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Background Relationships between right ventricular (RV) and left ventricular (LV) shape and function may be useful in determining optimal timing for pulmonary valve replacement in patients with repaired tetralogy of Fallot (rTOF). However, these are multivariate and difficult to quantify. We aimed to quantify variations in biventricular shape associated with pulmonary regurgitant volume (PRV) in rTOF using a biventricular atlas. Methods In this cross-sectional retrospective study, a biventricular shape model was customized to cardiovascular magnetic resonance (CMR) images from 88 rTOF patients (median age 16, inter-quartile range 11.8–24.3 years). Morphometric scores quantifying biventricular shape at end-diastole and end-systole were computed using principal component analysis. Multivariate linear regression was used to quantify biventricular shape associations with PRV, corrected for age, sex, height, and weight. Regional associations were confirmed by univariate correlations with distances and angles computed from the models, as well as global systolic strains computed from changes in arc length from end-diastole to end-systole. Results PRV was significantly associated with 5 biventricular morphometric scores, independent of covariates, and accounted for 12.3% of total shape variation (p Conclusion A biventricular atlas of rTOF patients quantified multivariate relationships between left–right ventricular morphometry and wall motion with pulmonary regurgitation. Regional RV dilation, LV reduction, LV septal-lateral flattening and increased RV strain were all associated with increased pulmonary regurgitant volume. Morphometric scores provide simple metrics linking mechanisms for structural and functional alteration with important clinical indices.

Right ventricular (RV) resynchronization therapy (CRT) with stimulation electrode implantation in the latest activation area guided by a high-density electroanatomic activation map was successfully performed in a patient with repaired tetralogy of Fallot (rToF), severe pulmonary regurgitation (PR), and severe dysfunction and dilatation of the right ventricle. An improvement in his clinical condition and intraventricular synchrony was achieved. There is a mechanical-electrical interaction in the right ventricle of patients with rToF; therefore, RV CRT in selected cases may be beneficial. Electroanatomic activation mapping can help achieve maximum clinical benefit by identifying the optimal stimulation site.

Background Data about the right ventricular (RV) mechanics adaptation to volume overload in patients with repaired tetralogy of Fallot (rToF) are limited. Accordingly, we sought to assess the mechanics of the functional remodeling occurring in the RV of rToF with severe pulmonary regurgitation. Methods We used three-dimensional transthoracic echocardiography (3DTE) to obtain RV data sets from 33 rToF patients and 30 age- and sex- matched controls. A 3D mesh model of the RV was generated, and RV global and regional longitudinal (LS) and circumferential (CS) strain components, and the relative contribution of longitudinal (LEF), radial (REF) and anteroposterior (AEF) wall motion to global RV ejection fraction (RVEF) were computed using the ReVISION method. Results Corresponding to decreased global RVEF (45 ± 6% vs 55 ± 5%, p 0.05) and AEF (0.38 ± 0.09 vs 0.39 ± 0.04, p > 0.05) to global RVEF was similar to controls. Accordingly, rToF patients showed lower 3D RV global LS (-16.94 ± 2.9 vs -23.22 ± 2.9, p 0.05). Conclusions 3DTE allows a quantitative evaluation of the mechanics of global RVEF. In rToF with chronic volume overload, the relative contribution of the longitudinal shortening to global RVEF is affected more than either the radial or the anteroposterior components.

La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente. Luego de más de siete décadas desde la primera cirugía paliativa, el pronóstico de esta enfermedad ha cambiado radicalmente. Con la mayor sobrevida de pacientes con tetralogía de Fallot hacia la adultez, el cardiólogo clínico enfrenta retos en el manejo de esta población, desde la regurgitación pulmonar severa hasta la falla cardíaca y arritmias ventriculares. En esta revisión se describirán los aspectos más relevantes del cuidado de pacientes adultos con esta enfermedad.

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

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.

Background : Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI. Methods : Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed. Results : Seven patients aged 17–60 years (median 26 years) had stenosed RV-PA conduits implanted 5–17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50–137 mmHg) to 12.7 ± 6.4 mmHg (5–20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10–22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events. Conclusions : Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Objectives The aim of this study was to demonstrate the safety and functionality of the Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (THV) in patients with dysfunctional, dilated right ventricular outflow tract (RVOT) greater or equal to moderate pulmonary regurgitation (PR). Background Significant variations in the size and morphology of the RVOT affect the placement of transcatheter pulmonary valves. The Alterra Prestent internally reduces and reconfigures the RVOT, providing a stable landing zone for the 29-mm SAPIEN 3 THV. Methods Eligible patients had moderate or greater PR, weighed >20 kg, and had RVOT diameter 27 to 38 mm and length >35 mm. The primary endpoint was device success, a 5-item composite: 1 Alterra Prestent deployed in the desired location, 1 SAPIEN 3 THV implanted in the desired location within the Prestent, right ventricular–to–pulmonary artery peak-to-peak gradient Results Enrolled patients (N = 15) had a median age and weight of 20 years and 61.7 kg, respectively; 93.3% were in New York Heart Association functional class I or II. Device success was 100%. No staged procedures were necessary. No THV dysfunction was reported to 6 months. No serious safety signals were reported. Conclusions This early feasibility study demonstrated the safety and functionality of the Alterra Adaptive Prestent in patients with congenital RVOT dysfunction and moderate or greater PR. Durability and long-term outcome data are needed.

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.

Graphical abstract, Highlights • Pulmonary blood supply is the main determinant of physiology in tetralogy of Fallot. • This case exhibits a highly abnormal pulmonary valve and blood flow pattern. • Circular shunting may spare fetal health yet lead to neonatal hypoxemia and shock. • Surgical intervention stabilizes pulmonary blood supply in this unusual physiology. • Comparison is made with known subtypes of tetralogy of Fallot.

