Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.

BACKGROUND: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. OBJECTIVE: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. MATERIALS AND METHODS: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. RESULTS: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. CONCLUSION: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.

Endovascular transatrial stenting of pulmonary vein stenosis after lung transplantation.

Pulmonary vein stenosis (PVS) and pulmonary vein occlusion (PVO) represent rare complications after lung transplantation (LTx), with limited therapeutic options and a high risk of graft loss. We present 2 cases of successful endovascular transatrial stenting following double LTx. A 60-year-old woman with chronic obstructive pulmonary disease who underwent double lobar LTx was diagnosed at postoperative day 72 with a high-grade PVS on the left side. A 22-year-old woman with idiopathic pulmonary arterial hypertension who underwent double LTx was diagnosed 9 days later with PVO of the left upper lobe vein. To avoid surgical reintervention, endovascular transatrial dilatation and stenting were performed successfully in both cases. Transatrial endovascular stenting of PVS or PVO after LTx seems an effective and safe treatment option that should be considered for these life-threatening complications and executed with care.

A Rare Report of Hypoplastic Coronary Arteries and Pulmonary Veins: A Case Report and Review of the Literature.

Congenital coronary artery anomalies are estimated to affect about 1% of the general population. Hypoplastic coronary artery disease is an uncommon subset associated with significant mortality regardless whether another major cardiac anomaly is present. In this case report, we present an extremely rare case of an infant with hypoplastic coronary artery disease and hypoplastic pulmonary veins. A literature review pertinent to the clinical findings is also contained herein.

Estenosis de vena pulmonar tras cirugía de reemplazo valvular / Pulmonary vein stenosis after valve replacement surgery

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Pulmonary cuff dysfunction after lung transplant surgery: A systematic review of the evidence and analysis of its clinical implications.

BACKGROUND: Pulmonary cuff dysfunction, either due to pulmonary vein obstruction, pulmonary vein stenosis, or pulmonary vein thrombosis, is an uncommon, yet serious complication after lung transplantation. Although there have been numerous reports of its occurrence, there is little consensus regarding the hemodynamic parameters associated with its presentation and diagnostic considerations. This systematic review summarizes the evidence surrounding pulmonary cuff dysfunction after lung transplantation surgery and empirically analyzes its implications. METHODS: Databases were examined for all articles and abstracts reporting on pulmonary cuff dysfunction. Data collected included: number of patients studied; patients' characteristics; incidences of pulmonary vein stenosis and pulmonary vein thrombosis; and timing and imaging modality utilized for diagnosis. RESULTS: Thirty-four full-text citations were included in this review. The point prevalence of pulmonary vein stenosis and thrombosis were 1.4% and 2.5%, respectively. The peak pulmonary cuff velocity associated with dysfunction was found to be 1.59 ± 0.66 m/sec. The diameter of the dysfunctional pulmonary vein was noted to be 0.48 ± 0.20 cm. The majority of diagnoses were made in the early post-operative period using transesophageal echocardiography. Overall, 41.3% of patients (26 of 63) required emergent procedural reintervention, and 32% of patients (20 of 63) diagnosed with pulmonary cuff dysfunction died during their hospital stay. CONCLUSIONS: This systematic review underscores the importance of identifying pulmonary cuff dysfunction after lung transplant surgery, and the usefulness of transesophageal echocardiography for detection of this complication. The clinical implications of these results warrant the further development of identification and management strategies for lung transplant patients.

Death or resolution: the "natural history" of pulmonary hypertension in bronchopulmonary dysplasia.

OBJECTIVES: The primary objective was to describe the early "natural history" of pulmonary hypertension (PH) in the premature population. The secondary objective was to describe factors associated with poor outcomes in the premature population with PH at 36 weeks post-menstrual age (PMA). STUDY DESIGN: Retrospective chart review of patients followed at our institution from 2000 to 2017 with echocardiographic (ECHO) evidence of PH at 36 weeks PMA, and born ≤ 32 weeks estimated gestational age (GA). Cox regression was used for survival analysis. RESULTS: Sixty-one patients with PH (26.5 ± 1.5 weeks at birth) were included. All PH patients had bronchopulmonary dysplasia (BPD), with 89% considered severe; 38% were small for gestational age. Necrotizing enterocolitis requiring surgery was common (25%). Use of post-natal steroids (HR 11.02, p = 0.01) and increased severity of PH (HR 1.05, p < 0.001) were associated with mortality. Pulmonary vein stenosis (PVS) was documented in 26% of the PH cohort, but not associated with increased mortality. ECHO estimation of pulmonary artery pressure (PAP) was available in 84%. PAP was higher in those who died (sPAP/sBP ratio 1.09 ± 27 vs 0.83 ± 20 %, p = 0.0002). At follow-up (mean 250 ± 186 weeks PMA), 72% of the PH cohort was alive. Most survivors (66%) had resolution of their PH on their most recent ECHO; 31% remained on PH therapy. CONCLUSION: PH resolved in most survivors in this study population. Mortality in those with BPD-PH was associated with male sex, post-natal steroid use, and increased severity of PH, but not with PVS.

