Left Superior Vena Cava Research Articles

Persistent left superior vena cava with confluence into the left atrium: Missing contrast of the pulmonary arteries in the computed tomographic pulmonary angiography in a patient with pulmonary artery embolism

Persistent left superior vena cava with confluence into the left atrium: Missing contrast of the pulmonary arteries in the computed tomographic pulmonary angiography in a patient with pulmonary artery embolism

Coronary Sinus Anomalies with Case Illustrations

Abstract Coronary sinus (CS) is the largest coronary vein that receives blood from all the venous tributaries of the heart. Its variations in anatomy are significant from the surgical point of view, especially in patients with complex congenital heart diseases (CHDs). CS anomalies are rare, but with increasing advancements in imaging, they are often diagnosed with cardiac computed tomography (CT). These are associated with other CHDs, so the radiologists are alerted to look further for different anomalies. The data in this study were collected from the routine contrast-enhanced CT examinations of the patients referred to the Department of Radiology, Narayana Hrudayalaya Hospital, Bengaluru, from January 2019 to August 2023. It was observed that the anomalies were seldom seen as a single entity and instead were seen as a part of the spectrum of anomalies such as persistent left superior vena cava (LSVC), Raghib complex, total anomalous pulmonary venous connection, and interrupted inferior vena cava. Out of these, the most common associated anomaly was found to be persistent LSVC. These anomalies are essential to detect in the preoperative assessment because they may cause significant morbidity in some cases if left uncorrected. Therefore, we must search for other cardiac anomalies when encountering CS anomalies.

Right-to-left-shunts in patients scheduled for neurosurgical intervention in semi-sitting position – a literature review based on two case scenarios

BackgroundNeurosurgery performed in the semi-sitting position provides advantages for certain procedures. However, this approach is associated with potential complications, particularly venous air embolism. Due to typically negative venous pressure at the wound site, air can be drawn into the veins. This risk is especially high in patients presenting with an intra- or extracardiac right-to-left-shunt. Transoesophageal echocardiography can be used to detect a patent foramen ovale or other possible pulmonary-systemic shunt before placing the patient in the sitting position.Case presentationIn this report, we present two young patients undergoing scheduled microsurgical vestibular schwannoma removal in a semi-sitting position who were diagnosed with congenital heart defects during routine perioperative assessment to detect possible intracardiac right-to-left shunts, using pre- and intraoperative transesophageal echocardiography (TEE) and additionally conducting an agitated saline bubble study under Valsalva manoeuvre. Patient A was diagnosed with a persistent left superior vena cava and Patient B with an unroofed coronary sinus (UCS). These findings confronted the anesthesiological and surgical teams with difficult individual decisions regarding further perioperative management.ConclusionsPerioperative transesophageal echocardiography is a diagnostic tool to both detect intraoperative position-related air embolisms and to rule out intracardiac right-to-left shunts, e.g. a patent foramen ovale, in order to decide for or against a (semi-)sitting position. Depending on the surgical circumstances a semi-sitting positioning of patients presenting with an intracardiac right-to-left-shunt, e.g. a PFO, can be feasible in individual cases if there is an implemented therapeutic algorithm to immediately terminate significant venous air entry. However, since certain other intra- or extracardiac right-to-left-shunts, such as here presented PLSVC or UCS, are rare, there is no definitive way of estimating the amount of entered air through detected shunts or anomalous vessels. Therefore, it is recommended to avoid a (semi-)sitting position in favour of a lateral or prone position for a patient undergoing intracranial surgery, once the perioperative TEE shows air bubbles in the left atrium or ventricle whose origins cannot be defined solely through TEE for certain in order to ensure patient safety by minimizing the risk of intraoperative paradoxical air embolisms.

Successful placement of a chest wall venous infusion port via persistent left superior vena cava: A case report.