Background: Long-term sequelae such as right ventricular dysfunction and reduced hemodynamic reserve are the main determinants of cardiovascular outcomes after repair of tetralogy of Fallot (TOF). Echocardiographic parameters at rest offer only partial information on impaired hemodynamics in these patients, and data during stress testing are lacking. The leg-positive pressure (LPP) maneuver has recently been reported to be able to apply acute preload stress. The aim of this study was to test the hypothesis that preload reserve is impaired and ventricular interaction is exacerbated in patients with TOF. Methods: In this prospective cross-sectional study, we recruited 44 consecutive patients with TOF and 30 normal control subjects. Echocardiography was performed both at rest and during LPP stress, and preload reserve was defined as the change between baseline stroke volume (SV) and that obtained during LPP stress. The eccentricity index was calculated as the ratio of the left ventricular anteroposterior to septal-lateral dimensions to quantify ventricular interaction. Results: LPP stress significantly increased SV from 73 ± 14 to 83 ± 16 mL (P < .01) in control subjects, while the increase in SV was significantly blunted (from 75 ± 19 to 79 ± 18 mL; P < .01 for interaction) in patients with TOF. The eccentricity index significantly changed during LPP stress in patients with TOF only from 1.07 ± 0.13 to 1.13 ± 0.14 (P < .01 for interaction). Patients with TOF were subdivided into two subgroups on the basis of the median value of increased response in SV (22 with sufficient and 22 with insufficient preload reserve). Multivariate analysis identified significant pulmonary regurgitation as the only independent determinant factor for insufficient preload reserve (odds ratio, 4.57; 95% CI, 1.048–19.90; P = .04). Conclusions: In patients after repair of TOF, ventricular interaction was exacerbated and preload reserve was impaired, especially in patients with significant pulmonary regurgitation. LPP stress testing may direct tailored treatment approaches, risk stratification, and clinical decision-making, such as more aggressive pharmacologic therapy, meticulous outpatient follow-up, or earlier reintervention.

Background Constrictive pericarditis (CP) is a pathological condition of the pericardium, resulting from fibrosis, scarring, and calcification of the pericardium. Other conditions have been reported to mimic ‘constrictive physiology’ despite the presence of an intact pericardium. However, there has been no report of pulmonary regurgitation (PR) mimicking the haemodynamic characteristics of CP. Case summary A 51-year-old woman was admitted to our institute because of severe right-sided heart failure. Transthoracic echocardiography revealed severe PR concomitant with significant dilatation of the right-sided heart. Septal bounce and the respiratory reciprocation of the transmitral and transtricuspid inflow velocities were also observed, indicating exacerbated ventricular interdependence. Cardiac catheter examination demonstrated elevated right atrial pressure with a prominent y descent, dip, and plateau waveform in the right ventricular pressure, and equalization of the diastolic pressure of all cardiac chambers, which are quite consistent with CP. On surgical inspection, however, there was no pericardial thickening or adhesion, indicating no obvious signs of CP. Discussion Pericardial constriction results from the relative relationship between intrapericardial volume and pericardial reserve. When the intrapericardial volume exceeds the physiological limit, the cardiac chambers compete with each other in a fixed pericardial space. In this case, prominent dilation of the right-sided chambers caused by severe PR resulted in overstretching of the pericardium above the pericardial reserve, which led to a characteristic haemodynamic picture that resembled CP. Thus, it is important to recognize the diagnostic pitfall in the preoperative evaluation of a ‘CP mimic physiology’.

Here we review the utility of strain measurements in patients with valvular diseases. The focus of the review is on using left ventricular global longitudinal strain in aortic regurgitation, aortic stenosis, and mitral regurgitation. We briefly cover the usefulness of right ventricular strain in these three disease states. Finally, we review the usefulness of right ventricular strain in mitral stenosis and tricuspid and pulmonary regurgitation.

The coexistence of tetralogy of Fallot and Ebstein’s anomaly is extremely rare. There are only a few case reports in the literature, and surgical options for the treatment are controversial. There is insufficient data on long-term follow-up of patients and management of complications. In this case report, we present a 20-year-old adult with operated tetralogy of Fallot, Ebstein’s anomaly, and Glenn anastomosis who underwent transcatheter pulmonary valve implantation for severe pulmonary insufficiency.

Background: Pulmonary regurgitation (PR) is the most common late complication in patients after repair of tetralogy of Fallot (TOF). Most patients remain asymptomatic over years, but eventually, the compensatory mechanisms fail, leading to right ventricular (RV) dilation and dysfunction, limited exercise capacity, ventricular arrhythmia, and sudden death. Aims: We aimed to evaluate associations between cardiac magnetic resonance (CMR) parameters and the need for either surgical or percutaneous pulmonary valve replacement (PVR) in asymptomatic patients with significant PR after repair of TOF. Methods: Of 209 patients with repaired TOF who had undergone a CMR study, we selected 61 asymptomatic patients with moderate‑to‑severe PR and followed them for up to 4 years (mean [SD], 21.4 [13.7] months). We excluded patients with residual ventricular septal defect, a peak RV outflow tract gradient of 30 mm Hg or higher, or at least moderate tricuspid regurgitation. Results: Receiver operating characteristic curve analyses revealed that the ratio of RV to left ventricular (LV) volume (RV/ LV ratio; threshold > 2.4) and PR fraction (PRF; threshold > 33%) had acceptable discriminatory capacity to differentiate between patients requiring PVR and those treated conservatively. The Cox proportional hazards regression and the Kaplan–Meier curves revealed that the RV / LV ratio and PRF was significantly associated with the need for PVR. The combination of the RV / LV ratio and PRF provided significant discrimination in terms of survival without PVR (P < 0.001; log‑rank test for trend). Conclusions: The RV/ LV ratio and PRF were significantly associated with the need for PVR in asymptomatic patients with isolated moderate‑to‑severe PR after repair of TOF.

