Search

Background and Aims: Patients with repaired tetralogy of Fallot remain at risk of life-threatening ventricular tachycardia related to slow-conducting anatomical isthmuses (SCAIs). Preventive ablation of SCAI identified by invasive electroanatomical mapping is increasingly performed. This study aimed to non-invasively identify SCAI using 3D late gadolinium enhancement cardiac magnetic resonance (3D-LGE-CMR)., Methods: Consecutive tetralogy of Fallot patients who underwent right ventricular electroanatomical mapping (RV-EAM) and 3D-LGE-CMR were included. High signal intensity threshold for abnormal myocardium was determined based on direct comparison of bipolar voltages and signal intensity by co-registration of RV-EAM with 3D-LGE-CMR. The diagnostic performance of 3D-LGE-CMR to non-invasively identify SCAI was determined, validated in a second cohort, and compared with the discriminative ability of proposed risk scores., Results: The derivation cohort consisted of 48 (34 ± 16 years) and the validation cohort of 53 patients (36 ± 18 years). In the derivation cohort, 78 of 107 anatomical isthmuses (AIs) identified by EAM were normal-conducting AI, 22 were SCAI, and 7 blocked AI. High signal intensity threshold was 42% of the maximal signal intensity. The sensitivity and specificity of 3D-LGE-CMR for identifying SCAI or blocked AI were 100% and 90%, respectively. In the validation cohort, 85 of 124 AIs were normal-conducting AI, 36 were SCAI, and 3 blocked AI. The sensitivity and specificity of 3D-LGE-CMR were 95% and 91%, respectively. All risk scores showed an at best modest performance to identify SCAI (area under the curve ≤ .68)., Conclusions: 3D late gadolinium enhancement cardiac magnetic resonance can identify SCAI with excellent accuracy and may refine non-invasive risk stratification and patient selection for invasive EAM in tetralogy of Fallot., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Surgical repair of Tetralogy of Fallot (ToF) is usually performed in the first months of life with low early postoperative mortality. During long-term follow-up, however, both right (RV) and left ventricular (LV) performances may deteriorate. Tissue Doppler imaging (TDI) and speckle tracking echocardiography (ST) can unmask a diminished RV and LV performance. The objective of the current study was to assess the cardiac performance before and shortly after corrective surgery in ToF patients using conventional, TDI and ST echocardiographic techniques. Thirty-six ToF patients after surgery were included. Transthoracic echocardiography including TDI and ST techniques was performed preoperatively and at hospital discharge after surgery (10 days to 4 weeks after surgery). Median age at surgery was 7.5 months [5.5-10.9]. Regarding the LV systolic function there was a significant decrease in interventricular septum (IVS) S' at discharge as compared to preoperatively (pre IVS S' = 5.4 ± 1.4; post IVS S' = 3.9 ± 1.2; p < 0.001) and in global longitudinal peak strain (GLS) (pre = - 18.3 ± 3.4; post = - 14.2 ± 4.1; p = 0.003); but not in the fractional shortening (FS). Both conventional and TDI parameters showed a decrease in diastolic function at discharge. Tricuspid Annular Plane Systolic Excursion and RV S' were significantly lower before discharge. When assessing the RV diastolic performance, only the TDI demonstrated a RV impairment. There was a negative correlation between age at surgery and postoperative LV GLS (R = - 0.41, p = 0.031). There seems to be an impairment in left and right ventricle performance at discharge after ToF corrective surgery compared to preoperatively. This is better determined with TDI and ST strain imaging than with conventional echocardiography.