Persistent left superior vena cava (PLSVC) is a rare congenital venous anomaly occurring in approximately 0.3% to 0.5% of the population. The presence of PLSVC complicates central venous catheter placement, increasing procedural risks. This case report describes the successful placement of a chest wall venous infusion port in a patient with PLSVC, offering valuable insights for managing similar cases and ensuring safer clinical outcomes. A 51-year-old female, 3 weeks post-right breast cancer surgery, was admitted for her first adjuvant hemotherapy session. She requested the placement of a venous infusion port due to the prolonged duration of chemotherapy. Imaging studies suggested the presence of PLSVC. Echocardiography revealed a dilated coronary sinus, and subsequent chest computed tomography and angiography confirmed the presence of PLSVC. A chest wall venous infusion port was inserted via the left internal jugular vein. Due to the PLSVC, the catheter was adjusted to ensure proper placement. The patient successfully completed chemotherapy without any complications or discomfort associated with the venous port. Imaging studies, including chest X-ray and computed tomography, confirmed proper port function and catheter positioning, with no evidence of thrombosis, infection, or other related issues. The patient remained in good overall condition throughout the treatment. Detailed preoperative evaluations, intraoperative imaging guidance, and postoperative follow-ups are crucial for the safe and effective management of PLSVC patients undergoing central venous catheter placement.

Prenatal diagnosis in fetal right aortic arch using chromosomal microarray analysis and whole exome sequencing: a Chinese single-center retrospective study

BackgroundRight aortic arch (RAA) is a common congenital aortic arch abnormality. Fetuses with RAA frequently have good outcomes after birth. However, chromosomal abnormalities and genetic syndromes suggest poor prognosis for these patients. So far the underlying genetic etiology is still not identified in most RAA patients based on traditional genetic techniques and a problem is still debated whether fetuses with isolated RAA should be referred for CMA. Our study aims to investigate the genetic etiology of fetuses with right aortic arch (RAA) by chromosomal microarray analysis (CMA) and whole exome sequencing (WES) and evaluate the efficacy of CMA in fetal isolated RAA.ResultsAmong these 153 fetuses, 99 (64.7%) with isolated RAA and 54 (35.3%) with non-isolated RAA; 25.5% (39/153) with additional intracardiac anomalies (ICA), and 19.0% (29/153) with extracardiac anomalies (ECA). Tetralogy of Fallot (n = 10) and persistent left superior vena cava (n = 11) are the most common ICA and ECA, respectively. CMA detected 15 clinically significant copy number variations (CNVs) in 14 cases (9.2%); microdeletion of 22q11.21 was the most common pathogenic CNVs (7.8%). The chromosomal abnormalities rate was higher in non-isolated RAA and RAA with ICA groups than in isolated RAA group (16.7% vs. 5.1%; 20% vs. 5.1%, both p < 0.05). From five cases further undergoing WES, a diagnostic variant in MTOR gene (c.7255G > A, de novo) was first reported in prenatal, extending the prenatal manifestation of Smith–Kingsmore syndrome (OMIM: 616638); a clinically relevant variant c.3407A > T in STAG2 was identified, being inherited from the healthy mother. Moreover, the premature birth and termination rates were higher in non-isolated RAA group than in isolated RAA group (11.1% vs. 1.0%; 37.0% vs. 2.0%, both p < 0.01).ConclusionsWe demonstrate that CMA and WES are useful diagnostic tools for fetal RAA, particularly non-isolated RAA, and all fetuses with RAA should be referred for CMA. The data probably aids in prenatal diagnosis and prenatal counseling of fetal RAA.

Maternal exposure to ambient ozone and fetal congenital heart defects: a national multicenter study in China.

Ambient O3 has demonstrated an aggravated increasing trend in the context of global warming. The available evidence of maternal exposure to ambient O3 on fetal congenital heart defects (CHD) is still limited, especially in high polluted areas. To examine associations of maternal exposure to ambient O3 during early pregnancy with fetal CHDs. We conducted a national multicenter study in 1313 hospitals from 26 provinces in China and collected a total of 27,817 participants athigh risk of CHD from 2013 to 2021. Exposure to ambient O3 during the embryonic period, preconception, the first trimester and periconception was assessed by extracting daily concentrations from a validated grid dataset at each subject's residential district. CHDs were diagnosed based on fetal echocardiography. Each 10 µg/m3 increase of exposure to ambient O3 during the embryonic period was approximately linearly associated with a 12.7% (odds ratio [OR]: 1.127, 95% confidence interval [CI]: 1.098, 1.155) increase in odds of pooled CHD (p < 0.001). The associations remain robust after adjusting for ambient PM2.5 and NO2 exposure. The odds of different types of CHD in association with ambient O3 exposure varied greatly. We observed significant association of ambient O3 exposure with ventricular septal defect (VSD), tetralogy of Fallot (TOF); pulmonary stenosis (PS), pulmonary atresia (PA), transposition of great arteries (TGA) and persistent left superior vena cava (PLSVC), with TOF demonstrating the strongest estimates (OR: 1.194, 95% CI:1.107, 1.288). The estimates for preconception, the first trimester and periconception demonstrate consistent findings with the main analyses, indicating stronger associations of ambient O3 exposure during the periconception period. Our study provides evidence that higher ambient O3 during early pregnancy was significantly associated with increased odds of fetal CHD. Our findings suggest that pregnant women, clinical practitioners, and policy makers need to pay more attention to the exposure to higher ambient O3 during early pregnancy to reduce the risk of developing CHD and to improve outcomes across the life span.

