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A 14-year-old child with recurrent cyanotic spells at the age of 9 months underwent echocardiography at that time, which revealed tetralogy of Fallot (TOF). Follow-up computed tomography angiography revealed TOF with valvular pulmonary stenosis and a right-sided aortic arch with mirror image branching. Interestingly, an unusual anomalous course of the left brachiocephalic vein was seen. Instead of coursing anterior to the aortic arch, the left brachiocephalic vein had a U-shaped course like a garland, where it descended vertically down behind the esophagus and the descending aorta, crossed the midline at T4 level of the vertebral body, and then ascended upwards to join the superior vena cava. This case highlights the role of computed angiography in depicting anomalous venous anatomy in TOF, which can have potential clinical implications., Competing Interests: The authors have no competing interests to declare. A written informed consent was obtained from the parent of the patient., (Copyright: © 2024 The Author(s).)

Purpose: We demonstrate the complexities of managing pediatric patients on extracorporeal membrane oxygenation (ECMO) therapy requiring neurosurgery, focusing on systemic anticoagulation, cardiac function, and medically refractory intracranial pressure (ICP)., Methods: A 3.5-year-old female with Tetralogy of Fallot developed severe ischemic cerebral edema following post-operative cardiac arrest and required ECMO. This case, along with four additional cases of children requiring neurosurgery while on ECMO, was examined., Results: Emergency neurosurgical intervention in the primary case led to significant improvement, highlighting the delicate balance between managing ECMO-induced anticoagulation and urgent neurosurgical needs. The additional cases had variable outcomes, emphasizing the challenges of caring for these critically ill patients., Conclusion: Successful management of children requiring ECMO support and neurosurgical intervention requires thoughtful multidisciplinary care. This report illustrates some of the nuances in such decision-making, and demonstrates one potential path to a good outcome., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Children with repaired tetralogy of Fallot (rToF) often have pulmonary regurgitation with right ventricular (RV) dilatation and dysfunction, whereas less is known about the effect on the left ventricle (LV). The aim was to investigate LV haemodynamic variables derived from non-invasive pressure-volume loops in children with rToF and how they compare to controls and previous research on adults., Materials and Methods: Ten children with rToF and pulmonary regurgitation (12 years [10-13], 6 males) and 10 age- and sex-matched healthy controls (12 years [10-14], 6 males) underwent brachial blood pressure in conjunction with cardiac magnetic resonance imaging. Pressure-volume loops were derived by brachial blood pressure together with LV volumes throughout the cardiac cycle in short-axis cine images yielding several haemodynamic variables, including arterial elastance. The RV endocardial border was delineated in end-diastole and end-systole., Results: Children with rToF and pulmonary regurgitation had larger RV end-diastolic volume (136 [114-156]) than controls (100 [94-112] ml/m 2 ; p  = 0.0015) and smaller LV end-diastolic volume (83 [58-91] ml/m 2 ) than controls (101 [92-110] ml/m 2 ; p  = 0.002). Arterial elastance was higher in children with rToF (1.5 [1.3-2.7] mmHg/ml) than in controls (1.1 [1.0-1.5] mmHg/ml; p  = 0.02). Heart rate was higher in children with rToF (77 [74-81] bpm) than in controls (69 [65-75] bpm; p  = 0.027)., Conclusion: Children with rToF had higher arterial elastance and heart rate than controls, likely due to increased sympathetic tone to compensate for impaired LV filling following pulmonary regurgitation. If this contributes to increased risk of adverse cardiovascular and cerebrovascular events remains to be studied.

Background: Patients with repaired tetralogy of Fallot (rTOF) continue to face a heightened risk of deteriorating cardiac function, and quantitative techniques of cardiac MRI-derived cardiac structure and function provide an opportunity to explore the causes and mechanisms of cardiac deterioration., Purpose: To explore right-left ventricular interdependence in rTOF patients before and after the onset of right ventricular (RV) heart failure., Study Type: Retrospective., Population: One hundred eighteen rTOF patients (21.85 [16.74, 29.20] years, 58 females) and 34 controls (23.5 [21, 26.5] years, 17 females) that underwent cardiac MRI were analyzed, with rTOF patients being further subdivided into those with preserved RV function (N = 54) and those that experienced RV heart failure (N = 64)., Field Strength/sequence: 3.0 T/balanced steady-state free precession sequence., Assessment: RV, left ventricular (LV), and septal strain; RV and LV volume., Statistical Tests: Chi-squared tests or Fisher's exact test, One-way ANOVAs with Bonferroni's post hoc test, Pearson/Spearman correlation, and multivariate backward linear regression analysis. A two-tailed P < 0.05 was deemed as the significance threshold., Results: The MRI-derived RV, LV, and septal strain decreased sequentially in controls, patients with preserved RV function, and patients with RV heart failure, with a good intra-observer (0.909-0.964) and inter-observer (0.879-0.937) agreement. Correlations between LV and RV strain were found to change sequentially with RV function and were the closest in rTOF patients with RV heart failure (r = -0.270 to 0.506). Correlations between RV volume and septal strain was variable in controls (r = 0.483 to -0.604), patients with preserved RV function (r = -0.034 to -0.295), and patients with RV heart failure (r = -0.026 to 0.500). Multivariate analyses revealed that the RV longitudinal strain was independently correlated with LV strain in three directions in rTOF patients with RV heart failure (Radial -0.70 [-1.33, -0.06]; Circumferential 0.44 [0.17, 0.72]; Longitudinal 0.54 [0.26, 0.81])., Data Conclusion: In rTOF patients, the coupling between RV volume and septal strain was broken during RV function compensation, and the adverse effect of RV on LV deformation was highest in patients with RV heart failure., Evidence Level: 4 TECHNICAL EFFICACY: Stage 5., (© 2023 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Abstract: Pentalogy of Fallot is a rare form of congenital cyanotic heart disease with a prevalence of 3/10,000 live births characterized by an association of Tetralogy of Fallot with Atrial Septal Defect. Pentalogy of Fallot with anorectal malformation is also a rare combination. Here we describe one of the rare case reports of a full-term, 38 weeks, female baby diagnosed with pentalogy of Fallot with imperforate anus and rectovaginal fistula at a tertiary care hospital. Pentalogy of Fallot combined with an imperforate anus and rectovaginal fistula is an exceptionally rare and complex congenital condition. The co- existence of these anomalies emphasizes the need for thorough prenatal and postnatal evaluation for early detection and management.

