scholarly journals Right-left ventricular shape variations in tetralogy of Fallot: associations with pulmonary regurgitation

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Charlène A. Mauger ◽  
Sachin Govil ◽  
Radomir Chabiniok ◽  
Kathleen Gilbert ◽  
Sanjeet Hegde ◽  
...  

Abstract 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 < 0.05). Increasing PRV was associated with RV dilation and basal bulging, in conjunction with decreased LV septal-lateral dimension (LV flattening) and systolic septal motion towards the RV (all p < 0.05). Increased global RV radial, longitudinal, circumferential and LV radial systolic strains were significantly associated with increased PRV (all p < 0.05). 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.

2013 ◽  
Vol 23 (6) ◽  
pp. 915-920 ◽  
Author(s):  
Cheul Lee ◽  
Chang-Ha Lee ◽  
Jae Gun Kwak

AbstractPulmonary valve replacement is being performed with increasing frequency in patients with various congenital heart diseases. Chronic pulmonary regurgitation after repair of tetralogy of Fallot is a typical situation that requires pulmonary valve replacement. Chronic pulmonary regurgitation after repair of tetralogy of Fallot can lead to right ventricular dilatation, biventricular dysfunction, heart failure symptoms, arrhythmias, and sudden death. Although pulmonary valve replacement can lead to improvement in functional class and a substantial decrease or normalisation of right ventricular volumes, the optimal timing of pulmonary valve replacement in patients with chronic pulmonary regurgitation is still unknown. There are several options for surgical pulmonary valve replacement. However, no ideal pulmonary valve substitute exists currently and most of the surgically implanted pulmonary valves will eventually require re-replacement. This article will review options and timing of surgical pulmonary valve insertion in patients with chronic pulmonary regurgitation after repair of tetralogy of Fallot.


2019 ◽  
Vol 20 (9) ◽  
pp. 1020-1026 ◽  
Author(s):  
Ganesh Kumar Gnanappa ◽  
David S Celermajer ◽  
Danyi Zhu ◽  
Rajesh Puranik ◽  
Julian Ayer

Abstract Aims Pulmonary regurgitation (PR) and right ventricular (RV) dilatation are common in repaired tetralogy of Fallot (rTOF). Left ventricular (LV) dysfunction is an important risk factor in rTOF. The effect of PR/RV dilatation on LV performance and RV-LV interactions in rTOF are incompletely understood. We examined LV responses and exercise capacity in rTOF, both before and after pulmonary valve replacement (PVR). Methods and results Cardiac magnetic resonance imaging scans in 126 rTOF patients (age 17.3 ± 7.6 years) were analysed, comparing subjects with indexed RV end-diastolic volume (RVEDVi) <170 mL/m2 (mild/moderate dilatation, n = 95) and RVEDVi ≥170 mL/m2 (severe dilatation, n = 31). Indexed PR volume (PRVi), RV end-systolic (RVESVi), RV end-diastolic (RVEDVi), RV stroke volume (RVSVi), net pulmonary forward flow (NPFFi), LV end-diastolic (LVEDVi), LV end-systolic (LVESVi), LV stroke volume (LVSVi), RV and LV ejection fraction (EF), and diastolic septal curvature were obtained. Peak aerobic capacity (VO2 max) was measured. In a subset (n = 30), measures were obtained pre-and-post surgical PVR. Compared to those with mild/moderate RV dilatation, patients with severe RV dilation had greater PRVi (38 ± 12 vs. 24 ± 9 mL/m2, P < 0.0001), NPFFi (53 ± 9 vs. 44 ± 11 mL/m2, P < 0.0001), LVEDVi (87 ± 14 vs. 73 ± 13 mL/m2, P < 0.0001), LVESVi (39 ± 12 vs. 30 ± 8 mL/m2, P < 0.0001), and LVSVi (48 ± 7 vs. 43  ±  8 mL/m2, P = 0.002) but lower RV ejection fraction (46 ± 8 vs. 53 ± 7%, P < 0.0001). Septal curvature and VO2 max were similar in both groups. After PVR, there was no change in LVEDVi, LVSVi, septal curvature, or VO2 max. Conclusions Chronic PR with severe RV dilatation is associated with increased NPFFi, LVEDVi, and LVSVi. This may potentially explain preserved exercise capacity in rTOF with severe PR and RV dilatation.


Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi

Abstract OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.


2018 ◽  
Vol 315 (6) ◽  
pp. H1691-H1702 ◽  
Author(s):  
Pia Sjöberg ◽  
Johannes Töger ◽  
Erik Hedström ◽  
Per Arvidsson ◽  
Einar Heiberg ◽  
...  

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.


2019 ◽  
Vol 21 (8) ◽  
pp. 906-913 ◽  
Author(s):  
Imran Rashid ◽  
Adil Mahmood ◽  
Tevfik F Ismail ◽  
Shamus O’Meagher ◽  
Shelby Kutty ◽  
...  

Abstract Aims The optimal timing for pulmonary valve replacement in asymptomatic patients with repaired Tetralogy of Fallot (rTOF) and pulmonary regurgitation remains uncertain but is often guided by increases in right ventricular (RV) end-diastolic volume. As cardiopulmonary exercise testing (CPET) performance is a strong prognostic indicator, we assessed which cardiovascular magnetic resonance (CMR) parameters correlate with reductions in exercise capacity to potentially improve identification of high-risk patients. Methods and results In all, 163 patients with rTOF (mean age 24.5 ± 10.2 years) who had previously undergone CMR and standardized CPET protocols were included. The indexed right and left ventricular end-diastolic volumes (RVEDVi, LVEDVi), right and left ventricular ejection fractions (RVEF, LVEF), indexed RV stroke volume (RVSVi), and pulmonary regurgitant fraction (PRF) were quantified by CMR and correlated with CPET-determined peak oxygen consumption (VO2) or peak work. On univariable analysis, there was no significant correlation between RVEDVi and PRF with peak VO2 or peak work (% Jones-predicted). In contrast, RVEF and RVSVi had significant correlations with both peak VO2 and peak work that remained significant on multivariable analysis. For a previously established prognostic peak VO2 threshold of &lt;27 mL/kg/min, receiver-operating characteristic curve analysis demonstrated a Harrell’s c of 0.70 for RVEF (95% confidence interval 0.61–0.79) with a sensitivity of 88% for RVEF &lt;40%. Conclusion In rTOF, CMR indices of RV systolic function are better predictors of CPET performance than RV size. An RVEF &lt;40% may be useful to identify prognostically significant reductions in exercise capacity in patients with varying degrees of RV dilatation.


2016 ◽  
Vol 27 (3) ◽  
pp. 498-506 ◽  
Author(s):  
Luís Almeida-Morais ◽  
Tiago Pereira-da-Silva ◽  
Luísa Branco ◽  
Ana T. Timóteo ◽  
Ana Agapito ◽  
...  

AbstractObjectiveThe role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients.MethodsPatients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI. Right ventricular longitudinal strain was evaluated in the four-chamber view using the standard semiautomatic method.ResultsIn total, 42 patients were included (61% male, 32±8 years). The mean right ventricular longitudinal strain was −16.2±3.7%, and the right ventricular ejection fraction, measured by MRI, was 42.9±7.2%. Longitudinal strain showed linear correlation with tricuspid annular systolic excursion (r=−0.40) and right ventricular ejection fraction (r=−0.45) (all p<0.05), which in turn showed linear correlation with right ventricular fractional area change (r=0.50), pulmonary regurgitation colour length (r=0.35), right ventricular end-systolic volume (r=−0.60), and left ventricular ejection fraction (r=0.36) (all p<0.05). Longitudinal strain (β=−0.72, 95% confidence interval −1.41, −0.15) and left ventricular ejection fraction (β=0.39, 95% confidence interval 0.11, 0.67) were independently associated with right ventricular ejection fraction. The best threshold of longitudinal strain for predicting a right ventricular ejection fraction of <40% was −17.0%.ConclusionsRight ventricular longitudinal strain is a powerful method for evaluating patients with tetralogy of Fallot. It correlated with echocardiographic right ventricular function parameters and was independently associated with right ventricular ejection fraction derived by MRI.


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