Severe Pulmonary Vein Stenosis Research Articles

Case report of the successful treatment of a rare complication of pulmonary vein stenting: atrial rupture and stent detachment

Pulmonary vein stenosis (PVS) is a relatively rare pulmonary vascular disease in clinical practice, and radiofrequency ablation (RFA) is a common cause of pulmonary vein stenosis. Owing to its nonspecific symptoms and irregular follow-up, PVS is often misdiagnosed. In severe cases, intervention is needed, with the main treatment options being pulmonary vein stenting and/or balloon dilation, both of which carry risks of serious complications. We report a patient with severe pulmonary vein stenosis who experienced right atrial wall rupture and stent detachment during an interventional procedure. Through active intraoperative intervention, we successfully avoided serious complications.

Exploring Stress and Coping in Caregivers of Children with Pulmonary Vein Stenosis: A Mixed-Method Study.

(1) Background: This mixed-method study aims to identify and describe factors associated with acute and long-term parenting stressors and coping strategies in caregivers of children with intraluminal pulmonary vein stenosis (PVS). (2) Methods: Parents of children with severe PVS were recruited from a large quaternary hospital to complete a survey that included demographics, the Pediatric Inventory for Parents (PIP), and the Coping Health Inventory for Parents (CHIP). We determined the Social Vulnerability Index (SVI) based on self-reported home address. A subset of caregivers completed a 60 min semi-structured interview via Zoom exploring the impact their child's diagnosis had on their family; experience managing stress in the hospital and at home; current resources and processes for coping; and potential recommendations for hospitals to build resilience and coping. We used multivariable linear regression to examine the association between SVI and parental stress and coping while adjusting for possible confounders. Thematic analysis identified themes related to stress and coping. Finally, we assessed instances of convergence and difference between the qualitative and quantitative results. (3) Results: Participants included 32 caregivers who were 91% female with a mean age of 39 years. The children of participants were 66% female, with a mean age of five years. The parents reported a high amount of stress with an average PIP score of 120, nearly 46 points higher than similar studies in the congenital heart community. We observed no significant associations between SVI and either parental stress or coping in adjusted models. We identified 13 themes, including medical care, hospital, family, support systems, and home medical routine or support. (4) Conclusions: Our study found high levels of illness-related parental stress among caregivers of children with PVS. Stress evolved over time from what caregivers described as 'survival mode' to a future-oriented outlook. Currently, caregivers rely heavily on support networks that are not available to all caregivers or may experience strain over time. Caregivers indicated that communication and parental role functioning were coping strategies that could be better supported by providers and health systems.

Comprehensive review of pulmonary vein stenosis post-atrial fibrillation ablation: diagnosis, management, and prognosis.

Pulmonary vein stenosis (PVS) can occasionally occur in the follow-up after pulmonary vein isolation (PVI) for atrial fibrillation (AF). During PVI, ablation is performed at the PV ostium or distal part, leading to tissue damage. This damage can result in fibrosis of the necrotic myocardium, proliferation, and thickening of the vascular intima, as well as thrombus formation, further advancing PVS. Mild-to-moderate PVS often remains asymptomatic, but severe PVS can cause symptoms, such as dyspnea, cough, fatigue, decreased exercise tolerance, chest pain, and hemoptysis. These symptoms are due to pulmonary hypertension and pulmonary infarction. Imaging evaluations such as contrast-enhanced computed tomography are essential for diagnosing PVS. Early suspicion and detection are necessary, as underdiagnosis can lead to inappropriate treatment, disease progression, and poor outcomes. The long-term prognosis of PVS remains unclear, particularly regarding the impact of mild-to-moderate PVS over time. PVS treatment focuses on symptom management, with no established definitive solutions. For severe PVS, transcatheter PV angioplasty is performed, though the risk of restenosis remains high. Restenosis and reintervention rates have improved with stent implantation compared with balloon angioplasty. The role of subsequent antiplatelet therapy remains uncertain. Dedicated evaluation is essential for accurate diagnosis and appropriate management to avoid significant long-term impacts on patient outcomes.

