Repair Of Tetralogy Research Articles

Factors Associated with Prolonged Mechanical Ventilation in Late Repair of Tetralogy of Fallot.

Research establishing factors associated with duration of mechanical ventilation after Tetralogy of Fallot repair, is mainly based on population presenting at early infancy. There are fewer reports regarding repair after infancy, during childhood and preadolescence. To compare two groups of late TOF repair based on post-operative invasive mechanical ventilation duration and explore associations with pre-operative clinical markers of severity of right ventricular outflow tract obstruction. A single-center retrospective cohort study based on medical charts review of patients older than 12months who underwent primary complete TOF repair between 2017 and 2023. Patients younger than 12months or those who had distinct anatomical variants were excluded. Pre-operative characteristics reflecting the severity of right ventricular outflow tract obstruction were collected, including baseline oxygen saturation, the occurrence of hypercyanotic spells, maximum right ventricular outflow tract pressure gradient. Perioperative variables included repair type, cardiopulmonary bypass and aortic cross-clamp duration, and rates of residual lesions. 280 patients from 27 countries were screened; 181 remained eligible and were significantly older than previously reported. 129 (71%) were extubated early post-operatively, while 52 (29%) had a longer ventilation course. In a multivariate analysis, only baseline oxygen saturation and repair type maintained a significant association with length of mechanical ventilation. In distinctly older patients undergoing TOF repair, those with lower base line saturation undergoing a non-valve preserving repair are more likely to have a longer course of mechanical ventilation. Other clinical markers reflecting the severity of right ventricular outflow tract obstruction were not independently associated with mechanical ventilation duration.

(Un)natural history of right ventricular function and exercise tolerance during adolescence following tetralogy of fallot repair

Abstract Introduction Patients with repaired tetralogy of Fallot (rTOF) develop progressive right ventricular (RV) dilatation and dysfunction in adulthood, which may have origins in childhood. Cohorts from previous eras studied patients who underwent primary repair at an older age with frequent use of palliative shunts. The trajectory of RV functional decline and its relation to exercise capacity and outcomes over childhood is poorly characterized. Purpose We sought to delineate changes in RV size, function, and exercise tolerance and occurrence of ventricular arrhythmia in a contemporary cohort of pediatric and adolescent rTOF. Methods We retrospectively reviewed serial echo, cardiac magnetic resonance (CMR) and clinical data from all rTOF patients between 2010-2020. We excluded patients with significant residual lesions, RV pressure overload (RVSP> half systemic) or without CMR data. Clinical data was recorded at 2-year intervals. We then compared CMR data and exercise tolerance at 10 to 16 years of age. Ventricular arrhythmias were defined based on Holter monitoring. Results We studied 95 rTOF patients (61% male). Pulmonary insufficiency fraction (31% vs 32%, p=0.8), RV size (RV end diastolic indexed volume 130 ml/m2 (117 – 167) vs. 140 ml/m2 (124 – 166), p=0.6) and RV free wall longitudinal strain (19.4±4.3% vs. 19.3±4.7%, p=0.86) remained stable at 10 and 16 years of age. CMR RVEF (51±6% vs. 47±5.3%, p=0.01) and exercise tolerance (95±17% vs. 81±14.4%, % predicted VO2, p=0.002, figure B) decreased with the advent of incident ventricular arrhythmias (figure A). There were no deaths. Conclusions Despite excellent survival in childhood, patients with rTOF develop worsening RV function, exercise intolerance and ventricular arrhythmias during adolescence. These are worrisome findings given the known increase in sudden death in the 3-4th decades of life that warrant further investigation into the underlying causes, their temporal development and therapeutic approaches. Figure 1: A-Kaplan-Meier curve of incident ventricular and atrial arrhythmias. B- percentage of predicted VO2 during childhood.

