Perventricular Transcatheter Pulmonary Valve Implantation in a Symptomatic 3-Year-Old Child With Repaired Tetralogy of Fallot.
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ABSTRACT:
SUBMITTER: Mejia E
PROVIDER: S-EPMC8311154 | biostudies-literature | 2021 May
REPOSITORIES: biostudies-literature
Publications
Perventricular Transcatheter Pulmonary Valve Implantation in a Symptomatic 3-Year-Old Child With Repaired Tetralogy of Fallot.
Mejia Ernesto E George Renelle S RS Lozier John S JS Bocks Martin L ML
JACC. Case reports 20210414 5
In select patients, transcatheter pulmonary valve replacement through a percutaneous approach can be challenging because of complicated anatomy or small patient size. In these patients, especially those weighing <20 kg, hybrid perventricular valve delivery may provide a preferred alternative approach. (<b>Level of Difficulty: Intermediate.</b>). ...[more]
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Project description:Transthoracic echocardiography plays a pivotal role in the diagnosis of complications, evaluation of hemodynamics, and management of patients with surgically repaired congenital heart disease. Late complications of surgically corrected tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease, include pulmonary regurgitation (PR), ventricular septal defect (VSD) patch leakage, and residual right ventricular outflow tract obstruction. We present a case of severe PR secondary to Bartonella endocarditis in an adult with a history of repaired TOF in which echocardiography was instrumental in the diagnosis of severe PR, residual VSD, and a right-to-left shunt through an unsuspected patent foramen ovale.
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Project description:BackgroundIn repaired tetralogy of Fallot (rTOF), abnormal left ventricular (LV) rotational mechanics are associated with adverse clinical outcomes. We performed a comprehensive analysis of LV rotational mechanics in rTOF patients using cardiac magnetic resonance (CMR) prior to and following surgical pulmonary valve replacement (PVR).MethodsIn this single center retrospective study, we identified rTOF patients who (1) had both a CMR ≤ 1 year before PVR and ≤ 5 years after PVR, (2) had no other intervening procedure between CMRs, (3) had a body surface area > 1.0 m2 at CMR, and (4) had images suitable for feature tracking analysis. These subjects were matched to healthy age- and sex-matched control subjects. CMR feature tracking analysis was performed on a ventricular short-axis stack of balanced steady-state free precession images. Measurements included LV basal and apical rotation, twist, torsion, peak systolic rates of rotation and torsion, and timing of events. Associations with LV torsion were assessed.ResultsA total of 60 rTOF patients (23.6 ± 7.9 years, 52% male) and 30 healthy control subjects (20.8 ± 3.1 years, 50% male) were included. Compared with healthy controls, rTOF patients had lower apical and basal rotation, twist, torsion, and systolic rotation rates, and these parameters peaked earlier in systole. The only parameters that were correlated with LV torsion were right ventricular (RV) end-systolic volume (r = - 0.28, p = 0.029) and RV ejection fraction (r = 0.26, p = 0.044). At a median of 1.0 year (IQR 0.5-1.7) following PVR, there was no significant change in LV rotational parameters versus pre-PVR despite reductions in RV volumes, RV mass, pulmonary regurgitation, and RV outflow tract obstruction.ConclusionIn this comprehensive study of CMR-derived LV rotational mechanics in rTOF patients, rotation, twist, and torsion were diminished compared to controls and did not improve at a median of 1 year after PVR despite favorable RV remodeling.
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Project description:We present the case of a 60-year-old male patient who underwent tetralogy of Fallot repair at 7 years of age and then developed severe degenerative mitral regurgitation during adulthood. Given the increased surgical risk (obesity, obstructive sleep apnea syndrome, and reoperation), the patient underwent a successful microinvasive mitral valve repair with neochordae implantation. (Level of Difficulty: Advanced.).
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Project description:Our goal was to compare results between a standard computed tomography (CT)-based strategy, the 'three-step preoperative sequential planning' (3-step PSP), for pulmonary valve replacement in repaired tetralogy of Fallot versus a conventional planning approach. We carried out a retrospective study with unmatched and matched groups. The 3-step PSP comprised the planning of mediastinal re-entry, cannulation for cardiopulmonary bypass (CPB) and the main procedure, using standard 3-dimensional videos. Operative times (skin incision to CPB, CPB time, end of CPB to skin closure and cross-clamp time) as well as postoperative length of stay and in-hospital mortality were compared. Eighty-two patients (49% classical tetralogy of Fallot) underwent an operation (85% with pulmonary homograft) with 1.22% in-hospital mortality. The 3-step PSP (n = 14) and the conventional planning (n = 68) groups were compared. There were no statistically significant differences in the preoperative characteristics. Differences were observed in the total operative time (P = 0.009), skin incision to CPB (P = 0.034) and cross-clamp times (74 ± 33 vs 108 ± 47 min; P = 0.006), favouring the 3-step PSP group. Eight matched pairs were compared showing differences in the total operative time (263 ± 44 vs 360 ± 66 min; P = 0.008), CPB time (123 ± 34 vs 190 ± 43 min; P = 0.008) and postoperative length of stay (P = 0.031), favouring the 3-step PSP group. In patients with repaired tetralogy of Fallot undergoing pulmonary valve replacement, preoperative planning using a standard CT-based strategy, the 3-step PSP, is associated with shorter operative times and shorter postoperative length of stay.
