rotation flap angioplasty technique to deal with acute-angle branch pulmonary artery stenosis without using any patch. Since 1998, 68 TOF patients have undergone this technique. During a median follow-up of 13.5 years, 20 (29%) patients underwent catheter or surgical-based intervention for branch pulmonary stenosis. Overall freedom from any intervention was 87%, 76%, 70%, and 59% at 1-year, 5-year, 10-year, and 20-year periods, respectively. I believe that this technique is reproducible and provides excellent intermediate and long-term outcomes in TOF patients with complicated branch pulmonary artery stenosis.

Exercise Limitation in Children after Tetralogy of Fallot Repair Is Not related to Right Ventricular Dysfunction: Single Egyptian Center Experience

Exercise Limitation in Children after Tetralogy of Fallot Repair Is Not related to Right Ventricular Dysfunction: Single Egyptian Center Experience

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.

A Rare Case of Prolapsing Accessory Tricuspid Valve Tissue into the Ventricular Septal Defect in Patient Undergoing Tetralogy of Fallot Repair: Role of Transesophageal Echocardiography

A Rare Case of Prolapsing Accessory Tricuspid Valve Tissue into the Ventricular Septal Defect in Patient Undergoing Tetralogy of Fallot Repair: Role of Transesophageal Echocardiography

Pediatric Heart-Lung Transplantation Through the Decades: A Single-Center Analysis

<h3>Purpose</h3> Pediatric heart-lung transplantation is an uncommon surgical approach utilized in children with end-stage cardiopulmonary failure. We sought to provide a detailed report of our experience at a high-volume institution over three decades. <h3>Methods</h3> We analyzed all pediatric heart-lung transplantations performed between 1993 and 2020. Medical records were reviewed for transplant indications, preoperative risk factors, postoperative complications, and survival outcomes. Patients were stratified and compared by surgical decade: 1993-2000, 2001-2010, and 2011-2020. Data are expressed as median (IQR) or percent. <h3>Results</h3> Twenty-two children were included in this study. One patient from the 1st decade with prior tetralogy of Fallot repair and aortic valve replacement experienced massive hemorrhage following sternotomy and passed away before the transplant could be performed. Median age at transplant was 13.8 (9.9-16.3) years. 2 (9.1%) children were supported on extracorporeal membrane oxygenation (ECMO) pre-transplant. 17 (77.3%) had congenital heart disease, including two with single-ventricle physiology. Median postoperative length of stay was 17 (10-51) days. All 5/22(23%) children on ECMO post-transplant died before discharge. Median follow-up for hospital survivors was 5.0 (2.9-7.9) years. Patients transplanted from 2010-2020 had the highest survival rate (Table). Transplant-free survival at 1, 3, and 5 years was 72.7%, 63%, and 52%, respectively. 3/11(14%) underwent redo bilateral lung transplantation due to severe bronchiolitis obliterans (BOS), with 2 alive at last follow-up. Children transplanted in the most recent decade had significantly lower rates of BOS. <h3>Conclusion</h3> The spectrum of children undergoing heart-lung transplant has changed over time, although postoperative ECMO remains an important risk factor for mortality. While survival after discharge appears promising in the most recent decade, discussions regarding long-term survival are warranted prior to listing.

Predictive Value of Right Ventricular Pressure Measurement for Residual Pulmonary Stenosis in Tetralogy Repair

Objective: The long-term outcome of tetralogy of Fallot repair depends on an adequate relief of right ventricular outflow tract obstruction and preservation of the pulmonary valve function. Since intraoperative transesophageal echocardiography is not routinely performed in small patients, we postulated that the post-bypass right ventricular pressure measured intraoperatively could predict residual pulmonary stenosis when evaluated by transthoracic echocardiography.Materials and Methods: Of the 187 patients who underwent tetralogy repair between 2012 and 2019 at Siriraj Hospital, Thailand, 95 with right ventricular pressure measurements and pre-discharge echocardiography were included in the study. Their intraoperative parameters, and postoperative outcomes were analyzed. The tolerable pressure cutoff was determined.Results: The median patient age was 3.9 years old (interquartile range 2.75–6). Fifty-three patients (54.6%) required the use of the transannular patch. Ten patients (10.3%) had significant residual pulmonary stenosis with a mean right ventricular systolic pressure of 64.0±10.6 mmHg compared with 48.7±14.4 mmHg for the other patients. There was an association between the pressure figure and the degree of residual pulmonary stenosis (rho=0.391, p=0.01). A systolic pressure above 49 mmHg predicted pulmonary stenosis with a likelihood ratio of 2.18 (1.94-2.80, 95%CI). The likelihood rose to 2.93 (2.44-4.01, 95%CI) if the pressure resulted in a right to left ventricular pressure ratio above 0.62. The patients whose figures did not exceed 49 mmHg experienced no significant residual obstruction, regardless of the pressure ratio.Conclusion: Intraoperative measurement of the right ventricular pressure can predict residual pulmonary stenosis after tetralogy repair with a reassuring cutoff of 49 mmHg