Development of right ventricular electromechanical dyssynchrony following surgical repair of tetralogy of Fallot in infants

Development of right ventricular electromechanical dyssynchrony following surgical repair of tetralogy of Fallot in infants

Comparing Parent Perception of Neurodevelopment after Primary versus Staged Repair of Neonatal Symptomatic Tetralogy of Fallot

To assess the association between primary and staged repair of neonatal symptomatic tetralogy of Fallot (sTOF) and neurodevelopmental outcomes in preschool through school-age children. Multicenter cohort (n=9 sites) study of patients with sTOF who underwent neonatal intervention between 2005 and 2017. The neurodevelopmental outcomes measures included caregivers' ratings of executive function with the Behavior Rating Inventory of Executive Function, and psychosocial functioning with the Behavior Assessment System for Children - third Edition (BASC-3). Results were compared with normative data and by treatment strategy (primary repair vs staged repair). A parent survey assessed history of disabilities and access to services related to neurodevelopment. Although the majority of patients (median age 8.3years, IQR 5.7-11.2) had median Behavior Rating Inventory of Executive Function and BASC-3 scores within the normal range, a proportion had clinically elevated (abnormal) scores, especially in the school-age patient subgroup (Behavior Rating Inventory of Executive Function 24%-30% and BASC 20%-37%). There were no statistically significant differences based on treatment strategy for either the Behavior Rating Inventory of Executive Function or BASC-3. However, lower birth weight, genetic syndrome, and medical complexity were significantly associated with worse executive function, and lower maternal education was associated in school-age children with lower executive and psychosocial functioning. Ongoing disabilities were relatively common (learning disability 35%, speech delay 33%, developmental delay 31%), although up to 50% of children were not receiving educational or developmental services. Elevated executive and psychosocial concerns are present in the patient population with sTOF. Although initial treatment strategy appears unrelated to neurodevelopmental outcomes, lower birth weight, genetic syndrome, and medical complexity and lower maternal education are risk factors. Early recognition of neurodevelopmental concerns can facilitate access to appropriate neurodevelopmental services in this high-risk group.

Accuracy of Intraoperative Transesophageal Echocardiographic Doppler Parameters in Assessing the Right Ventricular Diastolic Function After Repair of Tetralogy of Fallot in Pediatric Patients.

This study aimed to assess the accuracy of tricuspid spectral Doppler (E/A) and tissue Doppler parameters (E/E') to diagnose right ventricular diastolic dysfunction (RVDD) in comparison to American Society of Echocardiography (ASE criteria) in pediatric tetralogy of Fallot (TOF) patients after surgical repair. This prospective, observational study was done at a tertiary care hospital involving 40 pediatric TOF patients aged less than 2 years who underwent complete intracardiac repair with cardiopulmonary bypass (CPB). Echocardiographic observations were made using a pediatric transesophageal echocardiography probe after surgical repair in the post-CPB period. The ASE-described parameters (late diastolic forward flow in the main pulmonary artery, right atrial dilatation, inferior vena cava dilatation, and hepatic venous flow reversal) were acquired to diagnose the RVDD. The tricuspid Doppler parameters (E/A and E/E') were measured, and its predictive ability to diagnose RVDD was analyzed and compared with the ASE criteria. Based on the ASE criteria, 13 patients (32.5%) were diagnosed to have RVDD. We found that an E/E' ratio of ≥ 6.26 and an E/A ratio of >1.34 can be taken as the cutoff for diagnosing the RVDD. Based on the tricuspid Doppler parameters (E/A > 1.34 and E/E' >6.26), 17 patients (42.5%) were diagnosed to have RVDD, which was comparable to that of the diagnosis by ASE criteria (P > 0.05). The results suggested that the use of tricuspid Doppler parameters can be equally accurate and reproducible to the current ASE-described echocardiographic parameters for diagnosing RVDD after surgical repair of TOF.

Association Between Location of Pulmonary Outflow Obstruction and Right Ventricular Size and Function After Repair of Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals.

Quantification of right ventricular (RV) size and function after tetralogy of Fallot repair is critical for determining timing of reintervention and outcomes. Tetralogy of Fallot patients with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) are a unique group in which the RV is subjected to various loading conditions, allowing for direct comparison. Retrospective evaluation of RV echocardiographic indices in repaired pediatric TOF/PA/MAPCAs patients (2/2002 - 4/2018). Patients were categorized as having conduit stenosis (peak gradient ≥ 2.5m/s) and/or distal pulmonary artery (PA) stenoses (requiring catheter or surgical intervention and > 1/2 systemic RV pressures). A comparison group (N = 9) included distinct age-matched repaired patients without residual lesions. Indexed right heart dimensions were larger in patients with distal PA stenoses (N = 49) compared to conduit stenosis (N = 26), including RV end-diastolic and end-systolic dimensions (p < 0.01), despite earlier time to reintervention (p < 0.0001). RV fractional area change (FAC) was diminished in patients with distal PA stenoses compared to patients without residual lesions (p = 0.006). Patients with conduit stenosis demonstrated a marked decrement in tricuspid annular plane systolic excursion (TAPSE) z-scores. Repaired TOF/PA/MAPCAs patients with residual distal PA stenoses have larger right heart dimensions compared to patients with conduit stenosis, and decreased RV FAC compared to patients without residual lesions at 1-2years after repair, suggesting early surveillance and immediate intervention on stenotic PA segments appears warranted to decrease the risk of further deterioration of RV systolic function.