Background: There are limited data about the impact of timing of pulmonary valve replacement (PVR) on right heart reverse remodeling in patients with pulmonary regurgitation following intervention for isolated pulmonary valve stenosis (PS). This study compared differences in postprocedural right heart reverse remodeling after early versus late PVR (defined as PVR before versus after attainment of the conservative consensus criteria proposed by Bokma et al, 2018) in patients with prior intervention for PS, using patients with tetralogy of Fallot as the reference group., Method and Results: Right atrial reservoir strain and right ventricular free wall strain was measured at baseline, 1 and 3 years after PVR. There were 114 patients with PS (early PVR, 87 [76%]; late PVR, 27 [24%]) and 291 patients with tetralogy of Fallot (early PVR, 197 [67%]; late PVR, 96 [33%]). The PS group had greater improvement in right atrial reservoir strain at 1 year (12%±4% versus 8%±4%; P <0.001) and 3 years (15%±6% versus 9%±6%; P <0.001), and a greater improvement in right ventricular free wall strain at 1 year (12%±4% versus 7%±3%, P =0.008) and 3-years (16%±6% versus 12%±5%; P =0.01) after PVR compared with the tetralogy of Fallot group. There was no difference in right heart reverse remodeling between patients who underwent early versus later PVR within the PS group. In contrast, late PVR was associated with less right heart reverse remodeling within the tetralogy of Fallot group., Conclusions: These data suggest that patients with palliated PS presenting pulmonary regurgitation have a more benign clinical course, and hence delaying PVR in this population may be appropriate.

Background: Following surgical repair in infancy, a high proportion of children born with tetralogy of Fallot are surviving into adulthood, and it is important that these patients are monitored by an adult congenital heart disease specialist for problems and complications that can occur in the adult patient., Objective: To provide an overview on the management of adult patients with repaired tetralogy of Fallot and emphasise the need for specialist follow-up., Discussion: Following surgical repair of tetralogy of Fallot, it is important to have lifelong surveillance in adulthood for complications such as pulmonary valve dysfunction. Modern imaging modalities have helped with monitoring and the early detection of problems. Cardiac surgery has been refined over the years, leading to improved longevity. Over the past 23 years, percutaneous interventions have been developed to deal with valve complications and the results have been promising. Healthy behaviour, such as regular exercise, and behaviours to minimise the risk of endocarditis should be encouraged. Regular follow-up with an adult congenital heart disease specialist is important. Patients wishing to undertake a pregnancy should be supervised through a high-risk pregnancy clinic.

Abstract: Peri-operative management of cyanotic congenital heart disease in a patient of sickle cell disease (SCD) can be challenging. We report a case of Tetralogy of Fallot and homozygous SCD with history of multiple blood transfusions and sickle cell crises who underwent intracardiac repair. Hemoglobin S level was reduced from 75% pre-operative to 21.8% postoperative with a combination of pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB). Pre-operative optimization and safe intraoperative conduct were essential to avoid sickling crises., (Copyright © 2024 Copyright: © 2024 Annals of Cardiac Anaesthesia.)

Congenital eyelid imbrication syndrome is a rare eyelid finding where a long upper lid overlaps the lower lid when the eyes are closed. To date, congenital eyelid imbrication syndrome has been described in the literature less than 10 times. We present a case of congenital eyelid imbrication syndrome in a patient with trisomy 21 and tetralogy of Fallot on a prostaglandin E infusion to maintain a patent ductus arteriosus prior to definitive heart surgery. While on the infusion, the patient developed peripheral edema and flushing due to vasodilation. This coincided with eyelid swelling, conjunctival chemosis, and eversion of the eyelids. Upon cessation of the prostaglandin E1 infusion, his eyelid eversion resolved., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Introduction and Objectives: Our aim was to assess the impact of prosthetic pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) on changes in biventricular volumes and function and on adverse cardiac events., Methods: Adults with rTOF were identified from the SACHER-registry. Data from serial cardiac magnetic resonance imaging, echocardiography, exercise capacity and n-terminal pro b-type natriuretic peptide (NT-proBNP) were collected. The primary endpoint was right ventricular ejection fraction (RVEF) as measured by cardiac magnetic resonance. Secondary endpoints were biventricular volumes, left ventricular ejection fraction, exercise capacity and NT-proBNP levels, and time to adverse cardiac outcomes (atrial and ventricular arrhythmia, endocarditis). Associations between previous PVR and longitudinal changes in functional outcomes and time to adverse cardiac outcomes were analyzed using linear mixed-effects models and Cox proportional hazards models, respectively., Results: A total of 308 patients (153 with and 155 without PVR) with 887 study visits were analyzed. Previous PVR was not significantly associated with changes in RVEF (CE, -1.33; 95%CI, -5.87 to 3.21; P=.566). Previous PVR was associated with lower right ventricular end-diastolic volume but had no significant effect on left ventricular ejection fraction, exercise capacity, or NT-proBNP-levels. Previous PVR was associated with an increased hazard of atrial arrhythmias (HR, 2.09; 95%CI, 1.17-3.72; P=.012) and infective endocarditis (HR, 12.72; 95%CI, 4.69-34.49; P<.0001) but not with an increased hazard of sustained ventricular arrhythmias (HR, 0.64; 95%CI, 0.18-2.27; P=.490)., Conclusions: Previous PVR was not significantly associated with changes in RVEF but was associated with an increased risk of atrial arrhythmias and infective endocarditis., (Copyright © 2023 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

