TOF Repair Research Articles

Abstract 4143242: Right Ventricular Remodeling During the First Decade After Tetralogy of Fallot Repair: An Echocardiographic Study

Background: After TOF repair (TOFr), pulmonary regurgitation resulting in right ventricular (RV) dilatation and dysfunction is common. We have previously demonstrated that RV function (RVF) improves in the first two years after TOFr, however RV changes in the first decade after TOFr are less known. We investigated RVF and RV size (termed remodeling) using echocardiography during the first decade after TOFr. Methods: Retrospective cohort study of patients who underwent TOFr and were followed at our institution from 3/2012 to 12/2023. Those with at least two echocardiograms separated by one year were included. Two patients that received a pulmonary valve replacement were excluded. After confirming intra- and inter-reader reproducibility, RV remodeling was assessed offline by global longitudinal strain (GLS), free wall strain (FWS), fractional area change (FAC), end systolic area (ESA/BSA) and end diastolic area (EDA/BSA). RVF changes over time were assessed with multivariable linear mixed effects models adjusting for perioperative covariates. Two years after TOFr (Y2) was used as reference given RVF peaks at this point following surgery. Results: Out of 758 echocardiograms from 116 patients, 688 were suitable for analysis. Median age at TOFr was 3.5 months (IQR:1.92, 4.89). Compared to Y2, we identified the largest decline in RVF at Year 3, with no worsening from year to year until Y10. There were no associations of perioperative covariates with RVF except for ventriculotomy (FAC) and residual pulmonary stenosis (FWS). There was no progression in RV remodeling over the first decade after TOFr. (Graph, table). Conclusion: After the initial 2-year improvement following TOFr, RVF worsens but remains stable throughout the first decade with stable RV size accounting for BSA. Our findings augment the knowledge of RV remodeling during childhood and could help guide outpatient follow up, counseling, frequency of echocardiograms, and timing of the first cardiac MR.

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 (< 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.

Early surgical outcome for Tetralogy Of Fallot In An African Setting; A Tanzanian experience using retrospective analysis of hospital data

BackgroundTetralogy of Fallot (TOF) is typically treated in infancy but often done late in many resource-limited countries, jeopardizing surgical outcomes. This study examined the early results of children undergoing primary complete TOF repair at the Jakaya Kikwete Cardiac Institute (JKCI) in Tanzania, an emerging cardiac center in Eastern Africa.MethodsA retrospective cohort study of children ≤ 18 years undergoing primary TOF complete repair between 2019 and 2021 was conducted. Patients with complex TOF and those with obvious genetic syndrome were excluded. Data on socio-demography, pre-and postoperative cardiac complications, Intensive Care Unit (ICU) and hospital stay, and in-hospital and 30-day mortality were analyzed. Logistic regressions were employed to find the factors for mortality, ICU, and hospital stays.ResultsThe I02 children underwent primary TOF complete repair were majority male (65.7%; n = 67), with a median age of 3.0 years (IQR: 2–6), ranging from 3 months to 17 years.Only 20 patients (19.6%) were below one year of age. Almost all (90%; n = 92) were underweight, with a mean BMI of 14.6 + 3.1 kg/m2 Haematocrits were high, with a median of 48.7 (IQR: 37.4–59.0). The median oxygen saturation was 81% (IQR:72–93). Over a third of patients (38.2%; n = 39) needed Trans annular patch (TAP) during surgery. The median ICU stay was 72 h (IQR:48–120), with ICU duration exceeding three days for most patients. The median hospital stay was 8.5 days (IQR:7–11), with 70 patients (68.2%)experiencing an extended hospital stay of > 7 days. Bacterial sepsis was more common than surgical site infection (5.6%; n = 6 vs. 0.9%;n = 1). No patient needed re-operation for the period of follow up. The in-hospital mortality rate was 5.9%, with no deaths occurring in children less than one year of age nor after discharge during the 30-day follow-up period. No statistically significant differences were observed in outcomes in relation to age, sex, levels of hematocrit and saturations, presence of medical illnesses, and placement of TAP.ConclusionTOF repairs in this African setting at a national cardiac referral hospital face challenges associated with patients’ older age and compromised nutritional status during the surgery. Perioperative mortality rates and morbidity for patients operated at an older age remain elevated. It’s important to address these issues to improve outcomes in these settings.