Lipomatous hypertrophy of the interatrial septum: A benign cardiac mass.

Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare but benign cardiac tumor that can be found on cardiac imaging such as echocardiogram, or during surgery or an autopsy. Cardiac MRI is the best imaging modality to determine the borders of the tumor and its extension into the intraventricular septum and ventricular free wall. Patients require close monitoring because LHIS may cause right or left outflow tract obstruction or superior vena cava obstruction, requiring cardiac surgical intervention. This article describes a patient with LHIS who underwent cardiac surgery because of her increasing symptomatology.

Redo isolated tricuspid valve replacement in a patient with isolated persistent left superior vena cava: a case report

BackgroundRedo isolated tricuspid valve surgery has high in-hospital mortality and morbidity and is a challenging procedure. We report a successful case of redo isolated tricuspid valve replacement for structural valve deterioration of a bioprosthesis in a patient with isolated persistent left superior vena cava (PLSVC).Case presentationAn 81-year-old man with a history of tricuspid valve replacement using a porcine bioprosthetic valve 9 years previously presented with dyspnea on exertion. Right heart failure due to worsening transvalvular leakage in the bioprosthetic tricuspid valve was considered to be the cause of his symptoms, and the decision was made to replace the tricuspid valve. An isolated PLSVC is considered to be an obstacle in right-sided heart valve surgery. The PLSVC was located deep to the left of the pulmonary artery and, after some effort, was cannulated by obtaining an excellent surgical view using retraction sutures on the left side of the pericardium. Cardiopulmonary bypass was initiated after cannulation of the ascending aorta, PLSVC, and femoral vein. After cross-clamping of the ascending aorta, cold blood cardioplegic arrest was induced under moderate hypothermia, and the PLSVC and inferior vena cava were snared. The right atrium was opened and the prosthetic tricuspid valve was examined. One of the leaflets was shortened, which appeared to cause the transvalvular leak. The prosthetic valve was explanted, the annulus was trimmed, and a new bioprosthetic valve was implanted. The postoperative course was uneventful.ConclusionsIt is important to treat structural valve deterioration of a prosthetic tricuspid valve in a timely manner. We hope that our intervention timing and surgical strategy can help surgeons to consider early intervention in similar cases, even if there are surgical obstacles such as isolated PLSVC.

The Mediastinal Distortions

The case we present involves an atypically developed thoracic aorta, resulting in the consecutive displacement of the trachea and esophagus, combined with the unusual formation of vascular bridges between the ascending and descending thoracic aorta and the arising supraaortic branches. This represents a pathological entity further associated with a cardiac condition of Sick Sinus Syndrome and heterotopy of the excitation center in the pulmonary veins. Multiple descriptions of the combination of dextrocardia and Sick Sinus Syndrome have been reported by Aurora Bakalli et al. 2021 (1), as well as the combination of dextrocardia, persistent left superior vena cava, and Sick Sinus Syndrome by Gangliang Guo et al. 2017 (2), and another case of dextrocardia and Sick Sinus Syndrome by Junqian Luo et al. 2022 (3). Such combinations of anomalies have not been reported in larger statistical collections to date.

Prenatal Diagnosis of Persistent Left Superior Vena Cava Using High-Definition Flow Render Mode and Spatiotemporal Image Correlation.

