Unilateral Lung Congestion During Exercise in a Patient With a Single Pulmonary Vein Stenosis.
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ABSTRACT:
SUBMITTER: Kato T
PROVIDER: S-EPMC8311262 | biostudies-literature |
REPOSITORIES: biostudies-literature
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2024-07-10 | GSE267175 | GEO
Project description:We report a case of surgically repaired symptomatic mitral valve regurgitation (MR) in a 61-year-old woman with anomalous unilateral single pulmonary vein. A two-staged surgery was scheduled; first a catheter embolization of anomalous vessel to avoid recirculation of the blood into the left atrium during cardiopulmonary bypass, and second a mitral valve repair via right lateral thoracotomy. Learning objective Scimitar sign is a horn-like shape on plain chest radiograph. One of the possible diagnoses is partial anomalous pulmonary venous return (APVR), which often requires surgical interventions due to comorbidities of congenital heart disease and recurrent pneumonia [1–3]. Another is anomalous unilateral single pulmonary vein (AUSPV), which is generally asymptomatic, and therefore, requires no medical interventions. This case addresses the advantage of multidetector computed tomography (CT) and the safety of two-staged strategy.
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Project description:We present a rare case of hemoptysis secondary to isolated unilateral pulmonary vein atresia. Isolated pulmonary vein atresia is a rare condition in which patients typically acquire a diagnosis in infancy and early childhood [Mataciunas et al.; Pourmoghadam et al.]. Our patient presented during puberty with several previous episodes of hemoptysis prior to her admission and diagnosis. The initial diagnosis was suspected in our patient from chest computerized tomography (CT), and confirmed with cardiac catheterization and pulmonary angiography. Treatment aim is to preserve lung function and minimize irreversible pulmonary remodeling [Pourmoghadam et al.; Harrison et al.]. Conservative monitoring can be considered with milder or asymptomatic cases, while others may require preoperative collateral artery banding, surgical anastomosis between the pulmonary vein (PV) & left atrium (LA) and even pneumonectomy [Pourmoghadam et al.].
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Project description:The diagnosis of primary pulmonary vein stenosis is often overlooked because its symptoms overlap lung diseases and pulmonary arterial hypertension. Its diagnosis may be difficult because the condition is progressive and associated with other defects. We present a case of pulmonary vein stenosis in a newborn with stenosis of the left-sided common pulmonary vein, diffuse hypoplasia of the superior right pulmonary vein, and atresia of the inferior right pulmonary vein.
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Project description:Pulmonary vein stenosis results from a proliferative process that leads to the progressive obstruction of venous return to the left atrium. It is often resistant to catheterization and surgical based interventions and is frequently fatal when encountered in its severe form. Here, we describe three patients with severe, primary pulmonary vein stenosis that was progressing despite aggressive conventional management strategies. All three patients were initiated on combination chemotherapy with imatinib and sirolimus, drugs which have been previously shown to independently have potential benefit against PVS. Soon after the initiation of these therapies, all three patients experienced a stabilization of their disease process and clinical improvement. All three patients remain alive, with tolerable side effects from the medications. Although early in our experience and with only a small number of patients, combination chemotherapy with imatinib and sirolimus shows promise and merits further investigation as a therapeutic option for this aggressive disease.
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Project description:Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. We present a patient with PVS and a PIK3CA mutation, who demonstrated a good response to the targeted inhibitor, alpelisib.
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Project description:BackgroundDry weight assessment in hemodialysis (HD) remains a challenge. The aim of the study was to investigate the prevalence of subclinical pulmonary congestion using lung ultrasound (LUS) in maintenance HD patients with no clinical or bioimpedance signs of hyperhydration. The correlation between B-lines Score (BLS) and brain natriuretic peptide (BNP) was also evaluated.MethodsTwenty-four HD patients underwent LUS and BNP dosage at the end of the mid-week HD session, monthly for 6 months . LUS was considered as positive when BLS was >15. Hospitalizations and cardiovascular events were also evaluated in relation to the BLS.ResultsLUS+ patients at baseline were 16 (67%), whereas 11 (46%) showed LUS + in at least 50% of the measurements (rLUS+ patients). Only the rLUS+ patients had a higher number of cardiovascular events [p=0.019, OR: 7.4 (CI 95%. 1.32-39.8)] and hospitalizations [p=0.034, OR 5.5 (CI 95% 1.22- 24.89)]. A BNP level of 165 pg/ml was identified as cut-off value for predicting pulmonary congestion, defined by BLS >15.ConclusionPrevalence of pulmonary congestion as assessed by LUS and persistent or recurrent BLS >15 were quite prevalent findings in euvolemic HD patients. In the patients defined as rLUS+, a higher rate of cardiovascular events and hospital admissions was registered. BNP serum levels > 165 pg/ml resulted predictive of pulmonary congestion at LUS. In the dialysis care, regular LUS examination should be reasonably included among the methods useful to detect subclinical lung congestion and to adjust patients' dry weight.
| S-EPMC7818920 | biostudies-literature
Project description: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.
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Project description:BACKGROUND:Massive hemoptysis is a life-threatening condition. Massive hemoptysis caused by pulmonary vein stenosis (PVS) after radiofrequency catheter ablation for atrial fibrillation (AF) is rare. However, bilateral lung hemorrhage following bilateral PVS is extremely rare. CASE PRESENTATION:We herein describe a 62-year-old man with refractory massive hemoptysis after radiofrequency catheter ablation for AF, which was successfully controlled by surgical lobectomy and endovascular bilateral PV stenting. The hemorrhage was derived from the bilateral lungs following PV obstruction and bilateral PVS, which was definitively diagnosed by bronchoscopic examination. The patient had no recurrence of hemoptysis during a follow-up period of 30 months, and the PV stents had not narrowed as shown by computed tomography 30 months after stent placement. CONCLUSIONS:Massive hemoptysis can be caused by bilateral PVS after radiofrequency catheter ablation for AF, and hemorrhage from the bilateral lungs in such patients is extremely rare. Nevertheless, cardiologists, interventional radiologists, and pulmonologists should consider the potential for massive hemoptysis caused by PVS.
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