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.

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.

The many faces and outcomes of pulmonary vein stenosis in early childhood.

Pulmonary vein stenosis is a rare and poorly understood condition causing obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, increasingly severe dyspnea, hemoptysis, respiratory difficulty, recurrent respiratory tract infections/pneumonia, cyanosis, and subcostal retractions. On chest radiograph, the most frequent finding is increased interstitial, ground-glass and/or reticular opacity. Transthoracic echocardiography with pulsed Doppler delineates the stenosis, magnetic resonance imaging and multislice computerized tomography are used for further evaluation. Interventional cardiac catherization, surgical techniques, and medical therapies have been used with varying success as treatment options.

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.

Stent implantation for severe pulmonary vein stenosis or occlusion secondary to atrial fibrillation ablation.

BACKGROUND: Catheter interventional treatment of pulmonary vein stenosis or occlusion (PVS/O) following radiofrequency ablation (RFA) for atrial fibrillation (AF) remains a challenging field due to lacking randomized data and there are limited data about stenting. METHODS: All patients at our center who underwent pulmonary vein stenosis (PVS) stenting for PVS/O induced by RFA were retrospectively assessed. Clinical presentation, anatomic site of stenosis and hemodynamic information, as well as follow-up data, were collected and analyzed. RESULTS: From January 2010 to June 2018, 56 patients with PVS/O secondary to RFA were treated with 113 stents. Procedural success rate was 95.8%. Pressure gradients significantly reduced (p < 0.001) and vessel caliber markedly increased (p < 0.001) in all the stenotic sites, with immediate symptoms significantly improved. The overall in-stent restenosis (ISR) rate was 21.8% after a median follow-up period of 26 months (interquartile range: 16 to 35.5 months). The incidence of ISR was higher in patients treated with stent diameter ≤ 8 mm than those with stent diameter >8 mm (HR: 3.91; 95% CI 1.74-8.81; p = 0.001). CONCLUSIONS: PVS stenting is a safe and effective procedure for PVS/O secondary to RFA. Long-term patency and good clinical outcomes are gained after bigger-diameter stent implantation.

Diagnostic performance of CT angiography to detect pulmonary vein stenosis in children.

To assess the diagnostic efficiency of CT angiography (CTA) to detect pulmonary vein stenosis in children. We retrospectively identify patients between 0 and 3 years old with confirmed pulmonary vein stenosis with conventional angiography or surgery and available CTA. Patients without confirmed stenosis of the pulmonary veins were included as controls. We excluded patients with previous surgery involving the pulmonary veins, exclusively right-heart conventional angiography or insufficient data in the operation note to confirm the status of the pulmonary veins. Two pediatric radiologists evaluated and determine the presence of stenosis and the pulmonary veins affected. Disagreement between the readers were solved by consensus with a third reader. A pediatric cardiologist reviewed the available angiographic images to determine the presence of stenosis. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated. Kappa statistics was performed. 26 patients (15 boys, 11 Girls) were included. Conventional angiography (n = 20) and cardiothoracic surgery (n = 6) confirmed the diagnosis of pulmonary vein stenosis in 13 children. The diagnostic performance at patient level showed the sensitivity, specificity, PPV, and NPV were 84.6%, 92.3%, 91.6%, 87.5%, and 88.4%, respectively. The interobserver was k = 0.76. The performance at pulmonary vein level showed the sensitivity, specificity, PPV, and NPV were 63.3%, 97.4%, 90.4%,85.7% and 87.9%, respectively. The interobserver agreement was k = 0.62. Computed Tomography Angiography is an excellent and reliable image technique for ruling in pulmonary vein stenosis in young children.

Management outcomes of primary pulmonary vein stenosis.

OBJECTIVES: Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures. METHODS: A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed. Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial switch were excluded. Regression analyses were performed to examine characteristics, PPVS features, including severity score, and surgical details associated with treatment failures. RESULTS: Thirty-four children underwent initial surgical intervention for PPVS. Median age was 8.9 months (interquartile range, 5.9-18.4 months). Most patients (n = 31; 91%) had unilateral pulmonary vein involvement and the median PPVS severity score was 3.5 (interquartile range, 3-5). On competing risk analysis, 1 year following surgical repair, 9% of patients had died, 14% had undergone reintervention, and 77% were alive without reintervention; at 5 years the numbers were 9%, 30%, and 61%, respectively. Factors associated with mortality included bilateral disease and PPVS severity score >6. Bilateral disease and PPVS severity score >5 were associated with reintervention risk. CONCLUSIONS: Multidisciplinary management strategy is required for PPVS. Despite satisfactory early repair, patients continue to be at risk for recurrence and subsequent mortality, especially those with extensive primary involvement. The disappointing results underscore the need for multi-institutional collaborations to better understand this complex disease, establish management and follow-up protocols, and explore investigational treatment modalities that could modify the unfavorable outcome of this uncommon and challenging disease.