Pulmonary Vein Intervention for Severe Pulmonary Vein Stenosis After Atrial Fibrillation Ablation - A Retrospective Cohort Study.

Pulmonary vein (PV) stenosis (PVS) is a serious complication of atrial fibrillation (AF) ablation. The objective of this study was to describe interventional treatments for PVS after AF ablation and long-term outcomes in Japanese patients. This multicenter retrospective observational study enrolled 30 patients (26 [87%] male; median age 55 years) with 56 severe PVS lesions from 43 PV interventional procedures. Twenty-seven (90%) patients had symptomatic PVS and 19 (63%) had a history of a single AF ablation. Of the 56 lesions, 41 (73%) were de novo lesions and 15 (27%) were retreated. Thirty-three (59%) lesions were treated with bare metal stents, 14 (25%) were treated with plain balloons, and 9 (16%) were treated with drug-coated balloons. All lesions were successfully treated without any systemic embolic event. Over a median follow-up of 584 days (interquartile range 265-1,165 days), restenosis rates at 1 and 2 years were 35% and 47%, respectively. Multivariate Cox regression analysis revealed devices <7 mm in diameter (hazard ratio [HR] 2.52; 95% confidence interval [CI] 1.04-6.0; P=0.040) and totally occluded lesions (HR 3.33; 95% CI 1.21-9.15; P=0.020) were independent risk factors for restenosis. All PVS lesions were successfully enlarged by the PV intervention; however, restenosis developed in approximately half the lesions within 2 years.

Computed tomography imaging confirmation of severe pulmonary vein stenosis or occlusion: a valuable diagnostic tool.

Computed tomography imaging confirmation of severe pulmonary vein stenosis or occlusion: a valuable diagnostic tool.

Fatal Hemoptysis Secondary to Severe Pulmonary Veins Stenosis and Fibrosing Mediastinitis following Radiofrequency Ablation for Atrial Fibrillation: A Case Report and Review of the Literature

Fatal hemoptysis secondary to severe pulmonary veins stenosis and fibrosing mediastinitis is an exceptional late complication of radiofrequency ablation for atrial fibrillation. We report the case of a 53-year-old male with a history of atrial fibrillation treated by radiofrequency ablation and admitted in our center 6 months after the procedure because of aggravating dyspnea and fatigability. Transthoracic echocardiography showed moderate dilation of right heart cavities, severe pulmonary hypertension and a turbulent flow in superior pulmonary veins. The cardiologist suspected pulmonary vein(s) stenosis and so cardiac computed tomography (CT) angiography was performed, with findings of severe stenosis of the right superior, right inferior and left inferior pulmonary veins, near-occlusion of the left superior pulmonary vein and the vein draining the apical segment of the right lower lobe. The CT scan also revealed soft tissue attenuation of the mediastinum posterior to the left atrium suggesting fibrosing mediastinitis together with parenchymal findings consistent with pulmonary veno-oclusive disease and an area of hemorrhagic infarction. Fatal hemoptysis occurred 3 days later, before treatment was attempted. In conclusion, severe pulmonary vein stenosis and fibrosing mediastinitis are rare but devastating complications of radiofrequency ablation. Prevention and early diagnosis are the key elements as these entities are potentially life-threatening.

Pulmonary Vein Stenosis After Catheter Ablation of Atrial Fibrillation Using a Cryoballoon, Hot Balloon, or Laser Balloon.

