ACR Appropriateness Criteria® Pulmonary Arteriovenous Malformation (PAVM): 2023 Update.

Pulmonary arteriovenous malformations (PAVMs) occur in 30% to 50% of patients with hereditary hemorrhagic telangiectasia. Clinical presentations vary from asymptomatic disease to complications resulting from the right to left shunting of blood through the PAVM such as paradoxical stroke, brain abscesses, hypoxemia, and cardiac failure. Radiology plays an important role both in the diagnosis and treatment of PAVM. Based on different clinical scenarios, the appropriate imaging study has been reviewed and is presented in this document. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Evaluation of an Optimized Workflow for the Radiofrequency Catheter Ablation of Paroxysmal Atrial Fibrillation.

BACKGROUND A significant number of atrial fibrillation (AF) recurrences occur after initial ablation, often due to pulmonary vein reconnections or triggers from non-pulmonary veins. MATERIAL AND METHODS Patients with paroxysmal AF who underwent radiofrequency catheter ablation for the first time were enrolled. Base on propensity score matching (1: 1 matching), 118 patients were selected for an optimized workflow for the radiofrequency catheter ablation of paroxysmal AF (OWCA) group and a conventional group. Comparative analysis of the acute and 12-month clinical outcomes was conducted. Moreover, an artificial intelligence analytics platform was used to evaluate the quality of pulmonary vein isolation (PVI) circles. RESULTS PVI was successfully achieved in all patients. Incidence of first-pass isolation of bilateral PVI circles was higher (P=0.009) and acute pulmonary vein reconnections was lower (P=0.027) in the OWCA group than conventional group. The OWCA group displayed a significant reduction in the number of fractured points (P<0.001), stacked points (P=0.003), and a greater proportion of cases in which the radiofrequency index achieved the target value (P=0.003). Additionally, the contact force consistently met the force over time criteria (P<0.001) for bilateral PVI circles in the OWCA group, accompanied by a shorter operation time (P=0.017). During the 12-month follow-up period, the OWCA group exhibited a higher atrial arrhythmia-free survival rate following the initial ablation procedure than did the conventional group. CONCLUSIONS The optimized workflow for radiofrequency catheter ablation of paroxysmal AF could play a crucial role in creating higher quality PVI circles. This improvement is reflected in a significantly elevated 12-month atrial arrhythmia-free survival rate.

Recurrences after Pulsed Field Ablation of Atrial Fibrillation: Incidence, Mechanisms, Predictors, and Comparison with Thermal Energy.

Pulsed Field Ablation (PFA) is the latest and most intriguing technology for catheter ablation of atrial fibrillation, due to its capability to generate irreversible and cardiomyocytes-selective electroporation of cell membranes by delivering microsecond-lasting high-voltage electrical fields, leading to high expectations. The first trials to assess the clinical success of PFA, reported an arrhythmia-free survival at 1-year of 78.5%, while other trials showed less enthusiastic results: 66.2% in paroxysmal and 55.1% in persistent AF. Nevertheless, real world data are encouraging. The isolation of pulmonary veins with PFA is easily achieved with 100% acute success. Systematic invasive remapping showed a high prevalence of durable pulmonary vein isolation at 75 and 90 days (range 84-96%), which were significatively lower in redo procedures (64.3%). The advent of PFA is prompting a reconsideration of the role of the autonomic nervous system in AF ablation, as PFA-related sparing of the ganglionated plexi could lead to the still undetermined effect on late arrhythmias' recurrences. Moreover, a new concept of a blanking period could be formulated with PFA, according to its different mechanism of myocardial injury, with less inflammation and less chronic fibrosis. Finally, in this review, we also compare PFA with thermal energy.

Determination of Continuity Index Values in Atrial Fibrillation Ablation with Proactive Esophageal Cooling.

