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.

Current Endovascular Approach in Adult Patients with Pulmonary Vein Stenosis: A State-of-the-Art Approach.

Regarding to more invasive treatment of atrial arrhythmia (atrial fibrillation ablation) and pulmonary vein isolation (PVI), the rate of acquired pulmonary vein stenosis (PVS) is increasing and at present, PV ablation for AF has become the principal cause of PVS in adult patients. On the other way, by improvement in procedural techniques, equipment, and the experience of the operators, the incidence of PVS has been decreased. There is some controversy about the manner of follow-up of these patients and in most centers, just symptomatic patients are considered for imaging and treatment. Almost always, those with PV stenosis more than 70% or multiple PV involvement become symptomatic and if give them up without treatment, pulmonary symptoms and finally irreversible pulmonary hypertension will occurred. So, intensive pursue after the procedure is highly recommended. Whereas in pediatric patients with congenital or acquired PVS, the best treatment approach is surgery, in adult patients, the preferred type of treatment is the transcatheter intervention with high acute success rate. In this present review, we have scrutinized about the diagnostic modalities, the indications for intervention, the diverse treatment strategies, and principally clarify an accurate stepwise approach during transcatheter procedure.

Pulmonary vein stenosis mimicking interstitial lung disease.

Pulmonary vein stenosis (PVS) is a rare condition, often difficult to diagnose and associated with poor prognosis at advanced stages. Lung parenchymal abnormalities are indirect evidence of PVS and can manifest as multifocal opacities, nodular lesions, unilateral effusions, and interstitial septal thickening. These can lead to erroneous diagnoses of airway disease, pneumonia, malignancies or interstitial lung disease. This review summarizes the current literature about the approach to, evaluation and management of these patients. Our case report demonstrates that PVS is an under-recognized complication of cardiovascular surgery and should be considered in all patients presenting with respiratory symptoms after a cardiac procedure.

Pulmonary Vein Stenosis After Atrial Fibrillation Ablation: Insights From the ADVICE Trial.

BACKGROUND: Pulmonary vein (PV) stenosis is a complication of atrial fibrillation (AF) ablation. The incidence of PV stenosis after routine post-ablation imaging remains unclear and is limited to single-centre studies. Our objective was to determine the incidence and predictors of PV stenosis following circumferential radiofrequency ablation in the multicentre Adenosine Following Pulmonary Vein Isolation to Target Dormant Conduction Elimination (ADVICE) trial. METHODS: Patients with symptomatic AF underwent circumferential radiofrequency ablation in one of 13 trial centres. Computed tomographic (CTA) or magnetic resonance (MRA) angiography was performed before ablation and 90 days after ablation. Two blinded reviewers measured PV diameters and areas. PVs with stenosis were classified as severe (> 70%), moderate (50%-70%), or mild (< 50%). Predictors of PV stenosis were identified by means of multivariable logistic regression. RESULTS: A total of 197 patients (median age 59.5 years, 29.4% women) were included in this substudy. PV stenosis was identified in 41 patients (20.8%) and 47 (8.2%) of 573 ablated PVs. PV stenosis was classified as mild in 42 PVs (7.3%) and moderate in 5 PVs (0.9%). No PVs had severe stenosis. Both cross-sectional area and diameter yielded similar classifications for severity of PV stenosis. Diabetes was associated with a statistically significant increased risk of PV stenosis (OR 4.91, 95% CI 1.45-16.66). CONCLUSIONS: In the first systematic multicentre evaluation of post-ablation PV stenosis, no patient acquired severe PV stenosis. Although the results are encouraging for the safety of AF ablation, 20.8% of patients had mild or moderate PV stenosis, in which the long-term effects are unknown.

Is This My Home? A Palliative Care Journey Through Life and Death in the NICU: A Case Report.