Background: An anomalous coronary artery is reported in 2% to 23% of patients with tetralogy of Fallot (TOF). Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to vessels crossing the right ventricular outflow tract (RVOT). A meta-analysis on the prevalence of anomalous coronary arteries in TOF is lacking to date. Here, an overview of coronary anomalies in TOF is provided and implications for patient management are discussed., Methods: PubMed, Embase and Web of Science were searched. Analysis was done using Revman 5.3 (Cochrane Community, London). The primary analysis focused on the origin and proximal course of the right and left coronary arteries. In addition, the prevalence of large conus arteries and coronary arteriovenous fistulas (CAVF) was calculated., Results: Twenty-eight studies, encompassing 6956 patients, were included; 6% of TOF patients have an anomalous coronary artery. Hereof, 72% cross the RVOT; the majority of the remaining 28% courses behind the aorta. Six percent of patients have a large conus artery and 4% a CAVF. Other coronary anomalies include a left or right coronary artery from the pulmonary trunk or left or right pulmonary artery, coronary tree hypoplasia and anastomoses between coronary and bronchial arteries., Conclusions: The prevalence of coronary anomalies in TOF is 4-6%. In patients with an anomalous coronary artery, 72% cross the RVOT. The combined risk of encountering an anomalous coronary artery or a large conus artery crossing the RVOT is 10.3%. Coronary anatomy should be defined before surgery and the surgical approach adapted accordingly., Competing Interests: Declaration of competing interest None., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Objectives: In repaired tetralogy of Fallot, surgical pulmonary valve replacement (PVR) is in certain cases required. Our institution reported earlier about 26 patients who received a pulmonary homograft via PVR. To date, we have data from more than 17 years of follow-up. The aim of this retrospective study was to evaluate the late haemodynamic and clinical outcomes in this predefined patient group., Methods: Between 1993 and 2001, 26 patients underwent PVR for pulmonary regurgitation (58% men; 30.4 ± 8.9 years). The rates of mortality and of complications (re-PVR, ablation and cardioverter defibrillator implants) were analysed. Other main study outcomes were haemodynamic parameters determined from cardiovascular magnetic resonance imaging: pulmonary regurgitation; right ventricular (RV) end-diastolic volume; RV ejection fraction; left ventricular (LV) end-diastolic volume; LV ejection fraction; New York Heart Association functional class at the latest follow-up visit; and echocardiographic parameters of the right ventricle., Results: The median follow-up time was 17 ± 1.1 years. Overall freedom from complications was 61.5% (95% confidence interval 47.5-78.6%). One patient died 18 months after surgery of unknown causes. Two patients needed replacement of the homograft at 24 and 39 months after PVR. The indication in both patients was recurrence of severe homograft regurgitation with important RV dilatation. Six patients received an implantable cardioverter defibrillator at a median age of 41 years (interquartile range 36-47); 12 patients experienced supra- and/or ventricular arrhythmias and 6 of these needed ablation. There was no significant deterioration of haemodynamic function or functional class., Conclusions: The patients who underwent PVR exhibited long-term follow-up stabilization of RV function and impressive functional durability of the graft. After a follow-up of 17 years, 23 out of 26 patients (89%) were alive without redo PVR. Event-free survival was good (61.5%).