Radiofrequency (RF) ablation to perform pulmonary vein isolation (PVI) for the treatment of atrial fibrillation involves some risk to collateral structures, including the esophagus. Proactive esophageal cooling using a dedicated device has been granted marketing authorization by the Food and Drug Administration (FDA) to reduce the risk of ablation-related esophageal injury due to RF cardiac ablation procedures, and more recent data also suggest that esophageal cooling may contribute to improved long-term efficacy of treatment. A mechanistic underpinning explaining these findings exists through the quantification of lesion placement contiguity defined as the Continuity Index (CI). Kautzner et al. quantified the CI by the order of lesion placement, such that whenever a lesion is placed non-adjacent to the prior lesion, the CI is incremented by the number of segments the catheter tip has moved over. To facilitate real-time calculation of the CI and encourage further adoption of this instrument, we propose a modification in which the placement of non-adjacent lesions increments the CI by only one unit, avoiding the need to count potentially nebulous markers of atrial segmentation. The objective of this protocol is to describe the methods of calculating the CI both prospectively during real-time PVI cases and retrospectively using recorded case data. A comparison of the results obtained between cases that utilized proactive esophageal cooling and cases that used luminal esophageal temperature (LET) monitoring is then provided.

Scimitar syndrome with vein stenosis in an infant.

Scimitar syndrome is characterised by right lung hypoplasia and abnormal pulmonary venous return, known as the 'scimitar vein'. We report the case of an infant girl with scimitar syndrome who developed a severe respiratory distress mimicking asthma. Pulmonary hypertension (PH) was diagnosed, attributed to scimitar vein stenosis and a left-to-right shunt. Scimitar vein stenosis, a rare complication of scimitar syndrome, can lead to severe PH, highlighting the importance of prompt management in specialised care centres.

Targeting the Substrate for Atrial Fibrillation: JACC Review Topic of the Week.

The identification of the pulmonary veins as a trigger source for atrial fibrillation (AF) has established pulmonary vein isolation (PVI) as a key target for AF ablation. However, PVI alone does not prevent recurrent AF in many patients, and numerous additional ablation strategies have failed to improve on PVI outcomes. This therapeutic limitation may be due, in part, to a failure to identify and intervene specifically on the pro-fibrillatory substrate within the atria and pulmonary veins. In this review paper, we highlight several emerging approaches with clinical potential that target atrial cardiomyopathy-the underlying anatomic, electrical, and/or autonomic disease affecting the atrium-in various stages of practice and investigation. In particular, we consider the evolving roles of risk factor modification, targeting of epicardial adipose tissue, tissue fibrosis, oxidative stress, and the inflammasome, along with aggressive early anti-AF therapy in AF management. Attention to combatting substrate development promises to improve outcomes in AF.

Initial clinical experience with the balloon-in-basket pulsed field ablation system: acute results of the VOLT CE mark feasibility study.

AIMS: Pulsed field ablation (PFA) for the treatment of atrial fibrillation (AF) potentially offers improved safety and procedural efficiencies compared with thermal ablation. Opportunities remain to improve effective circumferential lesion delivery, safety, and workflow of first-generation PFA systems. In this study, we aim to evaluate the initial clinical experience with a balloon-in-basket, 3D integrated PFA system with a purpose-built form factor for pulmonary vein (PV) isolation. METHODS AND RESULTS: The VOLT CE Mark Study is a pre-market, prospective, multi-centre, single-arm study to evaluate the safety and effectiveness of the Volt™ PFA system for the treatment of paroxysmal (PAF) or persistent AF (PersAF). Feasibility sub-study subjects underwent phrenic nerve evaluation, endoscopy, chest computed tomography, and cerebral magnetic resonance imaging. Study endpoints were the rate of primary serious adverse event within 7 days and acute procedural effectiveness. A total of 32 subjects (age 61.6 ± 9.6 years, 65.6% male, 84.4% PAF) were enrolled and treated in the feasibility sub-study and completed a 30-day follow-up. Acute effectiveness was achieved in 99.2% (127/128) of treated PVs (96.9% of subjects, 31/32) with 23.8 ± 4.2 PFA applications/subject. Procedure, fluoroscopy, LA dwell, and transpired ablation times were 124.6 ± 28.1, 19.8 ± 8.9, 53.0 ± 21.0, and 48.0 ± 19.9 min, respectively. Systematic assessments of initial safety revealed no phrenic nerve injury, pulmonary vein stenosis, or oesophageal lesions causally related to the PFA system and three subjects with silent cerebral lesions (9.4%). There were no primary serious adverse events. CONCLUSION: The initial clinical use of the Volt PFA System demonstrates acute safety and effectiveness in the treatment of symptomatic, drug refractory AF.