BACKGROUND: With advancements in neonatology, patients in the neonatal intensive care unit (NICU) are living in the hospital with complex life-limiting illnesses until their first birthday or beyond. As palliative care (PC) becomes a standard of care in neonatology, a level IV NICU developed an interdisciplinary PC team with the mission to ease the physical, mental, and moral distress of the patients, families, and staff. This case report highlights the teamwork and long-term palliative care and ultimately end-of-life care that an infant received by this dedicated NICU palliative care team. CLINICAL FINDINGS: This case discusses a premature ex-27-week gestation male infant who initially presented to the emergency department at 5 months of age with significant tachypnea, increased work of breathing, and poor appetite. PRIMARY DIAGNOSIS: The primary diagnosis was severe pulmonary vein stenosis resulting in severe pulmonary hypertension. INTERVENTIONS: The severity of the infant's pulmonary vein stenosis was incurable. He required substantial life-extending surgical procedures and daily intensive care interventions. In addition to his life-extending therapies, the infant and his family received palliative care support by the NICU PC team and the hospital-wide PC team (REACH team) throughout his admission. This was specialized care that focused on easing pain and suffering while also addressing any social/emotional needs in the infant, his family, and in the hospital staff. The PC teams also focused on protecting the families' goals of care, memory making, and providing a positive end-of-life experience for the infant and his family. The infant's end-of-life care involved providing adequate pain and symptom management, education, and communication to his family about the dying process and allowing unlimited family time before and after his death. OUTCOMES: After 11 months in the NICU and despite aggressive therapies, he required more frequent trips to the cardiac catheterization laboratory for restenosis of his pulmonary veins. He was dependent on iNO to treat his pulmonary hypertension and he continued to require an ICU ventilator. His parents ultimately decided to pursue comfort care. He died peacefully in his mother's arms. PRACTICE RECOMMENDATIONS: The American Academy of Pediatrics and the National Association of Neonatal Nurses both have statements recommending that palliative care be standard of care in NICUs. Establishing a NICU-dedicated interdisciplinary PC team can improve outcomes for infants and families living in the NICU with complex life-limiting illnesses.

Severe pulmonary vein stenosis in association with IgG4 gammopathy: An unusual case.

IgG4-related disease (IgG4-RD)is a multifactorial and systemic immune-mediated disease. Pulmonary vein stenosis related to IgG4 is a rare entity. We report a case of a 57-year-old male who presented with vague symptoms of weakness, easy fatigue, shortness of breathing, chest tightness, and occasional palpitations. The patient was admitted, and investigations were arranged in the form of anterior mediastinotomy and pleural biopsies were taken to look for a possible diagnosis. The histopathology was reported as IgG4-related immunopathy. A transoesophageal echocardiogram was performed and this showed severe stenosis of left superior pulmonary vein. In patients with rare pathologies such as IgG4-RD, a transesophageal can be a useful diagnostic aid especially in patients with severely impaired renal functions which does not allow utilization of contrast computed tomography.

Management of arrhythmia recurrence in patients with pulmonary vein stenosis following atrial fibrillation ablation.

AIMS: Arrhythmia recurrence is a common clinical problem in patients with pulmonary vein stenosis (PVS) following catheter ablation of atrial fibrillation. The study sought to analyse the management of arrhythmia recurrence in patients with PVS. METHODS AND RESULTS: Retrospective analysis was performed on 29 patients with high-degree PVS. Follow-up contained clinical visits, Holter-electrocardiogram recordings and invasive pulmonary vein (PV) angiography and electrophysiological studies. Arrhythmia recurrence was observed in 18 patients (62%) after PVS formation. Fifteen of 18 patients (83.3%) with and 1 of 11 patients (9.1%) without arrhythmia recurrence had electrical PV reconnection (P = 0.0003). In 14 of 16 patients repeat pulmonary vein isolation (PVI) was conducted (radiofrequency ablation in 12 and cryoballoon ablation in 2 cases). Repeat PVI was successful in all PVs in nine patients and incomplete in five patients (failed attempt in two patients with stent implantation, no attempt in three patients with stenotic PVs). Freedom from arrhythmia recurrence was estimated at 56.3% [95% confidence interval (CI) 36.4-72.0%] after 24 months. At the end of the follow-up, complete PVI was documented in 19 of 23 patients (82.6%) with stable sinus rhythm and in 2 of 6 patients with arrhythmia recurrence (33.3%) (P = 0.0335). The Kaplan-Meier estimate of recurrence-free survival of restenosis after interventional PVS treatment was similar in patients without and with repeat ablation [75.6% (95% CI 57.0-94.3%) and 67.0% (95% CI 43.2-90.7%) after 500 days, P = 0.77]. CONCLUSIONS: Pulmonary vein reconnection is the major driver of arrhythmia recurrence in PVS patients. Repeat PVI is feasible and does not lead to progression of PVS or restenosis if the procedure is carefully performed.

