Pulmonary vein stenosis complicating catheter ablation of focal atrial fibrillation

INTRODUCTION: A recently described focal origin of atrial fibrillation, mainly inside pulmonary veins, is creating new perspectives for radiofrequency catheter ablation. However, pulmonary venous stenosis may occur with uncertain clinical consequences. This report describes a veno-occlusive syndrome secondary to left pulmonary vein stenosis after radiofrequency catheter ablation. METHODS AND RESULTS: A 36-year-old man who experienced daily episodes of atrial fibrillation that was refractory to antiarrhythmic medication, including amiodarone, was enrolled in our focal atrial fibrillation radiofrequency catheter ablation protocol. The left superior pulmonary vein was the earliest site mapped, and radiofrequency ablation was performed. Atrial fibrillation was interrupted and sinus rhythm restored after one radiofrequency pulse inside the left superior pulmonary vein. Atrial fibrillation recurred and a new procedure was performed in an attempt to isolate (26 radiofrequency pulses around the ostium) the left superior pulmonary vein. Ten days later, the patient developed chest pain and hemoptysis related to severe left superior and inferior pulmonary veins stenosis. Balloon angioplasty of both veins was followed by complete relief of symptoms after 2 months of recurrent pulmonary symptoms. The patient has been asymptomatic for 12 months, without antiarrhythmic drugs. CONCLUSION: Multiple radiofrequency pulses applied inside the pulmonary veins ostia can induce severe pulmonary venous stenosis and veno-occlusive pulmonary syndrome.     

An unusual cause of pulmonary vein stenosis: a case report and review of the literature

We describe the case of a patient with the Carney syndrome and several resections for recurrent left atrial myxomas who underwent autotransplantation of the heart with resection of the left and right atria and reconstruction of both atria with bovine pericardium. She subsequently presented with severe shortness of breath, ascites, and peripheral edema. She was found to have stenosis of all four pulmonary veins and severe pulmonary hypertension. We describe the echocardiographic findings and review the literature on assessment of acquired pulmonary vein stenosis.     

Intrastent sonotherapy in pulmonary vein restenosis: a new treatment for a recalcitrant problem

A 2 year old boy developed recurrent pulmonary vein stenosis after surgical repair of infradiaphragmatic pulmonary venous connection. He had required implantation of stents in the left and right sided pulmonary veins at 7 and 13 months of age, respectively. By 2 years of age he had undergone three catheterisation procedures and two surgical procedures to treat recurrent pulmonary vein stenosis. His right ventricular pressure was suprasystemic and catheterisation showed severe neointimal proliferation of both left and right sided stents. At this time the stents were dilated by balloon with simultaneous intrastent sonotherapy. Three months later the patient’s clinical improvement was significant, his right ventricular pressure had decreased, and Doppler velocity had decreased across both left and right sided stents.     

Successful stent treatment of pulmonary vein stenosis following atrial fibrillation radiofrequency ablation

Pulmonary vein stenosis is a known complication of radiofrequency ablation of atrial fibrillation foci. We report a case of severe unilateral pulmonary vein stenosis following radiofrequency ablation resulting in no perfusion to the right lung. Simple balloon dilation was unsuccessful in this patient. Stent placement in the stenotic right pulmonary veins resulted in reperfusion of the lung as documented by follow-up lung scan at one year.     

肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄

目的 分析先天性肺静脉狭窄患儿的临床特点,探讨肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄的疗效.方法 回顾性分析2008年1月至2009年12月收治的4例先天性肺静脉狭窄患儿的临床资料,其中男3例,女1例,年龄2个月～2岁11个月.均行胸片、心电图和超声心动图检查,其中2例行心脏多排计算机断层扫描检查,以期明确诊断.4例患者共有7支肺静脉狭窄,右上肺静脉3例,右下肺静脉1例,左上肺静脉1例,左下肺静脉2例.狭窄部位均在肺静脉开口处,均合并其他心血管畸形：室间隔缺损3例,房间隔缺损1例,动脉导管未闭2例,主动脉缩窄1例,术前均提示重度肺动脉高压.术前根据病情予强心、利尿、扩血管及抗感染等治疗.完善术前准备后在全身麻醉、胸骨正中切口、深低温、体外循环下行肺静脉内膜剥脱术.结果 术后患儿血流动力学稳定;机械通气时间3.7（2～4）d,重症监护时间4.2（3～5）d;围术期无一例死亡.随访18～26个月,患儿均存活,心功能（纽约心脏协会）Ⅰ级3例、Ⅱ级1例,生长发育均正常.结论 先天性肺静脉狭窄常合并各种心内畸形,超声心动图、心脏多排计算机断层扫描检查对诊断帮助较大.肺静脉内膜剥脱术治疗先天性肺静脉狭窄患儿安全、有效,手术成功率高,近期随访疗效较好,对于婴幼儿先天性肺静脉狭窄不失为一种治疗选择.     