We present a rare case of tetralogy of Fallot accompanied with dextrocardia, situs inversus and anomalous origin of the right coronary artery. Total repair was accomplished successfully using a minimally invasive left axillary thoracotomy., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Arrhythmias may originate from surgically unaffected right ventricular (RV) regions in patients with tetralogy of Fallot (TOF). We aimed to investigate action potential (AP) remodelling and arrhythmia susceptibility in RV myocardium of patients with repaired and with unrepaired TOF, identify possible correlations with clinical phenotype and myocardial fibrosis, and compare findings with data from patients with atrial septal defect (ASD), a less severe congenital heart disease., Methods: Intracellular AP were recorded ex vivo in RV outflow tract samples from 22 TOF and three ASD patients. Arrhythmias were provoked by superfusion with solutions containing reduced potassium and barium chloride, or isoprenaline. Myocardial fibrosis was quantified histologically and associations between clinical phenotype, AP shape, tissue arrhythmia propensity, and fibrosis were examined., Results: Electrophysiological abnormalities (arrhythmias, AP duration [APD] alternans, impaired APD shortening at increased stimulation frequencies) were generally present in TOF tissue, even from infants, but rare or absent in ASD samples. More severely diseased and acyanotic patients, pronounced tissue susceptibility to arrhythmogenesis, and greater fibrosis extent were associated with longer APD. In contrast, APD was shorter in tissue from patients with pre-operative cyanosis. Increased fibrosis and repaired-TOF status were linked to tissue arrhythmia inducibility., Conclusions: Functional and structural tissue remodelling may explain arrhythmic activity in TOF patients, even at a very young age. Surprisingly, clinical acyanosis appears to be associated with more severe arrhythmogenic remodelling. Further research into the clinical drivers of structural and electrical myocardial alterations, and the relation between them, is needed to identify predictive factors for patients at risk., (© 2023. The Author(s).)

Congenital diverticulum is an uncommonly detected cardiac lesion, especially in infancy. However, its association with cyanotic congenital heart disease is extremely rare. In the current work, we report a case diagnosed in the neonatal period with tetralogy of Fallot and pulmonary valve atresia associated with a large congenital diverticulum originating from the right ventricle., (© 2024 Wiley Periodicals LLC.)

A 3-day-old male neonate was referred to our hospital for a cardiac echocardiogram.

A 12-year-old female with pulmonary artery stenosis developed pseudoaneurysms due to septic embolism, requiring close follow-up.

Background and Aim: Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management., Methods: All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study., Results: We included 221 patients (116 males) with a median age of 19 (18-25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years., Conclusion: Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.

Introduction: Brain abscesses are rare but potentially fatal condition and can be associated with cyanotic congenital heart disease of which 5-18.7% of these patients that develop cerebral abscess commonly have tetralogy of Fallot (TOF)., Case Presentation: We report a case of 3-year-old Muganda male that presented with convulsions, cyanosis and difficulty in breathing. The patient had a combination intervention of medical treatment and surgical drainage of the abscess. Post-operative Computerized tomography scan images and pre-operative brain Computerized tomography scans were compared. The multiple rings enhancing lesions were reduced in number and sizes. The largest measured ring was 44 × 22.5×16mm compared to the previous; 42 × 41×36mm. The mass effect had reduced from 16 mm to 7.5 mm. The periventricular hypodensities persisted. Findings showed radiological improvement with residual abscesses, subacute subdural hematoma and pneumocranium. The patient was treated with intravenous ceftriaxone 1 g OD for six weeks and he showed marked improvement and was discharged home after 3 months., Conclusion: A comprehensive strategy involving medications, surgical drainage, and early neurosurgical consultation is vital in treating brain abscesses in uncorrected TOF. Early identification of the pathogen, appropriate antibiotic therapy, and vigilant follow-up through clinical assessments and imaging are crucial, potentially spanning a 4-8-week treatment., (© 2024. The Author(s).)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR)., Methods: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany)., Results: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude., Conclusion: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed., (© 2024 Wiley Periodicals LLC.)

