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The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter or surgical interventions. One fifth of these conditions usually involve the right ventricular outflow tract (RVOT). Percutaneous pulmonary valve replacement (PPVR) has been widely established as an alternative, less invasive option to surgical pulmonary valve replacement (SPVR). The variability of RVOT anatomy and size, the relative course of the coronary arteries, and the anatomy of the pulmonary artery branches are factors that determine the success of the intervention and the complication rates. Careful and reliable pre-interventional imaging warrants the selection of suitable candidates and minimizes the risk of complications. 2D and 3D fluoroscopy have been extensively used during pre- and peri-interventional assessment. Established imaging techniques such as cardiovascular magnetic resonance (CMR) and computed tomography (CT) and newer techniques such as fusion imaging have proved to be efficient and reliable tools during pre-procedural planning in patients assessed for PPVR.

We report our experience in simultaneously implanting multiple stents and valves mounted on a single balloon before and during transcatheter pulmonary valve placement.Heterogeneity and complexity of the right ventricular outflow tract (RVOT) may complicate stent deployment when preparing a landing zone for transcatheter pulmonary valve implantation.Retrospective analysis of patients from Children's Hospital of Colorado, USA; and Oslo University Hospital, Norway, undergoing transcatheter pulmonary valve replacement that had at least two stents mounted on a single balloon, deployed in the RVOT.Over a 42-month period, a total of 50 subjects from the two centers met inclusion criteria for the study. Subjects were predominantly male (58%), and the median age was 17 years (4-78 years). In six subjects (12%), there was need for prestenting with use of the double or triple stent piggyback technique. Forty subjects (80%) had a Melody ™ TPV implanted. In 45 cases (90%), one or more stents were mounted over the pulmonary valve using its delivery system, either the Ensemble for the Melody™ TPV or the Edwards Commander for the SAPIEN 3 THV. Thirty-seven subjects (74%) had one stent mounted and eight subjects (16%) had two stents mounted over the pulmonary valve for simultaneous deployment. No complications related to this technique were reported.The piggyback technique aims to simplify and facilitate adequate conduit preparation and valve insertion by minimizing manipulation across the outflow tract and decreasing the risk of stent distortion, misalignment, and embolization.

Serum biomarkers of myocardial fibrosis are considered markers of adverse outcome in adults with heart disease. Associations between biomarkers and clinical parameters in tetralogy of Fallot (TOF) has been understudied. We compared serum biomarker profiles with clinical and cardiac magnetic resonance (CMR) parameters of ventricular remodeling in patients with repaired TOF.Serum biomarkers [metalloproteinases MMP1 and MMP9, galectin-3, micro-RNA21 (miR21)), ST2, procollagen type I carboxy-terminal propeptide (PICP), and NTproBNP] were measured in TOF patients undergoing CMR. Associations between biomarkers and clinical and CMR variables were assessed using correlation coefficients, and linear and logistic regression.Sixty patients were investigated, of which 47% were male. Age at CMR and TOF repair was 15 years [interquartile range (IQR) 9, 22] and 3.2 months (IQR 0.8, 6.2), respectively. Twelve (20%) had prior pulmonary valve replacement (PVR). MMP1 values were higher among those with prior PVR (16.7 (IQR 7.9, 25.5) vs 14.4 (IQR 9.9, 24.9), p = 0.02). When stratifying MMP1 into low and high groups, higher MMP1 was associated with higher indexed right (RV) and left ventricular (LV) mass and RV mass:volume ratios after adjusting for PVR. No other associations between biomarkers and CMR parameters were identified.Only MMP1 was associated with markers of RV remodeling after TOF repair. As an enzyme involved in extracellular matrix degradation, MMP1 could be associated with fibrotic processes underlying RV remodeling, including dilation and hypertrophy. The additional biomarkers may not be specific towards cardiac remodeling. These findings merit further correlations with myocardial fibrosis measurements by CMR.

We aimed to explore the relation between temporal right heart growth and long-term outcomes in patients with pulmonary atresia with an intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvuloplasty (BPV).We performed echocardiography to measure pulmonary valve diameter, right atrial end-systolic area (RAA), and right ventricular end-diastolic area (RVA) before BPV 1 and 5 years after BPV. Primary and secondary end points were to explore temporal changes in right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs).In 31 patients, pulmonary valve diameter significantly increased after BPV, whereas tricuspid valve diameter remained unchanged throughout the 10.0-year follow-up (range, 5.8-14.0 years). After BPV, RAA temporally decreased, whereas RVA significantly increased. There were LAEs in 6 patients (19%), arrhythmias in 2, heart failure in 1, reintervention of the right ventricular outflow tract in 1, and reintervention for residual cyanosis in 2. The rate of freedom from LAEs at 5 and 10 years was 92% and 82%, respectively. Right atrial end-systolic area temporally decreased in patients without LAEs (P.01); however, RAA remained unchanged throughout the period in patients with LAEs (P = .16). Moderate or severe pulmonary regurgitation (hazard ratio = 23.0; 95% confidence interval, 1.3-385; P = .03) and the ratio of RVA to RAA at 1 year after BPV (hazard ratio = 6.3 × 10Disproportional right heart growth was observed in patients with pulmonary atresia with an intact ventricular septum after BPV. Pulmonary regurgitation and increased RAA are crucial for identifying the burden of LAEs among them.

Background Ventricular interdependence may account for altered ventricular mechanics in congenital heart disease. The present study aimed to identify differences in load-dependent right ventricular (RV)–left ventricular (LV) interactions in porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) by invasive admittance-derived hemodynamics in conjunction with noninvasive cardiovascular magnetic resonance (CMR). Methods Seventeen pigs were used in the study (7 with PS, 7 with PI, and 3 controls). Progressive PS was created by tightening a Teflon tape around the pulmonary artery, and PI was created by excising 2 leaflets of the pulmonary valve. Admittance catheterization data were obtained for the RV and LV at 10 to 12 weeks after model creation, with the animal ventilated under temporary diaphragm paralysis. CMR was performed in all animals immediately prior to pressure–volume catheterization. Results In the PS group, RV contractility was increased, manifested by increased end-systolic elastance (mean difference, 1.29 mm Hg/mL; 95% confidence interval [CI], 0.57-2.00 mm Hg/mL). However, in the PI group, no significant changes were observed in RV systolic function despite significant changes in RV diastolic function. In the PS group, LV end-systolic volume was significantly lower compared with controls (mean difference, 25.1 mL; 95% CI, -40.5 to -90.7 mL), whereas in the PI group, the LV showed diastolic dysfunction, demonstrated by an elevated isovolumic relaxation constant and ventricular stiffness (mean difference, 0.03 mL−1; 95% CI, -0.02 to 0.09 mL−1). Conclusions The LV exhibits systolic dysfunction and noncompliance with PI. PS is associated with preserved LV systolic function and evidence of some LV diastolic dysfunction. Interventricular interactions influence LV filling and likely account for differential effects of RV pressure and volume overload on LV function.