Pulmonary vein stenosis (PVS) after PV isolation (PVI) for atrial fibrillation (AF) is a severe complication that requires angioplasty. This study aimed to compare the reduction of the cross-sectional PV area (PVA) and the incidence of PVS after cryoballoon (CB)-PVI, hot balloon (HB)-PVI, or laser balloon (LB)-PVI. A total of 320 patients who underwent an initial catheter ablation procedure for AF using a CB, HB, or LB in 2 hospitals were included. They underwent contrast-enhanced multidetector CT before and 3 months after the procedure. In all 4 PVs, the reduction in PVA was more significant in the LB group than in the CB or HB groups, respectively. Moderate (50-75%) and severe (>75%) PVS were observed in 5.3% and 0.5% of the PVs, respectively. Although moderate PVS was more frequently observed in the LB group than in the CB or HB groups (8.2%, 3.8%, and 5.0%; P=0.03), the incidence of severe PVS was similar in the LB, CB, and HB groups (0.3%, 0.5%, and 1.0%; P=0.46). Symptomatic PVS requiring intervention occurred in 1 (0.3%) patient. Although the reduction in cross-sectional PVA and the incidence of moderate PVS after LB-PVI was more significant than after CB-PVI or HB-PVI, it rarely led to severe PVS. Symptomatic PVS requiring intervention was rare after the balloon ablation of AF.

Pulmonary vein stenosis post cryo AF ablation using single shot 4-minute freeze technique

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Magdi Yacoub foundation Introduction Cryo balloon ablation is widely used for AF ablation. Cryo ablation techniques include regular 2 shots per vein Vs single shot freeze technique. Studies showed that the single shot freeze technique is as effective as the double freezing shot, thus becoming the standard technique (1). There is a debate on whether the single shot freeze should be 3 minutes or 4 minutes. Some data suggest similar efficacy while other data suggest more durable PV isolation with the 4-minute technique (2). Severe pulmonary vein (PV) stenosis is a rare but a serious complication. The only available data on iatrogenic PV stenosis are from studies on the single 3-minute freeze technique (3). Purpose Assessment of the incidence of PVs stenosis using single shot 4-minute freeze technique. Methods We conducted a single center prospective study where we recruited 28 consecutive patients with 102 PVs scheduled for cryo AF ablation using 2nd generation cryo balloon using single shot 4-minute freeze technique as the standard technique. All patients had cardiac CT before ablation and at least 3 months post ablation where each PV’s cross-sectional area was measured. Measurements were validated by a single experienced operator. PV stenosis was classified to minimal, mild, moderate, and severe if the PV’s area decreased by 25%, 25-50%, 50-75% and more than 75% respectively. All patients were assessed regarding the presence of any symptom suggesting PV stenosis or other complications. Results Of the studied 102 PVs, only one vein (0.98%) had severe stenosis (asymptomatic) and one vein (0.98%) had moderate stenosis. Both were left superior pulmonary vein (LSPV). 13 veins (12.7%) developed mild stenosis, 74 veins (72%) showed minimal stenosis and 13 veins had no change in area. None of the studied patients developed phrenic nerve palsy or esophageal injury peri-procedure. Discussion Our results suggest low risk of developing severe PV stenosis using the 4-minute single shot freeze technique (0.98%). However, minimal, and mild pulmonary vein stenosis occurred in 84.7% of the patients denoting that this change is expected with the procedure. Tokutake et al studied the effect of using the single shot 3-minute freeze technique on PV area 3 months post cryo AF ablation and reported mild, moderate and severe (&amp;gt;75%) PV stenosis in 24.6%, 3.8% and 1.3% of PVs respectively (3). This shows that using the 4-minute freeze technique has a similarly low incidence of severe PV stenosis to the published data on the 3-minute technique. It appears that the LSPV is the most liable for this complication and this goes in line with the previous studies. Conclusion Using the 4-minute single shot freeze technique in cryo AF ablation appears to have a comparable low incidence of severe PV stenosis to the published data for the 3-minute freeze technique. The overall risk of severe PV stenosis is low. It appears that the LSPV is the most liable for this complication.