This study aimed to assess the use of two-dimensional (2D) ultrasound combined with high-definition flow (HD-flow) render mode and spatiotemporal image correlation (STIC) in diagnosing and classifying fetal persistent left superior vena cava (PLSVC). Overall, 114 cases of fetal PLSVC were diagnosed using 2D ultrasound combined with STIC, and 114 normal fetuses of the same gestational week were selected. These cases were retrospectively analyzed to evaluate the effectiveness of the diagnostic approach. All 114 PLSVC cases were diagnosed using 2D ultrasound combined with STIC. Although the diagnostic coincidence rate of PLSVC in the HD-flow combined with STIC was similar to that in the 2D ultrasound combined with HD-flow (96.8 vs 96.2%), 2D ultrasound with STIC enabled dynamic visualization of the PLSVC, furthering prenatal diagnosis. These cases were classified as type I PLSVC: 80 cases of type Ia, 29 cases of type Ib, and 5 cases of type Ic. Seventy isolated PLSVC cases (61.4%) were noted, whereas 44 cases (35.6%) were associated with concomitant structural abnormalities. Intracardiac structural malformations accounted for the highest proportion (n = 53, 58.89%), followed by single umbilical artery and facial/bodily abnormalities (n = 10, 11.11%). Combining HD-flow and STIC complements 2D ultrasound in diagnosing and classifying fetal PLSVC, demonstrating significant clinical relevance.

Extraction of intracardiac electrode located in the posterior interventricular vein, with a mechanical rotational cutting sheath, in a patient with persistent left superior vena cava

Extraction of intracardiac electrode located in the posterior interventricular vein, with a mechanical rotational cutting sheath, in a patient with persistent left superior vena cava

RF catheter ablation of AVNRT in a patient with interrupted inferior vena cava anomaly with hemiazygos continuity with persistent left superior vena cava

Intrahepatic interruption of the inferior vena cava (IVC) with continued hemizygous is a very rare abnormality and sometimes it may be accompanied by other cardiovascular abnormalities. Continuation of the hemizygous vein draining into the right atrium through the left superior vena cava (LSVC) is much rarer. In this paper, we have presented a patient who had simultaneous IVC interrupted with persistent LSVC and suffered from Atrioventricular nodal reentrant tachycardia (AVNRT). Finally, radiofrequencies (RF) catheter ablation for AVNRT was successfully performed through a left subclavian vein access.

Raghib Syndrome and Pulmonary Arterial Hypertension in a Pediatric Patient: Case Report and Literature Review

Background: Raghib syndrome is a rare malformation complex consisting of the drainage of the left superior vena cava (LSVC) into the left atrium, ostial atresia of the coronary sinus and an atrial septal defect (ASD). Case Report: This report aims to present the case of a child newly diagnosed with Raghib syndrome, complicated by pulmonary arterial hypertension, and to review previously published cases with the same diagnosis. A six-year-old female patient presented with signs and symptoms of heart failure (Ross III), reduced exercise tolerance and severe delay in stature and ponderal development. The imagistic work-up included echocardiography, followed by computer tomography (CT) and magnetic resonance imaging (MRI), through which a diagnosis of Raghib syndrome was established, complicated by pulmonary hypertension. As in other cases presented in the literature, MRI allowed for an accurate diagnosis, detecting the absent coronary sinus. The decision regarding the surgical closure of the ASD was made, with the patient having a favorable clinical evolution but with the persistence of elevated pulmonary artery pressure, for which Sildenafil therapy was instituted. Conclusions: The malformation complex consisting of an atrial septal defect, ostium atresia of the coronary sinus, uncovered coronary sinus, and persistent left superior vena cava, as identified through multiple imagistic investigations, was suggestive of the rare diagnosis of Raghib syndrome in this case. Among the limited number of cases of Raghib syndrome available in the literature, the present case is distinguished by the severity of the pulmonary artery hypertension at a very young age and in the absence of other concurrent cardiac malformations.