Objective: In tetralogy of Fallot (TOF), the dominant ventricular tachycardia substrates are slow-conducting anatomical isthmuses. Surgical correction has evolved, which might have influenced isthmus presence and dimensions., Methods: One hundred and forty-two postmortem TOF specimens (84/58 corrected/uncorrected) were studied for isthmus presence. Isthmus 1 is located between the tricuspid annulus and right ventricular (RV) outflow tract (RVOT) patch/RV incision, isthmus 2 between RVOT patch/RV incision and pulmonary valve, isthmus 3 between pulmonary valve and ventricular septal defect (patch), isthmus 4 between ventricular septal defect (patch) and tricuspid annulus. Isthmus width and thickness were measured., Results: Of 84 corrected postmortem TOF specimens (death: 6.6 years (4.0-11.5)), 83 demonstrated isthmus 1 (99%, width=25±10 mm, thickness=5±2 mm), 35 isthmus 2 (42%, width=10±9 mm, thickness=3±2 mm), 83 isthmus 3 (99%, width=10±6 mm, thickness=5±2 mm), and 5 isthmus 4 (6%, width=4±2 mm, thickness=2±1 mm). Transatrial-transpulmonary correction (n=49) as compared with transventricular correction (n=35) prevented isthmus 2 (0% vs 100%, P<0.001). Transatrial-transpulmonary correction at age <1 year (n=7) as compared with ≥1 year (n=42) required a smaller transannular RVOT patch (28±15 vs 45±14 mm, P<0.001). Mode and timing of correction did not influence presence and dimensions of isthmus 3. In corrected and uncorrected TOF specimens (death 1.8 years (0.5-6.6)), the range of isthmus 3 dimensions was broad (width: min=2 mm, max=32 mm; thickness: min=1, max 13 mm) across all ages. Isthmus 3 width and thickness were strongly correlated (r=0.65, P<0.001)., Conclusions: In TOF, the current routine use of transatrial-transpulmonary correction prevents isthmus 2. Correction <1 year reduces transannular patch size, which may influence isthmus 1 width later in life. Mode and timing of correction did not change prevalence and dimensions of isthmus 3, in which dimensions varied widely in uncorrected and corrected TOF., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Aims: The right ventricle (RV) remodels early after pulmonary valve replacement (PVR) in tetralogy of Fallot (TOF) patients. Previously reported preoperative thresholds to achieve early postoperative RV normalization were consistently close to 80 mL/m(2) for end-systolic volume (ESV) and 160 mL/m(2) for end-diastolic volume (EDV). Our objective was to determine whether these thresholds were also associated with mid-to-late RV normalization and clinical events., Methods and Results: Out of a multicentre cohort of 157 TOF patients who had undergone PVR, in 65 patients (62% male, age 29 ± 8 years, homograft in 98%) cardiovascular magnetic resonance (CMR) imaging was performed preoperatively and >3 years (6.3 years, interquartile range: 4.9-9.5) postoperatively. Mid-to-late haemodynamic outcome was classified as: 'RV normalization' [RV ejection fraction (EF) > 48% and RV EDV < 108 mL/m(2)] in 14 of 65 (22%) patients, 'intermediate' in 34 of 65 (52%) patients, and 'suboptimal' (RV EF < 45% and RV EDV > 120 mL/m(2)) in 17 of 65 (26%) patients. Preoperative RV ESV < 80 mL/m(2) was strongly associated with favourable mid-to-late haemodynamic outcome in a proportional odds model [common odds ratio (OR): 0.04 for worse class, 95% confidence interval (CI): 0.01-0.17]. During 7.8 ± 4.0 years follow-up after PVR, adverse clinical events (death, sustained ventricular tachycardia, or heart failure) occurred in 18 of 106 (17%) patients with preoperative CMR available. Patients with preoperative RV ESV > 95 mL/m(2) were at increased risk for unfavourable mid-to-late haemodynamic outcome (common OR: 25.5, 95% CI: 5.35-122) and events (hazard ratio: 2.89, 95% CI: 1.03-8.11)., Conclusion: In TOF patients who had undergone PVR, the best preoperative threshold to achieve mid-to-late RV normalization was RV ESV < 80 mL/m(2). Patients with preoperative RV ESV > 95 mL/m(2) were at increased risk for suboptimal haemodynamic outcome and adverse clinical events. Our findings may assist in timing of PVR., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Background: In patients with repaired tetralogy of Fallot (rTOF), multiple reoperations or percutaneous interventions after pulmonary valve replacement (PVR) may be necessary due to limited homograft durability. However, data to guide individualised prediction of homograft durability remain scarce. The aim of this study was to provide risk models for RV to pulmonary artery homograft durability., Methods: This retrospective multicentre study included consecutive patients with rTOF who had undergone PVR at an age of >12 years. Homograft dysfunction was defined as at least moderate pulmonary regurgitation (PR) or pulmonary stenosis (PS) (pressure gradient ≥36 mm Hg) as assessed by echocardiography. Reintervention was defined as percutaneous intervention or redo-PVR., Results: A total of 153 patients with rTOF were included (62% male, mean age at PVR 31±11 years, pulmonary homograft 96%, follow-up 9.6 years (IQR 5.9, 13.3)). Average freedom from homograft dysfunction and reintervention after 10 years was 74% and 89%, respectively. In multivariable Cox proportional hazards analysis, postoperative PS ≥20 mm Hg (HR 6.52, 95% CI 3.09 to 13.7), postoperative PR ≥ grade 1 (HR 3.13, 95% CI 1.45 to 6.74) and age at PVR <18 years (HR 3.52, 95% CI 1.64 to 7.53) were independently predictive for homograft dysfunction. In patients without any risk factor, 10-year freedom from homograft dysfunction and reintervention was excellent (91% and 96%, respectively) in contrast to patients with ≥2 risk factors (25% and 73%, respectively)., Conclusions: Individualised prediction of homograft durability in patients with rTOF can be guided by early postoperative echocardiography. In adult patients without early postoperative PS or PR, homograft dysfunction and reintervention are unlikely to occur within 10 years, and follow-up may be less stringent., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objective: Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR., Methods: In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included. Preoperative and postoperative imaging data and a composite of adverse clinical events (death, sustained ventricular tachycardia, heart failure, or supraventricular tachycardia) were collected. Multivariate Cox hazards regression analysis was used to determine which factors were predictive for adverse events after PVR., Results: A total of 129 patients (61% men, age at PVR 32.9±10.4 years) were included. The composite endpoint occurred in 39 patients during 8.4±4.2 years of follow-up. In multivariate analysis, severe preoperative TR (HR 2.49, 95% CI 1.11 to 5.52), right ventricular end-systolic volume (HR 1.02/mL/m(2), 95% CI 1.01 to 1.03) and age at PVR (HR 1.07/year, 95% CI 1.04 to 1.09) were predictive for adverse events. Early postoperative TR was not predictive for adverse events (p=0.96). In patients without any risk factor (age >40 years, right ventricular end-systolic volume >90 mL/m(2) or severe TR), 5-year event-free survival was 100% as compared with 61% in patients with two or three risk factors., Conclusions: In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background: Radiofrequency catheter ablation (RFCA) of ventricular tachycardia (VT) in repaired Tetralogy of Fallot focuses on isthmuses in the right ventricle but may be hampered by hypertrophied myocardium or prosthetic material. These patients may benefit from ablation at the left side of the ventricular septum., Methods and Results: Records from 28 consecutive repaired Tetralogy of Fallot patients from 2 centers who underwent VT ablation were reviewed. Ablation targeted anatomic isthmuses containing VT re-entry circuits, which were identified by 3-dimensional substrate, pace, and entrainment mapping. A left-sided approach was considered beneficial if (1) right-sided RFCA failed, (2) part of the circuit was mapped to the left side, and (3) left-sided RFCA resulted in isthmus transection and prevention of VT induction. In 4 of 28 patients (52±13 years; 75% men), inducible for 1.5 (quartiles, 1.0 - 2.0) VTs (335±58 ms), left-sided RFCA was performed. In 3 patients, RFCA at aortic sites terminated VT related to a septal isthmus and prevented reinduction. In 1 patient, with prior biventricular implantable cardioverter-defibrillator, diastolic activity was recorded at the left side of the septum in proximity to the His-bundle. RFCA prevented VT reinduction with anticipated complete atrioventricular block. The left-sided approach resulted in complete procedural success (transection of anatomic isthmus and noninducibility) and freedom of VT recurrence during follow-up (20±15 months) in all patients. Right-sided RFCA failure was likely because of septal hypertrophy in 2, overlying pulmonary homograft in 1, and overlying ventricular septal defect patch in 1., Conclusions: Left-sided RFCA for VTs dependent on septal anatomic isthmuses improves ablation outcome in repaired Tetralogy of Fallot., (© 2014 American Heart Association, Inc.)