Timing and indication for pulmonary valve replacement (PVR) in patients with repaired Tetralogy of Fallot (rToF) and pulmonary regurgitation (PR) are uncertain. To improve understanding of pumping mechanics, we investigated atrioventricular coupling before and after surgical PVR. Cardiovascular magnetic resonance was performed in patients ( n = 12) with rToF and PR > 35% before and after PVR and in healthy controls ( n = 15). Atrioventricular plane displacement (AVPD), global longitudinal peak systolic strain (GLS), atrial and ventricular volumes, and caval blood flows were analyzed. Right ventricular (RV) AVPD and RV free wall GLS were lower in patients before PVR compared with controls ( P < 0.0001; P < 0.01) and decreased after PVR ( P < 0.0001 for both). Left ventricular AVPD was lower in patients before PVR compared with controls ( P < 0.05) and decreased after PVR ( P < 0.01). Left ventricular GLS did not differ between patients and controls ( P > 0.05). Right atrial reservoir volume and RV stroke volume generated by AVPD correlated in controls ( r = 0.93; P < 0.0001) and patients before PVR ( r = 0.88; P < 0.001) but not after PVR. In conclusion, there is a clear atrioventricular coupling in patients before PVR that is lost after PVR, possibly because of loss of pericardial integrity. Impaired atrioventricular coupling complicates assessment of ventricular function after surgery using measurements of longitudinal function. Changes in atrioventricular coupling seen in patients with rToF may be energetically unfavorable, and long-term effects of surgery on atrioventricular coupling is therefore of interest. Also, AVPD and GLS cannot be used interchangeably to assess longitudinal function in rToF. NEW & NOTEWORTHY There is a clear atrioventricular coupling in patients with Tetralogy of Fallot (ToF) and pulmonary regurgitation before surgical pulmonary valve replacement (PVR) that is lost after operation, possibly because of loss of pericardial integrity. The impaired atrioventricular coupling complicates assessment of ventricular function after surgery when using measurements of longitudinal function. Left ventricular atrioventricular plane displacement (AVPD) found differences between patients and controls and changes after PVR that longitudinal strain could not detect. This indicates that AVPD and strain cannot be used interchangeably to assess longitudinal function in repaired ToF.

Introduction Transcatheter pulmonary valve implantation (TPVI) is a non-surgical method of treatment for patients with right ventricular outflow tract (RVOT) dysfunction after surgical repair of congenital heart defects (CHD). Aim To evaluate the long-term results of TPVI performed in a single center. Material and methods: Over 10 years, TPVI was performed in 100 patients (mean age: 26.4 ±8.1 years), using Melody Medtronic or Sapien Edwards valves. Results The initial success rate of TPVI was 93%. In 7 cases (5 urgent), a switch to surgical intervention was necessary due to periprocedural complications (all patients survived). Following TPVI, none of the 93 patients had severe pulmonary regurgitation. The pulmonary gradient decreased from 49.0 ±37.8 before to 27.6 ±14.9 mm Hg directly after TPVI (p < 0.0001). Right ventricular end-diastolic volume decreased, while NYHA class and pVO2 uptake significantly improved in 1 year after TPVI. Freedom from reintervention was 100% in 1 year. Freedom from serious adverse events was 86% in mean 5.5 years of observation. The main reason for reintervention was infective endocarditis (IE) (1.6% patients/year). Increased risk of IE was associated with severe PS before valve implantation and the suboptimal result of TPVI. The incidence of IE seems to be lower in patients treated permanently with antiplatelet therapy (1.8% vs. 0.9% patients/year, NS). Conclusions TPVI is a safe and effective method of treatment in patients with RVOT dysfunction after surgical correction of CHD. To achieve a good outcome, precise patient selection and rigorous IE prevention are necessary.

Lengthy exams and breath-holding limit the use of pediatric cardiac MRI (CMR). 3D time-resolved flow MRI (4DF) is a free-breathing, single-sequence exam that obtains magnitude (anatomic) and phase contrast (PC) data. We compare the accuracy of gadobenate dimeglumine-enhanced 4DF on a 1.5 T magnet to 2D CMR in children with repaired tetralogy of Fallot (rTOF) to measure pulmonary net flow (PNF) as a reflection of pulmonary regurgitation, forward flow (FF) and ventricular volumetry. Thirty-four consecutive cases were included. 2D PCs were obtained at the valve level. Using 4DF, we measured PNF at the valve and at the main and branch pulmonary arteries. PNF measured at the valve by 4DF demonstrated the strongest correlation (r = 0.87, p 0.001) and lowest mean difference (3.5 ± 9.4 mL/beat) to aortic net flow (ANF). Semilunar FF and stroke volume of the respective ventricle demonstrated moderate-strong correlation by 4DF (r = 0.66-0.81, p 0.001) and strong correlation by 2D (r = 0.81-0.84, p 0.001) with similar correlations and mean differences between techniques (p 0.05). Ventricular volumes correlated strongly between 2D and 4DF (r = 0.75-0.96, p 0.001), though 4DF overestimated right ventricle volumes by 11.8-19.2 mL/beat. Inter-rater reliability was excellent for 2D and 4DF volumetry (ICC = 0.91-0.99). Ejection fraction moderately correlated (r = 0.60-0.75, p 0.001) with better reliability by 4DF (ICC: 0.80-0.85) than 2D (ICC: 0.69-0.89). 4DF exams were shorter than 2D (9 vs. 71 min, p 0.001). 4DF provides highly reproducible and accurate measurements of flow with slight overestimation of RV volumes compared to 2D in pediatric rTOF. 4DF offers important advantages in this population with long-term monitoring needs.