Factors associated with early postoperative mortality after total anomalous pulmonary venous connection repair: A retrospective cohort study.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect where pulmonary venous plexus fails to connect with the left atrium (LA). Surgical repair is the primary treatment for TAPVC, but factors influencing outcomes are not fully understood. This study investigates the early outcomes of surgical repair for TAPVC and associated factors. A retrospective cohort analysis was conducted on TAPVC patients who underwent surgical repair between 2012 and 2022. Data were collected from medical records and supplemented with phone call validation. Demographic characteristics, surgical data, diagnostic tests, and outcomes were analyzed. Statistical analysis included chi-square, t-tests, and multivariate logistic regression using SPSS. A total of 88 patients underwent surgical repair for TAPVC, resulting in a mortality rate of 21.6%. Weight and bypass time were significantly associated with patient survival. Female patients had a higher likelihood of death. The anatomic type did not significantly influence mortality. Patients with pulmonary venous obstruction (PVO) experienced a higher mortality rate. Notably, ligation of the vertical vein in supracardiac and infracardiac types was associated with lower mortality. In conclusion, our study identifies several key factors contributing to higher mortality rates following TAPVC surgery, including low weight, female gender, prolonged bypass time, and preoperative vein obstruction. Highlighting the significance of surgical technique, particularly the sutureless approach, we advocate for its meticulous consideration to achieve improved outcomes. Furthermore, our findings indicate a potential decrease in mortality associated with vertical vein ligation, which may mitigate the risk of post-repair heart failure. We suggest further rigorous studies to gain comprehensive insights into TAPVC surgical interventions.

Role of Sleep-Disordered Breathing and Epicardial Connections in the Recurrence of Atrial Fibrillation.

The presence of epicardial connections (ECs) between the pulmonary veins (PVs) and atrium may contribute to atrial fibrillation (AF) recurrence. This study aimed to determine the impact of sleep-disordered breathing (SDB) on the presence of ECs and the interplay between SDB and ECs on AF recurrence.We retrospectively reviewed 400 consecutive non-valvular AF patients. Among them, 235 patients exhibiting a 3% oxygen desaturation index (ODI) of ≥ 10 events/hour underwent polysomnography to evaluate the SDB severity, measured by the apnea-hypopnea index (AHI). To facilitate the ablation of AF and ECs, a high-density mapping catheter (HDMC) was employed. AF recurrence was evaluated over a 12-month period post-AF ablation.The key findings included: 1) 63% of AF patients with ECs had SDB with an AHI ≥ 20 events/hour. 2) Despite achieving complete PV isolations and precise EC ablation using an HDMC, SDB presence was associated with an increased AF recurrence. 3) Continuous positive airway pressure therapy for SDB improved AF recurrence among the AF patients with both ECs and SDB (57% versus 73%; P = 0.016). 4) AHI (odds ratio [OR] = 1.91, ≥ 28.4 events/hour) and left atrial volume (LAV) (OR = 1.42, ≥ 128.3 mL) were independent predictors of the presence of ECs, and AHI (OR = 1.44, ≥ 27.8 events/hour) was an independent predictor of the presence of AF recurrence.It is essential for physicians to recognise the potential complexity of ECs and SDB in AF patients. Thus, screening and treating SDB in AF patients presenting with ECs might play a pivotal role in suppressing AF recurrence.

Impact of wall thickness on the tissue cooling effect of cryoballoon ablation.