Primary surgical repair of tetralogy of fallot at the Uganda Heart Institute: a ten-year review of 30day mortality and morbidity

BackgroundTetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources.ObjectiveThis study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated.MethodologyThis retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation.ResultsOut of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan’s, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team.ConclusionThe most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team.

Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation.

Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs. A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock-Taussig shunt at 2years of age with transannular patching at 18years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient's new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation. It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.

Staged therapeutic surgery for progressive pulmonary regurgitation and pacemaker induced cardiomyopathy after the tetralogy of fallot repair

BackgroundRecently, improvements in the repair of tetralogy of Fallot have increased the need for reoperation in adulthood, and it’s not rare that these reoperation candidates suffer from biventricular failure. However, there are no firm treatment guidelines, and each country, and even each facility, treats each case individually.Case presentationWe report the successful staged treatment of pulmonary regurgitation and pacemaker-induced cardiomyopathy with biventricular failure in adulthood in a case of complete atrioventricular block after tetralogy of Fallot repair in childhood. We planned a staged therapeutic strategy with preoperative left ventricular volume reduction with medication, following surgical pulmonary valve replacement concomitant epicardial lead implantation on the lateral basal wall, placed just beneath the generator pocket through 3rd intercostal space. in addition to postoperative intervention with a defibrillator to adjust cardiac resynchronization therapy, resulted in improvement of symptoms.ConclusionIn a patient with biventricular failure after TOF repair, a staged treatment strategy involving medication, PVR, and CRT with a combination of epicardial and intravenous leads could be a useful treatment worth trying before heart transplantation.

Technical performance scores associate with early prognosis of tetralogy of Fallot repair.

This study aimed to investigate the relationship between technical performance scores (TPS) and the early prognosis of tetralogy of Fallot repair (TOF). A retrospective study was conducted on TOF repair patients at our center from Oct 2017 to Oct 2022. Patients were classified into Class 1 (no residua), Class 2 (minor residua), or Class 3 (major residua) based on TPS derived from predischarge echocardiograms and need for reintervention. Statistical methods were used to assess the association between TPS and early prognosis. A total of 75 TOF repair patients (40% female, 60% male) were analyzed and categorized into TPS1 (24%), TPS2 (53.3%), and TPS3 (22.6%) based on pre-discharge echocardiographic findings. The median follow-up time was 7.0 months. The multivariable Cox regression analysis indicated that TPS3 scores are associated with a 12.68-fold increase in risk compared to TPS1 and TPS2 scores [95% CI = 12.68 (0.9∼179.28), P = 0.06]. The Spearman rank correlation analysis revealed a weak positive correlation between TPS classification and low cardiac output syndrome (r = 0.26, P = 0.03). However, there were no significant differences in ICU stay or duration of mechanical ventilation among the groups. TPS3 after intracardiac TOF repair is associated with higher risk of early re-intervention, highlighting the importance of close follow-up and monitoring in this patient population. Patients who develop low cardiac output syndrome in the early postoperative period may have residual defects that require prompt identification.

Predictive value of Cardiac Magnetic Resonance: new and old parameters in the natural history of repaired Tetralogy of Fallot

BackgroundPatients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients.Methods130 rTOF patients who underwent CMR (2006–2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome.ResultsUnivariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy.ConclusionsBesides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.

The Nakata index and McGoon ratio: correlation with the severity of pulmonary regurgitation after the repair of paediatric tetralogy of Fallot.

Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot.