Objective: To study the changes over time in biventricular size and function, and clinical parameters in patients after repair of tetralogy of Fallot (TOF) without subsequent pulmonary valve replacement (PVR)., Methods: We prospectively included 78 non-PVR patients (age 20(6-60)years at baseline), who were studied twice with a 5-year interval. Patients underwent magnetic resonance imaging for assessment of biventricular size and function. Exercise testing and electrocardiography were performed to determine peak oxygen uptake (peak VO2) and QRS duration. N-terminal prohormone brain natriuretic peptide (NT-proBNP) was assessed additionally., Results: Pulmonary regurgitation (PR), right ventricular (RV) volumes and QRS duration increased during 5-year follow-up (RV end-diastolic volume (EDV) 130 ± 30 ml/m(2) to 138 ± 34 ml/m(2); QRS 132 ± 27 msec to 139 ± 27 msec); peak VO2 decreased (96 ± 19% to 91 ± 17%). RV ejection fraction, RV effective stroke volume (eff.SV), and NT-proBNP levels remained unchanged. The slope of RVEDV increase was 1.6 ± 3.0 ml/m(2)/year, and depended on RVeff.SV, not on RVEDV, at baseline. Increase in RVEDV correlated with increase in QRS duration over time (r=0.28, p=0.016), and with decrease in RV mass/EDV ratio over time (r=-0.42, p<0.001), not with decrease in peak VO2. In subgroup analysis, patients with larger RVs at baseline showed larger increase in PR during follow-up and larger decrease in NYHA class over time., Conclusions: In TOF patients with moderate RV dilatation, RVEDV increased by 1.6 ± 3.0 ml/m(2)/year, irrespective of RV size at baseline, but depended on RVeff.SV at baseline. Despite limited progression in RV size, unfavourable changes occurred during 5 years follow-up, which suggests there is a need for close follow-up., (© 2013.)

Background: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients. The aim of the current study was to determine the role of residual PS in the need for PVR during follow-up in cToF patients., Methods: cToF patients were included if clinical follow-up after primary surgical correction had taken place for a minimum of 5 years. Patient characteristics, surgical factors, and postoperative factors were reviewed, with a special focus on the transpulmonic systolic gradient. Cox proportional hazards regression analysis was performed to identify predictors of PVR., Results: Of 171 cToF patients, 71 (41.5%) underwent PVR after 24.2 years (interquartile range, 16.8-31.6 years). Year of birth, older age at corrective operation, and patch use significantly predicted PVR during follow-up. By contrast, a mild residual PS in cToF patients (peak systolic gradient, 15-30 mm Hg) independently reduced the risk of PVR, as compared with patients without PS (hazard ratio, 0.47; p=0.02) and with moderate-to-severe PS (hazard ratio, 0.35; p=0.01)., Conclusions: In addition to the known risks factors for PVR, a postoperative mild residual PS reduces the risk of PVR during follow-up of cToF patients. This finding provides clinical evidence for a conservative PS relief during correction of ToF., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to analyze whether QRS duration, before and after pulmonary valve replacement (PVR), is related to long-term outcome in patients with tetralogy of Fallot (TOF)., Background: Key factors that determine outcome after PVR in adult TOF patients are largely unknown. Recognition of such factors assists the identification of patients at increased risk of adverse events., Methods: Adults who previously underwent total correction for TOF (n=90; age 31.4±10.3 years) and required PVR for pulmonary regurgitation were included. The QRS duration was measured pre-operatively and 6 months after PVR. The post-operative changes in QRS duration were calculated. Adverse events (death, re-PVR, ventricular tachycardia, and symptomatic heart failure) were noted during follow-up., Results: During 5.5±3.5 years of follow-up, 13 adverse events occurred. The 5-year event-free survival rate was 76% for patients with a pre-operative QRS duration>180 ms and 90% in patients with a QRS duration≤180 ms (p=0.037). For patients with a post-operative QRS duration>180 ms, 5-year event-free survival was 71%, whereas it was 91% for patients with a post-operative QRS duration≤180 ms (p=0.004). After multivariate correction, a post-operative QRS duration>180 ms (hazard ratio: 3.685, 95% confidence interval: 1.104 to 12.304, p<0.05) and the absence of a reduction in QRS duration post-PVR (hazard ratio: 6.767, 95% confidence interval: 1.704 to 26.878, p<0.01), was significantly associated with adverse outcome., Conclusions: Severe QRS prolongation, before or after PVR, and the absence of a reduction in QRS duration after PVR, are major determinants of adverse outcome during long-term follow-up of patients with TOF., (Copyright © 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