Bilateral pulmonary vein stenting for treatment of massive hemoptysis caused by pulmonary vein stenosis following catheter ablation for atrial fibrillation.

BACKGROUND: Massive hemoptysis is a life-threatening condition. Massive hemoptysis caused by pulmonary vein stenosis (PVS) after radiofrequency catheter ablation for atrial fibrillation (AF) is rare. However, bilateral lung hemorrhage following bilateral PVS is extremely rare. CASE PRESENTATION: We herein describe a 62-year-old man with refractory massive hemoptysis after radiofrequency catheter ablation for AF, which was successfully controlled by surgical lobectomy and endovascular bilateral PV stenting. The hemorrhage was derived from the bilateral lungs following PV obstruction and bilateral PVS, which was definitively diagnosed by bronchoscopic examination. The patient had no recurrence of hemoptysis during a follow-up period of 30 months, and the PV stents had not narrowed as shown by computed tomography 30 months after stent placement. CONCLUSIONS: Massive hemoptysis can be caused by bilateral PVS after radiofrequency catheter ablation for AF, and hemorrhage from the bilateral lungs in such patients is extremely rare. Nevertheless, cardiologists, interventional radiologists, and pulmonologists should consider the potential for massive hemoptysis caused by PVS.

Comparison of drug eluting versus bare metal stents for pulmonary vein stenosis in childhood.

OBJECTIVE: Comparison of outcomes using bare metal (BMS) and drug-eluting (DES) stents in pulmonary vein stenosis (PVS). BACKGROUND: PVS is a serious condition with frequent restenosis after surgical and percutaneous interventions. After experiencing encouraging results with DES, we sought to compare outcomes of BMS and DES in native and post-surgical PVS. METHODS AND RESULTS: A retrospective review of all patients who underwent stent implantation between 08/93 and 11/17 for PVS at Texas Children's Hospital was performed. BMS were used to treat 58 lesions in 37 patients and 105 DES used to treat 105 lesions in 41 patients. Mean age at first stent implant was 2.9 ± 3.5 years in BMS and 16.2 ± 18.8 months in DES group. Of those with follow-up catheterization, mean lumen loss rate from stent implant to first follow-up catheterization was 0.85 ± 1.47 mm/month over 6.4 ± 6.4 months in the BMS group (n = 44 lesions) compared to 0.16 ± 0.31 mm/month over 6.8 ± 7.4 months in the DES group (n = 86 lesions), p < .01. Follow-up for the BMS group was 14 months (6 days-22.3 years), with 13 mortalities, eight lesions were re-stented and six complete occlusions were noted. Follow-up for DES group (including four cross-overs) was 17.5 months (3 days-9 years), with 10 mortalities, seven lesions were re-stented, 11 had complete occlusion, 20 new adjacent lesions in the same vessel underwent stenting and 12 stents were intentionally fractured. CONCLUSION: DES have significantly lowered lumen loss rate when compared to BMS at medium term follow-up and can be fractured to enable larger diameters. Availability of larger diameter DES would be ideal.

Mortality in infants with bronchopulmonary dysplasia: Data from cardiac catheterization.

RATIONALE: Pulmonary hypertension (PH) is relatively common in infants with severe bronchopulmonary dysplasia (BPD), however, hemodynamic data and factors associated with mortality in this patient group are sparsely described in the literature. OBJECTIVES: To characterize the hemodynamics of former preterm infants with BPD and PH, as measured at cardiac catheterization, and to identify respiratory and cardiovascular predictors of mortality. METHODS: Single-center, retrospective cohort study, including, 30 patients born at less than 32-week gestational age (GA), who had an oxygen requirement at 36 weeks postmenstrual age and underwent cardiac catheterization between July 2014 and December 2017. RESULTS: Median GA at birth was 25 5/7 weeks (interquartile range [IQR], 24 4/7-26 6/7 weeks). Median birth weight was 620 g (IQR, 530-700 g). With a median of 23 months of follow up (IQR, 11-39 months), mortality as of July 2018 was 27% (8 of 30). The alveolar-arterial oxygen gradient as a measure of lung disease did not correlate with mortality (log-rank test P = 0.28). However, indexed pulmonary vascular resistance (PVR) of greater than 3 Woods units × m 2 showed a trend toward increased mortality (log-rank test P = 0.12). Pulmonary vein stenosis was the only predictor significantly associated with mortality (log-rank test P = 0.005). CONCLUSIONS: In this cohort, the severity of lung disease as assessed by impaired oxygenation at cardiac catheterization did not correlate with mortality. The only factor significantly associated with mortality was the presence of pulmonary vein stenosis on cardiac catheterization, although PVR may also be an important factor.