Abstract 12267: Timing and Mode of Death Following Treatment of Neonatal Symptomatic Tetralogy of Fallot

Introduction: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, using a staged (SR) or primary repair (PR) strategy. Independent of management, the risk of post-procedural mortality is considerable. Although risk factors for late mortality after TOF repair have been established, the modes of death and associated factors have not been defined in early childhood following treatment of sTOF. Methods: Neonates with sTOF who had an initial intervention from 2005-2017 at 9 centers of the Congenital Cardiac Research Collaborative were reviewed. Among the patients who died, additional details related to timing and cause of death, and last clinical follow-up, were obtained and described. Comparisons of patient characteristics between survivors and non-survivors were performed. Results: Of 572 sTOF patients (230 PR, 342 SR), there were 52 (9%) deaths at a median age of 82 (25 th - 75 th %ile: 28, 246) days . Timing of mortality included: inpatient post-palliation (n=9), interstage in SR pathway (n=11), inpatient post complete repair (CR) (SR, n=5; PR, n=11) and post-CR following hospital discharge (n=16). Death occurred within 30 days of palliation or repair in 52% of cases. Most deaths were due to cardiac etiologies, with only 6 including ≥1 non-cardiac cause of death (NEC (n=2), sepsis (n=1), CNS bleed (n=2), trauma (n=1)). Risk factors for mortality included presence of genetic syndrome, non-cardiac anomaly, prematurity, low birth weight, and need for ventilation prior to initial intervention (p&lt;0.05 for all). Modes and timing of death in the 16 patients who died after CR discharge are shown (Figure). In these, RV dysfunction at last echo was present in 50%. Conclusions: When death occurs after interventions for sTOF, it frequently occurs early, and relates to traditional risk factors. Amongst patients with childhood mortality following discharge from CR, sudden death and reintervention-related events predominate known causes.

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

BackgroundNeonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. ObjectivesThe objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. MethodsNeonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. ResultsWe included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). ConclusionsDespite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

Aortic Root Dilatation Measured by Cardiac Magnetic Resonance in Patients with Repaired Tetralogy of Fallot

Background: Aortic root dilatation (AoD) frequently occurs following repaired tetralogy of Fallot (rTOF). The objective of this study was to assess aortic dimensions, investigate the prevalence of AoD, and identify predictors of AoD in rTOF patients. Methods: A cross-sectional retrospective study was conducted in repaired TOF patients from 2009 to 2020. Aortic root diameters were measured by cardiac magnetic resonance (CMR). Severe AoD of the aortic sinus (AoS) was defined as a Z-score (z) of &gt;4, reflecting a mean percentile ≥99.99%. Results: Two hundred forty-eight patients, with a median age of 28.2 years (10.2–65.3 years), were included in the study. The median age at the time of repair was 6.6 years (0.8–40.5 years) and the median interval between the repair and CMR study was 18.9 years (2.0–54.8 years). The prevalence of severe AoD was found to be 35.2% when defined by an AoS z greater than 4 and 27.6% when defined by a AoS diameter ≥40 mm, respectively. A total of 101 patients (40.7%) had aortic regurgitation (AR), with 7 patients (2.8%) having moderate AR. Multivariate analysis revealed that severe AoD was only associated with the left ventricular end diastolic volume index (LVEDVi) and a longer duration after repair. The age at the time of repair for TOF was found not to be correlated with the development of AoD. Conclusions: After repair of TOF, severe AoD was found to be prevalent, but no fatal complications were observed in our study. Mild AR was also commonly observed. Larger LVEDVi and a longer duration after repair were identified as factors associated with the development of severe AoD. Therefore, routine monitoring of AoD is recommended.

Right Ventricle Myocardial Work in ToF-repair survivors: no longer behind the scenes

