Residual Pulmonary Regurgitation Research Articles

Primary surgical repair of tetralogy of fallot at the Uganda Heart Institute: a ten-year review of 30day mortality and morbidity

BackgroundTetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources.ObjectiveThis study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated.MethodologyThis retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation.ResultsOut of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan’s, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team.ConclusionThe most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team.

Severe Residual Pulmonary Stenosis after Surgical Repair of Tetralogy of Fallot: What’s Our Next Strategy?

This case report describes a 25-year-old woman with Tetralogy of Fallot (TOF) who underwent surgical repair at age 15. Eight years later, she presented with recurrent chest pain and was found to have residual severe pulmonary stenosis and regurgitation. This is a complication of TOF repair, particularly when the pulmonary valve is not preserved. The paper discusses the management of TOF, including initial palliative procedures and complete repair surgery. It highlights the long-term complications such as pulmonary valve regurgitation and residual stenosis, both of which may require re-intervention. The importance of long-term follow-up for TOF patients is emphasized.

Predictors of clinical outcome in adults with repaired tetralogy of Fallot and the prognostic value of surrogate markers of right ventricular dyssynchrony

Abstract Introduction Sequelae or residual lesions after surgical repair of tetralogy of Fallot (rTOF) are inevitable and have an impact on long-term outcome. So far, most of our attention was directed to negative consequences of residual pulmonary regurgitation. Less attention has been paid to the impact of right bundle branch block and intraventricular dyssynchrony on biventricular function, exercise capacity and clinical outcome. Purpose Using the Swiss Adult Congenital HEart disease Registry we aimed to investigate the 5-year clinical course of adults with rTOF late after surgical repair. We used echocardiography strain analysis as surrogate to quantify right and left ventricular dyssynchrony and correlated our findings with routine clinical measures from cardiopulmonary exercise testing, neurohumoral blood work and cardiac magnetic resonance imaging with the aim to identify patients at increased risk for an adverse clinical event. Methods 2D speckle-tracking strain analysis of the right and left ventricle was performed with dedicated software (Tomtec, Germany). RV dyssynchrony index was measured as the standard deviation of time to peak shortening (TTP) in six RV segments. Interventricular shortening delay (IVSD) was defined as the maximal delay of TTP between ventricular segments. We constructed a multivariate model with NT-proBNP, ECG, clinical, imaging and exercise testing variables to predict the composite outcome of all-cause mortality, relevant arrhythmias and hospital admission for decompensated heart failure. Results A total of 285 patients were included. During a mean follow-up of 48±21 months, 33 patient (12 %) suffered an adverse event with a mean time to event of 30 ± 21 months after the baseline visit. RV dyssynchrony index and IVSD did not differ statistically between the groups with and without events (49±31ms vs 41±18ms, p-value 0.21, 130±65ms vs. 103±50ms, p-value 0.9). In the multivariate Cox-regression model, NT-proBNP, right atrial area and peak heart rate at exercise testing were independent predictors of outcome. An integrated risk score with the median values of these 3 variables as cut-off was highly accurate in separating patients at low risk (score 0; n=39) and high risk (score 3; n=38) for an adverse event. After 6 years of follow-up, no patient at low risk (score 0) had an adverse event, while in 62% of patients at high risk (score 3) an adverse event occurred (p<0.001). Conclusion In our cohort of rTOF patients, echocardiographic surrogates of RV dyssynchrony did not correlate with clinical events. An integrated multimodality approach proved to be effective in predicting outcomes.

Abstract 14833: Association of RV Hemodynamics From 4D Flow CMR With RV Restrictive Physiology From Pediatric to Adult Repaired Tetralogy of Fallot