Objective: Residual sequelae after surgical repair of tetralogy of Fallot (rTOF) affect clinical outcome. We investigated the prognostic impact of right ventricular (RV) dyssynchrony in adults with rTOF years after the surgical repair., Methods: Patients from the Swiss Adult Congenital HEart disease Registry were included. NT-proBNP levels, echocardiography, exercise testing and MRI data were collected. An offline strain analysis to quantify RV-ventricular and interventricular dyssynchrony was performed. The standard deviation of the time-to-peak shortening (TTP) of six RV segments defined the RV Dyssynchrony Index (RVDI). Maximal difference of TTP between RV and left ventricular segments defined the interventricular shortening delay (IVSD). Predictors of a composite adverse event (arrhythmias, hospitalisation for heart failure and death) were identified by multivariate Cox regression analysis. Their median values were used to create a risk score., Results: Out of 285 included patients (mean age 34±14 years), 33 patients (12%) experienced an adverse event during a mean follow-up of 48±21 months. No correlation was found between RVDI, IVSD and clinical events. NT-proBNP, right atrial area and peak heart rate were independent predictors of outcomes. After 4 years-follow-up, no adverse events occurred in patients at low risk (score=0 points), while an adverse event occurred in 62% of patients at high risk (score=3 points, p<0.001)., Conclusion: In our cohort of adults with rTOF, surrogates of RV dyssynchrony did not correlate with outcomes. A multimodality approach was effective in predicting the risk for adverse events., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ.)

We report a case of a 44-year-old woman surgically treated for tetralogy of Fallot who experienced an acute and extensive Stanford A type aortic dissection despite the meticulous follow-up. While aortic dilatation is prevalent in individuals with repaired tetralogy of Fallot, aortic dissection represents a rare consequence, that when it appears, is progressive and usually detected during the check-up visits. In the case reported, the dissection was unexpected and severe, and the patient's clinical state worsened suddenly, leading to death after a few days. Constant awareness for aortic aneurysms is essential in the Fallot tetralogy population, nevertheless, several causes may contribute to the acute worsening of the clinical condition until the patient's death.

Although contemporary outcomes of initial surgical repair of tetralogy of Fallot (TOF) are excellent, the survival of adult patients remains significantly lower than that of the normal population due to the high incidence of heart failure, ventricular arrhythmias, and sudden cardiac death. The underlying mechanisms are only partially understood but involve an adverse biventricular response, so-called remodelling, to key stressors such as right ventricular (RV) pressure-and/or volume-overload, myocardial fibrosis, and electro-mechanical dyssynchrony. In this review, we explore risk factors and mechanisms of biventricular remodelling, from histological to electro-mechanical aspects, and the role of imaging in their assessment. We discuss unsolved challenges and future directions to better understand and treat the long-term sequelae of this complex congenital heart disease., (Copyright © 2024. Published by Elsevier Inc.)

Background: The aim of this study was to investigate the relationship among right ventricular (RV) dilatation, dysfunction, and electromechanical dyssynchrony (EMD) in patients with repaired tetralogy of Fallot (rTOF)., Methods: Data from a prospective rTOF registry of subjects with moderate or greater pulmonary regurgitation (PR) and contemporary imaging were analyzed. Electrocardiograms and echocardiograms were analyzed for EMD (prolonged QRS duration [QRSd], echocardiographic septal flash, and mechanical delay) and mechanical dispersion. The relationship among these, RV measurements on cardiac magnetic resonance, exercise capacity, and incident arrhythmia or death was analyzed with adjustment for PR., Results: In total, 271 patients with rTOF (42% women; median age, 32 years; interquartile range [IQR], 23-34 years) were included. Patients had moderate to severe PR (median PR fraction, 38%; IQR, 30%-47%), moderate to severe RV enlargement (median RV end-diastolic volume index, 161 mL/m 2 ; IQR, 138-186 mL/m 2 ) and mild RV systolic dysfunction (median RV ejection fraction [RVEF], 44%; IQR, 38%-48%). Eleven patients (4%) experienced ventricular arrhythmia or death. Presence of EMD was associated with larger RV size (RV end-diastolic volume index and RV end-systolic volume index, P = .006 and P < .001, respectively) and lower RVEF (P < .001). A sharp inflection in the relation among QRSd, RV size, and RVEF was observed when QRSd exceeded 150 msec (3.1% decrease in RVEF for every 20-msec increase in QRSd between 160 and 200 msec). Similar inflection points were observed for the mechanical delay between the RV basal-lateral and midseptal segments. The mechanical delay was higher in patients with vs without incident atrial arrhythmia (371 vs 276 msec, P = .014)., Conclusions: In adults with rTOF, EMD is independently associated with larger RV size, lower RVEF, and incident atrial arrhythmias., (Copyright © 2023 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Objective: We sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF)., Methods: A single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time., Results: Of 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045)., Conclusions: Patients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Patients with repaired tetralogy of Fallot (TOF) are at risk for ventricular tachycardia (VT) related to well-described anatomical isthmuses., Objective: The purpose of this study was to explore QRS morphology as an indicator of anatomical isthmus conduction., Methods: Patients with repaired TOF and complete right bundle branch block referred for transcatheter pulmonary valve replacement (PVR) or presenting with sustained VT underwent comprehensive 3-dimensional mapping in sinus rhythm. Electrocardiographic characteristics were compared to right ventricular (RV) activation and anatomical isthmus conduction properties., Results: Twenty-two patients (19 pre-pulmonary valve replacement and 3 clinical VT) underwent comprehensive 3-dimensional mapping (median 39 years; interquartile range [IQR] 27-48 years; 12 [55%] male). Septal RV activation (median 40 ms; IQR 34-46 ms) corresponded to the nadir in lead V 1 and free wall activation (median 71 ms; IQR 64-81 ms) to the transition point in the upstroke of the R' wave. Patients with isthmus block between the pulmonary annulus and the ventricular septal defect patch and between the ventricular septal defect patch and the tricuspid annulus (when present), were more likely to demonstrate lower amplitude R' waves in lead V 1 (5.8 mV vs 9.4 mV; P = .005), QRS fragmentation in lead V 1 (15 [94%] vs 2 [13%]; P < .001), and terminal S waves in lead aVF (15 [94%] vs 6 [40%]; P < .001) than those with intact conduction. During catheter ablation, these QRS changes developed during isthmus block., Conclusion: For patients with repaired TOF, the status of septal isthmus conduction was evident from sinus rhythm QRS morphology. Low-amplitude, fragmented R' waves in lead V 1 and terminal S waves in the inferior leads were related to septal isthmus conduction abnormalities, providing a mechanistic link between RV activation and common electrocardiographic findings., (Published by Elsevier Inc.)

