A Case Report of Pulmonary Vein Stenosis Within the Labyrinth of Fibrosing Mediastinitis.

This case centers on a 76-year-old male experiencing exertional dyspnea and hemoptysis, with a medical history marked by recurrent pulmonary embolism and chronic obstructive pulmonary disease (COPD). Notably, he resides in a histoplasmosis-endemic area. A computed tomography (CT) pulmonary embolism scan revealed notable findings, including an enlarged right lower pulmonary artery, vascular congestion, atelectasis, and a mass exerting pressure on the right lower pulmonary vein. Biopsy results identified the mass as fibrosing mediastinitis, likely attributed to histoplasmosis. A transthoracic echocardiogram indicated right ventricular dilatation, impaired function, and a right ventricular systolic pressure of 63 mm Hg. During right heart catheterization, the patient displayed disparate pulmonary artery wedge pressures (PAWPs) between the right and left sides. This discrepancy was linked to a blunted back wave from the left atrium to the catheter, induced by pulmonary vein compression. Although an infrequent phenomenon, the recorded asymmetry in PAWPs played a crucial role in guiding accurate patient management. The absence of subsequent evaluation of PAWP on the left side could have altered the treatment plan, potentially delaying appropriate patient care. This case emphasizes the necessity of thorough exploration with right heart catheterization when clinical symptoms warrant, highlighting the importance of standardized practices in such procedures.

Improving Outcomes in Pulmonary Vein Stenosis: Novel Pursuits and Paradigm Shifts.

Pulmonary vein stenosis (PVS) remains a clinical challenge, with progressive restenosis being common. In the past five years, we have seen an exponential increase in both clinical and scientific publication related to PVS. Central to progress in PVS clinical care is the paradigm shift towards collaborative, multidisciplinary care that utilizes a multimodality approach to treatment. This manuscript will discuss recent conceptual gains in PVS treatment and research while highlighting important outstanding questions and barriers.

Catheter ablation versus medical therapy in atrial fibrillation: an umbrella review of meta-analyses of randomized clinical trials.

This umbrella review synthesizes data from 17 meta-analyses investigating the comparative outcomes of catheter ablation (CA) and medical treatment (MT) for atrial fibrillation (AF). Outcomes assessed were mortality, risk of hospitalization, AF recurrence, cardiovascular events, pulmonary vein stenosis, major bleeding, and changes in left ventricular ejection fraction (LVEF) and MLHFQ score. The findings indicate that CA significantly reduces overall mortality and cardiovascular hospitalization with high strength of evidence. The risk of AF recurrence was notably lower with CA, with moderate strength of evidence. Two associations reported an increased risk of pulmonary vein stenosis and major bleeding with CA, supported by high strength of evidence. Improved LVEF and a positive change in MLHFQ were also associated with CA. Among patients with AF and heart failure, CA appears superior to MT for reducing mortality, improving LVEF, and reducing cardiovascular rehospitalizations. In nonspecific populations, CA reduced mortality and improved LVEF but had higher complication rates. Our findings suggest that CA might offer significant benefits in managing AF, particularly in patients with heart failure. However, the risk of complications, including pulmonary vein stenosis and major bleeding, is notable. Further research in understudied populations may help refine these conclusions.

Pulmonary vein narrowing after pulsed field versus thermal ablation.

AIMS: When it occurs, pulmonary vein (PV) stenosis after atrial fibrillation (AF) ablation is associated with significant morbidity. Even mild-to-moderate PV narrowing may have long-term implications. Unlike thermal ablation energies, such as radiofrequency (RF) or cryothermy, pulsed field ablation (PFA) is a non-thermal modality associated with less fibrotic proliferation. Herein, we compared the effects of PFA vs. thermal ablation on PV narrowing after AF ablation. METHODS AND RESULTS: ADVENT was a multi-centre, randomized, single-blind study comparing PFA (pentaspline catheter) with thermal ablation-force-sensing RF or cryoballoon (CB)-to treat drug-refractory paroxysmal AF. Pulmonary vein diameter and aggregate cross-sectional area were obtained by baseline and 3-month imaging. The pre-specified, formally tested, secondary safety endpoint compared a measure of PV narrowing between PFA vs. thermal groups, with superiority defined by posterior probability > 0.975. Among subjects randomized to PFA (n = 305) or thermal ablation (n = 302), 259 PFA and 255 thermal ablation (137 RF and 118 CB) subjects had complete baseline and 3-month PV imaging. No subject had significant (≥70%) PV stenosis. Change in aggregate PV cross-sectional area was less with PFA (-0.9%) than thermal ablation (-12%, posterior probability > 0.999)-primarily driven by the RF sub-cohort (-19.5%) vs. CB sub-cohort (-3.3%). Almost half of all PFA PV diameters did not decrease, but the majority (80%) of RF PVs decreased, regardless of PV anatomic location. CONCLUSION: In this first randomized comparison of PFA vs. thermal ablation, PFA resulted in less PV narrowing-thereby underscoring the qualitatively differential and favourable impact of PFA on PV tissue.