AIMS: Understanding of the tissue cooling properties of cryoballoon ablation during pulmonary vein (PV) isolation is lacking. The purpose of this study was to delineate the depth of the tissue cooling effect during cryoballoon freezing at the pulmonary venous ostium. METHODS AND RESULTS: A left atrial-PV model was constructed using a three-dimensional printer with data from a patient to which porcine thigh muscle of various thicknesses could be affixed. The model was placed in a 37°C water tank with a PV water flow at a rate that mimicked biological blood flow. Cryofreezing at the PV ostium was performed five times each for sliced porcine thigh muscle of 2, 4, and 6 mm thickness, and sliced muscle cooling on the side opposite the balloon was monitored. The cooling effect was assessed using the average temperature of 12 evenly distributed thermocouples covering the roof region of the left superior PV. Tissue cooling effects were in the order of the 2, 4, and 6 mm thicknesses, with an average temperature of -41.4 ± 4.2°C for 2 mm, -33.0 ± 4.0°C for 4 mm, and 8.0 ± 8.7°C for 6 mm at 180 s (P for trend <0.0001). In addition, tissue temperature drops were steeper in thin muscle (maximum temperature drop per 5 s: 5.2 ± 0.9°C, 3.9 ± 0.7°C, and 1.3 ± 0.7°C, P for trend <0.0001). CONCLUSION: The cooling effect of cryoballoon freezing is weaker in the deeper layers. Cryoballoon ablation should be performed with consideration to myocardial thickness.

[Thymoma with Partial Anomalous Pulmonary Venous Drainage from the Left Upper Lobe:Report of a Case].

A 73-year-old woman presented with left anterior chest pain and back pain. Computed tomography (CT) scan showed an anterior mediastinal tumor. It also showed partial anomalous pulmonary venous drainage (left superior pulmonary vein draining into the left brachiocephalic vein), and the tumor was located near the left brachiocephalic vein. The operation was performed through a median sternotomy to resect the thymus and tumor with partial resection of the left upper lobe due to the tumor's adhesion to the left upper lobe. One of the vascular anomalies encountered in adult thoracic surgery is partial anomalous pulmonary venous drainage. It is important to recognize the presence of such an anomaly on imaging and to anticipate the surgical procedure with a preoperative surgical technique.

A review regarding the article 'Multidisciplinary management strategies for atrial fibrillation.'

Catheter ablation has become an accepted first line therapy for paroxysmal atrial fibrillation (PAF), with pulmonary vein isolation (PVI) being the key element of ablation strategies. Catheter ablation of AF has been proven to be superior to antiarrhythmic drug (AAD) therapy regarding efficacy and improvement of survival in patients with heart failure. PVI has become a routine treatment procedure in recent decades especially for patients with symptomatic PAF. Recent research investigations have shown that AF recurrence still occurs in a significant number of patients after ablation. AF recurrence leads to a decrease in patients' quality of life and is related to an increased risk of cardiovascular events and mortality. As most arrhythmia recurrences take place within the first 3 months after the initial procedure, this time period is seen as a so-called "blanking period", in which ablation is not recommended, since up to 50 % of patients can become arrhythmia free in the long run. Ablation therapy, however, is less successful in patients suffering from long standing persistent AF, with a 20.3 % success rate after a single procedure and a 45 % success rate after multiple RFA procedures. Several scores have been established and validated to predict cardiac rhythm outcomes after catheter ablation. However, due to the lack of external validation of most of the scores, their efficacy in predicting recurrence of atrial fibrillation needs to be further evaluated in independent external studies.

Dual energy for pulmonary vein isolation using dual-energy focal ablation technology integrated with a three-dimensional mapping system: SmartfIRE 3-month results.