Objectives: To review outcomes after a uniform strategy of transventricular repair of tetralogy of Fallot., Methods: A total of 244 consecutive patients underwent transventricular primary repair of tetralogy of Fallot from 2004 to 2019. Median age at operation was 71 days; 57 (23%) patients were premature; 57 (23%) patients had low birth weight (<2.5 kg), and 40 (16%) had genetic syndromes. The diameter of pulmonary valve annulus, right pulmonary artery (PA), and left PA were 6.0 ± 1.8 mm (z score, -1.7 ± 1.3), 4.3 ± 1.4 mm (z score, -0.9 ± 1.2) and 4.1 ± 1.5 mm (z score, -0.5 ± 1.3)., Results: Three (1.2%) operative deaths were recorded. Ninety patients (37%) underwent transannular patching. Postoperative echocardiographic peak right ventricular outflow tract gradient decreased from 72 ± 27 mm Hg to 21 ± 16 mm Hg. Median intensive care unit and hospital stay were 3 and 7 days. The survival rate at 10 years was 94.6% ± 1.8%. Reintervention was required 86 times (55 catheter interventions) in 56 patients following tetralogy of Fallot repair. The freedom from all-cause reintervention rate at 10 years was 70.5% ± 3.6%. Cyanotic spells (hazard ratio, 2.14; 95% CI, 1.22-3.90; P < .01) and smaller pulmonary valve annulus z score (hazard ratio, 1.26; 95% CI, 1.01-1.59; P = .04) were associated with increasing risk of all reinterventions. Freedom from redo surgery for right ventricular outflow tract obstruction and right ventricular dilatation at 10 years were, respectively, 85.0% ± 3.1% and 98.7% ± 0.9%. Freedom from valve implantation was 96.7% ± 1.5% at 10 years., Conclusions: A uniform strategy of primary repair of tetralogy of Fallot through a transventricular approach resulted in low reoperation rate in the first decade. The need of pulmonary valve implantation was limited to <4% at 10 years., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Objectives: Tetralogy of Fallot (TOF) is a common congenital heart disease which should be corrected. The recommended time for the Tetralogy of Fallot Total Correction (TFTC) surgery is during the infancy for the possible difficulties during the surgery and the related issues. However, sometimes TOF is diagnosed and managed during the adulthood., Methods: This study is a descriptive and retrospective one which included all patients who underwent TFTC at the age of 15-year and older in 10 years (between the years 2010 and 2020) to identify short-term (in-hospital mortality, ICU stay, postoperative bleeding, respiratory complications after the surgery such as pulmonary edema, pneumonia, etc.) and one-year (left ventricle ejection fraction (LVEF), right ventricle (RV) ejection fraction, the severity of tricuspid and aortic regurgitation after surgery) outcomes. All data were taken from medical records at Rajaie Cardiovascular Medical and Research Center. Data were analyzed using SPSS 22., Results: 94 patients with the mean ± SD age of 26.7 ± 9.6 years were enrolled. Most of them were male (59.6%) (P-value: 0.009). In-hospital mortality in our study were 5.3%. Tricuspid regurgitation (TR) was significantly resolved after the surgery (P-value: 0.006). Of 17 (18.1%) patients with small or hypoplastic pulmonary artery (PA) branches, 14 patients had acceptable PA branch size after surgery., Conclusion: TFTC at an older age is safe with acceptable results. Age is not a contraindication for TFTC and surgery should be recommended if the patients are diagnosed with TOF in adulthood. Also, the TOF diagnosis should be considered in adult patients with suspicious signs and symptoms., (© 2023. The Author(s).)

Objective: To determine the 2-year neurodevelopmental outcomes for survivors of neonatal cardiac surgery for the most common right ventricular outflow tract obstructive lesions: tetralogy of Fallot and pulmonary atresia with a ventricular septal defect., Study Design: A single-center consecutive cohort of 77 children underwent neonatal surgery for tetralogy of Fallot or pulmonary atresia with a ventricular septal defect at ≤6 weeks of age between 2006 and 2017. The patients underwent a multidisciplinary neurodevelopmental assessment at 18-24 months of age. Survivor outcomes were compared by univariable and multivariable analyses., Results: The 2-year mortality was 7.8% (6/77) with a postoperative in-hospital mortality of 3.9% (3/77). Freedom from reintervention by cardiac catheterization or surgical intervention at 2 years was 36%. Functional and neurodevelopmental assessment for 69 of 71 survivors was completed at a mean age of 22.6 ± 4.0 months using the Bayley Scales of Infant and Toddler Development III. The mean neurodevelopmental outcome scores were 83.4 ± 16.5 for cognitive skills, 82.2 ± 18.7 for language skills, and 81.4 ± 18.1 for motor skills. Cognitive, language, and motor delay, defined as a score of <70, was identified in 25%, 25%, and 23% of patients, respectively. Multivariable analyses for factors associated with worse neurodevelopmental outcomes identified chromosomal anomalies (P < .001) and postoperative complications (P < .03)., Conclusions: Cyanotic tetralogy of Fallot and pulmonary atresia with ventricular septal defect requiring neonatal repair showed similar 2-year neurodevelopmental outcomes below normative values and a high prevalence of cognitive, language and motor delays., Competing Interests: Declaration of Competing Interest Financial support was initially provided by the Glenrose Rehabilitation Hospital Research Trust Fund with ongoing funding from the Registry and Follow-up of Complex Pediatric Therapies Project, Alberta Health and Wellness. The authors declare no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease (CHD) in infants. The four components that constitute ToF are an overriding aorta over the crest of the interventricular septum, obstruction in the right ventricular outflow tract, right ventricular hypertrophy, and a typically large non-restricted ventricular septal defect. ToF may also be associated with other extracardiac abnormalities, including patent ductus arteriosus or multiple aortopulmonary collateral arteries, which can impact the patient's survival. Patients with unoperated ToF rarely reach adulthood, and it is extremely rare to discover undiagnosed ToF in individuals over 60 years old. In this report, we describe an unusual case of a woman with unrepaired ToF who survived until the age of 71. She was fairly asymptomatic until the 7th decade of her life and complained only of dyspnea on exertion. The patient declined corrective cardiac surgery and preferred conservative management., (© 2023 Wiley Periodicals LLC.)