Propranolol palliation after right ventricular outflow tract stenting reduces the reintervention rate until complete repair of Fallot tetralogy and variants.

Stenting of the right ventricular outflow tract (RVOT) is a reasonable palliation in symptomatic infants with tetralogy of Fallot (ToF) and variants. However, this procedure needs reintervention until corrective surgery. To compare RVOT stenting followed with or without propranolol medication until complete repair of ToF and variants. Twenty-five cyanotic infants under 6 months of age with ToF and variants underwent RVOT stenting between March 2017 and May 2024 including the first 11 followed without propranolol medication and the next 14 followed with propranolol medication. Median age at initial RVOT stent implantation was 92 days and similar in both groups. At the time of this writing, total correction of ToF has been performed in 23 patients including 11 followed without propranolol medication and 12 followed with propranolol medication. Median age at the time of surgery was 258 days and similar in both groups. The rate of reintervention before complete repair was 8/25 (32%) during follow-up for recurrence of cyanosis, including 6/11 (54%) patients followed without propranolol medication and 2/14 (14%) in patients with propranolol medication (p = 0.041). The remaining 2 patients followed with propranolol medication required reintervention 150 and 170 days after initial intervention. Long-term treatment with propranolol given after RVOT stenting in ToF and variants, particularly under 3 months of age, may reduce the need for further interventions prior to complete repair.

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.

Right ventricular outlet tract reconstruction for tetralogy of Fallot: systematic review and network meta-analysis.

Concerns persist regarding pulmonary regurgitation after transannular patch repair (TAP) for tetralogy of Fallot. Despite the introduction of various architectural preservation techniques, the optimal strategy remains controversial. Our goal was to compare different right ventricular outlet tract reconstruction techniques. PubMed, EMBASE and Cochrane Central were searched through March 2024 to identify comparative studies on right ventricular outlet tract reconstruction techniques (PROSPERO ID: CRD42024519404). The primary outcome was mid-term pulmonary regurgitation, with secondary outcomes including postoperative mortality, postoperative pulmonary regurgitation, length of intensive care unit stays, postoperative right ventricular outlet tract pressure gradient and mid-term mortality. We performed a network meta-analysis to compare outcomes among TAP, valve-repairing (VR), TAP with neo-valve creation (TAPN) and valve-sparing (VS). Two randomized controlled studies and 32 observational studies were identified with 8890 patients. TAP carried a higher risk of mid-term pulmonary regurgitation compared to TAPN [hazard ratio, 0.53; 95% confidence interval (CI) (0.33; 0.85)] and VS [hazard ratio, 0.27; 95% CI (0.19; 0.39)], with no significant difference compared to VR. VS was also associated with reduced postoperative mortality compared to TAP [risk ratio, 0.31; 95% CI (0.18; 0.56)], in addition to reduced ventilation time. TAP also carried an increased risk of postoperative pulmonary regurgitation compared to the other groups. The groups were comparable in terms of length of intensive care unit stay, right ventricular outlet tract pressure gradient and mid-term mortality. VR was associated with a reduced risk of postoperative pulmonary regurgitation, whereas TAPN was associated with reduced risks of both postoperative and mid-term pulmonary regurgitation.

Results of Vertical Infra-Axillary Thoracotomy for Total Repair of Tetralogy of Fallot.