In post-operative Tetralogy of Fallot (TOF) patients cMRI data are used for determining right ventricle (RV) dilation to justify the pulmonary valve placement. Echocardiogram analysis of RV volumes has inherent accuracy issues. There are no data exploring the relationship of transthoracic echocardiography tricuspid valve (TV) annulus size and cMRI RV dilation. Retrospective study in post-operative TOF patients. Patients less than 21 years old and with high-quality transthoracic echocardiogram and MRI within 6 months of each other were included. We collected demographic data, type of initial repair, echocardiographic, and cMRI data. A single reviewer measured echocardiogram TV size. Boston z-scores were used. 77 echocardiogram and cMRI pairs were analyzed. Average age was 14 years old. There is not a strong linear relationship between TV z-score and indexed right ventricular end diastolic volume (RVEDVi) (R

Prestenting right ventricular outflow tracts (RVOTs) before transcatheter pulmonary valve replacement (TPVR) is essential. Optimus-XXL (AndraTec GmbH, Koblenz, Germany) is a new extra-large, balloon-expandable cobalt-chrome stent with promising technologies.From June 2020 to November 2020, 15 patients with congenital heart disease, dysfunctional RVOTs and target TPVR diameter ≥ 23 mm received Optimus-XXL stents before proceeding to TPVR using the SAPIEN valve (Edwards Lifesciences, Irvine, CA). Standard safety and outcomes were prospectively assessed.Patients' median age and weight were 25.8 years (range: 10.5-63.1 years) and 58 kg (range: 43.8-101 kg), respectively. Underlying diagnosis was tetralogy of Fallot (66.7%), and RVOTs were patched (80%). Fifteen bare-metal stents were implanted using femoral (n = 14) and jugular approaches (n = 1). One conduit rupture was immediately controlled with a covered Optimus-XXL. Median stent length was 43 mm (range: 33-57 mm), and median target expansion diameter was 28 mm (range: 23-30 mm). Two procedural incidents occurred during stent delivery and were percutaneously treated. Stent stability was documented during TPVRs immediately performed in 14 patients. Median stent shortening was 13.7%, and median percentage of intended stent expansion was 95.9%. There was no stent fracture on the short-term follow-up (median: 4.5 months).We report the first implantations of Optimus-XXL stents in dysfunctional RVOTs with excellent preliminary results. Optimus-XXL should be considered as a valuable adjunct in the armamentarium for routine and complex TPVR procedures.

The rate of morbidity and mortality related to pulmonary regurgitation and pulmonary stenosis are big concerns after the surgery for CHD. Percutaneous pulmonary valve implantation has been established as a less invasive technique compared to surgery with promising results according to long-term follow-up of the patients. There are only two approved valve options for percutaneous pulmonary valve implantation until now, which are Melody (Medtronic, Minneapolis, Minn, USA) and Sapien (Edwards Lifesciences, Irvine, Ca, USA). Both valves have limitations and do not cover entire patient population. Therefore, the cardiologists need more options to improve outcomes with fewer complications in a such promising area. Herein, we present a case series applying for pulmonary position in conduits and native right ventricular outflow tract of a new transcatheter valve system Myval ® which is designed for transcatheter aortic valve implantation procedures. This is the first patient series in which the use of Myvalv in dysfunctional right ventricular outflow tracts is described, after surgical repair of CHD.

Current indications for pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) rely on cardiovascular magnetic resonance (CMR) image-based indices but are inconsistently applied, lead to mixed outcomes, and remain debated. This study aimed to test the hypothesis that specific markers of biventricular shape may discriminate differences between rTOF patients who did and did not require subsequent PVR better than standard imaging indices. In this cross-sectional retrospective study, biventricular shape models were customized to CMR images from 84 rTOF patients. A statistical atlas of end-diastolic shape was constructed using principal component analysis. Multivariate regression was used to quantify shape mode and imaging index associations with subsequent intervention status (PVR, n = 48 vs. No-PVR, n = 36), while accounting for confounders. Clustering analysis was used to test the ability of the most significant shape modes and imaging indices to discriminate PVR status as evaluated by a Matthews correlation coefficient (MCC). Geometric strain analysis was also conducted to assess shape mode associations with systolic function. PVR status correlated significantly with shape modes associated with right ventricular (RV) apical dilation and left ventricular (LV) dilation (p p p p p

Background: Late pulmonary valve replacement following repair of tetralogy of Fallot may become necessary in patients with chronic pulmonary insufficiency. There is limited information on the long-term outcome of these prostheses, which is the focus of this study. Methods: We conducted a retrospective study of patients with repaired tetralogy of Fallot who underwent pulmonary valve replacement from 1990 to 2015 in our institution. We investigated imaging and clinical parameters including mortality and late adverse events (reintervention [surgical or transcatheter]), infective endocarditis, or arrhythmias requiring device implantation or ablation. Results: There were 69 patients divided into 3 groups: Carpentier-Edwards (n = 14), Contegra (n = 40), and pulmonary homograft (n = 15). The mean age at the time of pulmonary valve replacement was 21 ± 12 years. The mean follow-up was 8.5 ± 4.7 years. The mean preoperative and postoperative right ventricular end-diastolic volume index was 210 ± 42 and 120 ± 24 mL/m2, respectively. There were no mortalities. Late adverse events were observed in 23 (33%) patients: 15 (22%) reintervention (surgical or transcatheter), 11 (16%) endocarditis, and 11 (16%) arrhythmias. Overall, 1-, 5-, and 10-year freedom from surgical reintervention was 98.5%, 93.6%, and 79.3%, respectively. The Contegra group had significantly higher pulmonary valve gradients, a higher risk of developing late adverse events compared to Carpentier-Edwards ( P = .046) and pulmonary homograft ( P = .055) in multivariate analysis and increased risk for reintervention in the univariate analysis (hazard ratio: 3.4; 95% CI: 0.92-13; P value.066). Conclusion: Pulmonary valve replacement in patients with repaired tetralogy of Fallot has acceptable short- and intermediate-term outcomes. Contegra prosthesis had a higher risk of late adverse events with higher pulmonary valve gradients.

Transcatheter pulmonary valve replacement has become an attractive alternative to surgical approach in patients with dysfunctional right ventricular outflow tract. However, in certain cases, an unfavorable anatomy might complicate optimal valve deployment and stability. Several techniques have been described to reshape the landing zone and allow proper implantation of the transcatheter valve. Among them, the hybrid approach has gained attention as an interesting method for off-pump pulmonary valve replacement in patients with dilated right ventricular outflow tract. But to date, there is no standardized method to resize and reshape the landing zone for the stented valve. Here, we describe a reproducible method based on simple geometric rules to allow adequate remodeling of the main pulmonary artery to the desired dimensions in a single attempt, followed by perventricular implantation of a Venus P-valve.

Background: The aim was to determine the effect of pulmonary artery (PA) morphology on the branch pulmonary artery-regurgitation fraction (BPA-RF), the relationship of pulmonary insufficiency (PI) to BPA-RF and PA-distensibility, and factors (BPA-RF and PA-distensibility) associated with right ventricular function (RVF) in repaired tetralogy of Fallot (rTOF). Methods: A total of 182 rTOF patients (median age 17.1 years) were analyzed for length, angle of PA, BPA-RF, PI, and PA-distensibility, using magnetic resonance imaging. Results: The left PA had a significant greater RF than the right PA (median (interquartile range)): LPA 43.1% (32.6–51.5) and RPA 35.2% (24.7–44.7), p &lt, 0.001. The LPA was shorter with a narrower angle than the RPA (p &lt, 0.001). The anatomy of the branch-PA was not a factor for the greater LPA-RF (odds ratio, 95% confidence interval: CI, p-value): length 0.44 (0.95–2.00), p = 0.28, angle 0.63 (0.13–2.99), p = 0.56. There was a strong positive correlation between PI and BPA-RF-coefficients (95% CI), p-value: LPA 0.78% (0.70–0.86), p &lt, 0.001, RPA 0.78% (0.71–0.84), p &lt, 0.001 and between BPA-RF and distensibility-coefficients (95%CI), p-value: LPA 0.73% (0.37–1.09), p &lt, RPA 1.63% (1.22–2.03), p &lt, 0.001, respectively. The adjusted BPA-RF did not predict RVF, RPA (p = 0.434), LPA (p = 0.268). Conclusions: PA morphology is not a significant factor for the differential BPA-RF. The vascular wall in rTOF patients responds to chronic increased intravascular volume by increasing distensibility. BPA-RF is not a determinant of RVF.