Pulmonary cuff dysfunction after lung transplant surgery: A systematic review of the evidence and analysis of its clinical implications.

BACKGROUND: Pulmonary cuff dysfunction, either due to pulmonary vein obstruction, pulmonary vein stenosis, or pulmonary vein thrombosis, is an uncommon, yet serious complication after lung transplantation. Although there have been numerous reports of its occurrence, there is little consensus regarding the hemodynamic parameters associated with its presentation and diagnostic considerations. This systematic review summarizes the evidence surrounding pulmonary cuff dysfunction after lung transplantation surgery and empirically analyzes its implications. METHODS: Databases were examined for all articles and abstracts reporting on pulmonary cuff dysfunction. Data collected included: number of patients studied; patients' characteristics; incidences of pulmonary vein stenosis and pulmonary vein thrombosis; and timing and imaging modality utilized for diagnosis. RESULTS: Thirty-four full-text citations were included in this review. The point prevalence of pulmonary vein stenosis and thrombosis were 1.4% and 2.5%, respectively. The peak pulmonary cuff velocity associated with dysfunction was found to be 1.59 ± 0.66 m/sec. The diameter of the dysfunctional pulmonary vein was noted to be 0.48 ± 0.20 cm. The majority of diagnoses were made in the early post-operative period using transesophageal echocardiography. Overall, 41.3% of patients (26 of 63) required emergent procedural reintervention, and 32% of patients (20 of 63) diagnosed with pulmonary cuff dysfunction died during their hospital stay. CONCLUSIONS: This systematic review underscores the importance of identifying pulmonary cuff dysfunction after lung transplant surgery, and the usefulness of transesophageal echocardiography for detection of this complication. The clinical implications of these results warrant the further development of identification and management strategies for lung transplant patients.

Pulmonary vein stenosis with collateralization via esophageal varices: Long-term follow-up after successful treatment with drug-eluting stent.

OBJECTIVE: We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices. DESIGN: Case report SETTING: Tertiary children's hospital PATIENT: Single child through ages 2- to 11-year old INTERVENTIONS: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating in the need to induce intentional stent fracture. CONCLUSIONS: We highlight novel transcatheter techniques to treat this vexing condition, discuss mechanisms of disease treatment and progression, and present the only patient with this rare combination of lesions to have achieved both longstanding pulmonary vein patency and resolution of esophageal varices.

Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study.

