Atrial thrombus after total anomalous pulmonary venous connection repair.

A 6-year-old boy had previously undergone total anomalous pulmonary venous connection repair and postoperative pulmonary vein stenosis release. Magnetic resonance imaging revealed blood stasis caused by a collision between the inflow from the pulmonary veins and the outflow from the left atrial appendage. A surgical specimen revealed evidence of advanced thrombus attachment. Infra-cardiac total anomalous pulmonary venous connection with an antler appearance may be a risk factor for thrombus formation in the left atrial appendage and for postoperative pulmonary venous stenosis due to blood flow collision in the left atrium after total anomalous pulmonary venous connection repair.

De Novo and Progressive Pulmonary Vein Stenosis Following Pediatric Heart Transplantation: A Multicenter Retrospective Study.

BACKGROUND: Pulmonary vein stenosis (PVS) is a rare condition in which neointimal proliferation leads to venous and arterial hypertension. Little is known about PVS after heart transplant (HTx) in children. We sought to describe the characteristics and outcomes of children who develop PVS after HTx. METHODS: We performed a retrospective review of patients ≤18 years old who underwent HTx at two HTx centers between April 2012 and October 2023. Patients with PVS were identified via database queries. Cardiac diagnosis, PVS location and extent, and outcomes were recorded. RESULTS: Over 11.5 years, 422 patients underwent HTx across both centers. Nineteen patients with PVS (10 male) were identified, 15 with de novo PVS. Sixteen had underlying congenital heart disease (CHD), two with anomalous pulmonary venous return. PVS was diagnosed at a median of 2 months (range 2 weeks to 14 years) after HTx. At time of initial diagnosis, 13 patients had one-vessel PVS. At final follow-up, 7/19 (37%) had increases in the number of vessels involved. Six patients underwent surgery, and nine patients had stent or balloon angioplasty. Two patients were treated for pulmonary hypertension following PVS diagnosis. Three patients died from right heart failure secondary to PVS. CONCLUSIONS: This is the largest study to describe the characteristics of post-HTx PVS in children. PVS occurs in 4.5% of HTx, and underlying CHD is a strong risk factor. Multiple vessels can be involved and may require catheter-based or surgical intervention. Clinicians must be vigilant in monitoring the development of PVS in this population.

Pediatric Lung Transplantation for Pulmonary Vascular Diseases: Recent Advances and Challenges.

Pediatric lung transplantation for pulmonary vascular diseases has seen notable advancements and trends. Medical therapies, surgical options, and bridging techniques like extracorporeal membrane oxygenation and different forms of transplants have expanded treatment possibilities. Current challenges include ensuring patient adherence to post-transplant therapies, addressing complications like primary graft dysfunction and rejection, and conducting further research in less common conditions like pulmonary veno-occlusive disease and pulmonary vein stenosis. In this review article, the authors will explore the advancements, emerging trends, and persistent challenges in pediatric lung transplantation for pulmonary vascular diseases.

Efficacy of Drug-Coated Balloon Angioplasty in Pulmonary Vein Stenosis or Total Occlusion.

BACKGROUND: Current therapies for pulmonary vein stenosis (PVS) or pulmonary vein total occlusion (PVTO) involving angioplasty and stenting are hindered by high rates of restenosis. OBJECTIVES: This study compares a novel approach of drug-coated balloon (DCB) angioplasty and stenting with the current standard of care in PVS or PVTO due to pulmonary vein isolation (PVI). METHODS: A retrospective single-center study analyzed patients with PVS or PVTO due to PVI who underwent either angioplasty and stenting (NoDCB group; December 2012-December 2016) or DCB angioplasty and stenting (DCB group; January 2018-January 2021). Multivariable Andersen-Gill regression analysis assessed the risk of restenosis and target lesion revascularization (TLR). RESULTS: The NoDCB group comprised 58 patients and 89 veins, with a longer median follow-up of 35 months, whereas the DCB group included 26 patients and 33 veins, with a median follow-up of 11 months. The DCB group exhibited more PVTO (NoDCB: 12.3%; DCB: 42.4%; P = 0.0001), with a smaller reference vessel size (NoDCB: 10.2 mm; DCB: 8.4 mm; P = 0.0004). Follow-up computed tomography was performed in 82% of NoDCB and 85% of DCB, revealing lower unadjusted rates of restenosis (NoDCB: 26%; DCB: 14.3%) and TLR (NoDCB: 34.2%; DCB: 10.7%) in the DCB group. DCB use was associated with a significantly lower risk of restenosis and TLR (HR: 0.003: CI: 0.00009-0.118; P = 0.002). CONCLUSIONS: The novel approach of DCB angioplasty followed by stenting is effective and safe and significantly reduces the risk of restenosis and reintervention compared with the standard of care in PVS or PVTO due to PVI.