Lung histopathology evaluation of an X-MAID patient with a novel mutation in MSN

Background: The cell cytoskeleton is regulated by the ezrin-radixin-moesin (ERM) family of proteins, forming links between transmembrane proteins and the underlying actin cytoskeleton. Phosphorylation and activation of these proteins enable interactions with partners critically involved in shape regulation, such as actin filaments, transmembrane proteins, and scaffolding proteins. The MSN gene encodes moesin, which is ubiquitously expressed in lungs, spleen, kidney, endothelial cells of vessels, lymphocytes, and neutrophils. Deficiency or dysregulation of moesin, called X-linked moesin-associated immunodeficiency (X-MAID), is characterized by severe leukopenia affecting T cells, B cells, and neutrophils. To date, the clinical picture of patients with X-MAID is variable. Aim: We describe the presentation, immune-workup, and lung histopathology findings of a young male patient with X-MAID and multi-organ involvement, whose severe pulmonary vein stenosis necessitated a double lung transplant. Methods: A thorough review of the patient’s chart was performed. Results: The patient presented with a history of recurrent respiratory tract infections, oral thrush, and 3 major bacterial infections requiring admission and antibiotic therapy. His immune evaluation was remarkable for low T cells, and normal numbers of B and NK cells. At age 4 years he underwent a double lung transplant due to severe pulmonary vein stenosis and pulmonary hypertension. He further developed chronic kidney injury post-transplant. Clinical trio whole exome sequencing revealed a novel hemizygous variant in the MSN gene (c.278dupT; p.L93FfsX21), predicted to cause loss-of-function in moesin. Histologic evaluation of the lung tissue before transplantation identified profound abnormalities in alveoli formation. Conclusion: Patients with moesin deficiency may present during infancy or childhood with a severe form of the disease, including combined immunodeficiency with lymphopenia and neutropenia, while adults may have a milder clinical picture. The novel MSN mutation described here adds to the known spectrum of disease and highlights the non-redundant functions of moesin, particularly in the lung. Statement of Novelty: We report the first lung histopathological description of an X-MAID case, in a pediatric patient with recurrent infections, cytopenia, and autoimmunity who underwent a double lung transplant.

Safety and efficacy of balloon angioplasty compared to stent-based-strategies with pulmonary vein stenosis: A systematic review and meta-analysis.

Pulmonary vein stenosis (PVS) is an uncommon but known cause of morbidity and mortality in adults and children and can be managed with percutaneous re-vascularization strategies of pulmonary vein balloon angioplasty (PBA) or pulmonary vein stent implantation (PSI). To study the safety and efficacy outcomes of PBA vs PSI in all patient categories with PVS. We performed a literature search of all studies comparing outcomes of patients evaluated by PBA vs PSI for PVS. We selected all published studies comparing PBA vs PSI for PVS with reported outcomes of restenosis and procedure-related complications in all patient categories. In adults, PVS following atrial fibrillation ablation and in children PVS related to congenital etiology or post-procedural PVS following total or partial anomalous pulmonary venous return repair were included. The patient-centered outcomes were risk of restenosis requiring re-intervention and procedural-related complications. The meta-analysis was performed by computing odds ratios (ORs) using the random effects model based on underlying statistical heterogeneity. Eight observational studies treating 768 severe PVS in 487 patients met our inclusion criteria. The age range of patients was 6 months to 70 years and 67% were males. The primary outcome of the re-stenosis requiring re-intervention occurred in 196 of 325 veins in the PBA group and 111 of 443 veins in the PSI group. Compared to PSI, PBA was associated with a significantly increased risk of re-stenosis (OR 2.91, 95%CI: 1.15-7.37, P = 0.025, I 2 = 79.2%). Secondary outcomes of the procedure-related complications occurred in 7 of 122 patients in the PBA group and 6 of 69 in the PSI group. There were no statistically significant differences in the safety outcomes between the two groups (OR: 0.94, 95%CI: 0.23-3.76, P = 0.929), I 2 = 0.0%). Across all patient categories with PVS, PSI is associated with reduced risk of re-intervention and is as safe as PBA and should be considered first-line therapy for PVS.