Although valve sparing (VS) for patients with smaller pulmonary valves has been increasing, transannular patch repair( TAP) accounts for more than half of the total tetralogy of Fallot corrections worldwide. We use fresh autologous pericardial patches to perform a modified TAP procedure with pulmonary valve leaflet augmentation as proposed by Sung et al. We aimed to explore the early and midterm outcomes of this procedure.We retrospectively reviewed 37 patients( group TAP:12;group VS:25) who underwent total tetralogy of Fallot corrections from April 2018 to December 2021.No midterm mortality was observed at a median observation period of 20.4 months in both groups. The midterm rates of freedom from moderate or more pulmonary regurgitation( PR) were 64.2 % and 21.4% in group TAP and 100% and 100% in group VS at 1 and 3 years, respectively( p0.001). The midterm rates of freedom from pulmonary stenosis reintervention were 100% and 100% in group TAP and 96% and 96% in group VS at 1 and 3 years, respectively( p=0.51).TAP showed acceptable midterm survival and reintervention rate. Longer follow-up is essential considering the significantly higher PR in the postoperative period in group TAP.

During percutaneous pulmonary valve implantation, it is challenging to advance the delivery system loaded with a stent-valve through the right ventricular outflow tract. An extra-stiff wire can provide enough supporting and stable positioning to prevent the displacement of the guide wire in stent-valve delivery. In the 2 cases reported here, we used a snare to trap the extra-stiff wire and a large sheath to support the snare; thus, the extra-stiff wire was pulled to the distal end and provided strong support for the advancement of the delivery system. After 8 months of follow-up, the patients were in good condition with excellent valve function. We named this novel, efficient technique the “parallel anchor sheath snare” technique. This report highlights a novel delivery strategy for patients with tortuous right ventricular outflow tracts in percutaneous pulmonary valve implantation.

Background In this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot. Methods Between 2015 and 2018, 18 patients had anterior pulmonary valve repair at our institution, and 26 patients had both anterior and posterior pulmonary valve repair. Results Patients ranged from 6 months to 30 years of age. The median follow-up period was 8 months in the anterior augmentation group and 5 months in the anterior and posterior augmentation group. Postoperative echocardiograms indicated that only 2 patients (11%) in the anterior augmentation group had moderate or severe pulmonary insufficiency, compared with no patients in the anterior and posterior augmentation group. At follow-up, pulmonary insufficiency was seen in 3 patients (17%) in the anterior augmentation group and no patients in the anterior and posterior augmentation group. Conclusion Reconstruction of the native pulmonary valve accompanied by pulmonary cusp augmentation can decrease or even circumvent postoperative pulmonary insufficiency. Both anterior augmentation and anterior and posterior augmentation techniques are easily applied; however, we believe that the anterior and posterior augmentation technique is superior in terms of early postoperative and follow-up pulmonary insufficiency outcomes.

OBJECTIVES Since percutaneous pulmonary valve implantation (PPVI) was introduced to prolong the lifetime of surgically placed right ventricular to pulmonary artery conduits, valve technology has evolved and the indications for PPVI expanded to native and larger right ventricular outflow tracts. We explore how indications, patient populations and outcomes compare to surgical pulmonary valve replacement (PVR). METHODS This is a retrospective cohort study of PPVI and PVR procedures between 1998 and 2020 at a single UK centre. One hundred and twenty-eight patients underwent PPVI and 365 patients PVR. Primary outcome measures were survival, infective endocarditis and reintervention. RESULTS The most common indication for PVR was replacement of the native pulmonary valve for pulmonary regurgitation whereas PPVI was more commonly used to treat pulmonary stenosis in a previously placed bioprosthetic conduit or valve. Treatment indications for PPVI expanded over the study to include the native right ventricular outflow tract. Survival was similar for PPVI and PVR (92% PPVI and 96.8% PVR at 5 years; 85.8% PPVI and 95.1% PVR at 10 years). Preprocedural New York Heart Association class 3 and 4 was the most important predictor of poor outcome. Annualized infective endocarditis rate was significantly higher for the Melody PPVI (0.024 vs 0.0024/person/year for PVR, P CONCLUSIONS An increasing number of patients requiring PVR can now be treated percutaneously. A lifetime strategy for re-valving should be considered at the first valve implant.

One of the major obstacles preventing successful percutaneous pulmonary valve implantation (PPVI) is related to the close proximity of coronary artery branches to the expected landing zone. The aim of this study was to assess the frequency of coronary artery anomalies (CAAs) especially those associated with major coronary branches crossing the right ventricular outflow tract (RVOT) and to describe their relevance for the feasibility of percutaneous pulmonary valve implantation (PPVI). In our retrospective single-center study 90 patients were evaluated who underwent invasive testing for PPVI in our institution from 1/2010 to 1/2020. CAAs were identified in seven patients (8%) associated with major branches crossing the RVOT due to origin of the left anterior descending (LAD) or a single coronary artery from the right aortic sinus. In 5/7 patients with CAAs balloon testing of the RVOT and selective coronary angiographies revealed a sufficiently large landing zone distal to the coronary artery branch. While unfavorable RVOT dimensions prevented PPVI in one, PPVI was performed successfully in the remaining four patients. The relatively short landing zone required application of the “folded” melody technique in two patients. All patients are doing well (mean follow-up 3 years). CAAs associated with major coronary branches crossing the RVOT can be expected in about 8% of patients who are potential candidates for PPVI. Since the LAD crossed the RVOT below the plane of the pulmonary valve successful distal implantation of the valve was possible in 4/7 patients. Therefore these coronary anomalies should not be considered as primary contraindications for PPVI.

Percutaneous pulmonary valve implantation (PPVI) has been implemented as a novel alternative strategy to surgical pulmonary valve replacement. However, PPVI has an inevitable limitation: the large right ventricular outflow tract (RVOT) lesions exhibit variable geometry and significant pulmonary regurgitation (PR). To overcome this limitation, bilateral branch pulmonary artery (PA) valve implantations using Melody or Sapien valves have been attempted and have shown a reduction in right ventricular volume with clinical benefits in the intermediate term. Nevertheless, these trials also have constraints of large branch PA size. Recently, a feasibility study using the Pulsta valve (Tae Woong Medical Co, Gyeonggi-do, South Korea) for native RVOTs was reported; the diameter of the Pulsta valve ranges from 18 to 32 mm. Herein, we present a successful percutaneous bilateral branch PA valve implantation using two 32 mm Pulsta valves in a 59-year-old man who showed right heart failure with severe pulmonary regurgitation despite several open heart surgeries for tetralogy of Fallot. The main PA was measured to be 49 mm, and both the right and left PAs were measured to be 30 mm.

Introduction: Advancements in surgery and management have resulted in a growing population of aging adults with tetralogy of Fallot (TOF). As a result, there has been a parallel growth in late comp...