Abstract Funding Acknowledgements Type of funding sources: None. Background Tetralogy of Fallot is the most common cyanotic CHD and survivors after ToF repair are growing rapidly. Children and, more frequently, young adults spToF develop late complications: life-threatening ventricular arrhythmias and SCD remain a great concern in these patients. This study has the aim to analyse advanced Right Ventricle echocardiographic and CMR features sp-ToF in the long-term follow-up, finding non-invasive predictors of adverse prognosis. Methods This is a retrospective cohort study. 240 patients sp-ToF were included (mean age 13 ± 11 years). The electrocardiographic, echocardiographic, CMR and CPET data were reviewed retrospectively. Non-invasive RV Global myocardial Work Index (RVGWI) was calculated as the area of the RV pressure-strain loops. RV Global Constructive Work (RVGCW), RV Global Wasted Work (RVGWW) and RV Global Work Efficiency (RVGWE) were estimated. Patients were screened according to age at surgery, time since the radical repair at the follow-up, increased estimated RV systolic pressure, differences in instrumental findings after PVrep. A composite end-point of MACEs (syncope, sustained and non-sustained ventricular tachycardia, sudden cardiac death) was used. Results Older patients at surgery (≥12 months) have significantly reduced VO2 max at CPET, larger RA and LA areas and larger RV basal diameter. CPET values are not significantly different in patients with more than 25 years since the corrective surgery but echo and CMR-LVEF measured are slightly but significantly reduced and RVGWE is significantly lower. Patients with RVSP ≥ 60 mmHg have significantly higher RVGWW, worst RV FWLS, lower RVGCW and RVGWE. Patients who have undergone PVrep show improvement in RV GLS, RV FWLS and RVGWE and a RVGWW reduction. As reguards the CMR RV volume evaluation sp-ToF, patients with RVESVi ≥80ml/m2 have lower RVGWE and show an inverse correlation between RVEDVi and RVGWE. Sp-ToF patients with borderline/impaired predicted VO2 max at CPET have a reduced RV FWLS, higher RVGWW and an inverse correlation between METs and RVGWW. Patients who have suffered MACEs (2 NS-TV, 3 S-TV, 7 syncopes, 8 sudden deaths) hadsignificant impairment of RV systolic function (FAC, RV FWLS, CMR-RVFE), significantly higher RVGWW, lower RVGWE and RVGWI. Conclusion Long-term survival and clinical condition sp-ToF in infancy are generally good and late functional status in survivors could be excellent up to 25 years after radical correction. However, older patients at surgery have relative reduced functional status compared with those undergoing early repair. The advanced echocardiographic and CMR evaluation of RV function sp-ToF are usually related to worse prognosis, VT and reduced functional status. RV myocardial work evaluation is feasible in patients with repaired ToF, it may be a more sensitive indicator of RV fuctional impairment compared with standard echocardiographic parameters and is able to predict exercise capacity.

Right-sided weakness in a Rwandan patient with untreated Tetralogy of Fallot

BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease encountered in pediatrics with surgical repair being the definitive treatment. Long-term survival after surgical repair has improved; however, reported mortality rates in untreated TOF are significant. Associated complications include neurological sequelae such as brain abscess and stroke. In countries without early intervention for congenital heart disease (including TOF), delayed presentations and complications require recognition by healthcare workers.Case presentationA 22-year-old male with a history of untreated TOF presented to Rwanda’s tertiary university hospital, University Teaching Hospital of Kigali, with acute right-sided hemiparesis. Diagnostic imaging identified a left-sided brain lesion consistent with brain abscess and cardiac mass, concerning endocardial vegetation. He was managed with intravenous antibiotics but subsequently died due to complications of septicemia.DiscussionIn countries where surgical repair of TOF is not available, early recognition and medical management are key in temporizing the development of devastating sequelae. Describing the prevalence of CHD in Rwanda is urgent, requiring further research by which effective prevention and treatment strategies can be developed.

Surgical Repair of TOF: Outcome of Right Ventricular Outflow Tract Reconstruction Using Modified Monocusp at Pulmonary Valve Level- A Single Cardiac Hospital Experience in Bangladesh.