Introduction: Right ventricular restrictive physiology (RVRP) is common after repair of Tetralogy of Fallot (rTOF), especially with residual pulmonary regurgitation. Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) quantitates biventricular blood flow components and kinetic energy (KE) parameters, but their association with RVRP is unclear. We aimed to study 4D flow CMR parameters in adult and pediatric rTOF patients vs healthy controls and associations of 4D flow parameters with RVRP in rTOF. Methods: 103 rTOF patients (40 pediatric, 63 adult; age 24±17 years; 53M:50F) and 171 healthy controls (14 pediatric, 157 adult; age 40±15 years; 97M:74F) were prospectively recruited from centers in Singapore (2) and China (1) to undergo 4D flow CMR on multi-vendor scanners. LV and RV flow components—direct flow, retained inflow, delayed ejection flow and residual volume—and KE parameters (normalized to end-diastolic volume, KEi EDV )—global, peak systolic, average systolic, average diastolic, peak E-wave and peak A-wave—were analyzed. KE discordance was calculated as the ratio of RV and LV average systolic KEi EDV . Results: See Table. RVRP was identified in 70 patients (68%). rTOF with RVRP exhibited higher median RV stroke volume index, RV ejection fraction, RV direct flow, RV peak E-wave and E-wave/A-wave, and lower RV residual volume compared to rTOF without RVRP (all P<0.05). Using binary multivariable logistic regression analysis—and after adjustment for age, pulmonary regurtitation, LV and RV ejection fraction, RV direct flow was found to be independently associated with RVRP in rTOF (P<0.001). Conclusion: Patients with RVRP had higher RV direct flow, RVEF and RV peak E-wave energy, indicating the potential compensatory adaptations in patients with RVRP.

In vitro bench testing using patient-specific 3D models for percutaneous pulmonary valve implantation with Venus P-valve.

Due to the wide variety of morphology, size, and dynamics, selecting an optimal valve size and location poses great difficulty in percutaneous pulmonary valve implantation (PPVI). This study aimed to report our experience with in vitro bench testing using patient-specific three-dimensional (3D)-printed models for planning PPVI with the Venus P-valve. Patient-specific 3D soft models were generated using PolyJet printing with a compliant synthetic material in 15 patients scheduled to undergo PPVI between July 2018 and July 2020 in Central China Fuwai Hospital of Zhengzhou University. 3D model bench testing altered treatment strategy in all patients (100%). One patient was referred for surgery because testing revealed that even the largest Venus P-valve would not anchor properly. In the remaining 14 patients, valve size and/or implantation location was altered to avoid valve migration and/or compression coronary artery. In four patients, it was decided to change the point anchoring because of inverted cone-shaped right ventricular outflow tract (RVOT) ( n = 2) or risk of compression coronary artery ( n = 2). Concerning sizing, we found that an oversize of 2-5 mm suffices. Anchoring of the valve was dictated by the flaring of the in- and outflow portion in the pulmonary artery. PPVI was successful in all 14 patients (absence of valve migration, no coronary compression, and none-to-mild residual pulmonary regurgitation [PR]). The diameter of the Venus P-valve in the 3D simulation group was significantly smaller than that of the conventional planning group (36 [2] vs. 32 [4], Z = -3.77, P <0.001). In vitro testing indicated no need to oversize the Venus P-valve to the degree recommended by the balloon-sizing technique, as 2-5 mm sufficed.

Self-Expanding Pulmonary Valves in 53 Patients With Native Repaired Right Ventricular Outflow Tracts.

Self-expanding pulmonary valve grafts have been designed for percutaneous pulmonary valve implantation (PPVI) in patients with native repaired right ventricular (RV) outflow tracts (RVOTs). However, their efficacy, in terms of RV function and graft remodelling remain unclear. Patients with native RVOTs who received Venus P-valve (N=15) or Pulsta valve (N= 38) implants between 2017 and 2022 were enrolled. We collected data on patient characteristics and cardiac catheterization parameters as well as imaging and laboratory data before, immediately after, and 6 to 12 months after PPVI and identified risk factors for RV dysfunction. Valve implantation was successful in 98.1% of patients. The median duration of follow-up was 27.5 months. In the first 6 months after PPVI, all patients exhibited resolution of paradoxical septal motion and a significant reduction (P < 0.05) in RV volume, N-terminal pro-B-type natriuretic peptide levels, and valve eccentricity indices (-3.9%). Normalization of the RV ejection fraction (≥ 50%) was detected in only 9 patients (17.3%) and was independently associated with the RV end-diastolic volume index before PPVI (P= 0.03). Nine patients had residual or recurrent pulmonary regurgitation or paravalvular leak (graded as ≥ mild), which was associated with a larger eccentricity index (> 8%) and subsided by 12 months postimplantation. We identified the risk factors likely to be associated with RV dysfunction and pulmonary regurgitation following PPVI in patients with native repaired RVOTs. RV volume-based patient selection is recommended for PPVI of a self-expanding pulmonary valve, along with monitoring of graft geometry.

Right ventricular echocardiographic remodeling after pulmonary valve replacement in repaired Tetralogy of Fallot

BackgroundMany patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution. ResultsIn a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003–2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up. ConclusionsPulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery.