In patients with corrected tetralogy of Fallot (cToF), left ventricular (LV) dysfunction is closely related to right ventricular (RV) dysfunction, indicating adverse ventricular-ventricular interactions. However, the mechanism that links RV dysfunction to LV dysfunction remains unclear. In this prospective study, 32 patients with cToF and 19 controls were enrolled. With cardiac magnetic resonance imaging, biventricular ejection fractions were assessed. Using 2-dimensional speckle tracking, global and regional RV and LV strains and LV twist were assessed. To detect and characterize ventricular-ventricular interaction, the relation between global and regional RV mechanics and global and regional LV mechanics was assessed. Global RV strain, global LV strain, and LV twist were decreased in patients with cToF. Global RV strain correlated with global LV strain (r = 0.66, p <0.001) and LV twist (r = -0.72, p <0.001), indicating the presence of adverse ventricular-ventricular interaction. Furthermore, close relations were observed between apical RV strain and apical LV strain (r = 0.62, p <0.001) and apical LV rotation (r = -0.67, p <0.001). In conclusion, RV strain was significantly related to LV strain and LV twist in patients with cToF and controls. Furthermore, apical RV strain correlated with apical LV strain and apical LV rotation, indicating adverse apical ventricular-ventricular interactions., (2010 Elsevier Inc. All rights reserved.)

Background: The outcome of surgical correction of atrioventricular septal defect and tetralogy of Fallot has improved in recent years but is still reported to be associated with high mortality. Controversy exists about the need of a right ventriculotomy or a right ventricular to pulmonary artery conduit. The purpose of this study was to evaluate our results of atrioventricular septal defect and tetralogy of Fallot repair by transatrial-transpulmonary approaches., Methods: Between 1979 and 2007, 20 consecutive patients underwent correction of atrioventricular septal defect and tetralogy of Fallot. Five patients had undergone prior palliative shunts. In all patients, a transatrial-transpulmonary approach was used and repair was accomplished without a conduit. The two-patch technique was used to correct the atrioventricular septal defect. Clinical data were obtained by retrospective review of inpatient and outpatient clinical charts., Results: There was no in-hospital mortality and one late, noncardiac death. Six patients required eight reoperations, six for left atrioventricular valve insufficiency (repair: n = 4; replacement: n = 2), one for residual ventricular septal defect, and one for pulmonary artery branch obstruction. Follow-up was complete for all patients (median, 17 years; range, 1.5 to 28 years). All 19 survivors were in good clinical condition at last control, without medication, and in New York Heart Association class I (n = 18) or II (n = 1). Transesophageal echocardiography revealed good right ventricular function, low right ventricular outflow tract gradients (mean, 9 +/- 7.4 mm Hg), and trace pulmonary valve insufficiency (n = 11)., Conclusions: Atrioventricular septal defect and tetralogy of Fallot can be repaired with low mortality by the transatrial-transpulmonary approach without the use of a conduit.