Patient prosthesis mismatch (PPM) is an important factor of the outcome in transcatheter aortic valve implantation. However, the impact of PPM in transcatheter pulmonic valve implantation (TPVI) has not been studied. Based on the narrowest valve stent diameters in two views of fluoroscopy, internal geometric orifice area (GOA) of the valve stent was calculated and indexed by body surface area (BSA), deriving iGOA. To define PPM in TPVI, receiver operating characteristics (ROC) curve analysis for iGOA for predicting significant residual right ventricular outflow tract (RVOT) gradient was used to derive the optimal cut-off value of iGOA. Our cohort were divided into 2 groups: PPM versus non-PPM. The clinical data were compared between 2 groups. TPVI was performed using Melody valve in 101 patients. Significant RVOT residual pressure gradient (≥ 15 mmHg) was observed in 31 patients (39.6%). Over a mean follow up periods of 6.9 ± 2.7 years, 22 patients (21.8%) required repeat interventions (16 transcatheter, 11 surgical, and both in 5 patients). Based on the ROC analysis, the best cut-off value of iGOA was 1.25 cm2/m2 (area under the curve 0.873, p

Purpose Patients with repaired Tetralogy of Fallot (rTOF) will develop dilation of the right ventricle (RV) from chronic pulmonary insufficiency and require pulmonary valve replacement (PVR). Cardiac MRI (cMRI) is used to guide therapy but has limitations in studying novel intracardiac flow parameters. This pilot study aimed to demonstrate feasibility of reconstructing RV motion and simulating intracardiac flow in rTOF patients, exclusively using conventional cMRI and an immersed-boundary method computational fluid dynamic (CFD) solver. Methods Four rTOF patients and three normal controls underwent cMRI including 4D flow. 3D RV models were segmented from cMRI images. Feature-tracking software captured RV endocardial contours from cMRI long-axis and short-axis cine stacks. RV motion was reconstructed via diffeomorphic mapping (Deformetrica, deformetrica.org), serving as the domain boundary for CFD. Fully-resolved direct numerical simulations were performed over several cardiac cycles. Intracardiac vorticity, kinetic energy (KE) and turbulent kinetic energy (TKE) was measured. For validation, RV motion was compared to manual tracings, results of KE were compared between CFD and 4D flow. Results Diastolic vorticity and TKE in rTOF patients were 4.12¿±¿2.42 mJ/L and 115¿±¿27/s, compared to 2.96¿±¿2.16 mJ/L and 78¿±¿45/s in controls. There was good agreement between RV motion and manual tracings. The difference in diastolic KE between CFD and 4D flow by Bland-Altman analysis was - 0.89910 to 2 mJ/mL (95% limits of agreement: - 1.351¿×¿10-2 mJ/mL to 1.171¿×¿10-2 mJ/mL). Conclusion This CFD framework can produce intracardiac flow in rTOF patients. CFD has the potential for predicting the effects of PVR in rTOF patients and improve the clinical indications guided by cMRI.

Right ventricular outflow tract (RVOT) obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot, pulmonary atresia/stenosis and other conotruncal abnormalities etc. After surgical repair, these patients develop RVOT residual abnormalities of pulmonic stenosis and/or insufficiency of their native outflow tract or right ventricle to pulmonary artery conduit. There are also sequelae of other surgeries like the Ross operation for aortic valve disease that lead to right ventricle to pulmonary artery conduit dysfunction. Surgical pulmonic valve replacement (SPVR) has been the mainstay for these patients and is considered standard of care. Transcatheter pulmonic valve implantation (TPVI) was first reported in 2000 and has made strides as a comparable alternative to SPVR, being approved in the United States in 2010. We provide a comprehensive review in this space–indications for TPVI, detailed procedural facets and up-to-date review of the literature regarding outcomes of TPVI. TPVI has been shown to have favorable medium-term outcomes free of re-interventions especially after the adoption of the practice of pre-stenting the RVOT. Procedural mortality and complications are uncommon. With more experience, recognition of risk of dreaded outcomes like coronary compression has improved. Also, conduit rupture is increasingly being managed with transcatheter tools. Questions over endocarditis risk still prevail in the TPVI population. Head-to-head comparisons to SPVR are still limited but available data suggests equivalence. We also discuss newer valve technologies that have limited data currently and may have more applicability for treatment of native dysfunctional RVOT substrates.

Long-term results after tetralogy of Fallot (TOF) repair are determined by the extent of right ventricular remodeling to chronic pulmonary regurgitation entailing progressive RV dysfunction and a risk of developing ventricular arrhythmia. Pulmonary valve replacement (PVR) can alleviate this burden. As a predictor of ventricular arrhythmia, QRS duration remains a strong parameter in this decision. We performed a retrospective analysis of all PVR patients between 2005 and 2018, studying the time evolution of electrocardiographic parameters before and after PVR through linear mixed model analysis. 42 TOF patients underwent PVR. The median timespan between primary repair and PVR was 18 years (IQR 13-30). The indication for PVR was primarily based on the association of exercise intolerance (67%) and significant RV dilation on cMRI (median RVEDVi 161 ml/m2 IQR 133-181). Median QRS length was 155 ms (IQR 138-164), 4 (10%) patients had a QRS > 180 ms. QRS duration increased significantly before PVR, but barely showed regression after PVR. Changes of QRS duration after PVR were independent of RV dilation. In conclusion, when the decision for PVR in TOF patients is primarily based on RV volume and/or function threshold, QRS duration > 180 ms is rarely observed. In contrast with the significant increase of QRS duration before PVR, QRS length regression appears to be independent of the extent of RV dilation or QRS > 160 ms. Considering that the decision for PVR is based on mechanical RV characteristics, the utility of serial follow-up of QRS duration in contemporary operated TOF patients becomes questionable in absence of clinical arguments for ventricular arrhythmia.

Background Pregnant women with repaired tetralogy of Fallot (rTOF) are at increased risk of adverse outcomes. Although pre-pregnancy pulmonary valve replacement (PVR) may be considered in some women to attenuate risk, published data to support this practice are lacking. Our objective was to explore the impact of pre-pregnancy PVR on pregnancy outcomes in rTOF. Methods Women with rTOF and cardiovascular magnetic resonance imaging (CMR) before and after pregnancy were included if CMR studies were completed within 3 years of pregnancy. Subjects were compared according to presence (+) or absence (−) of PVR at pre-pregnancy CMR. Pregnancy outcomes (cardiovascular, obstetric, and fetal/neonatal) were documented. Results Of the 29 study women identified, 7 were PVR+ and 22 were PVR-. Post-pregnancy, the PVR- group demonstrated interval increase in indexed right ventricular end-diastolic volumes (RVEDVi) (157 ± 28 versus 166 ± 33 ml/m2, p = 0.003) and end-systolic volumes (RVESVi) (82 ± 17 versus 89 ± 20 ml/m2, p = 0.003) as compared with pre-pregnancy, but no significant change in RV ejection fraction, RV mass, or left ventricular measurements. In the PVR+ group, there were no interval changes in RV measurements pre-versus post pregnancy. Interval rate of change in RVESVi of PVR- exceeded PVR+ women (+3.7 ± 5.0 versus −2.2 ± 5.0 ml/m2/year, p = 0.03). Pregnancy outcomes did not differ in PVR+ versus PVR- women. Conclusions Pregnancy outcomes did not differ according to PVR status in our cohort. While RV volumes remained unchanged in PVR+ women, interval RV dilation was observed in PVR- women. Additional study of a larger population with longer follow-up may further inform clinical practice regarding pre-pregnancy PVR.