Repair with a pulmonary neovalve in tetralogy of Fallot: does this avoid ventricular dysfunction?

OBJECTIVESGiven the anatomical variations of tetralogy of Fallot (TOF), different surgical techniques can be used to achieve correction. Transannular patches (TAPs) are the most commonly used technique; they are associated with right ventricular dysfunction, the incidence of which can be reduced through pulmonary valve preservation.METHODSBetween January 2010 and July 2019, we performed 274 surgical corrections of tetralogy of Fallot at Fundación Cardioinfantil; 63 patients (23%) underwent repair with a TAP in addition to a pulmonary neovalve (Group I), 66 patients (24.1%) received a TAP without a pulmonary valve (Group II) and 145 patients (52.9%) had a repair with valve preservation (Group III). We analysed patient’s characteristics before, during and after surgery at a 30-day follow-up.RESULTSWe found that patients in Group III were older (P = 0.04). Group II had the lowest level of O2 saturation before surgery (82%, P = 0.001). Cardiopulmonary bypass and aortic cross-clamp times were longer in Group I (P < 0.001). Right ventricular dysfunction was less frequent in Group III (15.9%, P = 0.011). Severe residual pulmonary regurgitation was more common in Group II (21.9%, P = 0.001).CONCLUSIONSPreservation of the pulmonary valve is an important factor for immediate postoperative management of tetralogy of Fallot. Patients who were repaired with a TAP with or without a pulmonary neovalve had a higher incidence of right ventricular dysfunction than those with pulmonary valve preservation.

Flow vorticity relationships with right ventricular geometry in adult tetralogy of Fallot

Abstract Background Repaired tetralogy of Fallot adults (rToF) undergo right ventricular (RV) remodeling, in part due to volume overload of residual pulmonary regurgitation volume (PRV). Time-resolved phase-contrast cardiac magnetic resonance imaging (4D Flow MRI) enables the qualitative and quantitative measurement of altered blood flow patterns, including vorticity. Cardiac atlases allow for complex three-dimensional heart shapes to be expressed as morphometric scores. Those scores show the extent of geometrical shift and can help explore uncharted relationships between vorticity and architecture. Purpose We aimed to quantify vorticity, incorporating deep learning to enhance 4D Flow data, and correlate this with global cardiac parameters and morphometric scores. Methods 12 Adult rToF patients and 10 age-matched controls underwent 4D flow MRI and cine imaging. RV interventricular vorticity was calculated for outflow and inflow tracts. EDV, ESV and SV were computed from cines which were also used to build three-dimensional shape models. The biventricular models were projected onto an atlas generated from 95 rToF patients, and twenty-one principal component analysis shape modes were correlated with cardiac metrics and vorticity to identify global shape variations. Association between biventricular shape and vorticity was further analysed using multivariate multiple regression models. Results Strong correlation was found between PRV and the right ventricular outflow tract (RVOT) vorticity. PRV and RVOT vorticity both correlated with the same 3 shape modes (r=−0.55, −0.50 and 0.6 (p&amp;lt;0.05) respectively for PR and r=0.63, −0.82 and 0.60 (p&amp;lt;0.05) respectively for vorticity) i.e., the RV dilates with an increase in basal bulging, apical bulging and tricuspid annulus tilting with more severe regurgitation, as well as a smaller LV, and a paradoxical movement of the septum (Figure 1). However, RV vorticity correlated with 2 modes that did not correlate with PRV, (r=−0.62, −0.69, p&amp;lt;0.05). With higher vorticity the RV was longer, increased tilting of the tricuspid annulus and an increased basal bulge around the tricuspid area. The multivariate analysis model demonstrated that higher vorticity was associated with displacement of the pulmonary valve and change in the RVOT length and direction. A septal displacement towards the left ventricle was observed and increased apical flatness of the RV (Figure 1). Qualitatively, vorticity in rToF group was more heterogeneous than controls (Figure 2). Conclusions Vorticity is a novel marker based on the influence of blood motion providing new insight into early diagnosis and prognosis of cardiac disease. This is the first study to examine the relationships between vorticity and regional RV shape changes in rToF. Mode associations with vorticity were different to associations with PRV. More longitudinal studies are required for standardization of change in vorticity with the disease process. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The New Zealand heart foundation Mode variations and morphometric modelVorticity visualization and analysis

Cardiac index in adults with repaired tetralogy of Fallot: Are we missing the forest for the trees?