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 stenosis following catheter ablation of atrial fibrillation

PURPOSE OF REVIEW: This review provides an update on the mechanisms, incidence, and current management of significant pulmonary vein stenosis following catheter ablation of atrial fibrillation. RECENT FINDINGS: Catheter ablation involving the pulmonary veins and the surrounding left atrial tissue is increasingly used to treat atrial fibrillation. In parallel with the fact that these procedures may cure a substantial proportion of patients, severe complications have been observed. Pulmonary vein stenosis is a new clinical entity produced by radiofrequency energy delivery mainly within or at the orifice of the pulmonary veins. The exact incidence is currently unknown because the diagnosis is dependent on the imaging modality and on the rigor with which patients are followed up. The optimal method for screening patients has not been determined. Stenosis of a pulmonary vein may be assessed by combining anatomic and functional imaging using computed tomographic or magnetic resonance imaging, transesophageal echocardiography, and lung scanning. Symptoms vary considerably and may be misdiagnosed, leading to severe clinical consequences. Current treatment strategies involve pulmonary vein dilatation or stenting; however, the restenosis rate remains high. The long-term outcome in patients with pulmonary vein stenosis is unclear. Strategies under development to prevent pulmonary vein stenosis include alternate energy sources and modified ablation techniques. SUMMARY: Pulmonary vein stenosis following catheter ablation is a new clinical entity that has been described in various reports recently. There is much uncertainty with respect to causative factors, incidence, diagnosis, and treatment, and long-term sequelae are unclear.     

Multimodality imaging to diagnose pulmonary vein stenosis following atrial fibrillation ablation

BACKGROUND: Atrial fibrillation (AF) is increasing in prevalence. Although experienced centers performing radiofrequency pulmonary vein isolation procedures have reported success rates approaching 72%, in rare instances it is complicated by stenosis of one or more pulmonary veins. In this report we present a case of recurrent pulmonary vein stenosis (PVS) following radiofrequency ablation for AF.     

Thoracic Aortic Aneurysm Presenting as Pulmonary Vein Stenosis

We present the case of a 66-year-old woman presenting with pulmonary vein stenosis with a large descending thoracic aortic aneurysm. Pulmonary vein stenosis is a rare condition and can be caused by extrinsic compression, as well as by inflammatory diseases, congenital anomalies and related surgical repair, tuberculosis, and pulmonary veno-occlusive disease. With obstruction to pulmonary vein flow, the velocity increases and becomes continuous. The finding of turbulent antegrade flow in the left atrium through the use of transthoracic color flow Doppler and pulsed-Doppler warrants further investigation to evaluate known causes of pulmonary vein stenosis. We believe this is the first reported case of a patient with an aortic aneurysm causing pulmonary vein stenosis.     

Case Report: Pulmonary Vein Stenosis Following RF Ablation of Paroxysmal Atrial Fibrillation: Successful Treatment with Balloon Dilation

Paroxysmal atrial fibrillation and atrial tachycardia may originate from a focal source in one or multiple pulmonary veins. A focal origin facilitates a potential cure amendable to radiofrequency ablation. Herein we report the case of a 16 year old adolescent male with a tachycardia induced cardiomyopathy who presented with very frequent paroxysmal episodes of atrial fibrillation, atrial flutter and atrial tachycardia. The origin of the arrhythmia was mapped to the secondary branches of the left lower pulmonary vein using an octapolar micro-mapping catheter. Immediately following application of three radiofrequency lesions, angiography of the left lower pulmonary vein revealed a region of focal stenosis at the site of energy application, with delayed pulmonary venous emptying. Attempts to relieve any element of spasm using direct administration of nitroglycerin were unsuccessful. Three months later repeat catheterization revealed an unchanged region of tight anatomical stenosis. Balloon dilation of two stenotic areas resulted in dramatic relief of the obstruction and improved venous drainage. Recatheterization 6 months later revealed mild restenosis that was successfully redilated. Intracardiac ultrasound demonstrated focal constriction. Care should be exercised in attempting RF ablation in distal arborization sites of the pulmonary veins in children, because of the small caliber compared to adult subjects. Radiofrequency induced focal areas of stenosis may be amenable to balloon catheter dilation.     

Broken-heart syndrome (Takotsubo syndrome)

We report on the rare case of partial anomalous return of four pulmonary veins in the right atrium and superior vena cava with intact interatrial septum in a five-year-old child. There were few symptoms in contrast with the left ventricular output dependent on the flow of the left upper lobe vein and from the lingula. Reduced compliance to the left led to a severe picture of pulmonary venocapillary hypertension in the immediate postoperative period, mitigated by an 8-mm interatrial septal defect. The patient progressed well after the intervention.     

Thoracic Aortic Aneurysm Presenting as Pulmonary Vein Stenosis: A Case Presentation and Review of the Literature

We present the case of a 66-year-old woman presenting with pulmonary vein stenosis with a large descending thoracic aortic aneurysm. Pulmonary vein stenosis is a rare condition and can be caused by extrinsic compression, as well as by inflammatory diseases, congenital anomalies and related surgical repair, tuberculosis, and pulmonary veno-occlusive disease. With obstruction to pulmonary vein flow, the velocity increases and becomes continuous. The finding of turbulent antegrade flow in the left atrium through the use of transthoracic color flow Doppler and pulsed-Doppler warrants further investigation to evaluate known causes of pulmonary vein stenosis. We believe this is the first reported case of a patient with an aortic aneurysm causing 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.     