Echocardiography assessment of right ventricular dysfunction during postoperative period following total correction of tetralogy of Fallot

Background: Despite major advances in intracardiac surgery since Lillehei and Varco reported the first tetralogy of Fallot (TOF) repair by an open-heart procedure in 1954, right ventricular (RV) dysfunction remains an important clinical problem during the postoperative periods following TOF correction.Methods: This was a cross-sectional study conducted at the department of paediatric cardiology in Bangabandhu Sheikh Mujib medical university (BSMMU), Shahbag, Dhaka in collaboration with national heart foundation hospital and research institute (NHFH and RI) Mirpur, Dhaka, Bangladesh during the period from July 2018 to June 2019. Ethical consideration was approved by the proper concerns. The final sample size was 49. Data analysis was carried out by using the social science (SPSS) version 23.0 windows software. Results: The study was conducted on 49 patients, comprising 30 males and 19 females’ children, and their mean age was 75.38±52.22 months. RV dysfunction global was 63.3% at 24 hours of operation, significantly reduced 49% and 32.7% at 7th and 30th POD respectively. Chi-square test was done where p&lt;0.05. Global dysfunction was 13 (54.2) in ≤5 years, 18 (72.0) in &gt;5 years aged patients.Conclusions: RV dysfunction was more frequent after 24 hours of the postoperative period then it gradually decreases over a period of time

Early post-operative benefits of a pulmonary valve-sparing strategy during Fallot repair

BackgroundPatients undergoing complete repair of tetralogy of Fallot generally have an excellent prognosis. Unfortunately, pulmonary valve reconstruction with a transannular patch is generally required. Resulting pulmonary regurgitation is associated with late right ventricular dysfunction and morbidity. Early pulmonary regurgitation may also be poorly tolerated. This study compares immediate outcomes of non-valved transannular patch repair (NV-TAP) to a valve-sparing (V-Sp) approach. Methods67 patients, with tetralogy of Fallot and severe pulmonary annular hypoplasia underwent complete repair between 2010 and 2018.23 patients had a NV-TAP repair while a V-Sp technique was used in 44 patients. Mortality, length of stay in the intensive care unit and hospital, use of inotropes, duration of mechanical ventilation, use and duration of dialysis, chest drainage duration, reinterventions, and postoperative complications were compared. Immediate outcomes were assessed by biological data and postoperative echocardiography. ResultsICU stay was shorter in the V-Sp group (5 days vs 8 days, p = 0.009). At postoperative day 1, Vasoactive Inotropic Score and mean dosage of adrenaline were significantly lower in the V-Sp group. Duration of inotrope use was significantly shorter. Hospital stay, mechanical ventilation, and chest drainage duration, the incidence and duration of dialysis all showed a trend towards being lower in the V-Sp group. Surgical complications were similar despite longer cardiopulmonary bypass and aortic cross-clamp durations. ConclusionRepair of the native pulmonary valve by a V-Sp technique reduces length of stay in the ICU and the use of inotropic agents in the immediate post-operative period.

Right atrial function early after tetralogy of Fallot repair.