Background: Tetralogy of Fallot (TOF) is a common cyanotic congenital heart malformation that carries a high risk of right-to-left shunting. Anemia is characterized by decreased hemoglobin (Hb) levels that can affect tissue oxygen delivery and impact postoperative recovery in patients. Chronic hypoxia caused by right-to-left shunting of TOF could lead to compensatory increases in Hb to maintain systemic oxygen balance. This study aims to investigate whether preoperative Hb and blood oxygen saturation (SpO2) can predict adverse outcomes in children undergoing corrective surgery for TOF., Methods: This retrospective study included patients under 18 years of age who underwent corrective surgery for TOF at Fuwai Hospital between January 2016 and December 2018. Adverse outcomes, including in-hospital death, extracorporeal membrane oxygenation implantation, ICU stay > 30 days, and severe complications, were considered as the primary outcome. Univariable and multivariable logistic analyses were performed to identify independent risk factors for adverse outcomes. Propensity score-matched (PSM) analysis was also conducted to minimize the confounding factors., Results: A total of 596 children were included in the study, of which 64 (10.7%) experienced adverse outcomes. Hb*SpO2 < aaHb was identified as an independent risk factor for adverse outcomes (OR = 2.241, 95% CI = 1.276-3.934, P = 0.005) after univariable and multivariable logistic analyses. PSM analysis further confirmed the association between Hb*SpO2 < aaHb and adverse outcomes. Patients with Hb*SpO2 < aaHb had a significantly higher incidence of postoperative adverse outcomes, longer time of mechanical ventilation, and hospital stay, as well as higher in-hospital costs., Conclusions: Hb*SpO2 < aaHb is significantly associated with adverse outcomes in children undergoing corrective surgery for TOF. Clinicians can use this parameter to early identify high-risk children and optimize their postoperative management., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Objective: The purpose of this study was to compare pulmonary arterial (PA) growth and morbidity, mortality, reintervention and complete repair rates after modified transannular patching palliation (mTAP) versus modified Blalock-Taussig-Thomas shunt (mBTS) for palliation in infants with severe tetralogy of Fallot (TOF) with diminutive pulmonary arteries., Methods: This was a retrospective case review study of 107 patients (64 males) with severe TOF who underwent staged repair with either mTAP (n = 55) or mBTS (n = 52) over an 8-year period. Procedure-related PA growth and morbidity, mortality, reintervention and complete repair rates were compared., Results: Two deaths occurred in the mBTS group due to sudden cardiac arrest, and five patients needed reintervention after the mBTS procedure because of shunt thrombosis or stenosis. Postoperative complications of mBTS included sudden cardiac arrest, shunt thrombosis/stenosis, vocal cord palsy and diaphragmatic palsy. Unlike in the mBTS group, no death, severe complications or reintervention occurred in the mTAP group. Oxygen saturations post mTAP and mBTS were significantly higher, which improved from 67.73 ± 4.36% to 94.33 ± 2.19% in the mTAP group and from 68.24 ± 3.87% to 86.87 ± 3.38% in the mBTS group. The increase in oxygen saturation and pulmonary artery growth (from pre- to post palliation) was significantly better with mTAP than with mBTS palliation (p < 0.01). All 55 patients showed complete repair after mTAP, and the time from palliation to complete repair was significantly shorter in the mTAP group., Conclusions: In a severe form of TOF with the hypoplastic PA tree, mTAP seems to be a better strategy that is safe and better facilitates satisfactory pulmonary arterial growth until complete repair than the mBTS procedure.