Objective: To assess the effect of pulmonary valve replacement (PVR) on the repolarization of patients with tetralogy of Fallot., Background: Pulmonary valve regurgitation may cause right ventricular failure in adult patients with Fallot's tetralogy. In these patients, prolonged depolarization and disturbed repolarization are associated with ventricular arrhythmias and sudden cardiac death., Methods: Thirty Fallot patients (age 32+/-9 years, 19 male) eligible for PVR were studied with cardiac magnetic resonance imaging (CMR) before and 6 months after PVR. Electrocardiograms obtained during initial and follow-up CMR were analyzed and occurrence of ventricular arrhythmias was studied., Results: Right ventricular end-diastolic volume (RV EDV) decreased from 322+/-87 to 215+/-57 ml after PVR (P<0.0001). The spatial QRS-T angle normalized from 117+/-34 to 100+/-35 degrees , P=0.0004 (normal angle <105 degrees). QT dispersion and T-wave complexity did not change significantly. T-wave amplitude decreased from 376+/-121 to 329+/-100 microV (P=0.01). T-wave area decreased from 43+/-15 to 38+/-13 microV s (P=0.02). Decreases in T-wave amplitude and area were most prominent in the right precordial leads overlying the RV. Three patients had sustained ventricular arrhythmias and one patient died suddenly. These patients had a QRS duration >160 ms. No severe ventricular arrhythmias were found in patients with a RV EDV <220 ml, QRS-T angle <100 degrees , QT dispersion <60 ms or T-wave complexity <0.30., Conclusion: Normal repolarization indices may be associated with the absence of severe ventricular arrhythmias. PVR in Fallot patients with dilated right ventricles has a beneficial effect on electrocardiographic indices of repolarization heterogeneity.

An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implantation of a Melody pulmonary valve prosthesis to replace a stenotic and insufficient homograft in the pulmonary artery. Preoperatively, 2 of the children suffered from fatigue and dyspnoea on exertion The homografts had been implanted between the ages of 1-2, to establish surgical continuity between the right ventricle and the pulmonary artery. The anomalies were tetralogy of Fallot, pulmonary atresia with intact ventricular septum and pulmonary atresia with a ventricular septum defect. Percutaneous pulmonary valve replacement was successful in all 3 patients. After implantation, right ventricular pressure decreased to 30% of systemic pressure and regurgitation was not observed. All patients were discharged in a good condition on the day after the implantation. Percutaneous pulmonary valve replacement is a promising technique with good short-term results. In selected patients this percutaneous technique can substitute or postpone the surgical replacement ofa stenotic or insufficient homograft.

Background: To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery., Methods and Results: Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42+/-10% to 43+/-10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m2) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: beta=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m2 for normalization of RV end-diastolic volume or 82 mL/m2 for RV end-systolic volume., Conclusions: Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m2 or RV end-systolic volume was <82 mL/m2.

Background: Predicting changes in right ventricular (RV) size and function after pulmonary valve replacement (PVR) is important for timely reintervention in adult tetralogy of Fallot patients., Methods: We analyzed the influence of pulmonary regurgitation severity and RV size and function before PVR on the outcome of RV size and function after PVR in 27 adult Fallot patients who had cardiac magnetic resonance imaging before and after PVR. RV dimensions were indexed for body surface area., Results: Pulmonary regurgitation (48% +/- 11% of RV stroke volume) was not related to RV dimensions and function before PVR. Moreover, severity of pulmonary regurgitation did not influence changes in RV dimensions after PVR. The indexed RV end-systolic volume before PVR (mean, 98 mL/m2; range, 52 to 235 mL/m2) best predicted the indexed RV end-systolic volume after PVR (mean, 59 mL/m2; range, 24 to 132 mL/m2, r = 0.78, p < 0.001) and the indexed RV end-diastolic volume after PVR (mean, 107 mL/m2; range, 70 to 170 mL/m2, r = 0.73, p < 0.001). Baseline RV ejection fraction corrected for valvular insufficiencies and shunting (21% +/- 7%) best predicted the RV ejection fraction after PVR (43% +/- 10%, r = 0.77, p < 0.001)., Conclusions: Timing of PVR should be based on indexed RV end-systolic volume and corrected RV ejection fraction rather than on severity of pulmonary regurgitation.

Aims: To analyse the long-term outcomes after pulmonary valve replacement (PVR) in patients with a previous correction for tetralogy of Fallot., Methods and Results: In a retrospective study, 158 adult patients with a diagnosis of tetralogy of Fallot, who had undergone a PVR after initial total correction in childhood, were identified from the CONCOR (CONgenital CORvitia) registry. All patients underwent 175 PVRs between June 1986 and June 2005. To analyse the predictors for homograft dysfunction and adverse events (death, reoperations, balloon angioplasty), Cox-regression analysis was performed. Overall freedom from significant homograft dysfunction was 66% after 5 years and 47% after 10 years. We could not identify predictors for combined homograft dysfunction. Event-free survival was 78% at 10 years and 68% at 15 years after PVR. Both early significant pulmonary regurgitation (PR) (HR 6.8, P = 0.017) and pulmonary stenosis (PS) (HR 4.0, P = 0.037) after surgery were associated with adverse events. When analysing direct post-operative PR or PS, we observed that in patients with severe, pre-operative PR, right ventricular aneurysm/patch resection resulted in a lower post-operative PR (mean difference grade 0.38 +/- 0.14, P = 0.01). Less significant post-operative PS was associated with a higher diameter of the homograft (HR 0.37, P = 0.006)., Conclusion: While 47% of the patients in our study were free from homograft dysfunction at 10 years after PVR, event-free survival after PVR remained fairly good (78%). Significant residual lesions directly after surgery influenced event-free survival. A smaller diameter of the pulmonary homograft and severe pre-surgical PR were related to early homograft dysfunction after surgery.

