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.

Comparison between cryoballoon and hot balloon ablation in patients with paroxysmal atrial fibrillation.

PURPOSE: Pulmonary vein (PV) isolation using balloon ablation was developed as a technique for patients with paroxysmal atrial fibrillation (PAF). While most studies examined cryoballoon ablation (CBA), there have also been many reports on hot balloon ablation (HBA). We aimed to evaluate the clinical characteristics and outcomes between HBA and CBA. METHODS: In a total of 103 consecutive patients with PAF who underwent catheter ablation, 60 propensity score-matched (30 CBA and 30 HBA) patients were enrolled. The procedural differences and clinical outcomes between the two groups were analyzed. RESULTS: The requirement for additional touch-up ablation was more frequent in the left superior pulmonary vein (LSP) in the HBA group than in the CBA group. Pre-procedural computed tomography (CT) images showed that a thicker left pulmonary vein ridge and larger cross-sectional area of the LSPV were significantly associated with residual PV potentials after HBA. However, post-procedural CT images showed that PV stenosis (> 25%) was higher in the HBA group (33%) than in the CBA group (0%). PV stenosis after HBA was observed most frequently in the right superior PV (50%). The atrial fibrillation/atrial tachycardia-free survival rate during follow-up (365 ± 102 days) was similar between the two groups (CBA vs. HBA, 83% vs. 90%). CONCLUSIONS: Although both balloon modalities can relieve atrial arrhythmia after the procedure, careful attention is required during HBA procedures, especially for the right superior PV, to avoid PV stenosis.

Clinical Outcomes, Predictors, and Surgical Management of Primary Pulmonary Vein Stenosis.

BACKGROUND: Surgical outcomes for primary pulmonary vein stenosis (PPVS) remain unfavorable, and risk factors are still poorly understood. This study evaluated outcomes and risk factors after PPVS repair. METHODS: This retrospective study included 40 patients with PPVS who underwent surgical repair in Fuwai Hospital from 2010 to 2020. Adverse outcomes included overall death, pulmonary vein (PV) restenosis, and reintervention. A univariate and multivariate risk analysis was performed to determine risk factors. RESULTS: The mean follow-up duration was 37.5 ± 31.5 months. Sutureless technique was performed in 7 patients (17.5%), endovenectomy in 9 (22.5%), and patch venoplasty in 24 (60%). Bilateral PV involvement was documented in 12 patients (30%). Overall death, PV reintervention, and restenosis occurred in 15%, 12.5%, and 25% of patients, respectively. Freedom from overall death, PV reintervention, and restenosis at 5 years was 85% ± 6.3%, 88.9% ± 5.2%, and 65.1% ± 13.2%, respectively. Multivariate analysis revealed that bilateral PV involvement was an independent risk factor for death or PV reintervention (hazard ratio, 10.4; 95% confidence interval, 1.9-56; P = .006) and that involvement of the left inferior PV was an independent risk factor for postoperative restenosis of the left inferior PV (hazard ratio, 13.1; 95% confidence interval, 2.2-76.8; P = .004). CONCLUSIONS: Surgical treatment for PPVS remains a challenging issue with imperfect prognosis. Therefore, it is right and appropriate to take close surveillance on mild or moderate stenosis on a single PV. Bilateral and left inferior PV involvement are independent risk factors for adverse outcomes.

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.

Refractory Hemoptysis Caused by Severe Pulmonary Vein Stenosis after Multiple Catheter Ablations.

We herein report a 48-year-old man with a history of chronic atrial fibrillation (AF) and repeated hemoptysis after radiofrequency ablation. Contrast tomography showed soft tissue thickening of the left hilar region and left pulmonary vein stenosis. We performed bronchial artery embolization, but the hemoptysis did not disappear, and AF was not controlled. We performed left lung lobectomy and maze procedures since we considered surgical removal necessary as radical treatment. After the surgery, hemoptysis and atrial fibrillation did not recur. Refractory hemoptysis after catheter ablation is rare, but occasionally occurs in patients with severe pulmonary vein stenosis.

Pulmonary vein reconstruction for pulmonary vein stenosis and occlusion after surgical ablation.

Acquired pulmonary vein (PV) stenosis (PVS) is a complication following cardiac catheter intervention. However, very few cases of PVS after surgical ablation have been reported. We herein report a case of stenosis and occlusion at the left atrium to each pulmonary vein after surgical ablation. A 73-year-old woman who had received aortic valve replacement and pulmonary vein isolation 10 months earlier was diagnosed with congestive heart failure accompanied by pulmonary hypertension. Contrast-enhanced computed tomography revealed stenosis and complete occlusion of the left atrium to all four pulmonary veins. Surgical repair was performed via pericardial patch reconstruction of the left atrium to each PV. Treating multiple PV lesions with involvement of the left atrium wall requires tailored methods. However, there have been few reports concerning such methods of reconstruction. We herein report a method of reconstructing the left atrium and pulmonary veins at the same time.

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.

Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant.

Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Successful repair of infantile scimitar syndrome with contralateral pulmonary vein stenosis in an infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Percutaneous Pulmonary Vein Stenting to Treat Severe Pulmonary Vein Stenosis After Surgical Reconstruction.

A 36-year-old female underwent left lower lobectomy with left atrial and left upper pulmonary vein (LUPV) reconstruction with a bovine pericardial patch for an intrathoracic pheochromocytoma. Postoperatively, she developed shortness of breath and transesophageal echocardiography demonstrated LUPV stenosis with increased velocities. Computed tomography angiogram of the chest revealed LUPV stenosis at the left atrium ostium with an area of 39 mm2. Under angiographic and echocardiographic guidance, a 10 × 19 mm Omnilink Elite uncovered stent was deployed in the LUPV ostia. While reported following left atrial ablation, pulmonary vein stenting can be successful in a pulmonary vein surgically reconstructed with bovine pericardium.

Pulmonary vein narrowing after visually guided laser balloon ablation: Occurrence and clinical correlates.

INTRODUCTION: There are limited data focusing on pulmonary vein (PV) narrowing following ablation using a visually guided laser balloon (VGLB). We sought to assess the frequency and predictors of PV narrowing after VGLB ablation. METHODS AND RESULTS: Patients with paroxysmal atrial fibrillation treated with VGLB were screened. Study participants underwent contrast-enhanced computed tomography (CT) scanning before and 6 months after the procedure. We defined 25% to 49%, 50% to 74%, and 75% to 100% reduction in PV cross-sectional area as mild, moderate, and severe narrowing, respectively. Of 146 PVs in 38 patients analyzed, severe narrowing developed in two right superior and one right inferior PV. Moderate or severe narrowing occurred in 40 veins (27% of all PVs, 50% of the right superior, 22% of the right inferior, 21% of the left superior, and 14% of the left inferior PV). In PVs with moderate-severe narrowing, the baseline orifice area was significantly larger (4.1 [interquartile range, 3.2-4.8] vs 2.5 [1.9-3.3] cm2 , P < .0001), the narrowest region of stenosis was significantly more distal into the vessel (1.9 [0.7-2.9] vs 0 [0-1.7] mm from the orifice, P = .0006) and the total amount of energy delivered per vein was significantly greater (5190 ± 970 vs 4626 ± 1573 J, P = .018) than in PVs with mild or no significant narrowing. The baseline orifice area independently predicted moderate-severe narrowing in multivariate analysis (odds ratio, 1.8 [1.3-2.5] per 1 cm2 increase, P = .0003). No patient exhibited any signs or symptoms of PV stenosis. CONCLUSIONS: Baseline PV orifice area, ablating distally inside the veins, and total amount of laser energy are associated with PV narrowing after VGLB ablation.

Modified sutureless repair using left atrial appendage flap for acquired left-sided pulmonary vein stenosis.

Pulmonary vein stenosis is a well-known complication after radiofrequency catheter ablation of atrial fibrillation. Although surgical repair is indicated for younger patients and patients with multiple stenoses, the appropriate procedure for acquired pulmonary vein stenosis has not been established. In this study, we report the successful outcome of our modified sutureless technique using a left atrial appendage flap for left-sided pulmonary vein stenosis after radiofrequency catheter ablation.

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.

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single-secondary center experience.

BACKGROUND AND AIM OF THE STUDY: Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect. This study aimed to evaluate the outcome of isolated TAPVC repairs and the prognoses of affected patients in the last 12 years at a single center. METHODS: We retrospectively analyzed the medical records of 51 patients who underwent isolated TAPVC repair from 2007 to 2018. RESULTS: The median age at operation was 19 days, and the median body weight was 3.3 kg. Thirteen (25.5%) patients had emergency operations, and the median follow-up period was 29.54 ± 36.77 months. Early mortality was noted in five patients and late mortality was noted in one patient. Pulmonary vein stenosis was observed in 22 patients within 3 to 6 months after the operation, and six patients required reoperation or transcatheter interventions. Low birth weight, small left atrial volume, long operation time, and preoperative heart failure were identified as risk factors for mortality. CONCLUSIONS: Isolated TAPVC can rapidly lead to hemodynamic instability during the neonatal period and is associated with high mortality rates. Increasing the prenatal diagnosis rate and stabilizing the patients' condition before the operation are considered important for improving the surgical outcome.

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.

Video-Assisted Thoracoscopic Pneumonectomy for Complications of Congenital Pulmonary Venous Stenosis.

Congenital pulmonary venous stenosis is an uncommon congenital heart defect and is a consequence of failed incorporation of the common right or left pulmonary vein into the left atrium during embryologic development. Clinical manifestations include progressive exertional dyspnea, cough, chest pain, and hemoptysis. In this report, we describe a 37-year-old woman with known congenital unilateral pulmonary venous stenosis and a unilateral hypoplastic right pulmonary artery who presented with recurrent episodes of hemoptysis, requiring multiple catheter-based interventions, and symptomatic pleural effusions, requiring multiple large-volume thoracenteses. The patient underwent successful video-assisted thoracoscopic pneumonectomy with resolution of symptoms.