Diastolic dysfunction after repair for Tetralogy of Fallot (TOF) is associated with adverse long-term outcomes. Right atrial (RA) mechanics as a proxy of right ventricular (RV) diastolic function in the early post-operative period after surgical repair for TOF has not been reported. We sought to evaluate RA and RV strain prior to hospital discharge after TOF repair and to identify important patient factors associated with strain using a machine learning method. Single center retrospective cohort study of TOF patients undergoing surgical repair, with analysis of RA and RV strain from pre-and post-operative echocardiograms. RA function was assessed by the peak RA strain, systolic RA strain rate, early diastolic RA strain rate and RA emptying fraction. RV systolic function was measured by global longitudinal strain. Pre- and post-operative values were compared using Wilcoxon rank sum test. Gradient boosted machine (GBM) models were used to identify the most important predictors of post-operative strain. In total, 153 patients were enrolled, median age at TOF repair 3.5months (25th-75th percentile: 2.2- 5.2), mostly male (67%), and White (64.1%). From pre-to post-operative period, there was significant worsening in all RA parameters and in RV strain. GBM models identified patient, anatomic, and surgical factors that were strong predictors of post-operative RA and RV strain. These factors included pulmonary valve and branch pulmonary artery Z scores, birth weight, gestational age and age at surgery, pre-operative RV fractional area change and oxygen saturation, type of outflow tract repair, duration of cardiopulmonary bypass, and early post-operative partial arterial pressure of oxygen. There is significant worsening in RA and RV strain early after TOF repair, indicating early alteration in diastolic and systolic function after surgery. Several patient and operative factors influence post-operative RV function. Most of the factors described are not readily modifiable, however they may inform pre-operative risk-stratification. The clinical application of RA strain and the prognostic implication of these early changes merit further study.

Hand-sewn expanded polytetrafluoroethylene valved conduit for right ventricular outflow tract reconstruction.

Due to the lack of large-sized pulmonary valved conduit products in clinical practice, hand-sewn expanded polytetrafluoroethylene (ePTFE) valved conduit has been used for right ventricular outflow tract (RVOT) reconstruction in many heart centers around the world. This study aims to summarize the early results of the ePTFE valved conduit and the sewing technology of the conduit in combination with the latest progress, and to provide a reference for the application of ePTFE valved conduit. A total of 21 patients using ePTFE valved conduit for RVOT reconstruction in the Second Xiangya Hospital, Central South University from October 2018 to October 2020 were prospectively enrolled in this study. The age at the implantation of the conduit was 4.3 to 43.8 (median 15.1) years old, with weight of (38.9±4.1) kg. In this cohort, 14 patients underwent re-reconstruction of RVOT, including 12 patients with pulmonary regurgitation at 6.3 to 31.0 (median 13.8) years after tetralogy of Fallot (TOF) repair, and 2 patients with failed bovine jugular vein conduit (BJVC). Seven patients underwent Ross operations. Among them, 3 were for aortic valve stenosis, 2 were for aortic regurgitation, and 2 were for both stenosis and regurgitation. The ePTFE valved conduits were standard hand-sewn during the surgery. The 3 leaflets were equal in size with arc-shaped lower edge of the valve sinus. The free edge of the valve leaflets was straight with the length of about 1 mm longer than the diameter. The height of the valve sinus was 4/5 of the diameter. The junction of the valve leaflet was 3/4 of the height of the sinus. The designed leaflets were then continuous non-penetrating sutured into the inner surface of Gore-Tex vessel to make a valved conduit. Valved conduits with diameter of 18, 20, and 22 mm were used in 2, 9, and 10 cases, respectively. The surgical results, postoperative recovery time, and serious complications were summarized, and the changes of postoperative cardiac function status and hemodynamic status of the conduits were investigated. During the implantation of ePTFE valved conduit for RVOT reconstruction, 2 patients underwent mechanical mitral valve replacement with Ross operation, 2 patients with pulmonary regurgitation with repaired TOF underwent left and right pulmonary artery angioplasty, and 1 patient with failed BJVC underwent tricuspid valvuloplasty. The cardiopulmonary bypassing time for patients underwent re-reconstruction of RVOT was (130.9±16.9) min, with aorta clamping for 1 patient to repair the residual defect of the ventricular septum. The cardiopulmonary bypassing and aorta clamping time for Ross operation were (242.7±20.6) min and (145.6±10.5) min, respectively. The duration of postoperative ventilator assistance, intensive care unit stay, and hospital stay were 3.5 h to 7.7 d (median 17.1 h),11.2 h to 29.5 d (median 1.9 d), and 6.0 to 56.0 (median 13.0) d, respectively. All patients survived after discharge from hospital. The follow-up rate after discharge was 100% with median time at 15.0 (13.0 to 39.0) months. No death happened during the follow-up. One patient underwent stent implantation due to right coronary stenosis 2 months after Ross operation. One patient underwent balloon dilation due to right pulmonary artery ostium stenosis 1 year after re-reconstruction of RVOT. The cardiac function of all patients recovered to NYHA class I 6 months after operation. The peak pressure gradient across the valve measured by transthoracic echocardiography before discharge was (9.4±2.6) mmHg (1 mmHg=0.133 kPa), and (18.3±6.1) mmHg at the last follow-up. There was no significant increase in the gradient during the follow-up (P=0.134). No patient suffered from mild or more pulmonary regurgitation. Hand-sewn ePTFE valved conduit is feasible for RVOT reconstruction. It is a promising material for RVOT reconstruction which can effectively meet clinical need. In our experience, the ePTFE valved conduit is simple to manufacture with satisfactory early outcomes.In the application of ePTFE valved conduit, attention should be paid to implantation indications and postoperative anticoagulation management, especially to the preparation details of the valved conduit, to obtain better function and durability of the conduit after implantation.