Background/purpose: Prognostic factors remain unclear in patients undergoing transcatheter implantation of Venus P-valve for their severe pulmonary regurgitation associated with native right ventricular (RV) outflow tract. Method: Between January 2017 and October 2018, we prospectively collected data of patient characteristics, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) before and 6–12 months after valve graft implantation. Results: Fifteen patients (male: 8, median age: 24.8 years) were enrolled. The procedure success rate was 100%. The median follow-up was 16.3 months without any dysfunction of the valve graft. The cohort demonstrated a significant improvement in cardiac index (from 3.3 to 3.9 L/min/m2) and increase of percentage of New York Heart Association functional class I (P

OBJECTIVES Pulmonary valve replacement (PVR) performed for pulmonary valve regurgitation is the most common indication for reoperation during mid-to-long-term follow-up after tetralogy of Fallot repair. An aneurysmal dilation of the infundibulum is often associated secondary to the infundibulotomy performed in the first operation. The right ventricular outflow tract reconstruction with endo-exclusion aims to exclude the non-contractile segments of the dilated right ventricular. This study intends to assess the safety and efficiency of the endo-exclusion technique. METHODS Between January 2010 and December 2018, 86 patients underwent a PVR with (n = 46) or without (n = 40) endo-exclusion. The current study compares the outcomes in terms of survival, reintervention, structural valve deterioration, right ventricular function (volume and right ventricular ejection fraction) and pulmonary valve gradient. The median follow-up time was 4.45 years (1.9 months to 9.87 years). RESULTS There was no 30-day mortality. There was no difference in the freedom from reintervention at 7 years (without endo-exclusion, 97%, versus with endo-exclusion, 94%, log-rank = 0.68) or in the freedom from structural pulmonary valve deterioration at 7 years (without endo-exclusion, 94%, versus with endo-exclusion, 89%, log-rank = 0.94). No significant difference was observed in the indexed right ventricular end-diastolic volume (102.2 ± 34 ml/m2 in the PVR without endo-exclusion group and 93.3 ± 22 ml/m2 in the PVR with endo-exclusion group, P = 0.61). No significant difference was observed in the right ventricular function (right ventricular ejection fraction: 46 ± 11% in the PVR without endo-exclusion group and 46 ± 9% in the PVR with endo-exclusion group, P = 0.88). CONCLUSIONS PVR with or without endo-exclusion is a safe and effective procedure. PVR with endo-exclusion allows implantation without structural deformation of the valve and therefore excellent short- and medium-term results.

OBJECTIVES The present study was conducted to investigate the decrease in left ventricular stroke volume index (LVSVI) that is caused by pulmonary regurgitation-induced right heart dysfunction and its clinical implications before and after pulmonary valve replacement (PVR). METHODS Between January 2010 and December 2019, 30 adults who underwent surgical PVR for chronic pulmonary regurgitation with right ventricular dilation late after tetralogy of Fallot (TOF) repair were included. All patients were evaluated using cardiac magnetic resonance before PVR. The median interval from TOF repair to PVR was 29 [25th, 75th percentile: 25, 37] years. The median pulmonary regurgitation fraction and right ventricular end-diastolic volume index were 56 [48, 66] % and 203 [187, 239] ml/m2. Twenty-three patients (76.7%) were re-evaluated 1 year after PVR. RESULTS Before PVR, the median LVSVI was 40 [35, 46] ml/beat/m2. A lower LVSVI was associated with a longer interval from TOF repair to PVR (r = −0.40, P = 0.029) and a lower right ventricular ejection fraction (r = 0.52, P = 0.004). A lower LVSVI was not associated with a higher right ventricular end-diastolic volume index. LVSVI remained unchanged after PVR. The patients were subdivided into Normal-stroke volume index (SVI) and Subnormal-SVI groups using the preoperative LVSVI cut-off value of 35 mL/beat/m2. Compared with the Normal-SVI group, the Subnormal-SVI group had a higher incidence of ablation therapy before PVR (4.7 vs 2.3 patient-years, P = 0.044). After PVR, LVSVI in the Subnormal-SVI group was still lower (40 [34, 42] vs 44 [42, 47] ml/beat/m2, P = 0.038) despite the right ventricular end-diastolic volume index normalization. There was no difference in the clinical event incidence between the 2 groups during the follow-up period. Brain natriuretic peptide level in the Subnormal-SVI group was higher within 3 years after PVR (P = 0.046). CONCLUSIONS Reduced left ventricular stroke volume did not fully recover after PVR. PVR for patients with repaired TOF should be performed before the left ventricular stroke volume begins to decrease.

OBJECTIVES The aim of this study was to assess the impact of individual operator experience on outcomes after complete repair for tetralogy of Fallot. METHODS This is a retrospective cohort study. Consecutive patients who underwent TOF repair at a single institution were included and compared according to whether the primary operator was an experienced, high-volume operator (defined as an operator who performed at least 20 surgical procedures for congenital heart disease defined as complex by the Risk Adjustment for Congenital Heart Surgery classification per year for at least 3 consecutive years). The primary outcome was defined as a composite of death, or reintervention, or significant annular peak gradient, or significant pulmonary regurgitation. Multivariable logistic regression and Cox proportional-hazards model analyses were used to assess the relationships between operator experience and outcomes. RESULTS From January 2012 to December 2017, a total of 1760 patients with primary diagnosis of TOF underwent TOF repair by 37 operators. Of these, 5 operators (13.5%) were considered experienced, and 32 (86.5%) were considered less experienced. Complete follow-up data were available for 1728 complete repair for TOF patients with a median follow-up duration of 49 months; in 611 patients (35.4%), the surgery was performed by experienced operators, and in 1117 patients (64.6%), the surgery was performed by less experienced operators. Adjusted risks for the primary outcome and significant pulmonary regurgitation were lower for patients who were treated by experienced operators, both at discharge [adjusted odds ratio 0.67, 95% confidence interval (CI) 0.50–0.90; adjusted odds ratio 0.54, 95% CI 0.37–0.78, respectively] and at follow-up (adjusted hazard ratio 0.82, 95% CI 0.68–0.97; adjusted hazard ratio 0.70, 95% CI 0.56–0.87, respectively). The trend for the primary outcome during follow-up remained unchanged, even in most subgroups. CONCLUSIONS Increased surgeon experience is associated with improved risk-adjusted outcomes. These results have potentially important implications for individual training, quality improvement and hospital programmes in the context of complete repair for TOF. Registration Number http://www.chictr.org.cn number, ChiCTR2000033234.

Exercise capacity is a modifiable factor in patients with CHD that has been related to surgical outcomes in adults. We hypothesised that this was true for children undergoing surgical pulmonary valve replacement; therefore, the relationship of preoperative percent predicted peak oxygen consumption to surgical outcomes as measured by total hospital length of stay was explored.Methods:Single centre retrospective cohort study of patients aged 8–18 years who underwent surgical pulmonary valve replacement. The primary predictor was preoperative percent predicted peak oxygen consumption, and primary outcome was total hospital length of stay. Clinical, imaging, and cardiopulmonary exercise test data were reviewed and compared to total hospital length of stay. Cox proportional hazards regression was used to examine the association between total hospital length of stay and percent predicted peak oxygen consumption.Results:Three-hundred and seventy patients undergoing pulmonary valve replacement/conduit change between 2003 and 2017 at Boston Children’s Hospital were identified. Ninety had preoperative cardiopulmonary exercise tests within 6 months of surgery. Exclusion for inadequate exercise data (n = 3) and imaging data (n = 1) left 86 patients for review. Patients with percent predicted peak oxygen consumption ≥ 70% (n = 46, 53%) had shorter total hospital length of stay (4.4 days) than the 40 with percent predicted peak oxygen consumption Conclusion:Children undergoing surgical pulmonary valve replacement with better preoperative exercise capacity had shorter total hospital length of stay. Exercise capacity is a potentially modifiable factor prior to and after pulmonary valve replacement. Until more patients systematically undergo cardiopulmonary exercise tests, the full impact of optimisation of exercise capacity will not be known.

