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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.)

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.)

Pulmonary regurgitation is the most important residual lesion after initial surgical correction for pulmonary (sub)valvular stenosis in the early life of patients with tetralogy of Fallot or isolated pulmonary stenosis. Symptomatic or asymptomatic patients with severe right ventricular dilatation due to pulmonary regurgitation may benefit from pulmonary valve replacement. Surgery is ideally performed before the right ventricle becomes irreversibly damaged as a result of longstanding volume overload. However, the beneficial effects must be weighed up against the problems associated with degradation of the allograft, which often result in (numerous) reoperations. Owing to the higher risk of thromboembolic events in mechanical prosthesis and the lifetime need for anticoagulation, allografts are the most widely used prosthesis. Degradation of the allograft often leads to reoperation, mostly 10-20 years after initial implantation. For a patient receiving his first allograft at 20 years of age, several reoperations will have to be performed later in life. Percutaneous pulmonary valve implantation has the potential to decrease the number of surgical reoperations.

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.

Objective: To analyse the long-term course of QRS duration after pulmonary valve replacement in patients with a previous correction for tetralogy of Fallot., Setting: Tertiary referral centres., Methods: In a retrospective study, 99 adult patients with tetralogy of Fallot, who had undergone a first pulmonary valve replacement late after initial total correction, were identified from the CONCOR (CONgenital CORvitia) registry. Computer-generated QRS durations were obtained from 12-lead electrocardiogram ECG reports in the medical records. A mixed linear regression model was used to analyse the course of QRS duration over time and to identify risk factors for increase in QRS duration over time. Composite end point was created from sudden cardiac death, ventricular tachycardia or implantable cardioverter-defibrillator discharge., Results: In total, 99 patients (57% men, mean (SD) age at pulmonary valve replacement 29 (11) years) with a median follow-up of 4.9 (0.1-16) years were analysed. In patients with preoperative QRS <120 ms, surgery caused no significant change in QRS duration (increase 1.3 (7.9) ms; p = 0.65), and after surgery, QRS duration remained stable over time (increase 0.0064 (0.059) ms/year; p = 0.98). By contrast, in patients with a preoperative QRS of 150-180 ms or QRS > or =180 ms, surgery resulted in QRS shortening (mean decrease 9.9 (SE 4.3) ms, p = 0.021, and 12.2 (SE 2) ms; p<0.001, respectively). During follow-up, a QRS widening 1.1(1.3) ms/year (p<0.001) in both groups was observed. In patients with a preoperative QRS > or =180 ms, no significant difference was observed in the number of patients reaching the composite end point compared with patients with a preoperative QRS of 150-180 ms (25% vs 7%; p = 0.08). However, the former more often reached QRS > or =180 ms again after surgery compared with the latter (53% vs 13%; p = 0.02, respectively). None of the patients with a preoperative QRS > or =180 ms died during follow-up., Conclusion: In our study, we observed a decrease in QRS duration directly after surgery, followed by a steady increase, in patients with a preoperative QRS >150 ms. The beneficial effect of pulmonary valve replacement on QRS duration was transient. The risk of developing ventricular arrhythmias after surgery was substantial when preoperative QRS was > or =180 ms, but mortality remained low.

We present a 22 year old Moroccan woman with chronic severe pulmonary regurgitation, who becomes symptomatic in her fourth month of pregnancy. Cardiovascular magnetic resonance, during pregnancy, revealed a large pulmonary aneurysm and turbulent blood flow in the pulmonary trunk with severe pulmonary regurgitation. After gestation, the branch pulmonary arteries were assessed with magnetic resonance angiography and the severely dilated pulmonary arterial trunk and valve were replaced by a pulmonary homograft. We briefly review this rare syndrome, the management of pulmonary regurgitation during pregnancy and the role of CMR during pregnancy.

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.