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Project description:BackgroundPulmonary regurgitation (PR), though well tolerated for short term in patients with repaired tetralogy of Fallot (rTOF), could lead to right ventricular (RV) dysfunction, arrhythmias, and sudden cardiac death. Pulmonary valve replacement (PVR), considered as the gold-standard treatment for PR, is performed to mitigate these late effects. In this study, we aimed to evaluate the midterm outcomes and predictors of adverse clinical outcomes (ACO).MethodsFrom May 2014 to December 2017, 42 patients with rTOF undergoing surgical or transcatheter PVR in our department were retrospectively included. Cardiovascular magnetic resonance was performed before PVR (pre-PVR), early after PVR (early post-PVR), and midterm after PVR (midterm post-PVR). Medical history and individual data were collected from medical records. ACO included all-cause death, new-onset arrhythmia, prosthetic valve failure, and repeat PVR.ResultsThe median follow-up duration was 4.7 years. PVR was performed at a median age of 21.6 years. There was no early or late death. Freedom from ACO at 3 and 5 years was 88.1 ± 5% and 58.2 ± 9%, respectively. RV end-diastolic volume index (RVEDVI) and end-systolic volume index (RVESVI) significantly reduced early after PVR and further decreased by midterm follow-up (pre-PVR vs. early post-PVR vs. midterm post-PVR: RVEDVI, 155.2 ± 34.7 vs. 103.8 ± 31.2 vs. 95.1 ± 28.6 ml/m2, p < 0.001; RVESVI, 102.9 ± 28.5 vs. 65.4 ± 28.2 vs. 57.7 ± 23.4 ml/m2, p < 0.001). Multivariable analysis revealed that the occurrence of ACO was significantly increased in patients with lower left ventricular end-systolic volume index.ConclusionsA significant reduction of RV volume occurred early after PVR, followed by a further improvement of biventricular function by midterm follow-up. The midterm freedom from ACO was favorable.
| S-EPMC9120843 | biostudies-literature
Project description:Ventricular arrhythmia is an important cause of late death in patients with repaired tetralogy of Fallot (rTOF). By using an rTOF canine model, we investigated the role of repolarization alternans and its electrophysiological mechanisms.Six dogs received right ventricular outflow tract (RVOT) transannular patch, pulmonary valve destruction, and right bundle branch ablation to simulate rTOF. After 1 year, we performed high-resolution dual-voltage and calcium optical mapping to record action potentials and calcium transients on the excised right ventricular outflow tract wedges. Another 6 dogs without operation served as control. The rTOF group was more susceptible to action potential duration alternans (APD-ALT) and spatially discordant APD-ALT than control (threshold for APD-ALT: 516±36 vs 343±36 ms; P=0.017; threshold for discordant APD-ALT: 387±30 vs 310±14 ms; P=0.046). We detected 2 episodes of ventricular tachycardia in the rTOF group, but none in the control. Expressions of Kv4.3 and KChIP2 decreased in the rTOF group. Expression of connexin 43 also decreased in the rTOF group with a corresponding decrease of conduction velocity and might contribute to spatially discordant APD-ALT. We also found distinct electrophysiological features of the RVOT, including biphasic relationship between magnitude of APD-ALT and pacing cycle length, uncoupling of APD-ALT, and calcium transients alternans, and shortened APD, but unchanged, APD restitution in rTOF.We demonstrated novel electrophysiological properties of the RVOT. In an rTOF model, the RVOT exhibits increased susceptibility to temporal and spatially discordant APD-ALT, which was not totally dependent on calcium transient alternans.
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Project description:Pulmonary regurgitation is the most important sequellae after correction of Tetralogy of Fallot and has a considerable impact over the right ventricle. Surgery has demonstrated low early mortality after pulmonary valve replacement and good long-term outcomes, remaining nowadays the gold standard treatment of pulmonary regurgitation in rTOF patients. Nevertheless, transcatheter pulmonary valve implantation has emerged as a new, safe and efficient alternative to surgical valve replacement. In this review article, we try to evaluate and compare both techniques to find out which is the best therapeutic option in this patients.