ORTHOTOPIC HEART TRANSPLANTATION IN A PATIENT WITH A PERSISTENT LEFT SUPERIOR VENA CAVA: СASE REPORT

Introduction. Persistent left superior vena cava is the most common congenital venous abnormality of the thorax and has a prevalence of 0,3-0,5% among the general population. In most cases, the presence of a persistent left superior vena cava is asymptomatic and is usually a finding during surgery. Most often additional left superior vena cava coexists with superior vena cava in 80-90% cases. This feature is extremely important for choosing the optimal surgical tactics. Case Presentation. Patient Sh., 44 years old, was urgently hospitalized for orthotopic heart transplantation. From the anamnesis it is known that in 2017 the patient was diagnosed congenital heart disease: critical stenosis of the bicuspid aortic valve. However, the patient refused surgery at that moment. Conservative treatment was carried out. In 2022, the patient`s condition progressively worsened: severe exertional dyspnea, increased shortness of breath at rest, pitting edema in the lower extremities, interruptions in the work of the heart. The patient is diagnosed: dilated cardiomyopathy. The patient was included in the waiting list on the heart transplantation. In March 2023, patient underwent orthotopic heart transplantation with reconstruction a persistent left superior vena cava. A tubular conduit from the autopericardium with a diameter of 20 mm and a length of 5 cm was used for reconstruction. An anastomosis was formed between the distal part of the accessory left superior vena cava and the right atrial auricle. The patient was discharged without any complications and no signs of venous congestion in the upper body were detected during the follow-up examination after three months of observation. Conclusions. Our clinical observation demonstrates the feasibility of successful orthotopic heart transplantation using an autologous autopericardium graft in patients with a persistent left superior vena cava and shows encouraging immediate follow-up results.

Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients.

In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment details of CHD identified in patients born with ARM, from a unique large cohort of a very rare disease. We performed a retrospective single-center cohort study between January 2000 and July 2023. All consecutive patients with ARM were included. Outcomes were the number of patients with CHD, and screening percentage and percentage of patients diagnosed with CHD over 3 time periods (2000-2006, 2007-2014, 2015-2023). We used uni- and multi-variable logistic regression analyses to search for associations between CHD present and baseline characteristics. In total, 281 patients were included. Some 241 (85.8%) underwent echocardiography, of whom 80 (33.2%) had CHD. Screening percentage with echocardiography increased (74.1% vs. 85.7% vs. 95.9%, p < 0.001) and percentage of patients diagnosed with CHD remained similar over time (30.2% vs. 34.5% vs. 34.0%, p = 0.836). Atrial and ventricular septal defects (n = 36, n = 29), and persistent left superior vena cava (n = 17) were most identified. The presence of VACTERL-association or a genetic syndrome was independently associated with the presence of CHD. CHD were present in 33% of patients with ARM that underwent echocardiography. Over time, the number of CHD identified through screening remained similar. Patients with the presence of VACTERL-association or a genetic syndrome had a higher risk of having CHD. Therefore, acknowledging the potential presence of CHD in patients with ARM remains important.

Cryoballoon isolatiob of pulmonary vein in patient with persistent left superior vena cava and atresia of the right superior vena cava: case report

A clinical case of cryoballon ablation of pulmonary veins in a patient with atrial fibrillation with congenital heart disease: persistent left superior vena cava, atresia of the superior vena cava. The methods of investigation at the preoperative stage are described, which allow to plan the operation in advance, taking into account the peculiarities of the confluence of the main veins into the heart, and also some technical features of cryoballon ablation surgery are emphasized.

Injury to the sinus nodal artery during radiofrequency ablation of atrial fibrillation and atrial tachycardia: its prevalence and treatment with percutaneous coronary intervention

Abstract Background/Introduction Injury to the sinus node artery (SNA) leading to SN dysfunction during radiofrequency catheter ablation (RFCA) of atrial Fibrillation (AF) and atrial tachycardia (AT) has been reported. Purpose The purpose of this study was to elucidate the prevalence and clinical course of SNA injuries during RFCA of AF/AT and examine the efficacy of the percutaneous coronary intervention (PCI) to restore SN function. Methods We retrospectively analyzed data from 6,906 consecutive patients (pts) who underwent RFCA of AF/AT (6,379 pts with AF ablation and 527 pts with AT ablation) in two institutions between January 2013 and December 2022. Results Among 6,906 pts, 9 (0.13%) pts developed SN dysfunction due to SNA injury during RFCA (8 with AF, 1 with AT). Acute SNA injury (total occlusion or severe narrowing of the SNA) was confirmed by coronary angiography (CAG) during the RFCA session in 8/9 pts. In the remaining patient, SNA injury was suspected based on the CT angiography. The culprit sites of the SNA injury were located at the septal site of the superior vena cava-right atrium junction in 4 pts (44%), on the left atrial appendage ridge in 3 (33%), on the roof of the left atrium adjacent to the right superior pulmonary vein in 1 (11%), and in the persistent left superior vena cava in 1 (11%). PCI to the occluded SNA was performed in 8 of the 9 pts: 1) coronary blood flow of the SNA was restored by a 0.014" guidewire advancement into the occluded artery using a micro-catheter followed by direct nitroprusside injection in 4 pts; 2) the direct balloon angioplasty using 0.75-1.5 mm diameter balloons was required to maintain the blood flow of SNA in 3 pts; and 3) CAG revealed spontaneous recovery of the blood flow of SNA in one pt. The remaining patient did not receive the CAG/PCI. All 8 pts who underwent the PCI resulted in restoration of normal sinus rhythm. During a median of 44 months follow-up, all 8 pts who received the PCI maintained normal sinus rhythm, while one patient without PCI demonstrated persistent SN dysfunction. Conclusions SNA injury during RFCA is a rare but critical complication. Prompt recognition by CAG and subsequent PCI effectively restores SN function in pts with unexpected SN dysfunction during RFCA procedures.