Background and objectives: Basic components for Intra-cardiac repair of TOF are the same in every cardiac center except the timing which varies according to the capability of the team. Free PR is inevitable in case of a trans-annular patch which ultimately leads to RV dilatation, dysfunction, arrhythmia and failure with time. There is an ongoing endeavor to reconstruct the RVOT with the aim to avoid free PR in physiological repair. If RVOT reconstruction is done, RV dysfunction is absent or delayed which can avoid further re intervention. Monocuspid reconstruction of PV is commonly practiced in many centers but its long term outcome is poor though it helps to achieve aless stormy ICU course. Modified monocusp or bicuspid PV reconstruction is adopted in many centers using 0.1mm PTFE membrane. We represented the results of Modified Monocus PV reconstruction using a 0.1mm PTFE patch in repair of TOF with a trans-annular patch.&#x0D; Methodology: A total, 42 patients diagnosed as TOF were treated from January 2016 to October 2020. Age ranged from 18 months to 35 years, weighing 10 kg to 70 kg. 38 patients had TOF and 4 had DORV with PS. The trans-annular patch was followed by implantation of a 0.1-mm PTFE valve using posterior fixation.&#x0D; Results: Among the total42 patients,28 were male and 14 were females. Mean age was10.51±7.49 yrs and BSA was 0.90±0.34 kg/m2. Mean total bypass time was 187±31 min, cross-clamp time was 123.63±25.42 min. Out of 42 patients, 9(21.43%) had a PV gradient 0-10 mm/Hg, 24(57.14%) had 10-20 mm/Hg, and 9(21.43%) had &gt;20 mm/Hg in the post-operative echocardiogram. PR gradient was trivial in 7(16.67%), mild in 31(73.1%), moderate in 4(9.52%) patients. Follow-up echocardiogram revealed PR gradient remained trivial in 4(10%) patients, augmented from trivial to mild in 3(7.5%) and mild to moderate in 05(12.5%) patients. It remained moderate in 03(7.5%). There was no severe PR. ICU stay was 89±32.6 hours and mean hospital stay 11.48±2.1 days.&#x0D; Conclusions: Initial results using a transannular patch with a modified monocuspvalve to repair the RV outflow tract in cases of tetralogy of Fallot were promising. There were only a slight pressure gradient and mild regurgitation in most of the cases. A medium or long-term follow-up study is required to confirm these findings and compare them with results obtained using other techniques.&#x0D; AKMMC J 2021; 12(2): 82-90

Reintervention after repair of tetralogy of Fallot: a one-decade single-center experience

BackgroundReinterventions after tetralogy of Fallot repair (TOF) remains a common clinical problem. The objective of this study was to evaluate types of reintervention after TOF repair and identify the risk factors for reinterventions.MethodsThis retrospective study was conducted from 2010 to 2022 and included 171 patients with complete TOF repair. Patients were grouped according to the occurrence of reintervention into two groups: patients who did not have reintervention (n = 138) and those who required reintervention (n = 33).ResultsMedian follow-up was 36 (13–67) months. The first reintervention was required in 33 patients. Freedom from the first reintervention at 1, 3, 5, and 7 years was 91%, 85%, 81%, and 76%, respectively. Surgical reintervention was required in 12 patients and transcatheter intervention in 21 patients. Second reinterventions were required in 11 patients; 4 had surgery, and 7 had a transcatheter intervention. Third reinterventions were performed on two patients; one had surgery, and one had a transcatheter intervention. The most common interventions were performed at the level of pulmonary arteries (n = 17), followed by the pulmonary valve and the right ventricular outflow tract (n = 15). The risk of reintervention was associated with the low weight (HR: 0.65 (95% CI: 0.48–0.88); P = 0.005) and small LPA diameter (HR: 0.36 (95% CI: 0.21–0.60); P < 0.001) at the time of the primary intervention and the nonuse of the transannular patch (HR: 0.27 (95% CI: 0.08–0.85); P = 0.026).ConclusionsThe risk of reintervention is high after tetralogy of Fallot repair. In our experience, the smaller the left pulmonary artery and weight at the repair time increased the risk of reintervention. Using a transannular patch in our series was associated with a lower risk of reintervention.