Venous retrograde approach for endovascular angioplasty in chronic total pulmonary vein occlusion -a case report.

INTRODUCTION: Fibrosing mediastinitis (FM) is a rare disease characterized by excessive proliferation of fibrous tissue in the mediastinum and can cause bronchial stenosis, superior vena cava obstruction, pulmonary artery and vein stenosis, etc. CASE PRESENTATION: An aging patient with intermittent chest tightness and shortness of breath was diagnosed with FM associated pulmonary hypertension (FM-PH) by echocardiography and enhanced CT of the chest, and CT pulmonary artery (PA)/ pulmonary vein (PV) imaging revealed PA and PV stenosis. Selective angiography revealed complete occlusion of the right upper PV, and we performed endovascular intervention of the total occluded PV. After failure of the antegrade approach, the angiogram revealed well-developed collaterals of the occluded RSPV-V2b, so we chose to proceed via the retrograde approach. We successfully opened the occluded right upper PV and implanted a stent. CONCLUSIONS: This report may provide new management ideas for the interventional treatment of PV occlusion.

Post-RFA Pulmonary Vein Stenosis Mimicking Lymphangitic Carcinomatosis in a Patient With History of Liposarcoma.

ABSTRACT: A 58-year-old man with resected liposarcoma exhibited persistent irregular interlobular septal thickening of lung on CT concerning lymphangitic metastasis. FDG PET/CT was positive in these foci. There was a narrowing of the pulmonary veins, which had not been seen on the imaging conducted prior to radiofrequency ablation therapy for atrial fibrillation. Lung perfusion scanning demonstrated more extensive hypoperfusion. This case highlights the diagnostic pitfalls of interstitial thickening of the lung in cancer patients. Meticulous history review and nuclear imaging helped distinguish the postablation pulmonary venous stenosis from metastatic spread to the lung interstitium.

Interventions for Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) is a rare and unique disease of infants and young children. PVS is attended by high morbidity and mortality, and for many decades, effective therapy eluded the practitioner. However, in the most recent era, interventional techniques when employed in combination with systemic (primary) therapy have had a remarkable impact on outcomes in these at-risk children. Despite apparent complete relief of PVS in a discrete region of a pulmonary vein, stenosis reliably recurs and progresses. In this review, we discuss the current state-of-the-art interventional techniques, through the lens of our collective experiences and practices.

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.

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.

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

BACKGROUND: 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. METHODS AND RESULTS: 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. CONCLUSIONS: All PVS lesions were successfully enlarged by the PV intervention; however, restenosis developed in approximately half the lesions within 2 years.

Changes in pulmonary vein size and narrowing depend on the cardiac cycle before and after pulmonary vein isolation.

Accurate measurement of the pulmonary vein dimension (PVD) is important for determining stenosis and efficacy following pulmonary vein isolation (PVI). Little is known about the quantitative evaluation of the impact of the cardiac cycle on pulmonary vein (PV) morphology before and after PVI. This study aims to investigate variations in the ostial size of the PV during the cardiac cycle before and after PVI and the effect of the cardiac cycle on PV stenosis and reduction rate using cardiac computed tomography (CT). Sixty-eight patients with atrial fibrillation who underwent cardiac CT before and after PVI at our institution between 23 January 2021 and 5 February 2022 were retrospectively analyzed. The maximum and minimum PVD were measured at each segment before and after the PV. Each PV was evaluated according to the PVD reduction rate (ΔPVD), calculated as follows: (1 - post-PVD/pre-PVD) × 100 (%). The average dimension of all PVs at the end-diastolic frame was significantly reduced compared to that at the end-systolic frame before PVI. The average dimensions of the right superior and right inferior PV at the end-diastolic frame were significantly reduced compared with those at the end-systolic frame following PVI. The average reduction rate of dimension-classified stenosis of PVs, except for the left inferior PV at the end-diastolic frame, was significantly reduced compared with that at the end-systolic frame. The cardiac cycle affects PVD assessment, including PV stenosis, after PVI. PVD measurement is recommended to be unified to the end-systolic frame of the cardiac cycle to avoid underestimating PV stenosis before and after PVI, ensuring appropriate management and follow-up.

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.