Background Following the repair of TOF patients may be left with pulmonary regurgitation and a dilated right ventricle (RV), which in turn can lead to ventricular arrhythmias and sudden death. A prolonged QRS is a predictor of ventricular arrhythmias. However, whether subsequent pulmonary valve replacement (PVR) can reverse QRS‐prolongation is controversial. We hypothesized that changes in QRS duration following PVR are determined by preoperative QRS‐duration and RV volumes Methods A retrospective single‐center cohort study was conducted on 142 post‐TOF repair patients (mean age 25 ± 13 years) who underwent PVR between 1995 and 2019. Information on QRS duration and RV volumes measured by cardiac MRI (available in 83 patients) were collected. A linear mixed model was used to investigate the association between the preoperative QRS duration and RV volumes and the postoperative QRS duration. Results The QRS‐duration following PVR continued to increase in all subjects with a prolonged preoperative QRS‐duration(>160 ms, rate of increase of 0.87 msec ± 0.33 per year; p = .01), markedly raised RV end‐diastolic volume (RVEDV; ≥166 ml/m2, rate of increase of 2.0 msec ± 0.37 per year; p

Background Multiple congenital cardiac malformations require pulmonary valve replacement and/or right ventricular outflow (RVOT) reconstruction. Pulmonary valve replacement remains challenging in children owing to the limited growth potential of prosthetic valves. We evaluated outcomes in patients undergoing surgical implantation of a Melody valve in the RVOT. Methods Data were retrospectively collected for 23 patients undergoing surgical Melody valve implantation at Boston Children's Hospital between 2009 and 2019. We assessed postoperative valve function, reintervention rates, and mortality. Results Median age was 1.7 years (range, 2 months to 6 years); 12 patients were aged greater than 2 years (52%). Diagnosis was tetralogy of Fallot in 15 patients (65%); 15 had a prior RVOT operation (65%). The Melody valve was dilated before surgery to a median diameter of 14 mm (range, 10-20 mm). No patients had acute pulmonary regurgitation. One required transcatheter RVOT reintervention before discharge. Median follow-up was 3.7 years (range, 0.02-8.7 years) with moderate or greater pulmonary regurgitation in 2 patients. Catheter-based interventions (mean, 0.83 ± 1.07/patient) occurred at a median of 1 year (range, 16 days to 5.4 years) and included valve expansion for somatic growth (n = 10) and subsequent valve-in-valve replacement (n = 3). Three patients (13%) required surgical valve explant or replacement at a median of 1.0 year (range, 0.6-3.7 years) for Melody-specific indications. One-, 3-, and 5-year freedom from Melody-driven reoperation was 90%, 90%, and 83%, respectively. Conclusions The Melody valve can be surgically implanted in the RVOT of young patients with acceptable early results. These valves can be successfully dilated through transcatheter reintervention to accommodate growth.

右心室流出道功能障碍是成人先天性心脏病术后常见的血流动力学后遗症，因右心室流出道功能不全而再次手术较为常见。虽然外科手术的风险较低，但其创伤大、恢复慢、患者心理负担重，且未来再次手术的风险会随着手术次数增多而逐渐增加。对于这一类患者，经导管肺动脉瓣置换术是一种新的选择。随着技术的不断发展，新的经导管肺动脉瓣膜使得应用经导管瓣膜置换技术治疗这类患者成为可能。目前，右心室-肺动脉管道及生物肺动脉瓣的经导管肺动脉瓣置换术已经积累了较为丰富的经验，远期随访也得到了较好的血流动力学参数。然而，目前的技术不能完全解决具有复杂形态的自体右心室流出道功能障碍。该文描述了自体右心室流出道的复杂解剖形态、目前可用的经导管肺动脉瓣膜系统及未来发展中存在的问题。.

Background Until recently, a large right ventricle outflow tract interfered with the feasibility of standard transcatheter pulmonary valve replacement (PVR). We are describing our experience using a hybrid approach for PVR using a left anterior thoracotomy approach to allow for plication of the main pulmonary artery followed by a transcatheter PVR using a Sapien S3 valve. Methods and Results This is a single‐center, retrospective review of patients who were evaluated to be appropriate for a hybrid PVR approach. The patients' demographics, procedure details, and follow‐up data were collected. Between May 2018 and April 2021, a total of 11 patients presented for hybrid transcatheter PVR. The median age and weight were 24 years (interquartile range, 19–43 years) and 81.8 kg (interquartile range, 69–91 kg), respectively. Nine out of 11 patients received a transcatheter PVR after main pulmonary artery plication. There were no procedurally related deaths. One major complication was encountered in which the valve was malpositioned requiring successful surgical PVR. Minor complications included acute kidney injury (n=1) and a broken rib (n=1). The median length of stay was 4 days (interquartile range, 2–4 days), with median follow‐up of 7 months (interquartile range, 3–18 months). A well‐functioning pulmonary valve was observed in all patients at the last follow‐up. Conclusions A hybrid approach using left anterior thoracotomy with pulmonary artery plication followed by transcatheter Sapien S3 PVR provides a less‐invasive option for patients with an enlarged right ventricular outflow tract. Preliminary results demonstrated this to be a safe option with good short‐term outcomes.

Early surgical tetralogy of Fallot (ToF) repair involved patching across the pulmonic annulus (transannular patch [TAP] repair), which resulted in severe pulmonic regurgitation. Long-term outcome improvements were anticipated with modifications that preserved the pulmonic annulus (annulus-preserving [AP] repair). The objective of the present study was to evaluate the need for late reintervention in adults with AP repair and those with TAP repair.We conducted a retrospective review of adults (born 1981-1996) with childhood intracardiac ToF repairs at a tertiary care center. The primary cardiovascular outcome was need for reintervention after primary intracardiac repair of ToF. Secondary outcomes included a composite of death, heart failure, and ventricular arrhythmias.Two hundred thirty adults were included: 104 with AP repair and 126 with TAP repair. The median age at last follow up was 25 years (interquartile range [IQR] 20-28) and the median follow-up duration was 7.9 years (IQR 3.5-12). Reintervention of any type was significantly more common in the TAP group during both childhood and adulthood (72.2% TAP vs 20.2% AP, HR 5.5, 95% CI 3.4-9.0; P0.001). Pulmonary valve replacement (PVR) was almost 6 times more likely in adults with TAP repair (65.1% TAP vs 16.3% AP, HR 5.7, 95% CI 3.4-9.7; P0.001).Patients who had AP ToF repair had significantly fewer late reinterventions compared with TAP repair, with the majority of reinterventions due to PVR. More long-term follow-up is required.