Very High-Power Short-Duration, Temperature-Controlled Radiofrequency Ablation in Paroxysmal Atrial Fibrillation: The Prospective Multicenter Q-FFICIENCY Trial

BackgroundQDOT MICRO (QDM) is a novel contact force–sensing catheter optimized for temperature-controlled radiofrequency (RF) ablation. The very high-power short-duration (vHPSD) algorithm modulates power, maintaining target temperature during 90 W ablations for ≤4 seconds. ObjectivesThis study aims to evaluate safety and 12-month effectiveness of the QDM catheter in paroxysmal atrial fibrillation (AF) ablation using the vHPSD mode combined with conventional-power temperature-controlled (CPTC) mode. MethodsIn this prospective, multicenter, nonrandomized study, patients with drug-refractory, symptomatic paroxysmal AF underwent pulmonary vein (PV) isolation with QDM catheter with vHPSD as primary ablation mode, with optional use of the CPTC mode (25 to 50 W) for PV touch-up or non-PV ablation. The primary safety endpoint was incidence of primary adverse events within ≤7 days of ablation. The primary effectiveness endpoint was freedom from documented atrial tachyarrhythmia recurrence and acute procedural, repeat ablation, and antiarrhythmic drug failure. ResultsOf 191 enrolled participants, 166 had the catheter inserted, received RF ablation, and met eligibility criteria. Median procedural, RF application for ablating PVs, and fluoroscopy times were 132.0, 8.0, and 9.1 minutes, respectively. The primary adverse event rate was 3.6%. Imaging conducted in a subset of participants (n = 40) at 3 months did not show moderate or severe PV stenosis. The Kaplan-Meier estimated 12-month rate for primary effectiveness success was 76.7%; freedom from atrial tachyarrhythmia recurrence was 82.1%; clinical success (freedom from symptomatic recurrence) was 86.0%; and freedom from repeat ablation was 92.1%. ConclusionsTemperature-controlled paroxysmal AF ablation with the novel QDM catheter in vHPSD mode (90 W, ≤4 seconds), alone or with CPTC mode (25 to 50 W), is highly efficient and effective without compromising safety. (Evaluation of QDOT MICRO Catheter for Pulmonary Vein Isolation in Subjects With Paroxysmal Atrial Fibrillation [Q-FFICIENCY]; NCT03775512)

Abstract 13259: Genome Sequencing in Pediatric Patients With Pulmonary Vein Stenosis

Introduction: Pulmonary vein stenosis (PVS) is a rare and progressive disease of the vasculature, characterized by neointimal proliferation and is frequently lethal in pediatric patients. PVS is clinically heterogeneous, and its etiology remains unknown. Insight into the genetic basis of PVS can help to direct treatment interventions and improve clinical outcomes. Objectives: To examine the genetic mechanisms underlying PVS and to compare the genetic architecture in patients with severe disease to patients with disease stabilization. Methods: We performed genome sequencing in a cohort of 18 pediatric patients with PVS. The genomic data was analyzed for clinically reportable variants and biologically plausible candidates. We used a statistical overrepresentation test to investigate the overrepresentation of gene ontology (GO) terms in patients with aggressive disease compared with stabilized patients. Results: Seven patients (38.9%) had aggressive disease, resulting in death or lung transplant, 16 patients (88.9%) had concomitant congenital heart disease, and 11 patients (61.1%) had extracardiac anomalies. In two patients (11.1%) with a syndromic presentation, we identified Pathogenic/Likely Pathogenic variants in three genes: ANKRD11, associated with KBG syndrome, FOXP1 , and DYNC1H1 , associated with intellectual developmental disorders. In search of novel candidate genes, we identified de novo variants in PAK4 , AMBRA1 , and ANKRD50 , involved in cytoskeletal remodeling, mTOR-regulated autophagy, and protein trafficking, respectively. In patients with severe PVS, we found an overrepresentation of rare (minor allele frequency &lt;0.15%) coding variants in genes involved in blood vessel morphogenesis (FDR &lt;0.05), protein phosphorylation (FDR &lt;0.05), dynein intermediate chain binding (FDR &lt;0.05), and ATP binding (FDR &lt;0.05). No GO terms were overrepresented in the patients with stabilized disease. Conclusions: This exploratory cohort of patients with PVS provides further insight into the genetic contributions to PVS. These results suggest distinct genetic landscapes between progressive and stabilized disease. Genetic testing in larger cohorts is needed to further define the molecular pathology of pediatric PVS.