BACKGROUND: Primary pulmonary vein stenosis (PPVS) still carries a poor prognosis, and prognostic factors remain controversial. The aim of this study was to determine outcomes and prognostic factors after PPVS repair in the current era. METHODS: Thirty patients with PPVS and a normal pulmonary vein (PV) connection operated on in 10 European/North American centers (2000-2012) were included retrospectively. A specific PVS severity score was developed based on the assessment of each PV. Studied end points were death, PV reoperation, and restenosis. A univariate and multivariate risk analysis was performed. RESULTS: The mean number of affected PVs per patient was 2.7 ± 1.1. Sutureless repair was used in 21 patients (70%), endovenectomy was used in 5 patients, and patch venoplasty was used in 4 patients. Overall PV restenosis, reoperation, and mortality occurred in 50%, 40%, and 30% of patients respectively. Freedom from mortality, reoperation, and restenosis at 8 years of follow-up was 70% ± 8%, 62% ± 8%, and 47% ± 9%, respectively. Restenosis and mortality rates after sutureless repair versus nonsutureless repair were 57% (n = 12 of 21) versus 33% (n = 3 of 9) (p = 0.42) for restenosis and 38% (n = 8 of 21) versus 11% (n = 1 of 9) (p = 0.21) for mortality. Patients selected for a sutureless technique were younger and smaller and had more severe disease before operation. A postoperative high PVS score and pulmonary hypertension 1 month after the operation were independent risk factors for restenosis (hazard ratio [HR], 1.34; p = 0.002 and HR, 6.81; p = 0.02, respectively), reoperation (HR, 1.24; p = 0.01 and HR, 7.60; p = 0.02), and mortality (HR, 1.39; p = 0.01 and HR, 39.5; p = 0.008). CONCLUSIONS: Primary PVS still has a guarded prognosis in the current era despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by a new severity score are independent prognostic factors regardless of surgical technique.

Simultaneous arterial switch and totally anomalous pulmonary venous connection repair in a 5-hour-old child, complicated by pulmonary venous stenosis.

A 5-hour-old boy underwent arterial switch operation and totally anomalous pulmonary venous connection repair. Subsequently developed severe pulmonary venous stenosis was successfully treated with hybrid balloon angioplasty.

Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort.

BACKGROUND: Pulmonary vein stenosis is emerging as an important clinical problem in ex-premature infants. METHODS: We sought to describe the epidemiology of pulmonary vein stenosis affecting ex-premature infants by a multicenter retrospective cohort study of patients from seven children's hospitals diagnosed between 2000-2014. RESULTS: We identified 39 ex-premature patients (26 males, median gestational age 28 weeks range 22-36 weeks, birth weight 1.1 kg range 433-2645-g) with pulmonary vein stenosis. Median age at diagnosis was 6.5 months (1 month-6 years). Presentation with pulmonary hypertension occurred in 26/39 (67%) and 29/39 (74%) had bronchopulmonary dysplasia, 15 (39%) were born of twin pregnancies with unaffected twin siblings. A median of 5 (range 1-25) echocardiograms was performed prior to diagnosis. The diagnosis was made using echocardiography in 22/39 (56%), by multi-detector contrast computed tomography scan (CT) in 8/39 (21%), cardiac catheterization in 6/39 (15%) patients, magnetic resonance imaging in 3/39 (8%). Freedom from death or re-stenosis was 73% at 1-year, 55% at 2, 5, and 10 years. Factors associated with shorter survival or re-stenosis were stenosis of ≥3 pulmonary veins (P < 0.01), bilateral pulmonary vein stenosis (P < 0.01) small for gestational age (P = 0.05), aged <6 months at diagnosis (P < 0.01). CONCLUSION: Pulmonary vein stenosis of ex-premature infants is a complex problem with poor survival, delayed diagnosis, and unsatisfactory treatment. The lack of concordance in twins suggests epigenetic or environmental factors may play a role in the development of pulmonary vein stenosis. In ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia a focused echocardiographic assessment of the pulmonary veins is required with further imaging if the echocardiogram is inconclusive.

Drug-Coated Balloon Venoplasty for In-Stent Restenosis in a Patient With Recurrent Pulmonary Vein Stenosis Post Ablation for Atrial Fibrillation: Initial Experience With a New Treatment Technique.

Pulmonary vein stenosis (PVS) is an uncommon but serious complication following radiofrequency ablation for atrial fibrillation. Occurrence of this complication has risen with increased rates of ablation procedures, with >50,000 AF ablation procedures performed per year, and can occur within weeks to months post procedure. Currently, the main therapies for PVS include percutaneous interventions with balloon angioplasty and stenting, but these treatments are complicated by a high rate of restenosis. The optimal treatment for recurrent pulmonary vein in-stent restenosis has not been determined. We describe the novel use of a paclitaxel drug-coated balloon for the treatment of in-stent restenosis of the pulmonary veins.