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The Journal of the Association of... Jun 2024Partial anomalous pulmonary venous connection (PAPVC) is a congenital heart defect in which one or more pulmonary veins drain abnormally into the systemic venous...
Partial anomalous pulmonary venous connection (PAPVC) is a congenital heart defect in which one or more pulmonary veins drain abnormally into the systemic venous circulation, leading to the development of pulmonary arterial hypertension. It can be supracardiac type, draining into the superior vena cava or right atrium (also called cardiac type) and infracardiac type with drainage into the inferior vena cava (IVC). We present two cases-supracardiac and infracardiac types of PAPVC in this case report.
Topics: Humans; Scimitar Syndrome; Pulmonary Veins; Adult; Male; Female
PubMed: 38881145
DOI: 10.59556/japi.72.0529 -
The Journal of Asthma : Official... Jun 2024To investigate the clinical utility of small airway function indices for early identification of GOLD stage 0 chronic obstructive pulmonary disease (COPD).
OBJECTIVE
To investigate the clinical utility of small airway function indices for early identification of GOLD stage 0 chronic obstructive pulmonary disease (COPD).
METHODS
This retrospective study enrolled 137 participants at our institution between January 2017 and December 2018, comprising 40 healthy controls, 46 individuals with GOLD stage 0 COPD, and 51 patients with established COPD. Pulmonary function was assessed using the PowerCube spirometry system (GANSHORN, Germany). Parameters evaluated included forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), FEV1/FVC ratio, and small airway function indicators.
RESULTS
The COPD cohort exhibited significantly lower values across all lung function measures compared to the other two groups, particularly for dynamic lung volume parameters such as FEV1%predicted and FEV1/FVC%. Small airway function indices, including FEV3%predicted, FEF75%predicted, FEF50%predicted, FEF25%predicted, and MMEF%predicted, were markedly decreased in the COPD group (all -values <0.001). Receiver operating characteristic (ROC) curve analysis demonstrated that MMEF/FVC% and FEV3/FVC% had high diagnostic accuracy for COPD, with MMEF/FVC% exhibiting the optimal sensitivity and specificity.
CONCLUSION
Small airway function indices, especially MMEF/FVC%, can serve as effective tools for early identification of GOLD stage 0 COPD. Incorporation of these findings into clinical practice may facilitate early diagnosis and intervention, thereby improving treatment outcomes and patient quality of life.
PubMed: 38880950
DOI: 10.1080/02770903.2024.2368178 -
Scientific Data Jun 2024Pulmonary hypertension (PH) is a syndrome complex that accompanies a number of diseases of different etiologies, associated with basic mechanisms of structural and...
Pulmonary hypertension (PH) is a syndrome complex that accompanies a number of diseases of different etiologies, associated with basic mechanisms of structural and functional changes of the pulmonary circulation vessels and revealed pressure increasing in the pulmonary artery. The structural changes in the pulmonary circulation vessels are the main limiting factor determining the prognosis of patients with PH. Thickening and irreversible deposition of collagen in the pulmonary artery branches walls leads to rapid disease progression and a therapy effectiveness decreasing. In this regard, histological examination of the pulmonary circulation vessels is critical both in preclinical studies and clinical practice. However, measurements of quantitative parameters such as the average vessel outer diameter, the vessel walls area, and the hypertrophy index claimed significant time investment and the requirement for specialist training to analyze micrographs. A dataset of pulmonary circulation vessels for pathology assessment using semantic segmentation techniques based on deep-learning is presented in this work. 609 original microphotographs of vessels, numerical data from experts' measurements, and microphotographs with outlines of these measurements for each of the vessels are presented. Furthermore, here we cite an example of a deep learning pipeline using the U-Net semantic segmentation model to extract vascular regions. The presented database will be useful for the development of new software solutions for the analysis of histological micrograph.
Topics: Deep Learning; Hypertension, Pulmonary; Pulmonary Artery; Humans; Microscopy; Pulmonary Circulation
PubMed: 38879569
DOI: 10.1038/s41597-024-03473-z -
Journal of Orthopaedic Surgery and... Jun 2024Postoperative pulmonary complications (PPCs) are among the most severe complications following total hip arthroplasty revision (THAR), imposing significant burdens on...