Oclusión venosa pulmonar como complicación del tratamiento ablativo de la fibrilación auricular / Pulmonary Venous Occlusion as a Complication of Ablation Therapy for Atrial Fibrillation

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Progressive pulmonary vein stenosis in Down syndrome infant: a rare cause of pulmonary hypertension.

Pulmonary veins stenosis in a Down Syndrome infant with normal connection pulmonary vein is rare and precise incidence of this disease is unknown. We report a case of progressive multiple pulmonary vein stenosis in a Down Syndrome infant with congenital heart disease and transient myeloproliferative leukaemia. This case-report aims to improve awareness among physicians and sonographers of this disease and the importance of pulmonary vein assessment in congenital heart disease with unexplained pulmonary hypertension.

Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis: A case report and review of the literature.

INTRODUCTION: Fibrosingmediastinitis (FM) is caused by a proliferation of fibrous tissue in the mediastinum encasing the mediastinal viscera that results in compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of FM caused by extrinsic compression of pulmonary blood vessels. CASE PRESENTATION: Here, we present the case of a 47-year-old man who presented with a 10-year history of progressive hemoptysis and a 2-year history of shortness of breath, in whom a diagnosis of FM was made. Occlusion of the superior pulmonary veins was noted, with stenosis of the inferior pulmonary veins, leading to PH. Because the patient was a poor candidate for interventional catheterization, the preferred treatment for FM, his PH has been managed with diuretics, and he remains stable. CONCLUSIONS: FM is a serious, potentially life-threatening condition that is best managed in specialized centers.

Pulmonary vein stenosis with collateralization via esophageal varices: Long-term follow-up after successful treatment with drug-eluting stent.

OBJECTIVE: We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices. DESIGN: Case report SETTING: Tertiary children's hospital PATIENT: Single child through ages 2- to 11-year old INTERVENTIONS: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating in the need to induce intentional stent fracture. CONCLUSIONS: We highlight novel transcatheter techniques to treat this vexing condition, discuss mechanisms of disease treatment and progression, and present the only patient with this rare combination of lesions to have achieved both longstanding pulmonary vein patency and resolution of esophageal varices.

Pulmonary vein stenosis complicating radiofrequency catheter ablation for atrial fibrillation: A literature review.

Radiofrequency catheter ablation has become a widely used intervention in the treatment of atrial fibrillation. Pulmonary vein stenosis (PVS) is one of the most serious complications associated with this procedure; the degree of stenosis ranges from mild (<50%) to complete venous occlusion. The natural history of PVS and the risk of progression of existing PVS are uncertain. Symptomatic and/or severe PVS is a serious medical problem and can be easily misdiagnosed since it is an uncommon and relatively new medical problem, often has low clinical suspicion among clinicians, and has a non-specific presentation that mimics other more common respiratory or cardiac diseases. The estimated incidence varies in literature reports from 0% to 42% of ablation procedures, depending on technical aspects of the procedure and operator skill. Most patients with significant PVS remain asymptomatic or have few symptoms. Symptomatic patients usually present with dyspnea, chest pain, or hemoptysis and are usually treated with balloon angioplasty and/or stent placement. Little is known about the long term effect of PV stenosis/occlusion on the pulmonary circulation and the development of pulmonary hypertension. Evolving technology may reduce the frequency of this complication, but long term studies are needed to understand the effect of therapeutic atrial injury and adverse outcomes. This review summarizes the current literature and outlines an approach to the evaluation and management of these patients.

Chronic Left Lower Lobe Pulmonary Infiltrates During Military Deployment.

Deployment to Southwest Asia is associated with increased airborne hazards such as geologic dusts, burn pit smoke, vehicle exhaust, or air pollution. There are numerous ongoing studies to evaluate the potential effects of inhaled particulate matter on reported increases in acute and chronic respiratory symptoms. Providers need to be aware of potential causes of pulmonary disease such as acute eosinophilic pneumonia, asthma, and vocal cord dysfunction that have been associated with deployment. Other pulmonary disorders such as interstitial lung disease are infrequently reported. Not all deployment-related respiratory complaints may result from deployment airborne hazards and a broad differential should be considered. We present the case of a military member with a prolonged deployment found to have lobar infiltrates secondary to pulmonary vein stenosis from treatment for atrial fibrillation.