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.

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.

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.

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.

Doppler Echocardiographic Features of Pulmonary Vein Stenosis in Ex-Preterm Children.

BACKGROUND: Echocardiography is used to screen for the presence of pulmonary vein stenosis (PVS) in ex-preterm infants and children. However, there are no standard accepted criteria for screening or diagnosis of PVS by echocardiography. The aim of this study was to identify Doppler waveform features and Doppler systolic and diastolic velocity cutoff values associated with a diagnosis of PVS by cardiac catheterization. METHODS: In this retrospective observational study, the echocardiograms of ex-preterm children <3 years old who underwent cardiac catheterization at a single institution were reviewed. PVS on cardiac catheterization was defined by a mean pressure gradient of >3 mm Hg in the pulmonary vein, with angiographic evidence of stenosis. Pulmonary vein Doppler waveforms, from echocardiograms obtained before catheterization, in children with and without PVS were compared. Nonstenosed veins in patients with PVS were excluded. The systolic and diastolic velocities of blood flow, phasic flow, and return of the Doppler waveform to baseline were analyzed. RESULTS: Forty-seven children were analyzed in the study, 18 children with 25 stenosed pulmonary veins and 29 children with 78 nonstenosed pulmonary veins. Stenosed pulmonary veins had higher peak systolic and diastolic velocities and higher peak and mean pressure gradients as measured by spectral Doppler. Peak systolic and diastolic velocities had areas under the receiver operating characteristic curve of 0.89 (95% CI, 0.79-0.99) and 0.93 (95% CI, 0.85-0.99) for PVS, respectively, and a threshold velocity of 0.7 m/sec had sensitivity of 80% and 84% and specificity of 94%. There was no correlation between Doppler-derived pulmonary vein mean gradient and measured pulmonary vein mean gradient during cardiac catheterization in stenosed pulmonary veins. Presence of phasic flow in the pulmonary vein and return of the Doppler waveform to baseline were associated with a nonstenosed pulmonary vein (sensitivity of 94% and 60% and specificity of 52% and 60%, respectively). CONCLUSIONS: Systolic and diastolic Doppler velocities and features of the waveform can discriminate stenosed pulmonary veins confirmed by cardiac catheterization in ex-preterm children. These results suggest the use of lower systolic and diastolic Doppler velocity cutoff values than currently published to screen for PVS in ex-preterm children. These cutoff values require validation in prospective studies.

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.

Intravascular Ultrasound for Pulmonary Vein Stenosis Interventions in Congenital Heart Disease.

OBJECTIVE: Pulmonary vein stenosis (PVS) is aggressive, with high morbidity and mortality. Surgical and catheter interventions yield modest success, at best. Refinements in catheter interventions could potentially improve outcomes in this patient population. The goal of this study was to determine the utility of intravascular ultrasound (IVUS) for patients with congenital heart disease and PVS. METHODS: Single-center, retrospective review of patients with congenital heart disease and PVS undergoing diagnostic or interventional catheterizations from March 2015 to February 2020. IVUS of the pulmonary veins was performed using an Eagle Eye Platinum IVUS catheter (Volcano Corporation). RESULTS: Five patients underwent 6 procedures (2 diagnostic, 4 interventional). Median age was 1.5 years (range, 0.7-47.5 years) and weight was 8.8 kg (range, 7.3-61 kg). For the interventional procedures, mean pulmonary vein gradient was 8.7 mm Hg with reduction to 1.1 mm Hg (P<.001). Four patients had congenital PVS and 1 patient was post repair of Scimitar syndrome with an obstructed pulmonary venous baffle. Use of IVUS allowed confirmation of stent expansion and apposition, interval vessel growth after initial stenting, and detection of long-segment hypoplasia, unlikely to respond to intervention. There were no thrombotic complications related to IVUS use. CONCLUSIONS: IVUS of the pulmonary veins is safe and easy to perform, and provides detailed imaging of PVS to help guide therapy. For those requiring intervention, adequate stent apposition to the pulmonary vein walls, as well as limiting vessel overdilation, may minimize future in-stent stenosis and need for reintervention in this challenging disease.

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.

MRI detection of occult venous anomalies in a patient with Williams syndrome: a case report.

Williams syndrome is a multisystem, congenital disorder which is commonly associated with arterial stenoses: supravalvar aortic stenosis and peripheral pulmonary artery stenosis. Venous abnormalities have not been previously reported in children with Williams syndrome. We present a case of a 3-year-old girl with Williams syndrome and diffuse venous ectasia as detected by MRI.

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.

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.

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.

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.