Tricuspid Valve Blood Cysts Mimicking Thrombus or Vegetation on Echocardiogram in a Neonate.

A 2-month-old male infant, born premature with a birth weight of 865g, was found to have a tricuspid valve mass mimicking thrombus and vegetation by echocardiogram on the fourth day of life. The patient was treated with antibiotics and anticoagulation with no change in the size of the mass on serial follow-up echocardiography. The patient died of severe pulmonary vein stenosis and complex neurological disability. Postmortem cardiac examination revealed numerous cardiac blood cysts with two dominant ones (1.6 and 1.5mm) on the septal leaflet of the tricuspid valve, which based on the location and position corresponded to the suspected vegetation and thrombus on imaging. Cardiac blood cysts on valve leaflets are a common incidental finding during autopsy within the first 6months of life; however, they are rarely detected on imaging because of their minute size, often < 0.5mm. In this case, the sizable blood cysts were thought to represent thrombus or vegetation on echocardiogram, which influenced the patient management.

C41 AN UNUSUAL CASE OF “PULMONARY EMBOLISM”

Abstract Background Pulmonary vein stenosis (PVS) is a complication of catheter–based radiofrequency ablation (RFA) for atrial fibrillation (AF), caused by a vascular response which leads to replacement of necrotic myocardium with collagen. Clinical presentation can mimic pulmonary embolism (PE) and usually appears some months after procedure. Case report A 65–year–old man was hospitalized in March 2021 with a complaint of worsening dyspnoea on exertion, over the course of one month. He had a past medical history of persistent atrial fibrillation, because of which he underwent four pulmonary vein ablations (both RFA and crioablation), the last one in 2019. The previous echocardiogram showed a high systolic pulmonary arterial pressure (sPAP of 75 + 5 mmHg) with normal right ventricular function and dimensions. Despite D–dimer at admission was negative, a CT pulmonary angiography was performed and showed occlusion of left superior pulmonary vein and a severe stenosis of right superior pulmonary vein; ventilation–perfusion scintigraphy (V/Q scan) demonstrated widespread decreased perfusion of both superior lung lobes. The patient underwent angioplasty of right pulmonary vein and after the procedure sPAP greatly decreased (35 mmHg). Discussion Despite its reduction in incidence and its short–term presentation from procedure, severe symptomatic pulmonary vein stenosis must be considered among complications of ablation for atrial fibrillation. Delayed symptoms can also present years after procedure, even if in literature few similar cases are described. Ventilation–perfusion scintigraphy is a very sensible method to assess the functional significance of pulmonary vein stenosis and it also relates with symptoms’ severity. It should be performed in case of a high clinical suspicion, considering that the characteristic ventilation–perfusion mismatch of this condition can be seen in other diseases too, over all pulmonary embolism. Conclusions This case report emphasizes the importance of a thorough anamnesis and an exact interpretation of diagnostic imaging to correctly identify this AF ablation complication and optimize the patient’s therapeutic management. It must be considered that PV stenosis can develop late and with unusual symptoms, mimicking other diseases, like pulmonary embolism, which has the same scintigraphic imaging.

Impact of monitoring surface temperature during pulmonary vein isolation in a second-generation hot balloon system.