Conduit dilatation above 110% and TPVI in conduits16 mm is not recommended. However, if we want to reach normal values for RVOT diameters and diminish reintervention rates, pushing these boundaries is essential.Analysis of subsequent patients who underwent TPVI with Edwards Sapien valves in conduits ≤16 mm between 2010 and 2020.In n = 33 cases median age was 13 years (5-20 y) and median weight 47 kg (15-91 kg). Preexisting RVOT grafts were n = 28 Contegra® conduits and n = 5 homografts (12 mm n = 15; 14 mm n = 11; 16 mm n = 7). Implanted were the Sapien (n = 8), Sapien XT (n = 10) and Sapien 3 valve (n = 15) with 20 mm (n = 4), 23 mm (n = 19), 26 mm (n = 9) and 29 mm (n = 1). Mean minimal RVOT diameter after TPVI was 22,7 ± 2,3 mm (18-30 mm) which is 150% of the mean minimal RVOT diameter before TPVI (15,1 ± 4,3 mm). Covered stents were used in n = 10 cases. Contained conduit rupture occurred in n = 7 cases (21%). Residual RVOT gradients of 5,7 ± 4,9 mmHg (0-18 mmHg) showed adequate RV unloading.TPVI could be performed successfully in all patients. Dilatation above 150% and a valve/conduit diameter ratio up to 2,4 were well tolerated. There was a considerable amount of conduit rupture but all were confined without further need for intervention or surgery.

Aims To investigate pulmonary valve (PV) and right ventricular function by echocardiography in paediatric patients with repaired Tetralogy of Fallot (ToF), comparing PV preservation surgical strategies to standard transannular patch (TAP) repair. Methods and results All patients undergoing transatrial-transpulmonary repair for ToF at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 groups, according to the different techniques used (PV preservation strategy vs TAP repair). All patients underwent standard echo-Doppler study including RV areas, fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE); Pulmonary regurgitation (PR) was assessed by Color Doppler, continuous-wave (CW) Doppler, pressure half time (PHT) and PR index. By speckle tracking we measured also, in a subgroup of patients, right atrial strain (RAS), RV and left ventricle (LV) global longitudinal strain (RVGLS, LVGLS) and their time to peak (TTP) values. Eighty-two patients underwent a PV preservation strategy while 34 underwent a standard TAP repair. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p = 0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at Follow-up (HR: 1.85, 95% CI: 1.09, 3.15; p = 0.02). At a mean follow-up of 6.9 ± 0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9 ± 0.8% vs 42.5 ± 1.7%, P Conclusion Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible.

Pathophysiological differences in relief of pulmonary stenosis (PS) as opposed to pulmonary regurgitation (PR) by percutaneous pulmonary valve implantation (PPVI) remain elusive, but might impact current assessment of procedural success and ultimately indications.Invasive pressure measurements, cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed before pre-stenting (BMS), after BMS and after PPVI in patients with either PS or PR.In PS (n = 14), BMS reduced the right ventricular (RV) to systemic pressure ratio (0.8 ± 0.2 vs. 0.4 ± 0.1%; p .01), improved RF EF (53 ± 14 vs. 59 ± 12%; p = .01) but introduced free PR (PR fraction post 39 ± 12%; p .01) with no changes in effective RV stroke volume (SV). PPVI eliminated PR (PR fraction 5 ± 3%; p .01) and improved effective RV SV (p .01) with no changes in RV EF (p = .47). Peak VO2 improved significantly after BMS, with no changes following PPVI (26 ± 9 vs. 30 ± 11 vs. 31 ± 10 ml/kg*min). In PR (n = 14), BMS exaggerated PR (PR fraction post 47 ± 10) with reduction in effective RV SV (pre 43 ± 9 vs. post 38 ± 8 ml/mExercise capacity in patients with right ventricular outflow tract dysfunction is primarily afterload-dependent.

Background and objectivesChronic pulmonic regurgitation (PR) following repair of congenital heart disease (CHD) impairs right ventricular function that impacts peak exercise cardiac index (pCI). We aimed to estimate in a non-invasive way pCI and peak oxygen consumption (pVO2) and to evaluate predictors of low pCI in patients with significant residual pulmonic regurgitation after CHD repair.MethodWe included 82 patients (median age 19 years (range 10–54 years)) with residual pulmonic regurgitation fraction >40%. All underwent cardiac MRI and cardiopulmonary testing with measurement of pCI by thoracic impedancemetry. Low pCI was defined 2.ResultsLow pCI was found in 18/82 patients. Peak indexed stroke volume (pSVi) tended to compensate chronotropic insufficiency only in patients with normal pCI (r=−0.31, p=0.01). Below 20 years of age, only 5/45 patients had low pCI but near-normal (≥6.5 L/min/m2). pVO2 (mL/kg/min) was correlated with pCI (r=0.58, p=0.0002) only in patients aged >20 years. Left ventricular stroke volume in MRI correlated with pSVi only in the group of patients with low pCI (r=0.54, p=0.02). No MRI measurements predicted low pCI. In multivariable analysis, only age predicted a low pCI (OR=1.082, 95% CI 1.035 to 1.131, p=0.001) with continuous increase of risk with age.ConclusionsIn patients with severe PR, pVO2 is a partial reflection of pCI. Risk of low pCI increases with age. No resting MRI measurement predicts low haemodynamic response to exercise. Probably more suitable to detect ventricular dysfunction, pCI measurement could be an additional parameter to take into account when considering pulmonic valve replacement.

OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

Background Transannular patch, which results in pulmonary insufficiency (PI), is usually required during repair of tetralogy of fallot (TOF). In this study, we compared three types of pulmonary valve reconstruction techniques during transannular repair of TOF. Methods Between February 2014 and January 2018, 50 patients with TOF underwent primary repair with transannular patch. These patients were divided into three groups. In Group 1, (n = 15), a single gluteraldehyde-treated autologous pericardial patch (standard method) was reconstructed as monocusp. In Group 2, (n = 16) Nunn's bileaflet pulmonary valve reconstruction technique was used with pericardial patch. In Group 3, (n = 19), Nunn's bileaflet technique was performed with expanded polytetrafluoroethylene membrane. The outcomes of the patients and early and midterm competency of the pulmonary valves were analyzed. Results These techniques were significantly effective in early postoperative period. Freedom from moderate to severe PI were 73.3%; 100% and 89.4%, respectively. Mortality, duration of intensive care unit and hospital stay were similar between the groups. The mean follow-up period was 17.5 ± 13.0 (3-57) months. Freedom from moderate to severe PI decreased to 40%; 81.2% and 73.7%, respectively at the end of the follow-up period. Presence of moderate to severe PI was significantly higher in Group 1 (p = .018 between Groups 1 and 2, p = .048 between Groups 1 and 3). Conclusion All three pulmonary valve reconstruction techniques provided competent pulmonary valves. Nunn's bileaflet technique had better outcome at midterm. It has a potential to delay right ventricular dysfunction at long-term.

Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to vasoactive substances secreted by the tumor, of which serotonin is most prominent in the pathophysiology of CHD. Serotonin stimulates fibroblast growth and fibrogenesis, which can lead to cardiac valvular fibrosis. CHD primarily affects right heart valves, causing tricuspid and pulmonic regurgitation and less frequently stenosis of these valves. Left heart valves are usually spared because vasoactive substances such as serotonin are enzymatically inactivated in the lung vasculature. The pathology of CHD is characterized by plaque-like deposition of fibrous tissue on valvular cusps, leaflets, papillary muscles, chordae, and ventricular walls. Symptomatic CHD usually presents between 50 and 70 years of age, initially as dyspnea and fatigue. Echocardiography is the mainstay of imaging and demonstrates thickened right heart valves with limited mobility and regurgitation. Treatment focuses on control of the underlying carcinoid syndrome, targeting subsequent valvular heart disease and managing consequent heart failure. Surgical valve replacement and catheter-directed valve procedures may be effective for selected patients with CHD.