To demonstrate the efficacy of minimally invasive surgery via a vertical infra-axillary incision for complete tetralogy of Fallot (TOF) correction. In a study conducted from April to October 2023, 33 patients with TOF underwent total repair using this approach. On average, the patient age was 5.94 ± 2.68 months, weight was 6.49 ± 0.97 kg, and mean z-score index for the pulmonary valve annulus was -1.38 ± 0.92. Results also highlighted abnormal coronary artery pathways in 18.2% of cases, including 1 patient with dextrocardia and situs inversus. The average incision length was 4.01 ± 0.6 cm, with bypass and clamping times of 95.42 ± 33.19 min and 69.24 ± 28.15 min, respectively. Preservation of the pulmonary valve annulus was achieved in 67% of patients. No postoperative deaths occurred, and there were no significant ventilation differences between groups. After surgery, no severe pulmonary valve regurgitation was observed, with patients remaining in excellent condition throughout the 7-month follow-up. The pulmonary valve pressure gradient after the procedure was 23.97 ± 10.65 mm Hg, and no heart failure cases were reported per the Ross classification at the latest follow-up. The vertical infra-axillary incision approach for total TOF repair is safe, effective, and cosmetically advantageous.

Interaction between right atrial function reserve and liver stiffness after complete repair of tetralogy of fallot: a pilot study

Abstract Background Impaired right atrial mechanics is reported in patients with repaired tetralogy of Fallot (TOF) and is associated with liver stiffness. However, whether right atrial functional reserve correlates with liver stiffness is unknown. Purpose This study tested the hypothesis that right atrial reserve capacity is limited in patients with repaired TOF and explore its relationships with right ventricular function and liver stiffness. Methods 10 patients (45% male) aged 19.04 ± 3.76 at 16.70 ± 4.30 years after repair and 15 controls (60% male, aged 20.31 ± 1.25 years) were studied. RA mechanics was assessed by speckle-tracking echocardiography (STE) at rest and during bicycle exercise, with quantification of positive, negative, and total strain, and strain rates at ventricular systole (aSRs), early diastole (aSRed), and atrial contraction (aSRac). Right atrial function reserve (RAFR) is calculated as (∆RA total strain x [1-1/ RA total strain at baseline]). RV systolic and diastolic function was quantified using Doppler interrogation and STE. Hepatic shear wave velocity (c) and tissue elasticity (E) were measured using two-dimensional shear wave elastography. Results At rest, patients had significantly lower RA positive, negative and total strain, aSRs and aSRed than control subjects (all P&amp;lt;0.02). Shear wave velocity and hepatic E value were significantly higher in patients than controls (both P&amp;lt;0.02). Compared with controls, patients had significantly lower RA positive, negative and total strain, aSRs, aSRed, and aSRac at exercise (all P&amp;lt;0.05). No significant difference in hepatic shear wave velocity and tissue elasticity were found between patients and control subjects at exercise (all P&amp;gt;0.05). Both baseline liver stiffness indices correlated negatively with RAFR, RA positive and total strain and aSRed at exercise (p&amp;lt;0.05 for all). RV function parameters, including baseline trans-tricuspid early and late diastolic inflow velocity (E, A), systolic, early and late diastolic tricuspid annular velocity (s, e, a), isovolumetric acceleration (IVA), RV systolic and diastolic reserve correlated significantly with RA positive and total strain at exercise (P&amp;lt;0.05 for all). Conclusions In young adults with repaired TOF, right atrial function reserve is impaired, and is associated with increased liver stiffness and RV dysfunction.RA mechanics from rest to exerciseStress RA mechanics and liver stiffness

The impact of tricuspid regurgitation on the timing of pulmonary valve replacement in adult patients after Tetralogy of Fallot repair

Abstract Background Tricuspid regurgitation (TR) in patients with repaired tetralogy of Fallot (rTOF) is an important finding with a wide spectrum of etiologies. TR represents an important risk factor in adult patients with rTOF, influencing clinical outcomes and the requirement for additional surgical interventions. In this study, we reviewed the long-term outcomes of TR in ACHD patients with rTOF as well as its impact on the timing of surgical pulmonary valve replacement (PVR). Methods This was a retrospective analysis of a single institutional cohort which included 149 ACHD patients with a history of rTOF (transannular patch only) with CMR data, complete follow-up, and a recent (&amp;lt; 1 year) outpatient clinic visit. Results Overall follow-up time was 25.5 years (95% CI 24.5,26.4). Between 2002 and 2021, 93 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95% CI 15.5,17.7) and at a mean age of 17.9 years (95% CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase in the likelihood of requiring PVR (log-rank p = 0-.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = .0002) and affected time to PVR (13.9 versus 16.3 years, p = .0071), with equal preoperative RVEDVi at CMR. After PVR, RVEDVi normalized in all patients, regardless of the preoperative amount of TR (median 115.3 ml/m2 versus 112.3 ml/m2, p = .7). At a median time of 6.5 years from PVR, 13 patients (13/93, 14%) underwent redo PVR. Conclusions The preoperative severity of tricuspid regurgitation (TR) may have a substantial role on the likelihood of patients needing PVR at a younger age. This, in turn, could result in a reduced duration of freedom from valve failure. Replacing the pulmonary valve too early can effectively increase the lifetime number of procedures, preserving the function of the tricuspid valve at the time of TOF repair is of paramount importance.