BackgroundMany cardiac parameters have been associated with poor outcomes in patients with repaired tetralogy of Fallot (TOF) and significant residual pulmonary regurgitation (PR). However, the utility of cardiac index (CI) in these patients has never been studied. Our study aimed to assess if a low CI is associated with the development of adverse cardiac events in this population and compare it to other established cardiac parameters. MethodsAll patients with repaired TOF and significant PR who had a cardiac magnetic resonance imaging (CMR) at our institution were enrolled. CI was measured by CMR and their charts were reviewed for the development of the following outcomes: worsening NYHA class, admission for heart failure, arrhythmias, and sudden cardiac death. ResultsFifty-five patients were included in the study. Median age was 28 years and mean follow-up was 9.5 years. Eighteen patients (32.7%) developed one or more of the predefined outcomes. Their CI was significantly lower compared to patients without adverse events (CI 2.3 vs. 2.8 ​L/min/m2; p-value ​= ​0.0045). CI alone had a better yield in predicting adverse events when compared to the other combined CMR parameters (AUC 0.78 vs 0.61). Patients with a CI ​< ​2.4 had a 74.3% cumulative probability of developing adverse cardiac events at 10 years compared to 22.4% in patients with a CI ​≥ ​2.4 (p-value<0.0001). ConclusionIn adults with repaired TOF and significant residual PR, CI appears to be the best predictor of midterm adverse cardiac events. Whether it can be used for timing of pulmonary valve replacement remains to be studied.

Biomechanical Modeling to Inform Pulmonary Valve Replacement in Tetralogy of Fallot Patients After Complete Repair

BackgroundA biomechanical model of the heart can be used to incorporate multiple data sources (electrocardiography, imaging, invasive hemodynamics). The purpose of this study was to use this approach in a cohort of patients with tetralogy of Fallot after complete repair (rTOF) to assess comparative influences of residual right ventricular outflow tract obstruction (RVOTO) and pulmonary regurgitation on ventricular health. MethodsTwenty patients with rTOF who underwent percutaneous pulmonary valve replacement (PVR) and cardiovascular magnetic resonance imaging were included in this retrospective study. Biomechanical models specific to individual patient and physiology (before and after PVR) were created and used to estimate the RV myocardial contractility. The ability of models to capture post-PVR changes of right ventricular (RV) end-diastolic volume (EDV) and effective flow in the pulmonary artery (Qeff) was also compared with expected values. ResultsRV contractility before PVR (mean 66 ± 16 kPa, mean ± standard deviation) was increased in patients with rTOF compared with normal RV (38-48 kPa) (P < 0.05). The contractility decreased significantly in all patients after PVR (P < 0.05). Patients with predominantly RVOTO demonstrated greater reduction in contractility (median decrease 35%) after PVR than those with predominant pulmonary regurgitation (median decrease 11%). The model simulated post-PVR decreased EDV for the majority and suggested an increase of Qeff—both in line with published data. ConclusionsThis study used a biomechanical model to synthesize multiple clinical inputs and give an insight into RV health. Individualized modeling allows us to predict the RV response to PVR. Initial data suggest that residual RVOTO imposes greater ventricular work than isolated pulmonary regurgitation.

Impact of right atrium dimension on adverse outcome after pulmonary valve replacement in repaired Tetralogy of Fallot patients

The hemodynamic impact of residual pulmonary regurgitation (PR) in repaired Tetralogy of Fallot (rTOF) has been well demonstrated. However, markers driving the decision making process to indicate the ideal timing of pulmonary valve replacement (PVR) are still uncertain. Furthermore, very few studies have included the right atrium (RA) dilatation as a preoperative risk factor for post-PVR clinical adverse outcome. The aim of this study was to investigate the impact of pre-PVR right atrial dilation on adverse outcomes in rTOF. We retrospectively reviewed from our CMR database all rTOF patients who underwent CMR study before and after PVR. Detailed clinical and surgical history were collected, in addition to imaging data. The composite primary and secondary post-PVR end points were also recorded. The study cohort consisted of 41 patients (mean age at PVR repair 27.4 ± 10years). As expected, end-diastolic and end-systolic right ventricle (RV) volumes significantly decreased after PVR (p < 0.001). The RV reverse remodeling, defined by ΔRVEDVi and ΔRVESVi, was associated with both pre-PVR RVEDVi and RVESVi. The higher the pre-PVR RV volumes, more the RV reverse remodeling will be obtained post-surgery. Patients who experienced an adverse outcome were older at pre-PVR, they had a higher Nt-ProBNP, worse VO2/kg/min, more significant tricuspid regurgitation and more dilated RA. The association with the RA dilatation persists and becomes even more significant if we exclude patients who had tricuspid repair beside RVOT surgical reconstruction. Besides RV volumes and function, RA dimensions may play a pivotal role in the decision making of TOF patients.