Utility of simultaneous triple balloon inflation technique through a single 6-French sheath in treating pulmonary vein stenosis.

Pulmonary vein stenosis continues to pose significant challenges in children, frequently requiring repeated cardiac catheterisation procedures. This report describes a successful application of a "triple kissing balloon" technique to treat complex pulmonary vein stenosis in two patients, all accomplished with the use of a single 6-French sheath.

An extreme case of cor triatriatum mimicking hypoplastic left heart syndrome and combined pulmonary vein stenosis.

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

Hybrid approach to pulmonary vein stenting after Fontan palliation.

Pulmonary vein stenosis is poorly tolerated in patients who have undergone Fontan palliation and typically requires surgical or transcatheter intervention. Percutaneous transcatheter approaches to intervention can be technically difficult due to challenging anatomy. A hybrid per-atrial transcatheter approach for stenting pulmonary veins provides a direct approach to the pulmonary veins and has the potential to improve safety and efficacy of this complex intervention. We describe our experience with hybrid per-atrial pulmonary vein stenting in three patients with pulmonary vein stenosis following Fontan palliation.

Evaluation of paediatric pulmonary vein stenosis by cardiac CT angiography: a comparative study with transthoracic echocardiography and catheter angiogram.

AIM: To compare prospective electrocardiogram (ECG)-gated cardiac computed tomographic angiography (CCTA) with transthoracic echocardiography (TTE) and cardiac catheter angiography (CCA) for paediatric pulmonary vein (PV) stenosis. MATERIALS AND METHODS: Retrospective chart review was undertaken of all patients who underwent CCTA for PV evaluation over a 4-year period. Patient demographics, findings of CCTA, TTE, and CCA, as well as interventions performed, were recorded for each PV. RESULTS: Thirty-five patients were included (23 male patients). All patients had a prior TTE with time interval between TTE and CCTA ranging from 0 to 90 days. CCTA detected 92 abnormalities in 32 patients. TTE missed 16 PV abnormalities (16/92, 17%), detected 37 abnormalities with certainty (37/92, 40%), and was suggestive in 39 abnormalities (39/92, 42%). CCTA was negative for PV abnormalities when TTE was positive or suspicious in three patients. Nineteen patients underwent CCA (18 patients with 52 abnormalities and one patient with normal PV), confirming CCTA findings. Thirty-nine were treated with angioplasty/stenting (39/52,75%). Failed recanalisation occurred in three PVs (3/52, 6%) and no intervention was attempted for the rest as the gradient was not significant (10/52,19%). Nine patients underwent surgical repair (26/92, 28%). Five patients (14/92, 15%) were managed with no intervention based on CCTA findings and poor clinical prognosis. CONCLUSIONS: CCTA plays an important role in detecting paediatric PV stenosis and identifies additional findings compared to TTE that have direct surgical/interventional implications. CCTA complements TTE in imaging these patients and helps guide management.

Outcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) in children is a challenging condition with poor outcomes. Post-operative stenosis can occur after repair of anomalous pulmonary venous return (APVR) or stenosis within native veins. There is limited data on the outcomes of post-operative PVS. Our objective was to review our experience and assess surgical and transcatheter outcomes. Single-center retrospective study was performed including patients < 18 years who developed restenosis after baseline pulmonary vein surgery that required additional intervention(s) from 1/2005 to 1/2020. Non-invasive imaging, catheterization and surgical data were reviewed. We identified 46 patients with post-operative PVS with 11 (23.9%) patient deaths. Median age at index procedure was 7.2 months (range 1 month-10 years), and median follow-up was 10.8 months (range 1 day-13 years). Index procedure was surgical in 36 (78.3%) and transcatheter in 10 (21.7%). Twenty-three (50%) patients developed vein atresia. Mortality was not associated with number of affected veins, vein atresia, or procedure type. Single ventricle physiology, complex congenital heart disease (CCHD), and genetic disorders were associated with mortality. Survival rate was higher in APVR patients (p = 0.03). Patients with three or more interventions had a higher survival rate compared to patients with 1-2 interventions (p = 0.02). Male gender, necrotizing enterocolitis, and diffuse hypoplasia were associated with vein atresia. In post-operative PVS, mortality is associated with CCHD, single ventricle physiology, and genetic disorders. Vein atresia is associated with male gender, necrotizing enterocolitis, and diffuse hypoplasia. Multiple repeated interventions may offer a patient survival benefit; however, larger prospective studies are necessary to elucidate this relationship further.

[Pulmonary vein stenosis with pulmonary infarction secondary to primary mediastinal seminoma: a case report].

Pulmonary vein stenosis is a rare condition that is often underdiagnosed and misdiagnosed. The clinical and radiologic manifestations are unspecific such as cough, hemoptysis and pulmonary lesions and are therefore difficult to distinguished with pneumonia and tuberculosis. The present study is a successful case report of pulmonary vein stenosis and pulmonary infraction secondary to mediastinal seminoma. This case suggested that pulmonary vein stenosis should be considered when a mediastinal mass is accompanied by pulmonary opacites that cannot be explained by common causes such as infection.

Pathogenesis, Evaluation, and Management of Pulmonary Vein Stenosis: JACC Review Topic of the Week.

Pulmonary vein stenosis (PVS) can arise from several etiologies, including congenital, acquired, and iatrogenic sources. PVS presents insidiously, leading to significant delays in diagnosis. A high index of suspicion and dedicated noninvasive evaluation are key to diagnosis. Once diagnosed, both noninvasive and invasive evaluation may afford further insights into the relative contribution of PVS to symptoms. Treatment of underlying reversible pathologies coupled with transcatheter balloon angioplasty and stenting for persistent severe stenoses are established approaches. Ongoing refinements in diagnostic modalities, interventional approaches, postintervention monitoring, and medical therapies hold promise to further improve patient outcomes.

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

BACKGROUND: 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.Methods and Results: 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. CONCLUSIONS: 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.

Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.

BACKGROUND: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. OBJECTIVE: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. MATERIALS AND METHODS: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. RESULTS: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. CONCLUSION: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.

Predictors of Serious Adverse Events and High-Level Cardiorespiratory Support in Patients Undergoing Transcatheter Pulmonary Vein Interventions.

Patients with pulmonary vein stenosis (PVS) often require frequent transcatheter pulmonary vein (PV) interventions for management of restenosis. Predictors of serious adverse events (AEs) and need for high-level cardiorespiratory support (mechanical ventilation, vasoactive support, and/or extracorporeal membrane oxygenation) 48 h after transcatheter PV interventions have not been reported. This is a single-center retrospective cohort analysis of patients with PVS who underwent transcatheter PV interventions from 3/1/2014 to 12/31/2021. Univariate and multivariable analyses were performed using generalized estimating equations to account for within-patient correlation. 240 patients underwent 841 catheterizations involving PV interventions (median 2 catheterizations per patient [1,3]). At least one serious AE was reported in 100 (12%) cases, the most common of which were pulmonary hemorrhage (n = 20) and arrhythmia (n = 17). There were 14 severe/catastrophic AEs (1.7% of cases) including three strokes and one patient death. On multivariable analysis, age less than 6 months, low systemic arterial saturation (< 95% in patients with biventricular [BiV] physiology, < 78% in single ventricle [SV] physiology), and severely elevated mean PA pressure (≥ 45 mmHg in BiV, ≥ 17 mmHg in SV) were associated with SAEs. Age less than 1 year, hospitalization prior to catheterization, and moderate-severe RV dysfunction were associated with high-level support after catheterization. Serious AEs during transcatheter PV interventions in patients with PVS are common, although major events such as stroke or death are uncommon. Younger patients and those with abnormal hemodynamics are more likely to experience serious AEs and require high-level cardiorespiratory support after catheterization.