Primary versus staged repair for tetralogy of Fallot in symptomatic neonates: systematic review and meta-analysis.

The choice between primary repair and staged repair strategy for Tetralogy of Fallot remains a subject of debate in clinical practice. This review aims to compare the outcomes and efficacy of two surgical approaches in managing Tetralogy of Fallot among neonatal populations. Literature search was conducted across seven databases, identifying a total of 1393 relevant studies. Inclusion criteria encompassed comparative studies focusing on primary repair and staged repair for Tetralogy of Fallot in neonates. Quality of included studies was assessed using The Newcastle-Ottawa Scale for retrospective cohort studies. Data synthesis involved the extraction of post-operative outcomes. Meta-analysis was performed where feasible, pooling effect sizes to determine the overall impact of each repair strategy. Eight studies were selected for full-text appraisal. A total of 4464 Tetralogy of Fallot patients underwent surgical correction. The pooled mean patient age was 8.68 (±7.38) and 8.56 (±6.8) days for primary repair and staged repair, respectively. The primary repair was associated with a higher cardiac complications rate (odds ratio 1.50, 95% confidence interval 1.07 to 2.10) and transannular patch usage (odds ratio 2.62, 95% CI confidence interval 2.02 to 3.40). In contrast, staged repair was associated with longer hospital(mean difference11.84, 95% confidence interval 9.59 to 14.10) and ICU (mean difference 3.06, 95% confidence interval 1.64 to 4.47) length of stay. However, no substantial differences were observed in terms of mortality and reintervention rates between these two approaches. The findings highlight the need for well-designed research and emphasise the importance of personalised approaches to address the intricate nature of Tetralogy of Fallot management in this population. Adjusting surgical approach to patient features may be necessary to maximise surgical outcomes.

Higher left ventricular stroke volume is associated with aortic dilatation in repaired tetralogy of Fallot patients.

Aortic root dilation has been reported commonly after repair of tetralogy of Fallot. However, the rate and risk factors of progression of the dilation are not fully understood. This is a single-centre, retrospective study to assess the rate and factors associated with progressive dilatation of the aortic root in repaired tetralogy of Fallot patients using cardiac MRI. The presence of the significant aortic dilation and the progression of dilation between initial and follow-up cardiac MRI were examined. The study cohort comprised 72 patients with repaired tetralogy of Fallot. The median age at the initial cardiac MRI scan was 19.6 (interquartile range: 14.6-31) years, and the median follow-up interval was 4.3 (2.9-5.7) years. Median dimension of ascending aorta at initial and follow-up cardiac MRI was 27.0 (22.3-31.0) mm and 29.2 (25.0-32.1) mm, respectively. Significant aortic dilation (the percentage predicted ascending aorta ≥150%) was observed in 11 (15.2%) patients at the initial cardiac MRI and 24 (33.3%) at the follow-up cardiac MRI. The significant aortic dilation at follow-up cardiac MRI was associated with increased indexed left ventricular stroke volume (odds ratio 1.062, p = 0.023). Thirteen patients demonstrated the significant progressive dilation of aorta between initial and follow-up cardiac MRI. The progressive dilation was associated with left ventricular ejection fraction at initial cardiac MRI (odds ratio 1.135, p = 0.048). In patients with repaired tetralogy of Fallot, aortic dilation is common and progresses over time. Cardiac MRI is a valuable tool for identifying individuals at risk for progressive aortic dilation.