Novel Technique for Tetralogy of Fallot Repair with Transannular Patch Using Pedicled Own Pericardium.

In the surgical repair of the tetralogy of Fallot with a narrow pulmonary valve annulus, the transannular patch method is used for right ventricular outflow tract repair. To prevent pulmonary regurgitation and valve calcification, we created and applied a new transannular patch method using pedicled own pericardium in a 5-month-old boy with tetralogy of Fallot. After closing the ventricular septal defect as usual with a 0.4mm Gore-Tex sheet, we decided that the pulmonary valve could not be spared, because the pulmonary valve opening size was 6mm. After removing the right ventricular abnormal myocardium, each edge of the pedicled own pericardium patch was sewn from 5mm above the pulmonary valve commissures toward the basis of the pulmonary valve ring. The transannular patch was created using a Gore-Tex graft sawn to the right ventricular outflow tract. Echocardiography performed 6months post surgery showed no pulmonary stenosis and trivial pulmonary insufficiency.

Comparison of median sternotomy and left anterior mini-incision for pulmonary valve replacement following primary tetralogy of Fallot repair.

Pulmonary insufficiency requiring reintervention frequently occurs after primary tetralogy of Fallot repair. Repeat interventions present a challenge for both the surgeon and patient. We compare a minimally invasive, 5 cm left anterior mini-incision to redo median sternotomy for pulmonary valve replacement in tetralogy of Fallot patients. Following Internal Review Board approval, we conducted a single institution retrospective review of patients with tetralogy of Fallot who underwent pulmonary valve replacement via redo median sternotomy or left anterior mini-incision between 13 July, 2016 and 6 March, 2020. Twenty-three patients underwent pulmonary valve replacement following primary tetralogy of Fallot repair between March 2016 and March 2020. Twelve patients received a redo-median sternotomy from March 2016 to August 2018. Left anterior mini-incision was first offered in August of 2018 and was chosen by all eleven patients thereafter. The two groups had similar baseline characteristics including preoperative pulmonary valve dysfunction. Early trends suggest a longer cardiopulmonary bypass time for patients who received left anterior mini-incisions. Other outcomes were comparable, including operative times, blood product requirements, residual pulmonary valve dysfunction, postoperative pain, narcotic requirements, ICU length of stay, total length of stay, and postoperative complications. In patients who have previously undergone primary repairs of tetralogy of Fallot, outcomes for pulmonary valve replacement via left anterior mini-incision are comparable to those via redo median sternotomy.

Impact of right ventriculotomy for tetralogy of Fallot repair with a pulmonary valve–sparing procedure

ObjectivesThe study objectives were to reconfirm the superiority of the pulmonary valve-sparing procedure versus the transannular patch procedure for repair of tetralogy of Fallot and to evaluate the influence of a right ventriculotomy in the pulmonary valve-sparing procedure. MethodsBetween 1978 and 2003, 440 patients (aged <10 years) underwent tetralogy of Fallot repair. Of these patients, 242 (55.0%) underwent the transannular patch procedure, 106 (24.1%) underwent the pulmonary valve-sparing procedure without right ventriculotomy, and 92 (20.9%) underwent the pulmonary valve-sparing procedure with right ventriculotomy. End points focused on adverse events and included all-cause mortality, reoperation, catheter intervention, and symptomatic arrhythmias. To compare the outcomes of pulmonary valve sparing with and without right ventriculotomy, inverse probability weighting was applied to adjust for potential confounding factors. ResultsThe median follow-up period was 20.3 years (interquartile range, 10.7-27.6). In all cohorts, the pulmonary valve-sparing procedure was the independent factor that reduced adverse events after tetralogy of Fallot repair (hazard ratio, 0.47; 95% confidence interval, 0.23-0.94; P = .033). After weighting, there was no difference in overall survival or event-free survival in the pulmonary valve-sparing with and without right ventriculotomy group. However, the pulmonary valve-sparing with right ventriculotomy group exhibited a larger cardiothoracic ratio (beta: 6.01; 95% confidence interval, 2.36-9.66; P = .001), lower medication-free rate (odds ratio, 0.29; 95% confidence interval, 0.098-0.79; P = .019), and higher New York Heart Association functional classification (odds ratio, 2.99; 95% confidence interval, 1.36-6.80; P = .007) at the latest follow-up. ConclusionsRight ventriculotomy for tetralogy of Fallot repair with pulmonary valve-sparing did not increase major adverse events. However, negative impacts on current status cannot be ignored.