Objectives: The aim of this research was to assess right ventricular diastolic and systolic function before and after pulmonary valve replacement (PVR) in adult patients after repair of tetralogy of Fallot., Background: Pulmonary valve replacement (PVR) in adult patients late after repair of tetralogy of Fallot leads to rapid improvement of right ventricular (RV) systolic function., Methods: A total of 16 patients and 8 healthy subjects were included. Median age at initial repair was 4.9 (0.9 to 13.1) years, and mean age at PVR was 28.7 (19.5 to 45.6) years. Cardiac magnetic resonance imaging was performed before and 8 and 22 months after PVR. Right ventricular volumes and function as well as RV in- and outflow patterns were assessed., Results: The volume of the early filling of the RV (Evol) increased from 49.8 +/- 14.7 ml to 53.8 +/- 19.3 ml (not significant) and 62.0 +/- 18.9 ml, respectively (p < 0.05), whereas the volume of the atrial contraction (Avol) remained unchanged. Consequently, the Evol/Avol ratio increased from 1.4 +/- 0.7 before PVR to 1.6 +/- 0.7 at 8 months (not significant) and 2.3 +/- 1.2 at 22 months (p < 0.01). The Evol/Avol ratio was not significantly different from the healthy subjects at 22 months, indicating late recovery of diastolic function. Systolic function improved rapidly after PVR; the indexed RV end-systolic volume decreased from 93.7 +/- 33.0 ml/m2 to 60.9 +/- 18.4 ml/m2 (p < 0.01) and 54.8 +/- 21.0 ml/m2 (p < 0.01)., Conclusions: In adult patients late after total repair of Fallot, PVR leads to late improvement of diastolic function. We speculate that the rapid volume unloading after PVR increases systolic performance, whereas improvement in diastolic function requires long-term remodeling.

Aims: Late after total correction, Fallot patients with a long QRS duration are prone to serious arrhythmias and sudden cardiac death. Pulmonary regurgitation is a common cause of right ventricular (RV) failure and QRS lengthening. We studied the effects of pulmonary valve replacement (PVR) on QRS duration and RV volume., Methods and Results: Twenty-six consecutive Fallot patients were evaluated both pre-operatively and 6-12 months post-operatively by cardiac magnetic resonance (CMR). In this study, we present the computer-assisted analysis of the standard 12-lead electrocardiograms closest in time to the CMR studies. For the whole group, QRS duration shortened by 6+/-8 ms, from 151+/-30 to 144+/-29 ms (P=0.002). QRS duration decreased in 18 of 26 patients by 10+/-6 ms, from 152+/-32 to 142+/-31 ms. QRS duration remained constant or increased slightly in eight of 26 patients by 3+/-3 ms, from 148+/-27 to 151+/-25 ms. CMR showed a decrease in RV end-diastolic volume from 305+/-87 to 210+/-62 mL (P=0.000004). QRS duration changes correlated with RV end-diastolic volume changes (r=0.54, P=0.01)., Conclusion: Our study shows that PVR reduces QRS duration. The amount of QRS reduction is related to the success of the operation, as expressed by the reduction in RV end-diastolic volume.

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance.

Purpose: To assess the time course of right ventricular (RV) function improvement after pulmonary valve replacement (PVR) in patients 25.2 years +/- 7.0 after repair of tetralogy of Fallot., Materials and Methods: The medical ethics committee approved this study, and informed consent was obtained. Cardiac magnetic resonance (MR) imaging was performed before, 7 months after, and 19 months after PVR in 25 consecutive patients with tetralogy of Fallot with a 1.5-T MR imager. RV function was assessed with gradient-echo sequences in the short-axis plane. Pulmonary flow was assessed with a velocity-encoded phase-contrast sequence. Paired t test was used to evaluate follow-up data. Independent samples t test was used to assess differences based on the presence of recurrent pulmonary regurgitation (PR)., Results: Mean indexed RV end-diastolic volume decreased from 166.9 mL/m(2) +/- 41.3 before PVR to 113.5 mL/m(2)+/- 35.7 (P < .001) at 7-month follow-up and 111.7 mL/m(2)+/- 41.1 (P = .46) at 19-month follow-up. The RV ejection fraction was corrected for PR and improved from 25.0% +/- 7.7 before surgery to 44.1% +/- 11.9 (P < .001) and 45.2% +/- 11.1 (P = .39), at 7- and 19-month follow-up, respectively. Recurrent PR after PVR was found in 11 patients; 14 patients did not have recurrent PR. Total reduction of indexed RV end-diastolic volume at 19 months follow-up was more prominent in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05). Furthermore, improvement of RV ejection fraction corrected for regurgitation was more marked in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05)., Conclusion: In patients with tetralogy of Fallot, RV function improves rapidly after PVR and is sustained at 19-month follow-up in most patients; however, recurrence of PR after PVR appears to reduce recovery of RV systolic function., ((c) RSNA, 2004.)