BackgroundA surface temperature sensor can be used to visualize the effect of hot balloon (HB) catheters. This study evaluated the efficacy and safety of a second-generation HB system with surface temperature monitoring in patients with atrial fibrillation (AF).MethodsTwenty patients (age: 69.6 ± 9.7 years, 11 male participants) who underwent first-time pulmonary vein isolation (PVI) using a second-generation HB were included. For each pulmonary vein (PV), the acute isolation rate and effective therapeutic range of surface temperature were investigated.ResultsEighty-three PVs (including three right middle PVs) were isolated in 20 patients using an HB with a surface temperature sensor. Sixty-eight PVs were isolated using the first application. Fifteen PVs (left superior PV [LSPV], n = 7 [35%]; left inferior PV, n = 2 [10%]; right superior PV, n = 3 [15%]; right inferior PV, n = 3 [15%]) showed early intraoperative reconduction and required second applications. One LSPV required radiofrequency touch-up at the carina. The optimal balloon surface temperature and application time were evaluated, and a median value of 58 °C and integral value of 1000 °C·s were identified from the receiver operating characteristic curve to be useful effective indicators. However, for LSPV, the PV potential of carina or ridge likely often remained and needed to be independently considered. There was no periprocedural complication including severe pulmonary vein stenosis. During the observation period (median: 280 days, interquartile range: 261–318 days), 17 patients (85%) achieved and maintained sinus rhythm.ConclusionsSecond-generation HBs with a surface temperature sensor are expected to provide favorable outcomes in AF ablation treatment.

Primary Minimally Invasive Repair with Atriopericardial Anastomosis Technique for Pulmonary Vein Stenosis after Catheter Ablation.

A pulmonary vein (PV) stenosis is a rare adverse event associated with catheter ablation for atrial fibrillation, which can potentially impair quality of life. Although percutaneous PV angioplasty is performed as the initial treatment of choice, the incidence of restenosis is reported to be considerably high. Herein, we describe a successful case of severe PV stenosis treated by primary surgical repair using the atriopericardial anastomosis technique via right minithoracotomy.

Systemic Sirolimus Therapy for Infants and Children With Pulmonary Vein Stenosis

BackgroundAnatomic interventions for pulmonary vein stenosis (PVS) in infants and children have been met with limited success. Sirolimus, a mammalian target of rapamycin inhibitor, has demonstrated promise as a primary medical therapy for PVS, but the impact on patient survival is unknown. ObjectivesThe authors sought to investigate whether mTOR inhibition with sirolimus as a primary medical therapy would improve outcomes in high-risk infants and children with PVS. MethodsIn this single-center study, patients with severe PVS were considered for systemic sirolimus therapy (SST) following a strict protocol while receiving standardized surveillance and anatomic therapies. The SST cohort was compared with a contemporary control group. The primary endpoint for this study was survival. The primary safety endpoint was adverse events (AEs) related to SST. ResultsBetween 2015 and 2020, our PVS program diagnosed and treated 67 patients with ≥moderate PVS. Of these, 15 patients were treated with sirolimus, whereas the remaining patients represent the control group. There was 100% survival in the SST group compared with 45% survival in the control group (log-rank p = 0.004). A sensitivity analysis was completed to address survival bias using median time from diagnosis of PVS to SST. A survival advantage persisted (log-rank p = 0.027). Two patients on sirolimus developed treatable AEs. Patients in the SST group underwent frequent transcatheter interventions with 3.7 catheterizations per person-year (25th to 75th percentile: 2.7 to 4.4 person-years). Median follow up time was 2.2 years (25th to 75th percentile: 1.2 to 2.9 years) in the SST group versus 0.9 years (25th to 75th percentile: 0.5 to 2.7 years) in the control group. ConclusionsThe authors found a survival benefit associated with SST in infants and children with moderate-to-severe PVS. This survival benefit persisted after adjusting the analysis for survival bias. There were 2 mild AEs associated with SST during the study period; both patients were able to resume therapy without recurrence.