Reduced heart failure hospitalizations and improved quality of life in cardiac amyloidosis patients undergoing atrial fibrillation ablation

Abstract Background Cardiac amyloidosis (CA) has been associated with an increased risk of atrial arrhythmias and development and worsening of heart failure due to infiltration of the amyloid protein in the atrial wall. Few data exist regarding prognosis, symptoms, and quality of life after catheter ablation of atrial fibrillation in this category of patients. Objective The study evaluates the outcome of hospitalization for heart failure and quality of life in cardiac amyloidosis patients with atrial fibrillation after catheter ablation. Methods This is a study including 102 consecutive patients from two different centers with an established diagnosis of amyloidosis ATTR that underwent their first AF catheter ablation for persistent AF. They underwent pulmonary vein (PV) isolation + isolation of left atrial posterior wall and superior vena cava. Additionally, extrapulmonary triggers were identified and ablated. Post procedure, patients were followed up routinely with ECG during office visits, 7-day Holter monitor and event recorders during the first year followed by biannual checkups during the remaining part of the follow-up period. Quality of life (QoL) was measured using The Minnesota Living Heart Failure Questionnaire (MLHFQ) before Catheter ablation and after 18 months follow up; QoL was compared with T-test. Results 102 patients (mean age 72 ± 6) were included in the study. After 18 months follow up, 72 patients (70.6%) remained arrhythmia-free without antiarrhythmic drugs (Group 1), whereas 30 patients (29.4%) experienced recurrence (Group 2). In Group 1, 9 patients were hospitalized for heart failure, in contrast to 18 patients in Group 2, indicating a higher rate of heart failure hospitalizations associated with arrhythmia recurrence (p=0.02). The Quality of life in group 1 was 62 ±12 points before ablation and 63±16 points at 18 months follow up (p=0.17) while in group 2 was 68±16 points before ablation and 86±12 points at 18 months follow up (p&amp;lt;0.001). Conclusion Catheter ablation for AF in patients with cardiac amyloidosis is associated with reduced hospitalizations for heart failure, along with improvement in QoL.

Efficacy of catheter ablation in patients with persistent atrial fibrillation on chronic ibrutinib therapy