An increasing number of patients will require pulmonary valve replacement following repair of tetralogy of Fallot. Most of these patients have a history of surgical repair with a transannular patch (ie, pulmonary valve not preserved). Ten to twenty years after the initial repair, severe pulmonary regurgitation results in right ventricular dilation and dysfunction. There are multiple prosthetic valve options and techniques to use when performing pulmonary valve replacements. Herein, we describe our technique of bioprosthetic pulmonary valve replacement.

Resume Les pathologies de la voie droite sont historiquement les consequences de la chirurgie correctrice d’une malformation cardiaque congenitale en periode neonatale ou pediatrique (Tetralogie de Fallot, intervention de Ross). Ces chirurgies realisees dans l’enfance conduisent a l’apparition de dysfonctionnements de la voie droite (stenose, fuite) chez le grand enfant ou l’adulte jeune pour lesquels une nouvelle chirurgie serait a risque operatoire eleve. L’avenement des premieres valves percutanees en 2000 a permis d’ouvrir la voie de la revalvulation pulmonaire. La procedure de revalvulation pulmonaire percutanee offre une alternative credible a la chirurgie dans la population des patients porteurs d’une cardiopathie congenitale souvent multi operes donc a risque operatoire eleve. Les deux valves actuellement sur le marche francais (Melody®, SapienXT®) permettent de couvrir l’ensemble des indications exceptees les voies tres larges qui restent eminemment chirurgicales. Les equipes qui pratiquent ces actes sur des sujets jeunes doivent beneficier d’une experience suffisante pour limiter le risque procedural. Sur cette base, plusieurs equipes positionnent desormais la revalvulation pulmonaire percutanee en priorite sur la revalvulation chirurgicale qui n’est retenue qu’en cas d’impossibilite du traitement percutane.

BACKGROUND Little is known about the early time course of biventricular function and mechanics after tetralogy of Fallot (TOF) repair. We sought to evaluate and describe the evolution of the right ventricle (RV) after TOF repair in young infants and children using conventional echocardiographic parameters and global longitudinal strain (GLS). METHODS A retrospective review was performed of all patients with TOF and pulmonary stenosis who underwent repair from January 2002 to September 2015 and had at least 3 serial postsurgical echocardiograms spanning from infancy to early childhood (

We describe percutaneous repair of severe pulmonary regurgitation and a right ventricular outflow tract pseudoaneurysm in a 19-year-old patient after repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. A 3D printed model of his heart was used to simulate percutaneous repair with a closure device in the aneurysm neck and a Venus P-valve in the right ventricular outflow tract. The encouraging findings from the simulation allowed us to plan the complex procedure effectively with a successful outcome and avoidance of surgery.

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Percutaneous balloon pulmonary valvuloplasty is a preferred treatment in pulmonary valve stenosis children with higher success rate. It is important to study the risk factors of postoperative severe pulmonary regurgitation, residual stenosis, and re-stenosis.

Pulmonary regurgitation (PR) after repair of tetralogy of Fallot (rTOF) is associated with progressive right (RV) and left (LV) ventricular dysfunction and fibrosis. However, angiotensin II receptor blockade therapy has shown mixed and often disappointing results. The aim of this study was to serially assess changes in biventricular remodeling, dysfunction, and interactions in a rat model of isolated severe PR and to study the effects of angiotensin II receptor blockade. PR was induced in Sprague-Dawley rats by leaflet laceration. Shams (

Funding Acknowledgements Type of funding sources: None. Introduction and purpose The optimal timing for pulmonary valve replacement (PVR) in asymptomatic patients with repaired tetralogy of Fallot (TOF) and pulmonary regurgitation (PR) remains uncertain but is often guided by imaging characterization of the right ventricle. As cardiopulmonary exercise testing (CPET) performance is an accessible prognostic indicator, we assessed which CPET parameters best correlate with pulmonary regurgitation severity to potentially improve identification of high-risk patients. Methods A retrospective chart review was done from 2009 to 2018 on adult patients with repaired TOF who underwent maximal effort cardiopulmonary exercise testing with cycle ergometry and with concurrent pulmonary function testing. Demographics, standard measures of CPET interpretation, and major cardiovascular outcomes were collected. Results Cardiopulmonary exercise testing was performed in 54 adult repaired TOF patients (59% male), with a mean follow-up of 60 ± 33 months. The mean age was 34 ± 9 years. 30 patients (56%) had severe pulmonary regurgitation and 26 patients (48%) were submitted to PVR, with a 0% mortality rate. PVR was performed a mean 28 ± 7 years after TOF repair surgery. There was moderate to severe right ventricular dysfunction in 11 patients (20%). 12 patients (22%) had a hospitalization for heart failure. Arrhythmic events occurred in 9 patients (17%), mainly atrial fibrillation or atrial flutter (67%). 2 patients (4%) received an implantable cardioverter-defibrillator for secondary prevention of sudden cardiac death. Peak VO2 consumption (pVO2) showed no statistically significant correlation with severity of pulmonary regurgitation (HR 0.26, 95% CI 0.879-1.036, p= 0.262) or PVR (HR 0.92, 95% CI 0.829-1.028, p = 0.914), while percent of predicted pVO2 significantly correlated with severity of pulmonary regurgitation (HR 0.95, 95% CI 0.918-0.993, p = 0.020) and PVR (HR 0.94, 95% CI 0.886-0.992, p = 0.025). VE/VCO2 slope was not a significant predictor of severity of pulmonary regurgitation (HR 1.03, 95% CI 0.929-1.130, p = 0.622) or PVR (HR 1.04, 95% CI 0.952-1.128, p = 0.414) or) and neither cardiorespiratory optimal point (HR 0.94, 95% CI 0.786-1.120, p = 0.480) nor maximum end-tidal carbon dioxide pressure (PETCO2) (HR 0.93, 95% CI 0.846-1.037, p = 0.213) correlated with severity of pulmonary regurgitation or PVR. Conclusion Percent of predicted peak VO2 had the highest predictive power of all CPET parameters analysed in adult repaired TOF patients. Preoperative CPET could be an accessible way to identify high-risk patients earlier for PVR and should therefore be included in the routine assessment of these patients.