Pericardial effusion after congenital heart surgery

ObjectivePericardial effusion after cardiac surgery remains an important cause of morbidity and mortality. We describe the risk factors of pericardial effusion after congenital heart surgery through analyzing data from a nationwide, multi-institutional registry. MethodsThe Japan Congenital Cardiovascular Surgery Database, which reflects routine clinical care in Japan, was used for this retrospective cohort study. Multivariable regression analysis was done after univariable comparison of patients with pericardial effusion and no pericardial effusion. ResultsThe study enrolled 64,777 patients registered with the Japan Congenital Cardiovascular Surgery Database between 2008 and 2016; 909 of these had postoperative pericardial effusion (1.4%) and were analyzed along with 63,868 patients without pericardial effusion. Univariable analysis found no difference between the groups in terms of gender, early delivery, or preoperative mechanical ventilatory support. In the pericardial effusion group, cardiopulmonary bypass use was lower (58.4% vs 62.1%), whereas the cardiopulmonary bypass time (176.9 vs 139.9 minutes) and aortic crossclamp time (75.1 vs 62.2 minutes) were longer, and 30-day mortality was higher (4.1% vs 2.2%). Multivariable analysis identified trisomy 21 (odds ratio, 1.54), 22q.11 deletion (odds ratio, 2.17), first-time cardiac surgery (odds ratio, 2.01), and blood transfusion (odds ratio, 1.43) as independent risk factors of postoperative pericardial effusion. In contrast, neonates, infants, ventricular septal defect, atrial septal defect, tetralogy of Fallot repair, and arterial switch operation were correlated with a low risk of pericardial effusion development. ConclusionsThe incidence of postoperative pericardial effusion in congenital cardiac surgery was 1.4%. Trisomy 21, 22q.11 deletion, first-time cardiac surgery, and blood transfusion were identified as the principal factors predicting the need for pericardial effusion drainage.

Obesity and Over-Indexation of Right Ventricular Volumes—Potential Implications for Timing of Pulmonary Valve Replacement in Patients With Tetralogy of Fallot

In patients with pulmonary regurgitation (PR) after Tetralogy of Fallot (TOF) repair, cardiac MRI-derived right ventricular end diastolic volume index (RVEDVi)>150mL per m2 of body surface area (BSA) is sometimes considered an indication for pulmonary valve replacement. BSA however varies significantly with body habitus and RVEDVi can therefore under-represent right ventricular size in obese patients.

Variation in Body Surface Area Calculators Leads to Significant Changes in Indexed RV Volumes in Tetralogy of Fallot Patients

Indexed right ventricular end diastolic volume (RVEDVi) of greater than 150mL/m2 is often used as a threshold for pulmonary valve replacement in patients with severe pulmonary regurgitation (PR) post Tetralogy of Fallot (TOF) repair. There are multiple different formulae to estimate BSA and the method used in research papers isn’t always stated.

High Variability of Right Ventricular Volumes and Function in Adults with Severe Pulmonary Regurgitation Late After Tetralogy of Fallot Repair

Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ≥3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 ± 17.8ml/m2, change in RVEF: -1.0 ± 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2ml/m2; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p <0.001) and RVEF (0.815, 95% CI 0.697 to 0.887, p <0.0001) indicate high reliability of reported measurements. In conclusion, in asymptomatic adults with repaired tetralogy of Fallot with native right ventricular outflow tracts and severe pulmonary regurgitation, CMR measurements of RV volumes and RVEF remain stable during follow-up with variability between CMR studies in individual patients, as expected for interobserver and interstudy variability. Measurements derived from a single CMR study or changes occurring between 2 CMR studies should be used with caution for clinical decision-making.

Molecular signaling pathways in right ventricular impairment of adult patients after tetralogy of Fallot repair.