AIMS: Contact force (CF)-sensing radiofrequency (RF) catheters with an ablation index have shown reproducible outcomes for the treatment of atrial fibrillation (AF) in large multicentre studies. A dual-energy (DE) focal CF catheter to deliver RF and unipolar/biphasic pulsed field ablation (PFA), integrated with a three-dimensional (3D) mapping system, can provide operators with additional flexibility. The SmartfIRE study assessed the safety and efficacy of this novel technology for the treatment of drug-refractory, symptomatic paroxysmal AF. Results at 3 months post-ablation are presented here. METHODS AND RESULTS: Pulmonary vein isolation (PVI) was performed using a DE focal, irrigated CF-sensing catheter with the recommendation of PFA at posterior/inferior and RF ablation at the anterior/ridge/carina segments. Irrespective of energy, a tag size of 3 mm; an inter-tag distance ≤6 mm; a target index of 550 for anterior, roof, ridge, and carina; and a target index of 400 for posterior and inferior were recommended. Cavotricuspid isthmus ablation was permitted in patients with documented typical atrial flutter. The primary effectiveness endpoint was acute procedural success. The primary safety endpoint was the rate of primary adverse events (PAEs) within 7 days of the procedure. A prespecified patient subset underwent oesophageal endoscopy (EE; 72 h post-procedure), neurological assessment (NA; pre-procedure and discharge), and cardiac computed tomography (CT)/magnetic resonance angiogram (MRA) imaging (pre-procedure and 3 months post-procedure) for additional safety evaluation, and a mandatory remapping procedure (Day 75 ± 15) for PVI durability assessment. Of 149 patients enrolled between February and June 2023, 140 had the study catheter inserted (safety analysis set) and 137 had ablation energy delivered (per-protocol analysis set). The median (Q1/Q3) total procedure and fluoroscopy times were 108.0 (91.0/126.0) and 4.2 (2.3/7.7) min (n = 137). The acute procedural success rate was 100%. First-pass isolation was achieved in 89.1% of patients and 96.8% of veins. Cavotricuspid isthmus ablations were successfully performed in 12 patients [pulsed field (PF) only: 6, RF only: 5, and RF/PF: 1]. The PAE rate was 4.4% [6/137 patients; 2 pulmonary vein (PV) stenoses, 2 cardiac tamponades/perforations, 1 stroke, and 1 pericarditis]. No coronary artery spasm was reported. No oesophageal lesion was seen in the EE subset (0/31, 0%). In the NA subset (n = 30), microemboli lesions were identified in 2 patients (2/30, 6.7%), both of which were resolved at follow-up; only 1 was symptomatic (silent cerebral lesion, 3.3%). In the CT/MRA subset (n = 30), severe PV narrowing (of >70%) was detected in 2 patients (2/30, 6.7%; vein level 2/128, 1.6%), of whom 1 underwent dilatation and stenting and 1 was asymptomatic; both were associated with high index values and a small inter-tag distance. In the PV durability subset (n = 30), 100/115 treated PVs (87%) were durably isolated and 18/30 patients (60.0%) had all PVs durably isolated. CONCLUSION: A DE focal CF catheter with 3D mapping integration showed a 100% acute success rate with an acceptable safety profile in the treatment of paroxysmal AF. Prespecified 3-month remapping showed notable PVI durability. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05752487.

App-Based Mental Training to Reduce Atrial Fibrillation-Related Symptoms After Pulmonary Vein Isolation: MENTAL AF Trial.

BACKGROUND: Even after atrial fibrillation (AF) catheter ablation, many patients still experience relevant symptom burden. The objective of the MENTAL AF trial was to determine whether app-based mental training (MT) during the 3 months following pulmonary vein isolation reduces AF-related symptoms. METHODS AND RESULTS: Patients scheduled for pulmonary vein isolation were enrolled and randomized 1:1 to either app-based MT or usual care. Of 174 patients, 76 in the MT and 75 in the usual care group were included in the final analysis. The intervention was delivered by a daily 10-minute app-based MT. The primary outcome was the intergroup difference of the mean AF6 sum score, an AF-specific questionnaire, during the 3-month study period. Secondary outcomes included quality-of-life measures such as the AFEQT (Atrial Fibrillation Effect on Quality of Life). Mean age (SD) was 61 (8.7) years and 61 (41%) were women. The mean AF6 sum score over the study period was 8.9 (6.9) points in the MT group and 12.5 (10.1) in the usual care group (P=0.011). This referred to a reduction in the AF6 sum score compared with baseline of 75% in MT and 52% for usual care (P<0.001). The change in the AFEQT Global Score was 22.6 (16.3) and 15.7 (22.1), respectively; P=0.026. CONCLUSIONS: MENTAL AF showed that app-based MT as an adjunctive treatment tool following pulmonary vein isolation was feasible. App-based MT was found to be superior to standard care in reducing AF-related symptom burden and improving health-related quality of life. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04067427.