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: Outcomes in Children With Congenital Heart Disease and Prematurity.

Pulmonary vein stenosis (PVS) is a rare condition that has been linked to prematurity and congenital heart disease (CHD). Despite these associations, treatment options are limited and outcomes are guarded. We investigated differences in PVS outcomes based on the presence of CHD and prematurity, and risk factors for mortality or lung transplantation in PVS. Single-center retrospective cohort study of patients diagnosed with PVS between January 2005 and May 2016 and identified by ICD codes with chart validation. Cox proportional hazard models assessed risk factors for the composite outcome of mortality or lung transplantation. Ninety-three patients with PVS were identified: 65 (70%) had significant CHD, 32 (34%) were premature, and 14 (15%) were premature with CHD. Sixty-five (70%) underwent a PVS intervention and 42 (46%) underwent ≥2 interventions. Twenty-five subjects (27%) died or underwent lung transplant 5.8 months (interquartile range [IQR] 1.1, 15.3) after diagnosis. There was no difference in age at diagnosis or mortality based on presence of CHD or prematurity. PVS diagnosis before age 6 months and greater than 1 pulmonary vein affected at diagnosis were associated with higher mortality (hazards ratio [HR] 3.4 (95% confidence interval 1.5, 7.5), P = 0.003, and HR 2.1 per additional vein affected (95% confidence interval 1.3, 3.4), P = 0.004, respectively). Survival in children with PVS is poor, independent of underlying CHD or prematurity. Younger age and greater number of veins affected at diagnosis are risk factors for worse outcome. Understanding causal mechanisms and development of treatment strategies are necessary to improve outcomes.

Never Say Never: The Use of Nitric Oxide in Patients With Obstructed Pulmonary Veins: A Case Report.

Pulmonary vein stenosis (PVS) is a progressive disease with pulmonary hypertension (PH) as a major cause of morbidity and mortality. Traditional management of PH with inhaled nitric oxide (iNO) is typically avoided in PVS patients because, while iNO may reduce pulmonary vascular resistance, PH persists as pulmonary blood flow increases in the presence of a downstream resistive lesion. We report 3 cases with primary PVS and PH in which iNO was used to successfully decrease mean pulmonary artery pressures with clinical improvement. Based on this experience, we suggest that iNO can be used to treat PH in select patients with PVS.

Acquired Pulmonary Vein Stenosis After Radiofrequency Ablation for Atrial Fibrillation: Single-Center Experience in Catheter Interventional Treatment.

OBJECTIVES: The aim of the present study was to analyze and report a single-center experience with catheter interventional treatment of radiofrequency-induced pulmonary vein stenosis (PVS) following atrial fibrillation (AF) ablation. BACKGROUND: Catheter interventional treatment of radiofrequency-induced PVS following AF ablation remains a challenging field because of a lack of randomized data and treatment guidelines. METHODS: All patients at a single center who underwent catheter interventional treatment for radiofrequency-induced PVS were retrospectively assessed. RESULTS: From January 2004 to September 2017, the total rate of PVS following interventional AF ablation was 0.78% (87 of 11,103). Thirty-nine patients with PVS were treated with 84 catheter interventions: 68 (81%) with percutaneous transluminal balloon angioplasty (PTA) and 16 (19%) with stent implantation. The distribution of stent type was 3 drug-eluting stents (19%) and 13 bare-metal stents (81%). The overall restenosis rate was 53% after PTA versus 19% after stent implantation (p = 0.007) after a median follow-up period of 6 months (interquartile range: 3 to 55 months). The total complication rate for PTA was 10% versus 13% for stenting (p = NS). CONCLUSIONS: This study demonstrates significantly better outcomes in terms of restenosis after stent implantation versus PTA only, with comparable complication rates for these 2 options of interventional treatment of radiofrequency-induced PVS. In summary, despite the lack of randomized studies, the present data and currently available published studies seem to favor stent implantation as a first-line therapy in patients with radiofrequency-induced severe PVS.