INTRODUCTION Various valve repair techniques have been described for prevention of pulmonary insufficiency (PI) during right ventricular outflow tract (RVOT) reconstruction with transannular patch. Herein, we present the early results of an alternative technique of neopulmonary valve reconstruction using right atrial appendage (RAA) tissue. METHODS Between October 2019 and December 2019, 12 patients with tetralogy of fallot (TOF) (n = 10), TOF-absent pulmonary valve (n = 1) and intact ventricular septum-pulmonary atresia (n = 1) underwent RVOT reconstruction with neopulmonary valve that was created using RAA tissue. Median age and weight of patients were 9.9 months (5 months-14 years) and 9.8 kg (6.2-47), respectively. RESULTS No mortality or major events developed. Postoperative echocardiography revealed none/trace PI in 11 patients and mild PI in 1 patient. Median right ventricle/left ventricle ratio was 0.46 (0.35-0.65) and RVOT gradient was 20 mm Hg (0-30). Median cardiopulmonary bypass and aortic clamping times were 121 minutes (81-178) and 94 minutes (59-138), respectively. Operative times revealed statistically significant learning curve pattern in terms of cardiopulmonary bypass (r2 = .568; P = .005) and aortic clamping times (r2 = .635; P = .002). Median ventilation time, intensive care unit stay, and the length of hospital stay were 6 hours (2-48), 1 days (1-5), and 7 days (4-10), respectively. Longer perfusion time was not correlated with postoperative times. At median 6 months, echocardiography showed none/trivial PI in 11 patients and mild PI in one patient. CONCLUSION Early results showed that neopulmonary valve reconstruction using the RAA tissue may provide a reasonable alternative for RVOT reconstruction with transannular patch. But long-term results are needed.

OBJECTIVE This study sought to investigate the variation of right heart structure pre- and post-operation as risk factors for moderate to severe pulmonary regurgitation (PR) after repaired Tetralogy of Fallot and the best "cutoff" values for the transannular patch (TAP). METHODS We collected surgical, echocardiographic, and computed tomographic data of Teralogy of Fallot (TOF) patients over two years and calculated z-score values based on the echocardiographic data. Based on the PR level after follow-up, the patients were divided into two groups, trivial to mild PR and moderate to severe PR. A multivariate logistic regression analysis was performed, and the receiver operating characteristic curve analysis was used to find the best "cutoff" value for risk factors. RESULTS A total of 104 TOF patients were included in our cohort study. From the multivariate analysis, correction strategy (P = .002), difference in zRVOT (OR 1.974, 95% CI 1.354 to 2.878, P

Background A percutaneous approach for pulmonary valve replacement (PVR) is a feasible alternative to surgical PVR in selected patients with severe pulmonary regurgitation after repair of tetralogy of Fallot. However, large right ventricular outflow tract (diameter ≥ 25 mm) remains challenging. Methods This retrospective multicenter study enrolled consecutive patients with large right ventricular outflow tract who underwent percutaneous PVR (Venus P-valve, Venus MedTech Inc, Hangzhou, China) (n = 35) or surgical PVR (homograft valve; n = 30) between May 2014 and April 2017. Patients were followed up at 1, 3, 6, and 12 months, and yearly thereafter. Main study outcomes were pulmonary valve function and right ventricular function at discharge and midterm follow-up. Results PVR was successful in all patients. Percutaneous compared with surgical PVR group had: similarly distributed baseline characteristics; shorter hospitalization, intensive care unit stay, and endotracheal intubation duration; lower cost; lower pulmonary valve gradient before discharge; lower pulmonary valve regurgitant grade (mean difference, –0.63; 95% CI –1.11 to –0.20, P = .022), pulmonary valve gradient (mean difference, –5.7 mm Hg; 95% CI –9.4 to –2.2 mm Hg, P = .005), and right ventricular end-diastolic volume index (mean difference, –9.5 mL/m2; 95% CI –16.9 to –3.1 mL/m2, P = .022); and greater right ventricular ejection fraction (mean difference, 5.4%; 95% CI 2.4%-8.3%, P = .002) at median 36 months follow-up, without deaths in either group. Conclusions Percutaneous PVR using Venus P-valve appeared to be a safe, efficacious and minimally invasive alternative to surgical PVR in selected patients with large right ventricular outflow tract yielding better right ventricular and pulmonary valve function at midterm follow-up.

Tetralogy of Fallot is the most common cyanotic congenital disease, affecting 10% of children with congenital heart disease. The surgical management of patients with Tetralogy of Fallot leads, however, to significant detrimental effects on the right ventricle including pulmonary valve regurgitation. This experiment aimed to simulate different cases of pulmonary valve regurgitation with varying degrees of severity in order to observe the changes in flow structures present in the right ventricle. Planar time-resolved particle image velocimetry measurements have been performed on a custom-made double activation simulator reproducing flow conditions in a model of a right ventricle. Changes in flow characteristics in the right ventricle have been evaluated in terms of velocity fields and profiles, tricuspid inflow jet orientation and viscous energy dissipation. Our results show that pulmonary valve regurgitation significantly alters the flow in the right ventricle mostly by impairing the diastolic inflow through the tricuspid valve and by increasing viscous energy loss. This fundamental work should allow for a better understanding of such changes in the RV flow dynamics. It may also help in developing new strategies allowing for a better follow-up of patients with repaired TOF and for decision-making in terms of pulmonary valve replacement.

OBJECTIVES We aimed to investigate the safety, feasibility and early and mid-term outcomes of total repair of elective tetralogy of Fallot through a right subaxillary thoracotomy. METHODS Between May 2010 and September 2015, a retrospective review of patients with elective tetralogy of Fallot undergoing primary repair through a right subaxillary thoracotomy was performed. This study included 47 patients [27 males, 20 females; the mean age of 1.7 ± 1.5 years (ranging from 3 to 106 months); body weight of 9.7 ± 2.8 kg (ranging from 5 to 16 kg); McGoon ratio ≥1.5]. The ventricular septal defects were approached through a right atriotomy in 30 patients, a right ventriculotomy in 8 patients and the combination of a right atriotomy and ventriculotomy in 9 patients. Transannular and non-transannular patches were used in 20 and 27 patients, respectively. RESULTS There was 1 operative death (2.1%), and the others recovered uneventfully. The median cardiopulmonary bypass and aortic cross-clamping times was 79 ± 20.8 (45–136) and 50 ± 15.7 (25–94) min, respectively. The mechanical ventilation time, length of intensive care unit and postoperative hospital stay were 0.9 ± 0.6, 2.6 ± 1.1 and 10.7 ± 2.7 days, respectively. The main complications involved low cardiac output syndrome in 5 patients, arrhythmia in 6 patients, pulmonary infection in 4 patients, small residual shunt in 2 patients, mild residual right ventricular outflow tract obstruction in 2 patients, mild tricuspid regurgitation in 3 patients and atelectasis in 1 patient. The mean follow-up time was 82 ± 22.0 months, and no late deaths occurred during the follow-up. One (2.1%) patient received reintervention for residual significant pulmonary artery stenosis. That was in addition to 2 (4.3%) patients having mild residual right ventricular outflow tract, 1 (2.1%) patient with moderate tricuspid regurgitation and 3 (6.4%) patients with moderate pulmonary valve regurgitation to be followed up. CONCLUSIONS The right subaxillary thoracotomy, providing a safe and feasible alternative, can be performed with a low mortality rate and satisfactory early and mid-term outcomes for total repair of elective tetralogy of Fallot.