Paroxysmal Atrial Fibrillation Ablation Using a Novel Variable-Loop Biphasic Pulsed Field Ablation Catheter Integrated With a 3-Dimensional Mapping System: 1-Year Outcomes of the Multicenter inspIRE Study.

BACKGROUND: The inspIRE study (Study for Treatment of Paroxysmal Atrial Fibrillation [PAF] by Pulsed Field Ablation [PFA] System With Irreversible Electroporation [IRE]) evaluated safety and effectiveness of a fully integrated biphasic pulsed field ablation (PFA) system with a variable-loop circular catheter for the treatment of drug-refractory paroxysmal atrial fibrillation. METHODS: Subjects underwent pulmonary vein (PV) isolation with the PFA system, using at least 12 applications per vein; adenosine/isoproterenol was administered to confirm entrance block. Wave I assessed initial safety, including for esophageal lesions, silent cerebral lesions, and PV stenosis. Wave II (pivotal phase) tested (1) primary safety, incidence of early-onset primary adverse events, and (2) primary effectiveness, confirmed PV isolation with freedom from documented atrial arrhythmia at 12 months. The study design specified an interim analysis to determine early success once 30 subjects reached the 12-month follow-up and all subjects reached 3-month follow-up. RESULTS: Across 13 centers in Europe/Canada, 226 subjects were enrolled, met criteria for safety and effectiveness evaluations, and received PFA (Wave I, 40; Wave II, 186). Wave I demonstrated no esophageal thermal lesions or PV stenosis. Among 39 subjects with cerebral magnetic resonance imaging, silent cerebral lesions were detected in 4 of the first 6 subjects, after which workflow enhancements, including a 10-second pause between PFA applications, were implemented; subsequently, only 4 of 33 subjects had silent cerebral lesions. In the Wave II phase, no primary adverse events were reported. Upon declaring early success, 83 subjects reached 12-month follow-up. With 100% entrance block, PV isolation without acute reconnection was achieved in 97.1% of targeted veins. For Wave II, the primary effectiveness end point per Kaplan-Meier at the time of interim analysis was 70.9%; 12-month freedom from symptomatic atrial fibrillation/atrial flutter/atrial tachycardia recurrence and repeat ablation was 78.9% and 92.3%, respectively. Total procedure and transpired PFA times were 70.1±27.7 and 26.7±14.0 minutes, respectively. CONCLUSIONS: The inspIRE trial confirmed the safety and effectiveness of the novel mapping-integrated PFA system. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; unique identifier: NCT04524364.

Cardiovascular magnetic resonance pulmonary perfusion for functional assessment of pulmonary vein stenosis.

BACKGROUND: Cardiovascular magnetic resonance (CMR) imaging allows to combine pulmonary perfusion measurements and pulmonary venous angiography during a single-session examination with both imaging modules representing the basis for accurate diagnosis and therapeutic stratification of pulmonary vein (PV) stenosis. The present study investigated the clinical utility of dynamic pulmonary perfusion imaging integrated into a comprehensive CMR protocol for the evaluation of patients with suspected PV stenosis. METHODS: 162 patients with clinically suspected PV stenosis after catheter ablation of atrial fibrillation underwent a combined single-session CMR examination (cardiac cine imaging, dynamic pulmonary perfusion, and three-dimensional PV angiography). CMR angiography was used for visual grading of PV stenoses; dynamic pulmonary perfusion imaging was evaluated per lung lobe visually and quantitatively. RESULTS: All PV stenosis ≥90% showed a visible perfusion deficit of the corresponding lung lobe (60/60, 100%) while all PVs with luminal narrowing <50% exhibited normal pulmonary perfusion (680/680, 100%). However, every third 70-89% stenosis showed a normal pulmonary perfusion (10/31, 32%) while every fourth 50-69% PV stenosis was associated with hypoperfusion of the corresponding lung lobe (9/39, 23%). For quantitative pulmonary perfusion measurements, ROC analysis demonstrated high discriminatory power regarding PV stenosis detection with the highest AUC values for time-to-peak enhancement (cut-off value, 8.5 s). CONCLUSIONS: The combination of CMR angiography and CMR pulmonary perfusion allowed for assessment of the anatomical degree of PV stenosis and its hemodynamic impact on the pulmonary parenchymal level. Thus, the proposed comprehensive CMR protocol provided an efficient diagnostic work-up of patients with suspected PV stenosis.