Venous vascular closure system vs. figure-of-eight suture following atrial fibrillation ablation: the STYLE-AF Study.

AIMS: Simplified ablation technologies for pulmonary vein isolation (PVI) are increasingly performed worldwide. One of the most common complications following PVI are vascular access-related complications. Lately, venous closure systems (VCSs) were introduced into clinical practice, aiming to reduce the time of bed rest, to increase the patients' comfort, and to reduce vascular access-related complications. The aim of the present study is to compare the safety and efficacy of using a VCS to achieve haemostasis following single-shot PVI to the actual standard of care [figure-of-eight suture and manual compression (MC)]. METHODS AND RESULTS: This is a prospective, multicentre, randomized, controlled, open-label trial performed at three German centres. Patients were randomized 1:1 to undergo haemostasis either by means of VCS (VCS group) or of a figure-of-eight suture and MC (F8 group). The primary efficacy endpoint was the time to ambulation, while the primary safety endpoint was the incidence of major periprocedural adverse events until hospital discharge. A total of 125 patients were randomized. The baseline characteristics were similar between the groups. The VCS group showed a shorter time to ambulation [109.0 (82.0, 160.0) vs. 269.0 (243.8, 340.5) min; P < 0.001], shorter time to haemostasis [1 (1, 2) vs. 5 (2, 10) min; P < 0.001], and shorter time to discharge eligibility [270 (270, 270) vs. 340 (300, 458) min; P < 0.001]. No major vascular access-related complication was reported in either group. A trend towards a lower incidence of minor vascular access-related complications on the day of procedure was observed in the VCS group [7 (11.1%) vs. 15 (24.2%); P = 0.063] as compared to the control group. CONCLUSION: Following AF ablation, the use of a VCS results in a significantly shorter time to ambulation, time to haemostasis, and time to discharge eligibility. No major vascular access-related complications were identified. The use of MC and a figure-of-eight suture showed a trend towards a higher incidence of minor vascular access-related complications.

Comparing pulsed field electroporation and radiofrequency ablation for the treatment of paroxysmal atrial fibrillation: design and rationale of the BEAT PAROX-AF randomized clinical trial.

AIMS: Using thermal-based energy sources [radiofrequency (RF) energy/cryo energy] for catheter ablation is considered effective and safe when performing pulmonary vein isolation (PVI) in patients with paroxysmal atrial fibrillation (AF). However, treatment success remains limited and complications can occur due to the propagation of thermal energy into non-target tissues. We aim to compare pulsed field ablation (PFA) with RF ablation in terms of efficacy and safety for patients with drug-resistant paroxysmal AF. METHODS AND RESULTS: The BEAT PAROX-AF trial is a European multicentre, superiority, open-label randomized clinical trial in two parallel groups. A total of 292 participants were recruited in 9 high-volume European clinical centres in 5 countries. Patients with paroxysmal AF were randomized to PFA (FARAPULSE Endocardial Ablation System©, Boston Scientific) or RF using the CLOSE protocol with contact force sensing catheter (SmartTouch© catheter and CARTO© Biosense Webster). The primary endpoint will be the 1-year recurrence of atrial arrhythmia, and the major secondary safety endpoint will be the occurrence of acute (<7 days) procedure-related serious adverse events, or pulmonary vein stenosis, or atrio-oesophageal fistula up to 12 months. Additionally, five sub-studies investigate the effect of PFA on oesophageal safety, cerebral lesions, cardiac autonomic nervous system, durability of PVI as assessed during redo ablation procedures, and atrial and ventricular function. The study began on 27 December 2021 and concluded recruitment on 17 January 2024. Results will be available in mid-2025. CONCLUSION: The BEAT PAROX-AF trial aims to provide critical insights into the optimal treatment approach for patients with paroxysmal AF.