Background Great variability exists in repair strategies for the tetralogy of Fallot. While transannular patching, as introduced by Kirklin, was a breakthrough for primary repair, pulmonary regurgitation and a need for reoperation have led to the development of methods that preserve the natural morphology of the right ventricular outflow tract. Methods State-of-the-art details of tetralogy of Fallot repair are explained from the standpoint of architectural preservation, especially with regard to sparing native tissue such as the annulus, valve, or infundibulum. Particular attention is given to the latest technical details of each approach, benefits and disadvantages, and any long-term data available. Results The choice of procedure is complex and unique to each case as transannular patching alone may carry long-term pulmonary risks. Modifications that spare the annulus, valves, or infundibulum may thus be essential as preservation of natural morphology has resulted in excellent mid-term results. Conclusions The complexity of tetralogy of Fallot repair demands constant attention to clinical presentation and vigilance against long-term sequelae. Techniques will continue to improve over time as long-term data guides the refinement of these innovative surgical methods.

Pulmonary regurgitation after repair of tetralogy of Fallot (ToF) is almost inevitable and in the past has required repeat surgery for replacing the regurgitant pulmonary valve. After 2000, percutaneous pulmonary valve implantation (PPVI) with balloon-expandable valves has replaced the surgical approach in a select group of patients. However, PPVI with the commercially available balloon-expandable valves have limitations because of their size, being applicable to right ventricle–pulmonary artery conduits or bioprosthetic valves. They have not been considered suitable for native dilated right ventricular outflow tracts (RVOT) after a transannular patch repair technique, which form about 70–75% of the patients. Novel self-expanding pulmonary valves have been developed for use in such dilated native outflow tracts. Early experience with these valves, although limited, has been encouraging, suggesting that self-expandable pulmonary valves can be implanted safely, with good short- and mid-term results in patients with native RVOT. This chapter summarises the new transcatheter self-expanding valves available for the management of pulmonary regurgitation after surgical repair of ToF, in patients in whom the shape and size of the RVOT, have previously been considered unsuitable for transcatheter valve implantation.

Why would you ever operate on an asymptomatic person? Of course, it must be that you believe you are providing a prognostic benefit, that which outweighs any procedural risk. Severe pulmonary regurgitation (PR) following repair of congenital right ventricular outflow tract obstruction is a common occurrence, and though well tolerated in childhood and adolescence, it eventually leads to right ventricular (RV) dilatation and dysfunction, conferring an increased risk of arrhythmia, heart failure and death.1 Pulmonary valve replacement (PVR) is indicated at the onset of symptoms, but often patients are asymptomatic even in the presence of significant RV enlargement. Intervention before irreversible myocardial dysfunction manifests is crucial, but the optimal timing remains unclear. It is a stressful decision for clinicians; wait too long and risk irreversible ventricular dysfunction or operate too soon and expose patients to unnecessary risk. Consensus guidelines recommend PVR in asymptomatic patients with severe PR based primarily on RV volume cut-offs (see table 1).2 These recommendations are based on studies predicting near-normalisation of RV volumes following PVR.3 Operating at these stipulated volumes is believed to improve late prognosis, and since the publication of these putative markers of increased risk, PVR numbers have increased and are being performed at younger ages.4 This is a potential concern, given the existing knowledge gaps. View this table: Table 1 Recommendations for intervention after repair of tetralogy of Fallot PR and the chronic volume load on the RV are thought to be central in the pathophysiology of late cardiovascular complications in this situation, including heart failure, ventricular arrhythmia and sudden cardiac death (see figure …

Background:Severe pulmonary regurgitation may result in right ventricular volume overload and decreased right ventricular function. Severe pulmonary regurgitation can be predicted prior to repair of tetralogy of Fallot. The aim of this study was to determine the risk factors for severe pulmonary regurgitation in repaired tetralogy of Fallot with transannular patch.Methods:This was a cross-sectional study in 43 patients with repaired tetralogy of Fallot using transannular patch. This study was carried out in Dr. Cipto Mangunkusumo hospital during 2015 to 2018. Participants were followed up for routine examination using echocardiography. We used bivariate and multivariate logistic regression using STATA 12.1 to identify risk factors for severe pulmonary regurgitation in this population.Results:A total of 43 patients composed of 22 boys and 21 girls with repaired tetralogy of Fallot using transannular patch were enrolled in the study. Median age of participants was 6 years at admission (2.1–18.5 years) and 3.4 years (1–17 years) at repair. Median length of follow-up was 2.1(1–4.3) years. Risk factors associated with severe pulmonary regurgitation after tetralogy of Fallot repair were McGoon ratio > 1.8 (odds ratio = 6.9; 95% confidence interval = 1.6–30) and follow-up duration >1.9 years (odds ratio = 3.6; 95% confidence interval = 0.9–15.2).Conclusion:McGoon ratio > 1.8 and follow-up duration > 1.9 years are associated with severe pulmonary regurgitation after tetralogy of Fallot repair.

