Novel use of an atrial sensing leadless pacemaker to treat complete heart block in a patient with repaired tetralogy of Fallot with pre-existing dual-chamber pacemaker with ventricular lead fracture.
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ABSTRACT:
SUBMITTER: Needleman M
PROVIDER: S-EPMC7573471 | biostudies-literature | 2020 Oct
REPOSITORIES: biostudies-literature
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Novel use of an atrial sensing leadless pacemaker to treat complete heart block in a patient with repaired tetralogy of Fallot with pre-existing dual-chamber pacemaker with ventricular lead fracture.
Needleman Matthew M Symons John J Weber Lauren A LA Mulreany Michael M May Joseph J Haigney Mark C MC
HeartRhythm case reports 20200731 10
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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.
| S-EPMC6829761 | biostudies-literature
Project description:BackgroundSome adults suffer sudden cardiac death after previous surgical repair of tetralogy of Fallot (TOF), and in such cases, ventricular tachycardia is believed to be the most frequent cause of death. However, we report a case of cardiac arrest due to paroxysmal complete atrioventricular block in an adult with dextrocardia and repaired TOF.Case summaryA 49-year-old woman with dextrocardia and a history of surgical treatment for TOF lost consciousness three times. A previously implanted loop recorder showed a 60-second cardiac arrest, and complete atrioventricular block was diagnosed. An electrophysiological study showed prolongation of the His-ventricular interval but no ventricular tachycardia. A dual chamber pacemaker was implanted, and there has been no recurrence of syncope in the 23 months since implantation.DiscussionThere is little evidence for paroxysmal complete atrioventricular block in patients with repaired TOF. This case suggests that paroxysmal complete atrioventricular block can occur late after surgical repair of TOF, and research needs to elucidate whether it is the cause of sudden cardiac death in some patients with TOF.
| S-EPMC9671030 | 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.
| S-EPMC4845286 | biostudies-other
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.
| S-EPMC7475380 | biostudies-literature
Project description:Background and purpose Electromechanical 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. Methods Sixty-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). Results All 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). Conclusion RV 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
| S-EPMC9996188 | biostudies-literature
Project description:BackgroundA dual-chamber leadless pacemaker system has been designed for atrioventricular synchronous pacing using wireless, beat-to-beat, implant-to-implant (i2i) communication between distinct atrial and ventricular leadless pacemakers. The atrioventricular synchrony achieved across various ambulatory scenarios has yet to be systematically evaluated.MethodsA prospective, single-arm, unblinded, multicenter, international clinical trial of the leadless pacemaker system was conducted in patients with a conventional dual-chamber pacing indication enrolled from February 2022 to March 2023. Leadless pacemaker systems were implanted, and 12-lead Holter electrocardiographic recordings were collected 3 months after implantation over various postures/activities: sitting, supine, left lateral recumbent, right lateral recumbent, standing, normal walk, and fast walk. An independent Holter core laboratory performed a manual adjudication of the percent of atrioventricular synchronous beats using the standard 300-millisecond PR interval limit. Atrium-to-ventricle and ventricle-to-atrium i2i communication success rates were also assessed. Post hoc summary statistics describing the relationships between atrioventricular synchrony and i2i success, posture/activity, implantation indication, atrioventricular event, and heart rate were calculated.ResultsIn the evaluable population (n=384 of 464 enrolled [83%]; 61% male; age, 70 years; weight, 82 kg; 60% ejection fraction; 95% of beats evaluable), the mean atrioventricular synchrony of 98% of beats observed across all postures using the standard 300-millisecond limit was greater than both atrial-to-ventricular i2i (94%) and ventricular-to-atrial i2i (94%; P<0.001), exceeding both i2i values in 95% of patients. Atrioventricular synchrony was achieved in >95% of evaluable beats across all postures/activities, implantation indications, atrioventricular paced/sensed event combinations, and heart rate ranges (including >100 bpm).ConclusionsThis dual-chamber leadless pacemaker system demonstrated atrioventricular synchrony in 98% of evaluable beats at 3 months after implantation. Atrioventricular synchrony was maintained across postures/activities and remained robust for heart rates >100 bpm.
| S-EPMC11305627 | biostudies-literature
Project description:ObjectivesThis study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA).BackgroundTo date there is no robust risk stratification scheme to predict outcomes in adults with rTOF.MethodsConsecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers.ResultsThe primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value.ConclusionsWe present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.
| S-EPMC8821017 | biostudies-literature
Project description:Takotsubo syndrome or broken-heart syndrome is a rare form of nonischemic cardiomyopathy characterized by regional systolic dysfunction of the left ventricle without evidence of coronary artery disease or acute plaque rupture. This transient impairment in myocardial contractility leads to symptoms and signs that can mimic a myocardial infarction. We present a case of Takotsubo syndrome in a 47-year-old premenopausal woman with complex congenital heart disease who initially presented with acute onset of shortness of breath and chest tightness after a verbal altercation. Extremely rare cases of Takotsubo syndrome have been described in the congenital heart disease population in premenopausal women. This case emphasizes the need to highlight acquired cardiac disease in patients with adult congenital heart disease as this cohort continues to age.
| S-EPMC11775812 | biostudies-literature
Project description:Arterial desaturation following surgical repair of tetralogy of Fallot (TOF) is rare. In most instances, it results from residual right ventricular outflow tract obstruction, causing right-to-left shunt across residual interatrial or interventricular communication. In this report, we present an unusual scenario of arterial desaturation due to a recanalized left cardinal vein in a child with repaired TOF. We also discuss stepwise evaluation that led to successful identification and occlusion of the abnormal venous channel by percutaneous device closure.
| S-EPMC11573195 | biostudies-literature
Project description:BackgroundTetralogy of Fallot (TOF) is one of the most widespread cyanotic congenital heart disease (CHD), which can be successfully repaired in the neonatal period. However, residual problems and the surgical technique itself can create a favourable basis for arrhythmias and conduction abnormalities in these patients. Sometimes, these arrhythmias may worsen during pregnancy and require urgent intervention.Case summaryThis is a case of a 25-year-old woman, who underwent a surgical repair of TOF at the age of 2 years. She suffered an ischaemic stroke postoperatively, which was complicated with secondary seizures and syncope. These episodes were evaluated as epilepsy. The patient was admitted to our hospital with the above-mentioned complaints in the 10th week of pregnancy. A comprehensive cardiac examination was performed. Her presyncopal event was captured during Holter monitoring, which documented a severe dysfunction of the sinus node. She was diagnosed with postoperative sick sinus syndrome and implanted with a permanent dual-chamber pacemaker (PM). After the operation, the patient did not have any episodes of syncope or seizures and the PM check-up showed almost 30% of atrial pacing.ConclusionNo matter how obvious the neurological or other nature of syncope may seem, it is advised to exclude the cardiac origin of syncope, especially in patients with repaired CHD. One of the most common complications after surgery of CHD is rhythm and conduction disturbances. In some of these cases, permanent PM implantation can be unavoidable, even during pregnancy. The implantation of the PM device during pregnancy can be performed safely.
| S-EPMC9161714 | biostudies-literature