Abstract Background Ibrutinib represents a cornerstone in the management of B-Cells malignancies and chronic lymphatic leukaemia. This drug however was associated with a 5-fold increased risk of developing atrial fibrillation (AF). The consequences of AF in this category of frailty patients such as heart failure may lead to the need of Ibrutinib withdrawal, which is considered a disease modifying therapy. Purpose This study aims to evaluate the safety and efficacy of catheter ablation in managing AF in patients on chronic ibrutinib therapy. Methods this study evaluated 20 consecutive patients from two centres in Italy who developed persistent AF during chronic therapy with Ibrutinib for chronic lymphatic leukaemia or small lymphocytic lymphoma and underwent AF catheter ablation from 2016 to 2020. Antiarrhythmic drugs were withdrawn because of side effects and drug interaction. All receive pulmonary vein (PV) isolation + isolation of left atrial posterior wall (PW) and superior vena cava. A standardized protocol was performed to confirm persistent PVI and elicit any triggers originating from non-PV sites. Ablation of non-PV sites triggering runs of atrial tachyarrhythmias was left to operator's discretion. Exclusion criteria were age&amp;lt;18 years and history of AF before ibrutinib therapy. Patients were monitored for arrhythmia at quarterly office visits, ECGs, 7-day Holter monitoring and event recorders during the first year followed by biannual checkups during the remaining part of the follow-up period. Results 12 patients received PV+PW+SVC isolation plus ablation of non-PV trigger (Group 1) whereas 8 receive only PV+PW+SVC isolation (Group 2). 10 (83,3%) patients in group 1 remained arrhythmia-free without the need for additional antiarrhythmic drugs while only 3 (37,5%) in group 2 maintained sinus rhythms. Non pulmonary triggers were mostly found in left atrial appendage (5/12, 41,6%), coronary sinus (4/12, 33,3%) and crista terminalis (3/12 25%). More than one trigger was found in 4 (33.3) patients. After 22.3 ±3.6 months follow-up, patients with ablation of induced non-PV triggers had a significantly higher arrhythmia control than those whose triggers were not ablated (Chi – square p value: 0.035; Log rank p value: 0.014) (Fig.1). 5 patient that received left atrial appendage isolation underwent a left atrial appendage closure with Watchman device 3 months after atrial fibrillation ablation. Conclusion Catheter ablation in this category of patients seems to be a safe and effective tool for managing atrial fibrillation in patients with persistent AF on chronic ibrutinib therapy; an ablation strategy that includes as a first line therapy non pulmonary vein triggers appears to be an effective approach when compared to pulmonary vein plus posterior wall only. This approach will allow continuation of ibrutinib treatment, which markedly enhances the prognosis and diminishes the risk of adverse cardiovascular events in this patient population.Kaplan-Meier curve showing recurrence-fr

Prevalence of non pulmonary vein triggers and pulmonary pressure change in atrial fibrillation ablation for cardiac amyloidosis patients

Abstract Background Cardiac amyloidosis (CA) has been associated with an increased risk of atrial arrhythmias and high risk to develop pulmonary hypertension due to infiltration of the amyloid protein in the atrial wall. Though non-PV triggers are commonly seen in left atrium, they are also seen in the right side of the heart, so identifying them at the time of ablation is essential. Objective The study evaluates the prevalence of non-PV triggers during catheter ablation in atrial fibrillation (AF) patients affected by cardiac amyloidosis and how restoration of sinus rhythm affects pulmonary pressures. Methods This is a study including 74 consecutive patients with an established diagnosis of amyloidosis ATTR diagnosed with cardiac MRI or myocardial biopsy that underwent their first AF catheter ablation. All have a transthoracic echocardiogram before ablation. They underwent pulmonary vein (PV) isolation + isolation of left atrial posterior wall and superior vena cava. Additionally, extrapulmonary triggers, which are defined as ectopic triggers originating from sites other than PVs including left atrial appendage (LAA), coronary sinus (CS), interatrial septum, crista terminalis, mitral valve anulus were identified. Post procedure, patients were followed up routinely with ECG during office visits, echocardiogram, 7-day Holter monitor and event recorders during the first year followed by biannual checkups during the remaining part of the follow-up period. Student’s t-test was used to compare pulmonary pressure between the groups. Results Isoproterenol-challenge revealed non-PV triggers in 51 (68.9%) patients. These triggers were mostly mapped to LAA (39, 52.7%), CS (30, 40,5%), and crista terminalis (35, 47.3%). Besides, ectopic beats were seen originating from inter-atrial septum (19, 25.6%), mitral valve annulus (10, 13.5%). More than one trigger was found in (68%) patients. At 1-year after the ablation, 65 (87.8%) were arrhythmia-free off antiarrhythmic drugs. Moreover, in patients who maintained sinus rhythm post-ablation, there was either a non progression or a reduction in pulmonary pressures (PAPm before ablation: 44.8 ± 8.9 mmHg vs PAPm after ablation 45.1 ± 11.1 mmHg, P value =0.87). Conversely, those experiencing arrhythmic recurrences demonstrated an increase in pulmonary pressures (PAPm before ablation: 41.9 ± 7.7 mmHg vs PAPm after ablation 47.8 ± 10.2 mmHg, P value =0.019). Fig.1 Conclusion Our findings suggest that non-PV triggers are highly prevalent in cardiac amyloidosis AF patients including a high rate crista terminalis firing. Notably, restoration of sinus rhythm post-ablation was linked to stable or reduced pulmonary arterial pressures, whereas patients with arrhythmic recurrences experienced a significant increase.