Right ventricular (RV) dilatation is the determining prognostic factor in the long-term follow up of patients with repaired Tetralogy of Fallot (TOF). The objective of this study is to analyze whether the results vary depending on the timing of the complete repair and on the surgical technique applied. This is a retrospective longitudinal study in which patients with standard TOF were divided into 3 groups depending on their age at surgical repair: group 1 = Early repair (n = 12,1-8 months), group 2 = Late repair (n = 26, > 8 months), and group 3 = Late repair with previous palliative surgery (n = 17, > 8 months). Clinical, echocardiographic and cardiac magnetic resonance (CMR) data from patients that had received complete reparative surgery in our institution from January 2000 to March 2014 were analyzed and compared. 55 patients with echocardiogram and CMR studies (13.39 ± 3.59 years) were reviewed. All patients had at least moderate pulmonary regurgitation (PR). We observed a positive correlation between PR and right ventricular end-diastolic volume (r2 = 0.418; p = 0,004). Group 3 had more severe right ventricular dilatation than patients in groups 1 and 2 (p = 0.001). No differences in right ventricular end-diastolic volume, PR, and pulmonary trunk dimensions were observed between groups 1 and 2. Patients in group 3 had a longer hospital stay. Although all patients from our cohort had significant PR, age at surgery was not related to RV or pulmonary trunk dilatation. Previous palliative surgery was associated with more severe right ventricular dilatation and longer hospital stays. No differences were observed between early and late repair groups. Our study suggests that postponing TOF repair to a late stage does not improve the degree of PR or long-term morbidity from RV dilatation. Palliative surgery should be avoided if possible.

Background The most common post-surgical complication of tetralogy of Fallot (TOF) is pulmonary regurgitation (PR) which can lead to right ventricle (RV) dysfunction/failure. Cardiac magnetic resonance (CMR) is the imaging modality of choice to follow-up a repaired TOF. However, the conventional two-dimensional phase-contrast (2D-PC) flow usually underestimates PR as well as the pulmonary peak systolic velocity (PSV). Recently, four-dimensional (4D) CMR flow is introduced for more accurate quantitative flow assessment. This work aimed to compare between 4D-CMR and 2D-PC flow across the main (MPA), right (RPA), and left (LPA) pulmonary arteries (PAs) in surgically corrected TOF patients. Results This study was conducted on 20 repaired TOF patients (range 3–9 years, 50% males). All patients had CMR exam on 1.5T scanner. 4D-CMR and 2D-PC flows were obtained at the proximal segments of the MPA, RPA, and LPA. The stroke volume index (SVI), regurgitation fraction (RF), and PSV measured by 4D-CMR were compared to 2D-PC flow. The SVI across the PAs was nearly similar between both methods (P = 0.179 for MPA, 0.218 for RPA, and 0.091 for LPA). However, the RF was significantly higher by 4D-CMR in comparison to 2D-PC flow (P = 0.027 for MPA, 0.039 for RPA, and 0.046 for LPA). The PSV as well was significantly higher by 4D-CMR flow (P = 0.003 for MPA, < 0.001 for RPA, and 0.002 for LPA). The Bland-Altman plots showed a good agreement between 4D-CMR and 2D-PC flow for the SVI, RF, and PSV across the pulmonary arteries. Conclusion A good agreement existed between the two studied methods regarding pulmonary flow measurements. Because of its major advantage of performing a comprehensive flow assessment in a shorter time, 4D-CMR flow plays an important role in the assessment of patients with complex CHD especially in the pediatric group.

BACKGROUND AND OBJECTIVES We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. METHODS This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). RESULTS The median age of the patients was 14.0 months (interquartile range [IQR], 10.7-19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the follow-up, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2-17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. CONCLUSIONS Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age

Background Re-intervention after Ross procedure into the right ventricular outflow tract might be needed in patients in the long term. However, right ventricular outflow tract re-intervention indications are still unclear. Comprehensive assessment of total hemodynamics is needed. Case summary A 42-year-old Japanese woman was referred to our hospital for moderately severe pulmonary regurgitation and severe tricuspid regurgitation after a Ross–Konno procedure. Thirteen years after surgery, she developed atrial fibrillation and atrial flutter and complained of dyspnea. Electrophysiological studies showed re-entry circuit around the low voltage area of the lateral wall on the right atrium. Four-dimensional flow magnetic resonance imaging revealed moderate pulmonary regurgitation, severe tricuspid regurgitation, and a dilated right ventricle. Flow energy loss in right ventricle calculated from four-dimensional flow magnetic resonance imaging was five times higher than in normal controls, suggesting an overload of the right-sided heart system. Her left ventricular ejection fraction was almost preserved. Moreover, the total left interventricular pressure difference, which shows diastolic function, revealed that her sucking force in left ventricle was preserved. After the comprehensive assessments, we performed right ventricular outflow tract reconstruction, tricuspid valve annuloplasty, and right-side Maze procedure. A permanent pacemaker with a single atrial lead was implanted 14 days postoperatively. She was discharged 27 days postoperatively. Echocardiography performed 3 months later showed that the size of the dilated right ventricle had significantly reduced. Discussion A four-dimensional imaging tool can be useful in the decision of re-operation in patients with complex adult congenital heart disease. The optimal timing of surgery should be considered comprehensively.