Comparison of postoperative outcomes of milrinone versus dobutamine in tetralogy of Fallot with transannular patch

Background: Since the original description of TOF, its management modalities are continuously evolving. Present modality includes complete correction of the pathology by intra-cardiac repair with or without using transannular patch. Various finer aspects of intraoperative and postoperative management of the TOF repair are still evolving. Aims and Objectives: In this single centre study we are aiming to compare short term outcomes in patients of tof operated with transannular patch repair treated postoperatively with dobutamine vs milrinone. Materials and Methods: Total 100 patients undergoing TOF repair with transannular patch were grouped with respect to the inotropes used. One group who received milrinone and other who received dobutamine. Postoperative outcomes depending upon the need of other inotropic support, duration of ventilatory support, icu stay, inotropic support, hospital stay, morbidity and mortality are compared between the groups. Observations: No significant difference was seen in both groups for mean ventilation time, duration of icu and hospital stay. Only parameter which was significant was increase in usage of adjuvant inotropic support in milrinone group which were depicted in terms of increased VIS (Vasoactive inotrope score). Almost equal incidence of adverse events were noted in both the groups. Conclusion: Milrinone and dobutamine are fairly comparable to each other in cases of TOF repair with transannular patch. Dobutamine being a cheaper alternative have a better scope in developing countries like India.

966 ADVANCED ECHOCARDIOGRAPHIC AND CMR FEATURES OF PATIENTS WITH REPAIRED TETRALOGY OF FALLOT IN THE LONG-TERM FOLLOW-UP

Abstract Advanced echocardiographic and CMR features of patients with repaired Tetralogy of Fallot in the long-term follow-up. Anna Gozzi1, Jolanda Sabatino1, Elena Cuppini1, Irene Cattapan1, Alessia Basso1, Jennifer Fumanelli1, Angela Di Candia1, Martina Avesani1, Domenico Sirico1, Biagio Castaldi1, Elena Reffo1, Alessia Cerutti1, Roberta Biffanti1, Giovanni Di Salvo1 1Department for Women's and Children's Health, University Hospital of Padova, Pediatric and Congenital Cardiology Unit, Via Nicolo` Giustiniani, 2, 35128 Padova, Italy Background Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Children and, more frequently young adults, with repaired ToF develop late complications. Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in patients with repaired tetralogy of Fallot. The aim of this study was to describe and analyse advanced echocardiographic and CMR features of patients with repaired ToF in the long-term follow-up and to find non-invasive predictors of adverse prognosis. Methods This is a retrospective cohort study. Consecutive 200 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 19 ± 8. The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively. Right myocardial work index (RWI) was calculated as the area of the right ventricular pressure strain loops. From RWI, Right Constructive Work (RCW), Wasted Work (RWW) and Work Efficiency (RWE) were estimated. Patients were screened according the age at surgery (&amp;lt;12 months and &amp;gt;12 months) and the time since the repair (&amp;lt; 25 years and ≥ 25 years). A composite end-point of major adverse cardiovascular events (sudden cardiac death, sustained and non-sustained ventricular tachycardia, resuscitated sudden cardiac death, or syncope) was used. Results CPET values were not significantly different in patients with more than 25 years since the corrective surgery. Left ventricular ejection fraction measured either by CMR or echocardiography were slightly but significantly reduced in patients with more than 25 years since the corrective surgery. Patients with older age at surgery (&amp;gt;12 months) have significantly reduced V02max at CPET, larger RA and LA areas and larger RV basal diameter. During a median follow-up of 2.9 years, 1 patient died suddenly, 5 had documented ventricular tachycardia, and other 4 had syncope. On univariate analysis, echocardiographic left atrial area/BSA (P=0.044) and right ventricular longitudinal strain (P=0.010) were significantly related to the combined end point. Patients with reported VT at Holter ECG presented significantly reduced right ventricular function in terms of CMR-EF, echocardiographic FAC and RWI. Finally, TOF patients with borderline/impaired predicted VO2 max at CPET presented with significantly reduced RV freewall longitudinal strain and right ventricular myocardial work index. Conclusion Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF - operated patients could be excellent up to 25 years after the repair. However, patients with older age at surgery have relative reduced functional status compared with those undergoing early repair. The echocardiographic and CMR evaluation of RV function are usually related to worse prognosis, recurrency of VT and reduced functional status in patients with repaired TOF. Right ventricular myocardial work index is feasible in patients with repaired TOF; disadvantageous right ventricular work may be a more sensitive indicator of right ventricular impairment compared with standard echocardiographic parameters, and is able to predict exercise capacity.