Pentalogy of Fallot is a rare congenital cyanotic heart disease; few patients with uncorrected disease survive to childbearing age. Cardiovascular changes during pregnancy and delivery can lead to haemodynamic instability, while anaesthesia can cause right-to-left shunting and worsen hypoxaemia.We present the learning points from the anaesthetic management of an obstetric patient with uncorrected pentalogy of Fallot. We describe the successful application of general anaesthesia, choice of transoesophageal echocardiography for real-time haemodynamic monitoring and management, and the comprehensive multidisciplinary care of this high cardiovascular risk obstetric patient perioperatively. We also review the literature and discuss the anaesthetic management of patients with pentalogy of Fallot going for caesarean section., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Discrete central endovascular pulmonary arterial lesions raise clinical concern for malignancy such as primary pulmonary artery sarcoma. We present a case of a female in her late teens who had an obstructive mid right pulmonary artery lesion found on follow-up imaging 15 years after Tetralogy of Fallot repair. The lesion was in the vicinity of a previously ligated Blalock-Taussig shunt and causing right PA stenosis with delayed perfusion to the right lung, and a flow-related distal left PA aneurysm. The lesion was excised and confirmed histologically to be inflammatory in nature. Intraoperative microbiology demonstrated growth of the Kytococcus species, and she was managed with 6 weeks of intravenous antibiotics, with a full recovery., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Dilatation of the aortic root structures or ascending aorta is often observed in patients with Fallot-type anomalies. We aimed to determine the dilation rate of the aortic structures and investigate strategies for managing this phenomenon., Methods: In this retrospective study, we enrolled 66 out of 801 patients who underwent corrective surgery for Fallot-type anomalies (tetralogy of Fallot [TOF] and Fallot-type double outlet right ventricle [DORV]) between 2004 and 2020. These 66 patients had follow-up cardiac computed tomography (CT) angiography images taken at least 5 years after the initial CT study. We analyzed the diameters and aortic cross-sectional area/height ratio (AH) of the aortic annulus, sinus of Valsalva, sinotubular junction, and ascending aorta between the initial and follow-up CT scans. "Dilatation" was defined as a z-score over 2 for each aortic structure., Results: The median age at the initial and follow-up CT scans was 5.9 years (interquartile range [IQR]: 0.4 ~ 12.4) and 15.9 years (IQR 9.3 ~ 23.4), respectively. The median CT interval (from initial to latest CT) was 9.5 years (IQR 6.6 ~ 12.0). The sinus of Valsalva exhibited the most significant dilation (32.8 mm at follow-up CT) over the study period. The AH ratio increased significantly in all four aortic structures. The patient's age was significantly associated with higher AH in the follow-up CT. Aortic dilatation was present in 74.2% of patients at the initial CT and increased to 86.4% at the follow-up CT., Conclusion: In Fallot-type anomalies, the AH ratio of aortic root structures significantly increased over an average period of approximately 9.5 years. The number of patients diagnosed with aortic dilatation also increased. Based on our observations in this study, these patients' group should be considered for more frequent follow-up examinations, as significant dilatation could occur in their mid-20 s., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: To identify the association between preoperative low muscle mass and early postoperative outcomes in pediatric patients undergoing total correction of tetralogy of Fallot (TOF)., Design: A retrospective cohort study., Setting: A single university hospital in Seoul, South Korea., Participants: Pediatric patients (≤3 years) who underwent total correction of TOF between May 2008 and February 2018., Interventions: None., Measurements and Main Results: Cross-sectional areas of the pectoralis and erector spinae muscles were measured using preoperative chest computed tomography (CT) scans, and adjusted to body surface area to define muscle mass index. The patients were divided into sarcopenia, presarcopenia, and no sarcopenia groups based on cutoff values determined using the mean and SD of the muscle mass index in the third z-weight quintile. Of a total of 330 patients included in the final analysis, 13 were associated with the sarcopenia group, 57 in the presarcopenia group, and 260 in the no sarcopenia group. The sarcopenia group exhibited a higher incidence of major adverse events than the presarcopenia and no sarcopenia groups, respectively (38% v 25% v 18%; p = 0.033). Logistic regression analyses revealed that only younger age at the time of surgery was significantly associated with major adverse events (odds ratio 0.82; 95% CI 0.72-0.94, p = 0.003)., Conclusions: The incidence of sarcopenia, as assessed by preoperative chest CT, was low in pediatric patients undergoing total correction of TOF, and preoperative sarcopenia did not predict early postoperative major adverse events., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Patients with repaired tetralogy of Fallot are at elevated risk for ventricular arrhythmia and sudden cardiac death. Over the past decade, the pathogenesis and natural history of ventricular tachycardia has become increasingly understood, and catheter ablation has emerged as an effective treatment modality. Concurrently, there has been great progress in the development of a versatile array of transcatheter valves that can be placed in the native right ventricular outflow tract for the treatment of long-standing pulmonary regurgitation. Although such valve platforms may eliminate the need for repeat cardiac operations, they may also impede catheter access to the myocardial substrates responsible for sustained macro-reentrant ventricular tachycardia. This manuscript provides the rationale and design of a recently devised multicenter study that will examine the clinical outcomes of a uniform, preemptive strategy to eliminate ventricular tachycardia substrates before transcatheter pulmonary valve implantation in patients with tetralogy of Fallot., Competing Interests: Declaration of Competing Interest Drs. Aboulhosn and Levi report a relation with Edwards Lifesciences Corp. that includes consulting or advisory and with Medtronic Inc. that includes consulting or advisory. The remaining authors have no competing interests to declare., (Published by Elsevier Inc.)

