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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Project description:We reported the RNAseq analyses of pulmonary vein tissues at the age of postnatal day 21 (P21) on SD rats with or without pulmonary vein stenosis. Pulmonary vein stenosis was induced by performing pulmonary vein banding surgery within 24 hours postnatally. RNAseq analyses of the pulmonary vein tissues was generated at P21 from 3 sham-operated rats and 3 pulmonary vein banding rats. The results revealed that there were 1124 differentially expressed genes between PAB and sham group at P7, among which 714 were upregulated and 410 were downregulated.
2024-07-10 | GSE267175 | GEO
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: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.
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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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Project description: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.
| S-EPMC8471903 | biostudies-literature
Project description:Pulmonary vein stenosis (PVS) is a rare condition that has been linked to prematurity and congenital heart disease (CHD). Despite these associations, treatment options are limited and outcomes are guarded. We investigated differences in PVS outcomes based on the presence of CHD and prematurity, and risk factors for mortality or lung transplantation in PVS. Single-center retrospective cohort study of patients diagnosed with PVS between January 2005 and May 2016 and identified by ICD codes with chart validation. Cox proportional hazard models assessed risk factors for the composite outcome of mortality or lung transplantation. Ninety-three patients with PVS were identified: 65 (70%) had significant CHD, 32 (34%) were premature, and 14 (15%) were premature with CHD. Sixty-five (70%) underwent a PVS intervention and 42 (46%) underwent ≥2 interventions. Twenty-five subjects (27%) died or underwent lung transplant 5.8 months (interquartile range [IQR] 1.1, 15.3) after diagnosis. There was no difference in age at diagnosis or mortality based on presence of CHD or prematurity. PVS diagnosis before age 6 months and greater than 1 pulmonary vein affected at diagnosis were associated with higher mortality (hazards ratio [HR] 3.4 (95% confidence interval 1.5, 7.5), P = 0.003, and HR 2.1 per additional vein affected (95% confidence interval 1.3, 3.4), P = 0.004, respectively). Survival in children with PVS is poor, independent of underlying CHD or prematurity. Younger age and greater number of veins affected at diagnosis are risk factors for worse outcome. Understanding causal mechanisms and development of treatment strategies are necessary to improve outcomes.
| S-EPMC6440879 | biostudies-literature
Project description:Supplemental Digital Content is available in the text. Background: Transient pulmonary congestion during exercise is emerging as an important determinant of reduced exercise capacity in heart failure with preserved ejection fraction (HFpEF). We sought to determine whether an abnormal cardiac energetic state underpins this process. Methods: We recruited patients across the spectrum of diastolic dysfunction and HFpEF (controls, n=11; type 2 diabetes, n=9; HFpEF, n=14; and severe diastolic dysfunction attributable to cardiac amyloidosis, n=9). Cardiac energetics were measured using phosphorus spectroscopy to define the myocardial phosphocreatine to ATP ratio. Cardiac function was assessed by cardiovascular magnetic resonance cine imaging and echocardiography and lung water using magnetic resonance proton density mapping. Studies were performed at rest and during submaximal exercise using a magnetic resonance imaging ergometer. Results: Paralleling the stepwise decline in diastolic function across the groups (E/e′ ratio; P<0.001) was an increase in NT-proBNP (N-terminal pro-brain natriuretic peptide; P<0.001) and a reduction in phosphocreatine/ATP ratio (control, 2.15 [2.09, 2.29]; type 2 diabetes, 1.71 [1.61, 1.91]; HFpEF, 1.66 [1.44, 1.89]; cardiac amyloidosis, 1.30 [1.16, 1.53]; P<0.001). During 20-W exercise, lower left ventricular diastolic filling rates (r=0.58; P<0.001), lower left ventricular diastolic reserve (r=0.55; P<0.001), left atrial dilatation (r=–0.52; P<0.001), lower right ventricular contractile reserve (right ventricular ejection fraction change, r=0.57; P<0.001), and right atrial dilation (r=–0.71; P<0.001) were all linked to lower phosphocreatine/ATP ratio. Along with these changes, pulmonary proton density mapping revealed transient pulmonary congestion in patients with HFpEF (+4.4% [0.5, 6.4]; P=0.002) and cardiac amyloidosis (+6.4% [3.3, 10.0]; P=0.004), which was not seen in healthy controls (–0.1% [–1.9, 2.1]; P=0.89) or type 2 diabetes without HFpEF (+0.8% [–1.7, 1.9]; P=0.82). The development of exercise-induced pulmonary congestion was associated with lower phosphocreatine/ATP ratio (r=–0.43; P=0.004). Conclusions: A gradient of myocardial energetic deficit exists across the spectrum of HFpEF. Even at low workload, this energetic deficit is related to markedly abnormal exercise responses in all 4 cardiac chambers, which is associated with detectable pulmonary congestion. The findings support an energetic basis for transient pulmonary congestion in HFpEF.
| S-EPMC8601674 | biostudies-literature
Project description:Pulmonary vein stenosis (PVS) is a rare, frequently lethal disease with heterogeneous phenotypes and an unclear etiology. Limited studies have reported associations between PVS and congenital heart disease (CHD), chronic lung disease (CLD), and/or prematurity; however, to date, there have been no studies that report detailed clinical syndromic phenotypes and the potential role of genetics in PVS. An existing registry of multivessel PVS patients seen at Boston Children's Hospital (BCH) was queried between August 2006 and January 2017 for all existing genetic testing data on these patients. PVS was defined as an intraluminal pulmonary venous obstruction in ≥2 vessels with mean pressure gradients > 4 mmHg. One-hundred-and-fifty-seven patients (46% female, with a median age at PVS diagnosis of 3 months) formed the cohort. Seventy-one (45%) patients had available genetic testing information. Of the 71 patients, a likely genetic diagnosis was found in 23 (32%) patients: 13 (57%) were diagnosed with Trisomy 21 (T21), five (22%) with Smith-Lemli-Opitz Syndrome, five (22%) had other pathologic genetic disease, and 24 (33%) had variants of unknown significance. The majority of 13 patients with T21 and PVS had common atrioventricular canal (CAVC) (10, 77%) and all had severe pulmonary hypertension (PHTN), which led to their PVS diagnosis. In our study, PVS was associated with T21, the majority of whom also had CAVC and PHTN. Therefore, complete assessment of the pulmonary veins should be considered for all T21 patients, especially those with CAVC presenting with PHTN. Furthermore, prospective standardized genetic testing with detailed clinical phenotyping may prove informative about potential genetic etiologies of PVS.
| S-EPMC7916466 | biostudies-literature