The impact of a residual atrial communication in patients undergoing complete repair for tetralogy of Fallot: A propensity score–matched analysis

BackgroundSurgeons may leave a residual atrial-level communication during complete repair of tetralogy of Fallot (TOF) in anticipation of restrictive right ventricle physiology or as routine practice. We investigated the impact of closing the interatrial communication at the time of definitive TOF repair. MethodsWe retrospectively reviewed TOF patients who underwent definitive repair at age <12 months between June 2000 and January 2023. Propensity score matching identified 82 patients with a patent interatrial communication and 50 patients with no interatrial communication on postoperative echocardiography (as-treated analysis). The primary endpoint was maximum vasoactive-inotropic score (VIS) as a surrogate for low cardiac output syndrome. ResultsA total of 132 patients (median age, 3.5 months; interquartile range [IQR], 1.8-5.8 months) were matched. There was no difference in maximum VIS (patent interatrial communication: 5.0 [IQR, 4.8-9.0] vs no interatrial communication: 6.0 [IQR, 5.0-8.0]; P = .78). Additionally, the duration of inotrope therapy (3.0 [IQR, 2.0-4.0] days vs 3.0 [IQR, 1.3-4.0] days; P = .57), peak lactate (2.2 [IQR, 1.9-3.0] mmol/L vs 2.3 [IQR, 1.9-3.2] mmol/L; P = .58), time to lactate clearance (0.2 [IQR, 0.0-0.3] days vs 0.1 [IQR, 0.0-0.3] days; P = .57), chest tube duration (4.0 [IQR, 3.0-6.0] days vs 4.0 [IQR, 3.0-5.0] days; P = .23), and length of intensive care unit stay (5.0 [IQR, 3.0-7.0] days vs 5.0 [IQR, 3.0-7.0] days; P = .71) were similar in the 2 groups. The median duration of follow-up was 5.5 years (IQR, 2.7-9.9 years). Among patients with a residual communication, patency rates were 93.6% at discharge and 53.7% at latest follow-up, with most having bidirectional shunting across the defect. ConclusionsClosure of the atrial-level communication during complete TOF repair does not significantly impact the immediate postoperative course or mid-term outcomes. Further investigation is warranted to better understand how patency influences long-term outcomes.

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.

Effect of Tadalafil on Heart Function in Children with Severe Pulmonary Regurgitation Following Surgical Repair of Tetralogy of Fallot: A Prospective Study

Background: Tetralogy of Fallot (TOF) is the most prevalent cyanotic congenital heart defect. Pulmonary regurgitation (PR) is a common sequela following most surgical repairs for TOF. Tadalafil might reduce pulmonary vascular resistance after Tetralogy of Fallot total correction (TOFTC). Objectives: This study evaluated the efficacy of tadalafil in reducing PR volume and improving heart function among TOFTC children with severe PR, using transthoracic echocardiography, particularly focusing on changes in N-terminal pro-b-type natriuretic peptide (NT-proBNP). Methods: The present study was conducted on TOFTC patients consecutively between September 2019 and August 2020 at Shiraz University of Medical Sciences, Shiraz, Iran. M-mode and two-dimensional (2D) Doppler echocardiography were performed, and NT-proBNP levels were measured in 20 patients before and one month after tadalafil administration. SPSS version 23.0 was utilized to analyze all results. Results: The patients' ages ranged from 25 to 128 months. The mean age and weight of the patients were 67.9 ± 34.5 months and 21.1 ± 6.9 kg, respectively. Tadalafil administration did not significantly improve Doppler and tissue Doppler parameters; however, it increased the pulmonary valve pressure gradient and velocity-time integral. Additionally, tadalafil had no significant effect on improving NT-proBNP levels. The Spearman correlation test did not show any significant correlation between the pulmonary valve pressure gradient and velocity-time integral with age, weight, and NT-proBNP. Conclusions: Tadalafil increased the pulmonary valve pressure gradient and velocity-time integral in TOFTC patients with severe PR; however, it did not affect NT-proBNP levels or tissue Doppler parameters.