Background: Postcongenital heart surgery pulmonary regurgitation requires subsequent pulmonary valve replacement. We sought to compare the outcomes of pulmonary valve replacement after using bioprosthetic valves, porcine versus pericardial bioprosthesis. Method: Retrospective single-center study of consecutive pulmonary valve replacement in patients with pulmonary regurgitation following initial congenital cardiac surgery. From 2004 to 2016, 82 adult patients (53 males, 29 females) underwent pulmonary valve replacement at a mean age of 28.7 ± 8 years (range 18-52 years) with a mean time to pulmonary valve replacement of 24 ± 7 years (range 13-43 years). Porcine bioprosthetic valves (group 1, n = 32) and pericardial valves (group 2, n = 50) were used. Cardiac magnetic resonance imaging was performed (n = 54) at a mean of 18 ± 13 months before and 24 ± 21 months after pulmonary valve replacement. Results: No significant difference was seen between the groups except that the mean follow-up was longer for group 1 (5.02 ± 2.06 vs 4.08 ± 3.21 years). In-hospital mortality was 1.1%. Follow-up completeness was 100% with no late death. Mean right ventricular end-systolic and end-diastolic volumes reduced significantly in both the groups ( P < .001), whereas right ventricular ejection fraction remained unchanged (group 1, P = .129; group 2, P = .675) . Only the left ventricular end-diastolic volume increased in both the groups, but the increase was significant for group 2 only (group 1, P = .070; group 2, P = .015), whereas the left ventricular end-systolic and ejection fraction remained unchanged in both the groups. There was no reoperation for pulmonary valve replacement. Freedom from intervention was 93.8% (group 1) and 100% (group 2) at eight years after pulmonary valve replacement ( P = .407). Conclusion: Midterm outcomes of pulmonary valve replacement in our adult cohort were satisfactory. Both types of bioprosthetic valves performed comparably for eight years and were a good option in adults.

Patients with repaired tetralogy of Fallot (TOF) have not uncommonly fragmented QRS complexes (fQRS) on surface electrocardiogram. We suggested that the right heart hemodynamics might be correlated with both the number of leads showing fQRS (fQRS extent) and the QRS duration. A retrospective analysis of the magnetic resonance imaging (MRI) derived data of 30 patients following total correction of TOF was performed. The number of leads showing fragmentation (fQRS extent) as well as the QRS duration were assessed and tested for correlation with the right ventricular volumes and severity of pulmonary regurgitation (PR) as detected by MRI. Twenty-one patients had fQRS in at least 2 consecutive leads. fQRS correlated with larger right ventricular volumes and lower ejection fraction. fQRS in 5 leads was an accurate parameter in identifying an indexed right ventricular end diastolic volume >150 ml/m2 with a sensitivity of 87.5% and a specificity of 85.7%. Moreover, it was the most accurate parameter in detecting a PR fraction > 45%. To conclude, assessment of fQRS extent and duration is a simple, available, and an accurate parameter in identifying patients with significant right ventricular hemodynamic changes in repaired TOF patients.

OBJECTIVES Many surgeons develop unique techniques for unmet needs for right ventricular outflow reconstruction to resolve pulmonary regurgitation after corrective surgery for congenital heart diseases. Expanded polytetrafluoroethylene (ePTFE) stands out as a reliable synthetic material, and clinical results with handmade ePTFE valves have been promising. This review focuses on the historical evolution of the use of ePTFE in pulmonary valve replacement and in the techniques for pioneering the translation of the handmade ePTFE trileaflet design for the transcatheter approach. METHODS We searched for and reviewed publications from 1990 to 2020 in the Pubmed database. Nineteen clinical studies from 2005 to 2019 that focused on ePTFE-based valves were summarized. The evolution of the ePTFE-based valve over 3 decades and recent relevant in vitro studies were investigated. RESULTS The average freedom from reintervention or surgery in the recorded ePTFE-based valve population was 90.2% at 5 years, and the survival rate was 96.7% at 3 years. CONCLUSIONS Non-inferior clinical results of this ePTFE handmade valve were revealed compared to allograft or xenograft options for pulmonary valve replacement. Future investigations on transferring ePTFE trileaflet design to transcatheter devices should be considered.

Simultaneous pulmonary and aortic endocarditis is extremely rare, and there is no consensus on its surgical management. Here, we report a case of infective endocarditis of pulmonary and aortic valves complicated by severe pulmonary regurgitation due to complete damage of valve cusps. We performed pulmonary valve reconstruction using autologous pericardium using Ozaki’s technique, with excellent outcomes.

Epic Supra valves have been used off-label in the pulmonary position. We aim to evaluate the durability of Epic valves in the pulmonary position. We performed a retrospective review of all Epic valves placed in the pulmonary position from October 2008 to May 2019. Time-to-event analysis was performed using Kaplan-Meier estimates to evaluate freedom from valve intervention, moderate pulmonary regurgitation, and peak velocity greater than 3.5 m/s. Valve dysfunction was a composite of all 3 end points. A total of 79 patients had Epic valves implanted in the pulmonary position. Median age was 18.5 years (15th–85th percentile 11.2–41.0). In total, 1 (1%) 19 mm valve, 4 (5%) 21 mm valves, 8 (10%) 23 mm valves, 23 (29%) 25 mm valves, and 43 (54%) 27 mm valves were implanted. There were no deaths or transplants. Median follow-up was 3.1 years (interquartile range 1.0–5.5). At 5 years, freedom from valve intervention was 95%, freedom from valve dysfunction was 68%, freedom from moderate pulmonary regurgitation was 73%, and freedom from peak velocity greater than 3.5 m/s was 82%. Epic Supra valves provide an acceptable valve replacement in the pulmonary position for children and adults. Longer follow-up is needed to determine valve durability through the entirety of the valve life expectancy.