Abstract 13633: Differences in Right and Left Ventricular Remodeling After Tetralogy of Fallot Repair in Patients With and Without Pulmonary Valve Replacement

Background: Pulmonary valve replacement (PVR) is required to address residual lesions after tetralogy of Fallot repair (rTOF). We wished to determine the progression of ventricular dilation after rTOF, using cardiac magnetic resonance (CMR) data in children, to determine age related RV and LV volume differences in patients who did or did not require PVR. Methods: Single center, retrospective longitudinal study, including 214 patients with rTOF who had primary repair before 24 months and at least one CMR performed from 2000-2020. During follow-up, 142 patients (66.3%) had PVR at median age 12 years (IQR 9-15.5). Linear mixed effect modeling was used to evaluate time-related changes in ventricular volumes by log-transformed age at CMR adjusted for sex and regurgitant lesion status. Patients were grouped as per need for PVR during follow-up. Results: A total of 323 CMR from 201 patients performed before PVR were analyzed. The median age at first CMR was 9.4 years (IQR 5.9-12.3): 126 (62.7%) patients had one CMR, 34 (16.9%) had 2 and 41 (20.4%) had 3 or more. Prior to PVR there was steeper time-related RV dilation compared to non-PVR requiring patients: RVEDVi = 19.4 (CI 15.1 - 23.7) vs 2.8 (CI -2.5 - 8.2) ml/m 2 /log2year, p&amp;lt0.001; RVESVi = 11.9 (8.9 - 14.8) vs 0.8 (CI -2.9 - 4.4) ml/m 2 /log2year, p&amp;lt0.001. A time-related increase in LV volume also occurred in the PVR group compared to non-PVR: LVEDVi = 7.2 (CI 4.7 - 9.7) vs 1.5 (CI -1.6 - 4.6) ml/m 2 /log2year, p=0.005; LVESVi = 3.2 (CI 1.9 - 4.6) vs -0.4 (CI -2.1 - 1.3) ml/m 2 /log2year, p=0.001) (Figure 1). The factors associated with PVR were presence of regurgitant residual lesion, more prolonged QRS duration and transannular patch as surgical technique for TOF repair. Conclusion: Early and significantly increased progression of RV dilation and LV dilation are identifiable by CMR in patients destined to require PVR following rTOF. Early CMR surveillance may be indicated to permit early identification of progressive remodeling.

Del Nido Cardioplegia for patients of adult congenital heart disease.

Objective: To assess the efficacy of del Nido cardioplegia for patients of adult congenital heart diseases undergoing cardiac surgery. Study Design: Descriptive Observational Case Series. Setting: Department of Paediatric Cardiac Surgery, Faisalabad Institute of Cardiology, Faisalabad. Period: October 2019 to September 2021. Material &amp; Methods: All consecutive patients of age fifteen years or above who underwent open-heart surgery for congenital heart disease by using ante grade del Nido cardioplegia during the study period were included. Dedicated hospital database used to retrieve the data of patients regarding type of surgery for CHD and operative or postoperative outcome parameters. The data was entered in excel sheet and descriptive analysis done. Results: One hundred and twenty five patients underwent open heart bypass surgery for congenital heart disease by using del Nido cardioplegia during the study period. Majority of patients were male (n=83, 66.4%) with male to female ratio 1.97:1. Sixty eight percent were less than thirty years of age. Primary repair of TOF was the most frequently performed surgery (49.6%, n=62) while 28.8% had repair of VSD. Average bypass time was 136 ±57 minutes and average cross clamp time of 103 ± 44 minutes. Total duration of mechanical ventilation was 6.6 ±11.9 hours, ICU stay of 38 ±34 hours and stay in the hospital of 5.2 ± 2.6 days. Cardiac tamponade was seen in 0.8% of patients (n=1) while moderate LV systolic dysfunction in 1.6 % of patients. Conclusion: Del Nido cardioplegia solution is as safe as conventional blood cardioplegia with short ventilation time, ICU stay and lowest postoperative complications.