Background: In patients with the tetralogy of Fallot, QRS prolongation predicts malignant ventricular arrhythmias. QRS prolongation may result from right ventricular dilatation. The relation of ECG markers to biventricular wall mass and volumes has not been assessed., Objective: To investigate the relations of surface ECG markers of depolarisation and repolarisation to right and left ventricular volume and biventricular wall mass., Methods: 37 Fallot patients (mean (SD) age 17 (9) years) were studied 14 (8) years after surgical repair; 34 had important pulmonary regurgitation. Left and right ventricular size was assessed from tomographic magnetic resonance imaging (MRI), and the amount of pulmonary regurgitation by velocity mapping MRI. QT, QRS, and JT duration and interlead dispersion markers were derived from a standard 12 lead ECG., Results: Mean QRS duration was significantly prolonged (133 (31) v 91 (11) ms in controls), as were dispersion of QRS (36 (17) v 20 (6) ms), QT interval (87 (48) v 42 (20) ms), and JT interval (93 (48) v 42 (19) ms). Biventricular volumes were increased (right ventricular end diastolic volume, 129 (41) v 70 (9) ml/m(2); left ventricular end diastolic volume, 83 (16) v 69 (10) ml/m(2)), as was right ventricular wall mass (24 (7) v 17 (2) g/m(2)). QRS duration correlated best with right ventricular mass (r = 0.55, p < 0.01)., Conclusions: In patients operated on for tetralogy of Fallot and with pulmonary regurgitation, ECG predictors of ventricular arrhythmias are influenced by several mechanical factors that may occur simultaneously. These include increased right ventricular volume, but also increases in left ventricular volume and in right and left ventricular wall mass.

Background: Pulmonary regurgitation (PR) late after total correction for tetralogy of Fallot may lead to progressive right ventricular (RV) dilatation and an increased incidence of severe arrhythmias and sudden death. Timing of pulmonary valve replacement (PVR) is subject to discussion, because the effect of PVR on RV function in adults is unclear. In this study, MRI was used to assess the effect of PVR on RV function and PR. Clinical improvement was established by means of the NYHA classification., Methods and Results: Twenty-six adult patients were included. Cardiac MRI was performed at a median of 5.1+/-3.4 months before and 7.4+/- 2.4 months after PVR. Mean preoperative PR was 46+/-10% (range, 25% to 64%). After PVR, 20 of 26 patients (77%) showed no residual PR, 5 patients showed mild residual PR, and 1 patient showed moderate PR. RV end-diastolic volume (RV-EDV) decreased from 305+/-87 to 210+/-62 mL (P<0.001), and RV end-systolic volume (RV-ESV) decreased from 181+/-67 to 121+/-58 mL (P<0.001). No significant change was found in RV-EF (42% versus 42%). However, RVEF corrected for regurgitations and shunting increased from 25.2+/-8.0% to 43.3+/-13.7% (P<0.001). Mean validity class improved from 2.0 to 1.3 (P<0.001)., Conclusions: In adult patients with PR and RV dilatation, late after total correction of tetralogy of Fallot, MRI measurements show remarkable hemodynamic improvement of RV function after PVR and improvement of validity. We therefore advocate a less restrictive management concerning PVR in these patients.

Objectives: To analyze the results of pulmonary valve insertion late after initial repair of Fallot's tetralogy. Pulmonary insufficiency (PI) after correction of Fallot's tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time., Methods: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7+/-11.9 years. The mean age at initial repair was 6.4+/-7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3+/-9.1 years (2.7-40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up., Results: Follow-up is complete and had a mean duration of 1.7+/-1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3+/-0.6 to 1.4+/-0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged., Conclusions: PI late after correction of Fallot's tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients.

A 42-year-old man underwent assumed total surgical repair of an acyanotic tetralogy of Fallot: a perimembranous ventricular septal defect (VSD) was closed with a dacron patch and myectomy of the infundibulum of the right ventricular outflow tract was performed. Reexamination eight years later revealed a large recurrent VSD and by surprise a narrow supravalvular ridge above rudimentary pulmonary cusps in the pulmonary trunk, leading to a pressure drop of 70 mmHg across the supravalvular stenosis. The latter finding was not recognized during the operation eight years before. The diagnosis could noninvasively be established by means of magnetic resonance imaging. During revision surgery the VSD was closed and an aortic homograft was inserted as conduit between the right ventricle and the pulmonary artery.