Diagnosis of pulmonary venous obstruction by Doppler echocardiography

A 1 1/2-year-old female patient with atrial and ventricular septal defects and severe pulmonary arterial hypertension was found to have discrete stenosis of left upper and middle lobe pulmonary veins. The diagnosis was made by cross-sectional and Doppler echocardiography. The findings were confirmed at cardiac catheterization and operation. Pressure gradients across the pulmonary vein stenosis as obtained by Doppler echocardiography correlated well with those measured at cardiac catheterization.     

Catheter intervention for abnormal pulmonary venous drainage

Transcatheter interventions for congenital heart anomalies are constantly improving. Although correction of anomalous pulmonary venous connection is routinely achieved through surgery, there are rare instances where the abnormal pulmonary vein has dual connections to both left atrium and the major systemic veins. Under these circumstances catheter based treatment might become a feasible option. We report a case of dual supply vertical vein connected to left upper pulmonary vein and innominate vein which was successfully obstructed by an occluder device leading to improvements in patient’s condition.     

The use of a wire control catheter to treat complex pulmonary artery or vein anatomy

The difficult performance of certain percutaneous interventions in the field of congenital heart disease is well known. Crossing pulmonary arteries in patients who have previously undergone surgical repair or stenotic pulmonary veins in infants can be typical examples of these technical challenges in the catheterization laboratory. The Venture wire 6 Fr control catheter (St Jude Medical) is compatible with a steerable tapered radiopaque tip that can be manually angulated (up to 90°) by clockwise rotation of a knob located in the proximal handle. This mechanism directs any 0.014″ guidewire and provides back-up support. This catheter has been successfully used in coronary artery intervention for crossing severely tortuous vessels, extreme angulations of side-branch ostia, jailed stents, saphenous vein graft anastomoses, and chronic total occlusions. We report the first use of the Venture wire control catheter (St Jude Medical) in the field of congenital heart disease. Patient #1 was diagnosed with pulmonary atresia and ventricular septal defect and had a proximally migrated stent in the pulmonary trunk and severe left pulmonary artery stenosis. We have used this catheter in order to cross this stent and perform left pulmonary artery stent placement. Patient #2 had postoperative vein restenosis after surgery. The Venture catheter was used to reach the obstructed insertion of the right medium lobe pulmonary vein from a transseptal approach. Techniques from coronary interventional colleagues can help interventional cardiologists in the field of congenital heart disease to treat complex situations.     

Isolated congenital left-sided pulmonary vein stenosis in an adult patient misdiagnosed as primary pulmonary hypertension

Pulmonary vein stenosis (PVS) is a rare condition, seen usually in association with congenital heart disease or secondary to various acquired causes. Isolated PVS, in adults, especially in absence of congenital heart disease is extremely uncommon. We report PVS of left sided pulmonary veins in an 18-year-old male, who had been till then diagnosed as primary pulmonary hypertension (PPH).     

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment

Background and purposeSinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium.Summary of caseWe report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect.ConclusionsInterventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.     

Univentricular heart and pulmonary stenosis with a right-to-left shunt between the innominate and pulmonary veins after Glenn operation: a case report

A 12-year-old girl with a univentricular heart and pulmonary stenosis, who had undergone Glenn operation at two years of age and Fontan operation at 11 years of age, had a right-to-left shunt via collaterals between the innominate and pulmonary veins. This right-to-left shunt was detected by two-dimensional contrast echocardiography. The contrast material was injected into the left antecubital vein appeared in the left atrium, and this was confirmed by innominate venography. The contrast material injected into the left innominate vein passed via the collaterals into the pulmonary veins bilaterally. No such cases have so far been reported. In our experience, in four of six cases after Glenn operation, including the present case, the abnormal collateral circulation from the superior vena cava to the atrium was detected by two-dimensional contrast echocardiography. This case is interesting embryologically and suggests that the connection between the pulmonary and systemic veins which is present in early fetal life, and subsequently disappears, was present after birth.     

Tight mitral stenosis with normal unilateral pulmonary capillary pressure

There are few reports of severe mitral stenosis with normal pulmonary artery wedge pressure. In order to illustrate this problem we present two adult patients with these abnormalities. Both patients had intact interatrial septum with partial anomalous pulmonary venous drainage, one to the inferior vena cava (The Scimitar Syndrome) and the other to the superior vena cava. In the first case the right pulmonary artery wedge pressure was 11 mm Hg. In the second case this pressure was only 8 mm Hg. Pulmonary arteriograms are of paramount importance in the diagnosis and surgical management. In both cases the surgical procedure with mitral commissurotomy and transplant of the anomalous pulmonary veins to the left atrium was successful.