Objective: The purpose of this study is to assess the feasibility, effectivity, and safety of a novel self-expandable valve system, Pulsta® transcatheter heart valve in patients with tetralogy of fallot and severe pulmonary regurgitation after transannular patch repair., Background: Severe pulmonary regurgitation after tetralogy of fallot repair is a life-threatening problem and should be treated by pulmonary valve implantation. Although percutaneous pulmonary valve implantation has been ever increasingly used for this purpose, available balloon-expandable valves have limitations and cannot be used by most patients. Pulsta® transcatheter heart valve is a new self-expandable valve system and offers a new solution to be used in patients with different types of native right ventricular outflow tract geometry., Patients and Methods: Ten patients with severe regurgitation after tetralogy of fallot repair with a transannular patch have been enrolled in the study according to echocardiographic examination. MRI was used in asymptomatic patients to delineate the indication and the right ventricular outflow tract geometry. Pulsta® transcatheter heart valve implantation was performed in ten patients, and preprocedural, procedure, and 6 months follow-up findings of the patients were evaluated., Results: Pulsta® pulmonary valve implantation was performed in ten patients successfully without any severe complications. Valve functions were perfect in six of ten patients, while the others had insignificant regurgitation by echocardiographic examination at the end of 6 months follow-up., Conclusions: This study showed that Pulsta® transcatheter heart valve is a feasible, effective, and safe method in the treatment of severe pulmonary regurgitation due to transannular patch repair in patients with tetralogy of fallot.

Objectives: For patients with tetralogy of Fallot (TOF) who are not suitable candidates for primary corrective surgery or have a high surgical risk, transcatheter right ventricular outflow tract (RVOT) stent implantation is considered a safe and effective palliative intervention. This study aims to investigate the therapeutic outcomes of RVOT stent implantation in neonates and infants with TOF in comparison with the modified Blalock-Taussig shunt (mBTS) and to compare the impact of the 2 palliative interventions on arterial oxygen saturation and pulmonary artery development in pediatric patients., Methods: Clinical data of 32 patients with TOF admitted to the Second Xiangya Hospital of Central South University from March 2011 to March 2021 were retrospectively collected. The patients were divided into an mBTS group (undergoing mBTS, n =15) and a stent implantation group (undergoing RVOT stenting, n =17) according to the surgical procedures. The 2 groups were assessed and compared in the surgical-related arterial oxygen saturation, postoperative complication rate, mortality rate, and re-intervention rate. The development of the patients' main pulmonary artery, right pulmonary artery, and left pulmonary artery was assessed by z-scores according to echocardiographic results., Results: The children in the stent implantation group were younger and less weighed compared with the mBTS group (both P <0.05). Compared with the preoperative period, children in the stent implantation group had significantly higher arterial oxygen saturation [(75±17)% vs (96±3)%, P =0.026]; z-scores of pulmonary trunk [(-2.82±1.27) points vs (0.86±0.77) points, P =0.014], right pulmonary artery [(-1.88±0.59) points vs (-0.28±0.71) points, P =0.011], and left pulmonary artery [(-2.34±0.36) points vs (-1.67±0.36) points, P =0.036] were significantly increased. However, there were no significant differences in arterial oxygen saturation and pulmonary artery z-scores between pre- and post-mBTS procedures (all P >0.05)., Conclusions: RVOT stent would have good surgical outcomes used in TOF patients with low weight and severe comorbidities. It also leads to an higher postoperative oxygen saturation and better promotion of pulmonary artery growth with RVOT stent compared to mBTS.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Ventricular dysfunction and cardiac arrhythmias are well-documented complications in patients with repaired TOF. Whether intrinsic abnormalities exist in TOF cardiomyocytes is unknown. We establish human induced pluripotent stem cells (hiPSCs) from TOF patients with and without DiGeorge (DG) syndrome, the latter being the most commonly associated syndromal association of TOF. TOF-DG hiPSC-derived cardiomyocytes (hiPSC-CMs) show impaired ventricular specification, downregulated cardiac gene expression and upregulated neural gene expression. Transcriptomic profiling of the in vitro cardiac progenitors reveals early bifurcation, as marked by ectopic RGS13 expression, in the trajectory of TOF-DG-hiPSC cardiac differentiation. Functional assessments further reveal increased arrhythmogenicity in TOF-DG-hiPSC-CMs. These findings are found only in the TOF-DG but not TOF-with no DG (ND) patient-derived hiPSC-CMs and cardiac progenitors (CPs), which have implications on the worse clinical outcomes of TOF-DG patients., (© 2023. Springer Nature Limited.)

Background: Patients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF., Methods: Thirty two rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3-6 months post-surgery., Results: RV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI) ≥ 160 ml/m2 or right ventricular end-systolic volume index (RVESVI) ≥ 80 ml/m2 in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI = 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI < 160 ml/m2 and RVESVI < 80 ml/m2, and the two parameters were higher than normal values. Compared with the RV mild dilatation group, patients of RV moderate-severe dilatation have worse cardiac function before surgery (right ventricular ejection fraction, 38.92(9.19) % versus 48.31(5.53) %, p < 0.001; Left ventricular ejection fraction, 59.80(10.26) versus 66.41(4.15), p = 0.021). Patients with RV moderate-severe dilatation faced longer operation time and more blood transfusion during operation (operation time, 271.53(08.33) min versus 170.53(72.36) min, p < 0.01; Intraoperative blood transfusion, 200(175) ml versus 100(50) ml, p = 0.001). Postoperative RV moderate-severe dilatation patients have poor short-term prognosis, which was reflected in a longer postoperative hospital stay (6.59 [2.12] days versus 9.80 [5.10] days, p = 0.024) and a higher incidence of hypohepatia (0[0] % versus 4[26.7] %, p = 0.023). Patients with RV dilatation score > 2.35 were diagnosed with RV moderate-severe dilatation (AUC = 0,882; Sensitivity = 94.1%; Specificity = 77.3%)., Conclusions: RV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score > 2.35, the patient is indicated for further CMR examination and treatment., (© 2023. BioMed Central Ltd., part of Springer Nature.)