Assessment and Management of Pulmonary Vein Occlusion After Atrial Fibrillation Ablation.

OBJECTIVES: This study sought to evaluate the sensitivity of noninvasive imaging in the assessment of severely stenosed and occluded pulmonary veins, and examine clinical outcomes following percutaneous intervention. BACKGROUND: PV stenosis (PVS) is a rare complication of atrial fibrillation ablation, but is associated with significant morbidity. Patients present with nonspecific pulmonary symptoms that can result in delayed diagnosis and progression to PV occlusion. The assessment and management of PV occlusion has rarely been described. METHODS: This was a prospective observational study performed from 2000 to 2014. RESULTS: Computed tomography identified 124 patients with severe PVS, including 46 patients with at least 1 occluded vein. Patients with PV occlusion more frequently presented with cough (64.1% vs. 32.8%; p = 0.002) and hemoptysis (39.1% vs. 14.1%; p = 0.0015) and were more likely to have pulmonary parenchymal consolidation (77.3% vs. 41.7%; p = 0.0002). Intervention was attempted in 65 occluded veins and a residual microchannel was identified in 22 (34.0%). Balloon angioplasty was performed in 11, and 11 were treated with stenting. Over 3 years the rates of restenosis were similar for patients with PVS and PV occlusion (47.0% vs. 35.0%; p = 0.24). Among patients with PV occlusion, stenting significantly reduced the rate of restenosis (hazard ratio: 3.97; 95% confidence interval: 1.14 to 13.85; p = 0.03). CONCLUSIONS: Veins deemed occluded on noninvasive imaging require invasive characterization, as residual microchannels may be present in one-third of patients. In patients with a microchannel, intervention can be performed with either balloon angioplasty or stenting. Recurrence remains a common problem; however, stenting significantly reduces the rate of subsequent restenosis.

Key technical steps in dual-pathway repair of congenital pulmonary vein stenosis.

We present the case of a 2-year-old girl with congenital stenosis of the left inferior pulmonary vein associated with a large perimembranous ventricular septal defect. The child underwent repair of the left inferior pulmonary vein with autologous left atrial appendage as a pedicled tube, followed by closure of the ventricular septal defect. Important technical steps to minimize the restenosis rate are highlighted.

Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era.

BACKGROUND: The optimal management and prognostic factors of postsurgical pulmonary vein stenosis remain controversial. We sought to determine current postsurgical pulmonary vein stenosis outcomes and prognostic factors in a multicentric study in the current era. METHODS: Seventy-five patients with postsurgical pulmonary vein stenosis who underwent 103 procedures in 14 European/North American centers (2000-2012) were included retrospectively. A specific pulmonary vein stenosis severity score was developed on the basis of the assessment of each pulmonary vein. End points were death, pulmonary vein reintervention, and restenosis. A univariate and multivariate risk analysis was performed. RESULTS: Some 76% of postsurgical pulmonary vein stenosis occurred after repair of a total anomalous pulmonary venous return. Sutureless repair was used in 42 of 103 procedures (41%), patch veinoplasty was used in 28 procedures (27%), and endarterectomy was used in 16 procedures (16%). Overall pulmonary vein restenosis, reintervention, and mortality occurred in 56% (n = 58/103), 49% (n = 50/103), and 27% (n = 20/75), respectively. Sutureless repair was associated with less restenosis (40% vs 67%; P = .007) and less reintervention (31% vs 61%; P = .003). Mortality after sutureless repair (20%; 7/35) tends to be lower than after nonsutureless repair (33%; 13/40) (P = .22). A high postoperative residual pulmonary vein stenosis score at the time of hospital discharge was an independent risk factor for restenosis (hazard ratio [HR], 1.55; P < 10-4), reintervention (HR, 1.33; P < 10-4), and mortality (HR, 1.37; P < 10-4). The sutureless technique was an independent protective factor against restenosis (HR, 0.27; P = .006). CONCLUSIONS: Postsurgical pulmonary vein stenosis still has a guarded prognosis in the current era. The sutureless technique is an independent protective factor against restenosis. The severity of the residual disease evaluated by a new severity score is an independent risk factor for poor outcomes regardless of surgical technique.