Biventricular Shape Modes Associated with Clinical History and Pulmonary Regurgitation in Repaired Tetralogy of Fallot

Surgical repair of tetralogy of Fallot (ToF) often involves damage to the right ventricular (RV) outflow tract typically resulting in pulmonary valve impairment and subsequent pulmonary regurgitation. Residual pulmonary regurgitation leads to progressive RV remodeling in the form of RV dilation and apical bulging, and plays an important role in long‐term outcomes. Patients with severe pulmonary regurgitation and RV decompensation undergo pulmonary valve replacement (PVR) to reduce the risk of arrhythmia, heart failure, and sudden death. However, the clinical decision on whether and when to perform PVR remains unclear. We sought to quantify biventricular shape modes associated with clinical history and pulmonary regurgitation prior to valve replacement using a biventricular geometric atlas constructed using principle component analysis. In this cross‐sectional retrospective study, biventricular shape models were customized using cardiovascular magnetic resonance images from 97 repaired ToF patients. Patients were divided into two groups: those that subsequently had PVR and those that did not. Regression and clustering techniques were used to quantify shape variations associated with type of surgical repair, time following surgical repair, time leading up to valve replacement, and pulmonary regurgitant volumes. Principal components of biventricular shape variation were associated with RV dilation and apical bulging, overall size and sphericity, and valve plane orientation. Biventricular shape modes were quantified using statistical shape modeling techniques and are correlated with clinical history and pulmonary regurgitation.Support or Funding InformationFunding was provided by the National Institutes of Health 1R01HL121754. SG acknowledges National Institutes of Health NHLBI 1T32HL105373. CM acknowledges New Zealand Heart Foundation Grant 1695. AY acknowledges Health Research Council Grant 17/234.

RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION IN AN ADULT WITH REPAIRED TETRALOGY OF FALLOT PRESENTING AS TET SPELLS

Adults with repaired TOF may face sequelae like residual RVOT stenosis and pulmonary regurgitation (PR) that warrant re-intervention. Optimal timing of re-intervention is uncertain. 21F with Trisomy 21, complete AV canal defect and TOF s/p complete repair presented with worsening dyspnea on

A LOW CARDIAC INDEX IS ASSOCIATED WITH CLINICAL EVENTS IN ADULT PATIENTS WITH REPAIRED TETRALOGY FALLOT

Cardiac index (CI) was shown to be the best predictor to cardiac hospitalization and cardiac interventions in patients with congenital heart disease. However, it has not been studied specifically in patients with tetralogy of Fallot (TOF). The goal of our study was to see if a low CI in TOF patients was associated with clinical deterioration. All patients with TOF who underwent a transannular patch repair with residual severe pulmonary regurgitation (PR) and who had a cardiac magnetic resonance imaging (MRI) were enrolled in the study. CI was measured from their first MRI performed as an adult and clinical charts were reviewed for the development of any of the following clinical outcomes: 1) atrial arrhythmias; 2) ventricular arrhythmias or sudden cardiac death; 3) worsening NYHA functional class; 4) admission for heart failure. Sixty patients (52% male) were included in the study. Mean age at MRI was 20.5 years and mean follow up was 9.5 years. Twenty-one patients (35%) developed outcomes: SVT in nine patients, VT in three patients, worsening NYHA in nine patients and heart failure requiring admission in five patients. Patients with clinical outcomes had a significantly lower CI compared to patients who had no clinical events (CI 2.05 L/min/m2 vs. 3.09 L/min/m2; p<0.001, 95% confidence interval). In adults with TOF repair and residual severe PR, a low cardiac index correlates with midterm clinical outcomes.

