scholarly journals Complex treatment of recurrent pulmonary vein stenosis

Author(s):  
Я. Б. Микичак

Pulmonary vein stenosis (PVS) in children is a rare heart disease characterized by high recurrence and mortality rates. Objective. We describe a case of recurrent PVS, which was treated surgically and subsequently with repeated balloon angioplasties. Materials and methods. Echocardiogram showed total anomalous pulmonary venous connection, mixed form. A CT scan of the chest confirmed the diagnosis. Month later after initial surgery angiography detected the presence of stenosis in all pulmonary veins. Results. Currently patient has moderate residual stenosis in each PV. His prognosis is considered bleak due to continuous requirement in aggressive interventions in order at least temporarily resolve pulmonary vein stenosis. Conclusions. Pulmonary vein stenosis still bears high recurrence, reintervention and mortality rates. Rigorous surveillance and combination of surgical and interventional treatment is warranted in this subset of patients to maintain quality of life.

2020 ◽  
Vol 36 (6) ◽  
pp. 580-584
Author(s):  
Dana Jones

Surveillance of patients with congenital heart disease is difficult in some clinical settings. This case study follows the progress of a patient, born with prenatally missed diagnoses of tetralogy of Fallot (TOF) and total anomalous pulmonary venous return (TAPVR), for over two years. The patient acquired pulmonary vein stenosis following initial TOF/TAPVR repair, which made the case an interesting longitudinal study. Multiple cardiac catheterizations for stents and balloon angioplasties of the pulmonary veins were necessary. This case study illustrates this patient’s success with treatment options such as chemotherapy medications, as well as the failure of each treatment. It was also important to note that the interval of time between each interventional treatment weighed heavily upon results of frequent echocardiograms.


Children ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 193
Author(s):  
Ryan Callahan ◽  
Zachary Gauthier ◽  
Shuhei Toba ◽  
Stephen P. Sanders ◽  
Diego Porras ◽  
...  

Preliminary intravascular ultrasound (IVUS) images of suspected pediatric intraluminal pulmonary vein stenosis (PVS) demonstrate wall thickening. It is unclear how the IVUS-delineated constituents of wall thickening correlate with the histology. We analyzed six postmortem formalin-fixed heart/lung specimens and four live patients with PVS as well as control pulmonary veins using IVUS and light microscopic examination. In PVS veins, IVUS demonstrated wall thickening with up to two layers of variable echogenicity, often with indistinct borders. Histologically, the veins showed fibroblastic proliferation with areas rich in myxoid matrix as well as areas with abundant collagen and elastic fibers. Discrete vein layers were obscured by scarring and elastic degeneration. A lower reflective periluminal layer by IVUS corresponded with hyperplasia of myofibroblast-like cells in abundant myxoid matrix. The hyper-reflective layer by IVUS extended to the outer edge of the vessel and corresponded to a less myxoid area with more collagen, smooth muscle and elastic fibers. The outer less reflective edge of the IVUS image correlated with a gradual transition into adventitia. Normal veins had a thin wall, correlating with histologically normal cellular and extracellular components, without intimal proliferation. IVUS may provide further understanding of the anatomy and mechanisms of pediatric pulmonary vein obstruction.


Author(s):  
Y. Mykychak ◽  
D. Kozhokar ◽  
I. Yusifli ◽  
O. Yachnik ◽  
V. Zakharova ◽  
...  

Pulmonary vein stenosis (PVS) is a rare but serious condition characterized by obstruction of extrapulmonary segments of pulmonary veins which leads to progressive pulmonary hypertension. Objective. In this study we aimed to determine relationship between clinical features and histopathologic findings in patients with PVS. Material and methods. We retrospectively reviewed 34 consecutive patients who underwent PV stenosis repair. Surgical wedge biopsy specimens were collected intraoperatively in 11 patients and reviewed using light microscopy. Results. Affected pulmonary veins in patients with primary PVS were characterized by diffuse stenosis extending into the lung parenchyma. In post-surgical group stenosis was found in a limited segment of pulmonary vein at its ostium. Microscopically, abnormal intimal proliferation was identified in both patient groups. Scaring was predominant finding in patients with post-surgical PVS. Conclusion. In patients with PVS, pathophysiological mechanism influences the severity and extent of clinical manifestations. A comprehensive understanding of this mechanism may improve results of the treatment.


2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
John J. Lee ◽  
Denis Weinberg ◽  
Rishi Anand

Pulmonary vein stenosis is a well-established possible complication following an atrial fibrillation ablation of pulmonary veins. Symptoms of pulmonary vein stenosis range from asymptomatic to severe exertional dyspnea. The number of asymptomatic patients with pulmonary vein stenosis is greater than originally estimated; moreover, only about 22% of severe pulmonary vein stenosis requires intervention. We present a patient with severe postatrial fibrillation (AF) ablation pulmonary vein (PV) stenosis, which was seen on multiple imaging modalities including cardiac computed tomography (CT) angiogram, lung perfusion scan, and pulmonary angiogram. This patient did not have any pulmonary symptoms. Hemodynamic changes within a stenosed pulmonary vein might not reflect the clinical severity of the obstruction if redistribution of pulmonary artery flow occurs. Our patient had an abnormal lung perfusion and ventilation (V/Q) scan, suggesting pulmonary artery blood flow redistribution. The patient ultimately underwent safe repeat atrial fibrillation ablation with successful elimination of arrhythmia.


