scholarly journals Outcomes in Establishing Individual Vessel Patency for Pediatric Pulmonary Vein Stenosis

Children ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 210
Author(s):  
Ryan Callahan ◽  
Kimberlee Gauvreau ◽  
Audrey C. Marshall ◽  
Laureen M. Sena ◽  
Christopher W. Baird ◽  
...  
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Multivariable Analysis ◽  
Dose Intensity ◽  
Drug Dose ◽  
Pulmonary Vein Stenosis ◽  
Vessel Patency ◽  
Modal Treatment ◽  
The Individual ◽  
Vein Stenosis

The purpose of this study was to determine what patient and pulmonary vein characteristics at the diagnosis of intraluminal pulmonary vein stenosis (PVS) are predictive of individual vein outcomes. A retrospective, single-center, cohort sub-analysis of individual pulmonary veins of patients enrolled in the clinical trial NCT00891527 using imatinib mesylate +/− bevacizumab as adjunct therapy for the treatment of multi-vessel pediatric PVS between March 2009 and December 2014 was performed. The 72-week outcomes of the individual veins are reported. Among the 48 enrolled patients, 46 patients and 182 pulmonary veins were included in the study. Multivariable analysis demonstrated that patients with veins without distal disease at baseline (odds ratio, OR 3.69, 95% confidence interval, CI [1.52, 8.94], p = 0.004), location other than left upper vein (OR 2.58, 95% CI [1.07, 6.19], p = 0.034), or veins in patients ≥ 1 y/o (OR 5.59, 95% CI [1.81, 17.3], p = 0.003) were at higher odds of having minimal disease at the end of the study. Veins in patients who received a higher percentage of eligible drug doses required fewer reinterventions (IRR 0.76, 95% CI [0.68, 0.85], p < 0.001). The success of a multi-modal treatment approach to aggressive PVS depends on the vein location, disease severity, and drug dose intensity.

scholarly journals Correlation of Intravascular Ultrasound with Histology in Pediatric Pulmonary Vein Stenosis

Children ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 193
Author(s):  
Ryan Callahan ◽  
Zachary Gauthier ◽  
Shuhei Toba ◽  
Stephen P. Sanders ◽  
Diego Porras ◽  
...  
Keyword(s):  
Pulmonary Vein ◽  
Intravascular Ultrasound ◽  
Pulmonary Veins ◽  
Pulmonary Vein Stenosis ◽  
Elastic Fibers ◽  
Gradual Transition ◽  
Wall Thickening ◽  
Thin Wall ◽  
Myxoid Matrix ◽  
Vein Stenosis

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.

scholarly journals Relationship between Clinical Features and Histopathologic Findings in Patients with Pulmonary Vein Stenosis

2019 ◽  
pp. 61-63 ◽  
Author(s):  
Y. Mykychak ◽  
D. Kozhokar ◽  
I. Yusifli ◽  
O. Yachnik ◽  
V. Zakharova ◽  
...  
Keyword(s):  
Pulmonary Vein ◽  
Clinical Features ◽  
Pulmonary Veins ◽  
Clinical Manifestations ◽  
Lung Parenchyma ◽  
Pulmonary Vein Stenosis ◽  
Pathophysiological Mechanism ◽  
Patient Groups ◽  
Vein Stenosis ◽  
Histopathologic Findings

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.

scholarly journals Asymptomatic Pulmonary Vein Stenosis: Hemodynamic Adaptation and Successful Ablation

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
John J. Lee ◽  
Denis Weinberg ◽  
Rishi Anand
Keyword(s):  
Atrial Fibrillation ◽  
Pulmonary Artery ◽  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Lung Perfusion ◽  
Atrial Fibrillation Ablation ◽  
Clinical Severity ◽  
Pulmonary Vein Stenosis ◽  
Multiple Imaging ◽  
Vein Stenosis

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.

CT Visualization and Histopathological Assessment of Cryoablation in Pulmonary Veins

2009 ◽  
Author(s):  
Mithun M. Shenoi ◽  
Xiaoqing Zhang ◽  
Ramji T. Venkatasubramanian ◽  
Erin D. Grassl ◽  
Lenny George ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
The United States ◽  
The Body ◽  
Pulmonary Vein Stenosis ◽  
Invasive Technique ◽  
Freezing Process ◽  
Ectopic Beats ◽  
Vein Stenosis

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.

The Effect of the Cryosurgical Cox-Maze Procedure on Pulmonary Veins Diameter and Left Atrial Size

2009 ◽  
Vol 4 (4) ◽  
pp. 209-216
Author(s):  
Naama R. Bogot ◽  
Amir Elami ◽  
Dorith Shaham ◽  
Philip M. Berman ◽  
Jacob Sosna ◽  
...  
Keyword(s):  
Pulmonary Vein ◽  
Left Atrial ◽  
Statistical Significance ◽  
Pulmonary Veins ◽  
Nonparametric Test ◽  
Maze Procedure ◽  
Pulmonary Vein Stenosis ◽  
Venous Anatomy ◽  
Lesion Pattern ◽  
Vein Stenosis

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.

scholarly journals Pulmonary vein stenosis and the pathophysiology of “upstream” pulmonary veins

2014 ◽  
Vol 148 (1) ◽  
pp. 245-253 ◽  
Author(s):  
Hideyuki Kato ◽  
Yaqin Yana Fu ◽  
Jiaquan Zhu ◽  
Lixing Wang ◽  
Shabana Aafaqi ◽  
...  
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Pulmonary Vein Stenosis ◽  
Vein Stenosis

scholarly journals Aspiration Is Associated with Poor Treatment Response in Pediatric Pulmonary Vein Stenosis

Children ◽  
2021 ◽  
Vol 8 (9) ◽  
pp. 783
Author(s):  
Maria Niccum ◽  
Ryan Callahan ◽  
Kimberlee Gauvreau ◽  
Kathy J. Jenkins
Keyword(s):  
Treatment Response ◽  
Pulmonary Vein ◽  
Single Ventricle ◽  
Cohort Analysis ◽  
Multivariable Analysis ◽  
Pulmonary Vein Stenosis ◽  
Poor Treatment ◽  
Male Patients ◽  
Vein Stenosis ◽  
Poor Treatment Response

Intraluminal pulmonary vein stenosis is a disease with significant morbidity and mortality, though recent progress has been made using multimodal therapy with antiproliferative agents. The aim of this study was to evaluate the association between aspiration and poor treatment response in patients with intraluminal pulmonary vein stenosis. A retrospective, single-center cohort analysis was performed of patients treated with a combination of imatinib mesylate and multimodal anatomic relief between March 2009 and November 2019. Analysis focused on 2-ventricle patients due to small numbers and clinical heterogeneity of single ventricle patients. Among the 84 patients included, 15 had single ventricle physiology and 69 had 2-ventricle physiology. Among the 2-ventricle group, multivariable analysis revealed that patients with clinical aspiration had nearly five times higher odds of poor treatment response than patients without aspiration (OR 4.85, 95% CI [1.37, 17.2], p = 0.014). Furthermore, male patients had higher odds of poor treatment response than their female counterparts (OR 3.67, 95% CI [1.04, 12.9], p = 0.043). Aspiration is a novel, potentially modifiable risk factor for poor treatment response in pediatric multi-vessel intraluminal pulmonary vein stenosis in patients with 2-ventricle physiology.

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