Three-dimensional analysis of regional right ventricular shape and function in repaired tetralogy of Fallot using cardiovascular magnetic resonance

BackgroundPatients with surgically repaired tetralogy of Fallot (rTOF) often have residual pulmonic valve regurgitation, leading to abnormal remodeling and dysfunction of the right ventricle often requiring pulmonic valve replacement. We tested the hypothesis that 3D analysis of right ventricular (RV) shape and function may reveal differences in regional adaptive remodeling that occurs in patients with rTOF, depending on whether a transannular patch (TAP) was utilized. MethodsForty patients with rTOF who underwent cardiac magnetic resonance imaging (1.5 T), including 20 with and 20 without TAP, and 10 normal controls were studied. Images were analyzed to measure RV endocardial curvature and global and regional volume and function. ResultsRV ejection fraction (EF) was 42 ± 11% in TAP and 38 ± 9% in no-TAP (p = 0.19), both lower than 54 ± 3% in controls (p < 0.01). Left ventricular (LV) EF was 54 ± 9% in TAP, 54 ± 8% in no-TAP (p = 0.87) and 61 ± 16% in controls (both p < 0.01). Indexed LV end-diastolic volumes were higher in no-TAP than in TAP subgroup (p = 0.02). With TAP, mid RV septum showed lower curvature during diastole (less convex), than the mid and apical free walls and free wall adjacent to the RV outflow tract (RVOT; more convex). There were no differences in curvature during systole between rTOF subgroups but mid and RVOT free walls showed higher curvature versus controls. ConclusionsThis is the first study to comprehensively describe the influence of TAP on changes in regional RV shape in patients with rTOF. Understanding these differences may help guide therapeutic options for residual pulmonary valve regurgitation in rTOF patients.

Long-term outcomes following transatrial versus transventricular repair on right ventricular function in tetralogy of Fallot.

Outcomes after repair of tetralogy of Fallot (TOF) are good with either a transventricular (TV) or transatrial (TA) approach. We sought to determine if there is a relationship between the TV or TA approach and right ventricular (RV) function, and the role of residual pulmonary regurgitation (PR) on the long-term outcomes. This was a retrospective cohort multicentric study on survivors after surgical repair of TOF (TA versus TV approach, ±transannular patch) between 1990 and 2004. All patients underwent magnetic resonance imaging to assess RV volume, function, and PR. Patients were matched for length of follow-up and age. Clinical adverse events were retrieved from institutional databases. Seventy-nine patients (TA/TV = 37/42, median age 0.3 and 1.0 yrs, respectively) were included. At a median follow-up of 16.6 years (12.5-20.3), there were no differences in freedom from reintervention (either catheter or surgical), RV volumes, function, and PR between the TA and TV groups. Pulmonary valve (PV) replacement was significantly less frequent in the TA subgroup (P = 0.033) and patients with a preserved PV showed significantly lower RV volumes and less adverse events at follow-up. There is no significant difference in RV volumes and function between the TA and TV. However, the TA approach seems to be protective against PV replacement in the long-term. When PV is not preserved at repair, residual pulmonary regurgitation is a significant cause of late RV dysfunction and dilation, and is associated with a higher rate of late adverse events.

At what age should tetralogy of Fallot be corrected?

Tetralogy of Fallot can be corrected with very low mortality at any age, even in neonates, but this does not necessarily mean that it should be corrected in the neonatal period. Although there are many advantages to early correction, a high proportion of these neonates have residual stenosis or pulmonary regurgitation that impairs ventricular function and may require further surgery or implantation of a pulmonary valve. Before we had the ability to correct this anomaly with low mortality in small children, a variety of palliative procedures had to be performed. Today, with better understanding of the anatomy of tetralogy of Fallot, we should consider what forms of palliation will increase growth of the right ventricular outflow tract in order to reduce the complications of very early surgery.

Erratum: Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair

[This corrects the article on p. 235 in vol. 56, PMID: 23807889.].

NT-proBNP as a biomarker of right ventricular dilatation and pulmonary regurgitation in Tetralogy of Fallot

Background After surgical correction of Tetralogy of Fallot (TOF), residual pulmonary regurgitation (PR), right ventricular (RV) dilatation and RV failure have been associated with adverse clinical events and are considered criteria for pulmonary valve replacement. Cardiovascular magnetic resonance imaging (CMR) is the gold-standard for evaluation of these parameters. However it is an expensive technique which is not readily available in all hospitals and outpatient clinics. NT-proBNP is a biomarker which may overcome these CMR limitations and might be used as a cost-effective biomarker for monitoring patients with TF. The aim of this study is to evaluate whether NTproBNP plasma levels may help as a biomarker for monitoring ventricular dilatation, pulmonary valve regurgitation and heart failure in patients with repaired TF