Impact factors of in-stent restenosis following successful stent implantation for acquired severe pulmonary vein stenosis post atrial fibrillation ablation

Objective: To investigate the influencing factors of in-stent restenosis (ISR) following successful stent implantation in patients with ablation-associated severe pulmonary vein stenosis (PVS) who undergo atrial fibrillation ablation. Methods: Data of patients who underwent pulmonary vein angiography to confirm PVS after radiofrequency ablation for atrial fibrillation (AF) and received pulmonary vein stenting at Shanghai Chest Hospital from March 2010 to December 2017 were retrospectively analysed. All patients were followed up for a long period of time (pulmonary vein contract-enhanced CT within 6 to 12 months after operation was performed, and pulmonary angiography was performed if CT indicated stenosis>50%). The incidence of ISR was recorded. According to angiography, the patients were divided into ISR group and non-ISR group. The clinical and intraoperative imaging characteristics and interventional data were compared between the two groups. Logistic regression was used to analyse the influencing factors of ISR. Results: A total of 47 patients ((47.1±12.2) years old) were enrolled in this study, including 28 males(59.6%). There were 19 cases in ISR group and 28 cases in non-ISR group. Compared with the non-ISR group, the ISR group received more pulmonary vein isolation ((2.8±0.9) vs. (1.8±1.3), P=0.02), and the interval between last ablation and stenting was longer ((19.4±9.6) vs. (13.0±12.4), P=0.03). The incidence of ISR in patients with stent diameter≤8 mm was significantly higher than those with stent diameter>8 mm (33.3%(20/60) vs. 8.1%(3/37), P<0.01). Logistic regression analysis found that the number of radiofrequency ablation>1 (OR=2.1, 95%CI 1.3-3.9, P=0.02), and the time from the last ablation to stent placement>12 months (OR=1.5, 95%CI 1.1-2.5, P=0.03), reference diameter of stenosed distal vessel (OR=0.7, 95%CI 0.5-0.9, P=0.04), post procedural minimal luminal diameter (OR=0.4, 95%CI 0.2-0.8, P=0.02) and stent diameter (OR=0.6, 95%CI 0.3-0.9, P=0.03) were independent factors of ISR. Conclusions: The greater number of radiofrequency ablations and the longer time from the last ablation to stent placement increase the risk of ISR. The larger reference diameter of the stenosed distal vessel, stent diameter and post procedural minimal luminal diameter are the protective factors of ISR.

Combined pulmonary vein stenosis stenting and left atrial appendage occlusion in a patient with hemoptysis after atrial fibrillation ablation

BackgroundPulmonary vein stenosis (PVS) after radiofrequency ablation for non-valvular atrial fibrillation (AF) is an uncommon but serious complication. PVS stenting can rapidly restore pulmonary flow and improve symptoms with long-term low incidence of restenosis. However, high risk of thrombosis remains if AF is recurrent, especially for CHA2DS2-VASc > 2.Case presentationA 67-year-old man with diabetes, hypertension and a history of stroke underwent radiofrequency pulmonary vein isolation for persistent AF 1 year ago. Six months later he developed recurrent respiratory infection and massive hemoptysis. Computed tomography pulmonary angiography revealed severe left pulmonary vein stenosis. Simultaneous percutaneous PVS stenting and left atrial appendage occlusion were performed to resolve recurrent hemoptysis and prevent stroke. The clinical follow-up indicated a good short and mid-term result with significant improvement of symptoms.ConclusionsSimultaneous PVS stenting and left atrial appendage occlusion is feasible and effective in patients with recurrence of AF and hemoptysis induced by radiofrequency ablation for AF.

Severe pulmonary vein stenosis requiring angioplasty after hot balloon ablation for pulmonary vein isolation

Severe pulmonary vein stenosis requiring angioplasty after hot balloon ablation for pulmonary vein isolation