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Project description:Background and purposeElectromechanical dyssynchrony, manifested by right bundle branch block and regional wall mechanical dysfunction, contributes to inefficient RV function in repaired Tetralogy of Fallot (ToF). This study aims to evaluate the synchronicity of multiple RV walls using two-dimensional multi-plane echocardiography (2D-MPE) in order to augment current understanding of the mechanisms behind RV dyssynchrony.MethodsSixty-nine adult ToF patients [aged 33 (23-45) years; 61% male] and twenty-five matched healthy controls underwent deformational analysis of the RV lateral, anterior, inferior and septal walls following 2D-MPE acquisitions. RV synchronicity was assessed by the intra-RV deformation delay between each basal RV wall and mid-septal segment in addition to mechanical dispersion calculated across four, six and eight segments (MD).ResultsAll RV wall-septum delays plus MD-4 and MD-6 indices were significantly greater in ToF patients compared to healthy controls (p < 0.001-0.03). In ToF patients, the lateral and anterior RV walls were last to reach peak deformation and anterior wall longitudinal strain was lower (p = 0.001). Post systolic shortening of at least one RV wall segment was identified in 19 (28%) ToF patients. Despite similar ECG characteristics, lateral and anterior wall-septum delays were significantly longer in patients with greater degrees of dyssynchrony (73 [37-108]ms vs. 37 [0-63]ms, p = 0.006; 91 [52-116]ms vs. 41 [1-69]ms, p = 0.013), although RV ejection fraction (RVEF) was not significantly lower. MD-4 and MD-8 indices displayed moderate negative associations with RVEF, strengthened by inclusion of lateral wall longitudinal strain (r = 0.64/0.65; p ≤0.01).ConclusionRV dyssynchrony in ToF is characterised by electromechanical delays between the lateral, anterior and septal walls, with anterior wall dysfunction likely associated with surgical repair of the RV outflow tract. Prospectively, 2D-MPE may have an emerging role evaluating RV mechanical response to electrical resynchronisation therapy.
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Project description:ObjectivesRisk factors associated with adverse cardiac events (cardiac AEs) after pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot are incompletely understood. In this study, we aimed to determine the relationship between histological myocardial fibrosis and cardiac AEs after PVR in patients with rTOF.MethodsWe consecutively collected clinical, cardiac magnetic resonance, echocardiography and electrocardiogram data of 51 patients with rTOF who underwent surgical PVR. The right ventricular outflow tract tissue was collected during the PVR and the degree of histological myocardial fibrosis was determined by a tailor-made automated image analysis method of picrosirius red staining.ResultsThe median follow-up time was 4.9 years, and 14 patients had cardiac AEs (a composite of heart failure admission and arrhythmia) during follow-up. The total analysis area of myocardial samples was 5782.18 mm2, and the median percentage of myocardial fibrosis was 20.6% (interquartile range 16.7-27.0%), which were significantly elevated in patients with cardiac AEs compared with patients without cardiac AEs (24.1% vs 19.7%, P = 0.007). Right ventricular ejection fraction and left ventricular end-systolic volume index were significantly associated with myocardial fibrosis in multivariable stepwise linear regression analysis (R2 = 0.238). Cox proportional hazards regression identified degree of myocardial fibrosis [hazard ratio 1.127; 95% confidence interval (CI) 1.047-1.213; P = 0.001] and age at PVR (hazard ratio 1.062; 95% CI 1.010-1.116; P = 0.019) were associated with increased risk of cardiac AEs. The incidence of adverse cardiac events was significantly increased when myocardial fibrosis >20.1% and age at PVR >18.2 years.ConclusionsHistological myocardial fibrosis was associated with biventricular systolic functions in rTOF. Higher myocardial fibrosis and older age at PVR are independent risk factors for the adverse cardiac events after PVR in patients with rTOF.
| S-EPMC10023241 | biostudies-literature
Project description:BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. It is generally diagnosed and surgically repaired early in life, with good overall outcomes.Case presentationWe report the case of a patient incidentally diagnosed with paucisymptomatic TOF at the age of 56 years old, during investigations for carbon monoxide poisoning. The patient had a history of thyroidectomy, arterial hypertension, and four uncomplicated vaginal deliveries.ConclusionsThis case shows us that some patients with TOF can reach older ages without surgical correction. Late surgical repair should be meticulously decided on a case basis.
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