Right ventricular impairment (RVI) secondary to altered hemodynamics contributes to morbidity and mortality in adult patients after tetralogy of Fallot (TOF) repair. The goal of this study was to describe signaling pathways contributing to right ventricular (RV) remodeling by analyzing over lifetime alterations of RV gene expression in affected patients. RV tissue was collected at the time of cardiac surgery in 13 patients with a diagnosis of TOF. RNA was isolated and whole transcriptome sequencing was performed. Gene profiles were compared between a group of 6 adults with signs of RVI undergoing right ventricle to pulmonary artery conduit surgery and a group of 7 infants, undergoing TOF correction. Definition of RVI in adult patients was based on clinical symptoms, evidence of RV hypertrophy, dilation, dysfunction or elevated pressure on echocardiographic, cardiovascular magnetic resonance, or catheterization evaluation. Median age was 34 years in RVI patients and 5 months in infants. Based on P adjusted value <0.01, RNA sequencing of RV specimens identified a total of 3,010 differentially expressed genes in adult patients with TOF and RVI as compared to infant patients with TOF. Gene Ontology and Kyoto Encyclopedia of Genes databases highlighted pathways involved in cellular metabolism, cell-cell communication, cell cycling and cellular contractility to be dysregulated in adults with corrected TOF and chronic RVI. RV transcriptome profiling in adult patients with RVI after TOF repair allows identification of signaling pathways, contributing to pathologic RV remodeling and helps in the discovery of biomarkers for disease progression and of new therapeutic targets.

Outcomes for unplanned reinterventions following paediatric cardiac surgery for tetralogy of Fallot.

Advances in surgical techniques and post-operative management of children with CHD have significantly lowered mortality rates. Unplanned cardiac interventions are a significant complication with implications on morbidity and mortality. We conducted a single-centre retrospective case-control study for patients (<18 years) undergoing cardiac surgery for repair of Tetralogy of Fallot between January 2009 and December 2019. Data included patient characteristics, operative variables and outcomes. This study aimed to assess the incidence and risk factors for reintervention of Tetralogy of Fallot after cardiac surgery. The secondary outcome was to examine the incidence of long-term morbidity and mortality in those who underwent unplanned reinterventions. During the study period 29 patients (6.8%) underwent unplanned reintervention, and were matched to 58 patients by age, weight and sex. Median age was 146 days, and median weight was 5.8 kg. Operative mortality was 7%, and 1-year survival was 86% for the entire cohort (cases and controls). Hispanic patients were more likely to have reinterventions (p = 0.04) in the unadjusted analysis, while Asian, Pacific Islander and Native American (p = 0.01) in the multi-variate analysis. Patients that underwent reintervention were more likely to have post-op arrhythmia, genetic syndromes and higher operative and 1-year mortality (p < 0.05). Unplanned cardiac interventions following Tetralogy of Fallot repair are common, and associated with increased operative, and 1-year mortality. Race, genetic syndromes and post-operative arrhythmia are associated with increased odds of unplanned reinterventions. Future studies are needed to identify modifiable risk factors to minimise unplanned reinterventions.

Does interatrial communication affect post-operative course of children undergoing tetralogy of Fallot repair? Single centre retrospective cohort study: propensity score matching.

During tetralogy of Fallot repair, leaving or even create an interatrial communication may facilitate post-operative course particularly with right ventricle restrictive physiology. The aim of our study is to assess the influence of atrial communication on post-operative course of tetralogy of Fallot repair. Retrospectively, we studied all children who had tetralogy of Fallot repair (2003-2018). We divided them into two groups: tetralogy of Fallot repair with interatrial communication (TOFASD) group and tetralogy of Fallot repair with intact atrial septum (TOFIAS) group. We performed propensity match score for specific pre- or intra-operative variables and compared groups for post-operative outcome variables. Secondarily, we looked for right ventricle restrictive physiology incidence and influence of early repair performed before 3 months of age on post-operative course. One hundred and sixty children underwent tetralogy of Fallot repair including (93) cases of TOFIAS (58%) and (67) cases of TOFASD (42%). With propensity matching score, 52 patients from each group were compared. Post-operative course was indifferent in term of positive pressure ventilation time, vasoactive inotropic score, creatinine and lactic acid levels, duration and amount of chest drainage and length of intensive care unit and hospital stay. Right ventricle restrictive physiology occurred in 38% of patients with no effects on outcome. 12/104 patients (12%) with early repair needed longer pressure ventilation time (p = 0.003) and intensive care unit stay (p = 0.02). Leaving interatrial communication in tetralogy of Fallot repair did not affect post-operative course. As well, right ventricle restrictive physiology did not affect post-operative course. Infants undergoing early tetralogy of Fallot repair may require longer duration of positive pressure ventilation time and intensive care unit stay.