Early outcomes of experience warm surgery in children undergoing complete repair of tetralogy of Fallot in developing countries

ObjectivesWhile significant evidence supports the benefits of normothermic cardiopulmonary bypass (NCPB) over hypothermic techniques, many institutions in developing countries, including ours, continue to employ hypothermic methods. This study aimed to assess the early postoperative outcomes of normothermic cardiopulmonary bypass (NCPB) for complete surgical repair via the Tetralogy of Fallot (TOF) within our national context.MethodsWe conducted this study in the Pediatric Cardiac Intensive Care Unit (PCICU) at the University Children’s Hospital. One hundred patients who underwent complete TOF repair were enrolled and categorized into two groups: the normothermic group (n = 50, temperature 35–37 °C) and the moderate hypothermic group (n = 50, temperature 28–32 °C). We evaluated mortality, morbidity, and postoperative complications in the PCICU as outcome measures.ResultsThe demographic characteristics were similar between the two groups. However, the cardiopulmonary bypass (CPB) time and aortic cross-clamp (ACC) time were notably longer in the hypothermic group. The study recorded seven deaths, yielding an overall mortality rate of 7%. No significant differences were observed between the two groups concerning mortality, morbidity, or postoperative complications in the PCICU.ConclusionsOur findings suggest that normothermic procedures, while not demonstrably effective, are safe for pediatric cardiac surgery. Further research is warranted to substantiate and endorse the adoption of this technique.

Electrocardiogram changes post surgical repair of tetralogy of Fallot with valve-sparing versus transannular patch: A retrospective observational study.

Introduction: Long-term survival after tetralogy of Fallot (TOF) repair depends on several factors including the extent of chronic right ventricular adaptation to surgery. QRS duration (QRSd) is an important determinant of life-threatening arrhythmia post-TOF repair. This single-center retrospective study was designed to evaluate changes in QRSd post-TOF repair using either a pulmonary valve-sparing approach (VSA) or transannular patch (TAP). Methods and results: Data from patients who underwent TOF repair between January 2016 and December 2019 were analyzed to compare the changes in the QRSd following intracardiac repair after VSA (Group 1) or TAP (Group 2). Among the 105 patients who underwent TOF surgical repair, 60 were included in the study (Group 1:30 vs. Group 2:30). Electrocardiograms (ECGs) were recorded pre- and post-surgery. The primary outcome was to compare the change in QRSd (ΔQRSd) before and after surgery between the two groups. The mean length of postoperative follow-up was 35.9 months in group 1 and 34.47 months in group 2. The mean [SD] difference in QRSd values (QRSd2 - QRSd1) was shorter in Group 1 (45.67 [22.79] ms) than in Group 2 (49.63 [23.76] ms); however, these differences were not statistically significant (p = 0.428). The PR interval was similar between the two groups in both preoperative and postoperative ECG. Conclusion: At the short-term follow-up, both surgical approaches (VSA and TAP) resulted in similar QRSd post-TOF repair. Studies with longer follow-up periods are required to evaluate the association of the surgical approach with prolongation of QRSd and mortality.

Complex Pulmonary Artery Rehabilitation in Children with Alagille Syndrome: An Early Single-Center Experience of a Successful Collaborative Work.

Objective: In this paper, we share our single-center experience of successful multidisciplinary management of patients with Alagille syndrome. In addition, we aim to highlight the need for an Alagille program for effectively managing these patients, in general, and particularly peripheral pulmonary artery stenosis associated with this syndrome. Study Design: This is a retrospective review of six children with Alagille syndrome and advanced liver involvement who underwent pulmonary artery reconstruction between 2021 and 2022. Cardiac diagnosis, co-existing liver disease burdens, management approach, and short-term outcomes were analyzed. Results: All the patients underwent one-stage extensive bilateral branch pulmonary rehabilitation. Concomitant procedures included repair of tetralogy of Fallot in one patient and repair of supravalvar pulmonary artery stenosis in two. One patient had balloon pulmonary branch angioplasty before surgery. In all patients, there was a decrease in right ventricular systolic pressure post-operatively. Three patients underwent liver transplantation for pre-existing liver dysfunction. At a median 3-year follow-up, all the patients were alive with their right ventricular systolic pressure less than half of their systemic systolic pressure. One patient underwent balloon angioplasty due to new and recurrent left pulmonary artery stenosis 13 months after surgery. Conclusion: Pulmonary arteries can be successfully rehabilitated surgically in the presence of complex branch disease. Patients with advanced liver disease can undergo successful complex pulmonary artery reconstruction, which can facilitate their future liver transplantation course. A multidisciplinary team approach is a key for successful management of Alagille patients.