Right ventricular outflow tract (RVOT) dysfunction is common among individuals with congenital heart disease (CHD). Surgical intervention often carries prohibitive risks due to the need for sequential pulmonary valve (PV) replacements throughout their life in the majority of cases. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with RVOT dysfunction.In this review, we examine the pathophysiology of RVOT dysfunction, indications for tPVR, and the procedural aspect. Advancements in clinical application and valve technology will also be covered.tPVR is widely accepted as an alternative to surgery to address RVOT dysfunction, but still significant numbers of patients with complex RVOT morphology deemed not suitable for tPVR. As the technology continues to evolve, new percutaneous valves will allow such complex RVOT patient to benefit from tPVR.

Deleterious long-term effects of chronic pulmonary regurgitation after repair of tetralogy of Fallot have become evident during the last decades. Subsequently, some groups have developed strategies to spare the pulmonary valve function at the time of repair with good early results. However, mid-term outcomes are scarce in the literature and in some cases controversial. The aim of our study is to report our results mid-term with valve-sparing repair of tetralogy of Fallot. We retrospectively reviewed patients undergoing tetralogy of Fallot repair and having preservation of the pulmonary valve with intraoperative dilation at our institution. From June 2009 through June 2017, 42 patients underwent valve-sparing tetralogy of Fallot repair. Median age and weight at surgery were 5.2 months and 7.2 kg. Median preoperative pulmonary valve diameters and Z scores by echocardiography were 6.4 mm (range 4.5-11 mm) and -2.3 (range -1.3 to -4.5). No patient died in our series. For a median follow-up of 45 months, the pulmonary valve has grown by Z score (P0.0001) as well as the pulmonary trunk (P= 0.00216). Significant pulmonary regurgitation has developed in 9 patients (21.4%). No patient has required reintervention/reoperation for recurrent right ventricular outflow tract obstruction. Patients with tetralogy of Fallot who had valve-sparing repair with intraoperative dilation of the pulmonary valve show good early and mid-term results with respect to right ventricular outflow tract obstruction. The pulmonary valve annulus and the pulmonary trunk grow through follow-up. Progressive development of significant pulmonary regurgitation is seen in more than 20% of patients. Long-term data with this approach and comparison with a population of patients undergoing a transannular patch repair are required to establish the real utility of this approach.

Congenital heart defects that involve obstruction to the right ventricular outflow tract are common. Surgical repair involves early relief of right ventricular outflow tract obstruction, which typically results in pulmonary regurgitation and large irregularly shaped "native" right ventricular outflow tract. This type of anatomy represents the majority of patients who could potentially benefit from transcatheter pulmonary valve therapy. Currently approved balloon-expandable devices were not designed for this application and the unique anatomy of these patients presents tremendous challenges for designing a valve that is. This article explores those challenges and the newest self-expanding devices designed to treat this challenging population.

We report a case of a 15-year-old female who underwent combined hybrid pulmonary valve replacement and transcatheter atrial septal defect device closure, which was performed due to severe volume overload of the right side of the heart secondary to pulmonary regurgitation and atrial septal defect.

Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy.A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037].Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.

Intracardiac hemodynamic forces have been proposed to influence remodeling and be a marker of ventricular dysfunction. We aimed to quantify the hemodynamic forces in patients with repaired tetralogy of Fallot (rToF) to further understand the pathophysiological mechanisms as this could be a potential marker for pulmonary valve replacement (PVR) in these patients. Patients with rToF and pulmonary regurgitation (PR) > 20% ( n = 18) and healthy control subjects ( n = 15) underwent MRI, including four-dimensional flow. A subset of patients ( n = 8) underwent PVR and MRI after surgery. Time-resolved hemodynamic forces were quantified using 4D-flow data and indexed to ventricular volume. Patients had higher systolic and diastolic left ventricular (LV) hemodynamic forces compared with control subjects in the lateral-septal/LV outflow tract ( P = 0.011 and P = 0.0031) and inferior-anterior ( P < 0.0001 and P < 0.0001) directions, which are forces not aligned with blood flow. Forces did not change after PVR. Patients had higher RV diastolic forces compared with control subjects in the diaphragm-right ventricular (RV) outflow tract (RVOT; P < 0.001) and apical-basal ( P = 0.0017) directions. After PVR, RV systolic forces in the diaphragm-RVOT direction decreased ( P = 0.039) to lower levels than in control subjects ( P = 0.0064). RV diastolic forces decreased in all directions ( P = 0.0078, P = 0.0078, and P = 0.039) but were still higher than in control subjects in the diaphragm-RVOT direction ( P = 0.046). In conclusion, patients with rToF and PR had LV hemodynamic forces less aligned with intraventricular blood flow compared with control subjects and higher diastolic RV forces along the regurgitant flow direction in the RVOT and that of tricuspid inflow. Remaining force differences in the LV and RV after PVR suggest that biventricular pumping does not normalize after surgery. NEW & NOTEWORTHY Biventricular hemodynamic forces in patients with repaired tetralogy of Fallot and pulmonary regurgitation were quantified for the first time. Left ventricular hemodynamic forces were less aligned to the main blood flow direction in patients compared with control subjects. Higher right ventricular forces were seen along the pulmonary regurgitant and tricuspid inflow directions. Differences in forces versus control subjects remain after pulmonary valve replacement, suggesting that altered biventricular pumping does not normalize after surgery.