2007 ◽  
Vol 15 (5) ◽  
pp. 438-440 ◽  
Author(s):  
Shin Takabayashi ◽  
Hideto Shimpo ◽  
Kazuto Yokoyama ◽  
Masaki Kajimoto ◽  
Koji Onoda

An 11-month-old boy with congenital pulmonary vein stenosis, partial anomalous pulmonary venous connection, and ventricular septal defect is described. Angiocardiography demonstrated stenosis between the right upper pulmonary vein and high superior vena cava and obstruction of the right lower pulmonary vein. For pulmonary vein stenosis, we performed transverse sutured plasty for the right upper pulmonary vein, followed by right lower lobectomy. In some patients, combined management for pulmonary vein stenosis is effective.


2016 ◽  
Vol 27 (5) ◽  
pp. 870-876 ◽  
Author(s):  
Brian Kogon ◽  
Jan Fernandez ◽  
Subhadra Shashidharan ◽  
Kirk Kanter ◽  
Bahaaldin Alsoufi

AbstractBackgroundPatients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.MethodsWe reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.ResultsA total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).ConclusionThe surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.


Author(s):  
Mithun M. Shenoi ◽  
Xiaoqing Zhang ◽  
Ramji T. Venkatasubramanian ◽  
Erin D. Grassl ◽  
Lenny George ◽  
...  

Over 2 million adults in the United States are affected by atrial fibrillation (AF), a common cardiac arrhythmia that is associated with decreased survival, increased cardiovascular morbidities, and a decrease in quality of life. Atrial fibrillation can be initiated by ectopic beats originating in the myocardial sleeves surrounding the pulmonary veins [1]. Pulmonary vein (PV) isolation via radiofrequency ablation is the current gold standard for treating patients with drug-refractory AF [2]. However, cryoablation is emerging as a new minimally-invasive technique to achieve PV isolation. Cryoablation is fast gaining acceptance due to its minimal tissue disruption, decreased thrombogenicity, and reduced complications (RF can lead to low rate of pulmonary vein stenosis) [2]. One important question in regard to this technology is whether the PV lesion is transmural and circumferential and to what extent adjacent tissues are involved in the freezing process. As ice formation lends itself to image contrast in the body, we hypothesized that intraprocedural CT visualization of the iceball formation would allow us to predict the extent of the cryolesion and/or provide us with a measure of the adjacent tissue damage.


Author(s):  
Naama R. Bogot ◽  
Amir Elami ◽  
Dorith Shaham ◽  
Philip M. Berman ◽  
Jacob Sosna ◽  
...  

Objective The Cox-Maze procedure using cryoablation results in transmural lesions, which follow the lesion pattern of the cut-and-sew Cox-Maze procedure. The purpose of our study was to evaluate the effect of the Cox-Maze procedure on left atrial and pulmonary vein size using computed tomography angiogram (CTA). An additional aim was to evaluate pulmonary vein anatomic variability. Methods Six patients (four women and two men; ages 39–63 years, mean age 54.3) underwent chest CTA 1 day before and 38 to 104 days (mean 62.6 days) after the cryosurgical Cox-Maze procedure. Measurements of pulmonary vein ostia and left atrial cranio-caudal, left-to-right and anterior-posterior diameters were derived by consensus. The change in diameters after therapy was compared using the Wilcoxon nonparametric test for paired measurements. Four patients (1 woman and 3 men; age 57–73 years; mean age 59) were evaluated with postoperative CTA alone 296–530 days (mean 447) after surgery, for the development of postoperative pulmonary vein stenosis. A single patient underwent preoperative CTA, but surgery was not performed. Pulmonary venous anatomy was recorded in all 11 patients. Results Sinus rhythm was restored in all operated patients. No focal ostial stenosis of the pulmonary veins was observed. The quantitative assessment in the six patients with preoperative and postoperative studies disclosed only slight changes in pulmonary vein diameter with either reduction or dilatation of no more than 20% from baseline (P > 0.05). There was a consistent trend toward decrease in left atrial dimensions, which did not reach statistical significance. Six patients (55%) had standard pulmonary venous anatomy and five patients (45%) had at least one variation in their pulmonary vein anatomy. Conclusions In this study, we found that a very intensive cryoablation protocol around the pulmonary veins did not result in pulmonary vein stenosis. In addition, a relatively high incidence of anatomic variations of the pulmonary veins was documented.


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