BACKGROUND
Postoperative pulmonary complications (PPCs) are among the most severe complications following total hip arthroplasty revision (THAR), imposing significant burdens on individuals and society. This study examined the prevalence and risk factors of PPCs following THAR using the NIS database, identifying specific pulmonary complications (SPCs) and their associated risks, including pneumonia, acute respiratory failure (ARF), and pulmonary embolism (PE).
METHODS
The National Inpatient Sample (NIS) database was used for this cross-sectional study. The analysis included patients undergoing THAR based on NIS from 2010 to 2019. Available data include demographic data, diagnostic and procedure codes, total charges, length of stay (LOS), hospital information, insurance information, and discharges.
RESULTS
From the NIS database, a total of 112,735 THAR patients in total were extracted. After THAR surgery, there was a 2.62% overall incidence of PPCs. Patients with PPCs after THAR demonstrated increased LOS, total charges, usage of Medicare, and in-hospital mortality. The following variables have been determined as potential risk factors for PPCs: advanced age, pulmonary circulation disorders, fluid and electrolyte disorders, weight loss, congestive heart failure, metastatic cancer, other neurological disorders (encephalopathy, cerebral edema, multiple sclerosis etc.), coagulopathy, paralysis, chronic pulmonary disease, renal failure, acute heart failure, deep vein thrombosis, acute myocardial infarction, peripheral vascular disease, stroke, continuous trauma ventilation, cardiac arrest, blood transfusion, dislocation of joint, and hemorrhage.
CONCLUSIONS
Our study revealed a 2.62% incidence of PPCs, with pneumonia, ARF, and PE accounting for 1.24%, 1.31%, and 0.41%, respectively. A multitude of risk factors for PPCs were identified, underscoring the importance of preoperative optimization to mitigate PPCs and enhance postoperative outcomes.
Topics: Humans; Arthroplasty, Replacement, Hip; Risk Factors; Postoperative Complications; Male; Female; Retrospective Studies; Incidence; Aged; Middle Aged; Cross-Sectional Studies; Databases, Factual; Pulmonary Embolism; Reoperation; Length of Stay; Lung Diseases; United States; Pneumonia; Adult; Aged, 80 and over; Respiratory Insufficiency; Inpatients
PubMed: 38877587
DOI: 10.1186/s13018-024-04836-3 -
Journal of Cardiothoracic and Vascular... Mar 2024
PubMed: 38876811
DOI: 10.1053/j.jvca.2024.03.032 -
Circulation Jun 2024Endothelial cell (EC) apoptosis and proliferation of apoptosis-resistant cells is a hallmark of pulmonary hypertension (PH). Yet, why some ECs die and others proliferate...
BACKGROUND
Endothelial cell (EC) apoptosis and proliferation of apoptosis-resistant cells is a hallmark of pulmonary hypertension (PH). Yet, why some ECs die and others proliferate and how this contributes to vascular remodeling is unclear. We hypothesized that this differential response may: (1) relate to different EC subsets, namely pulmonary artery (PAECs) versus microvascular ECs (MVECs); (2) be attributable to autophagic activation in both EC subtypes; and (3) cause replacement of MVECs by PAECs with subsequent distal vessel muscularization.
METHODS
EC subset responses to chronic hypoxia were assessed by single-cell RNA sequencing of murine lungs. Proliferative versus apoptotic responses, activation, and role of autophagy were assessed in human and rat PAECs and MVECs, and in precision-cut lung slices of wild-type mice or mice with endothelial deficiency in the autophagy gene (). Abundance of PAECs versus MVECs in precapillary microvessels was assessed in lung tissue from patients with PH and animal models on the basis of structural or surface markers.
RESULTS
In vitro and in vivo, PAECs proliferated in response to hypoxia, whereas MVECs underwent apoptosis. Single-cell RNA sequencing analyses support these findings in that hypoxia induced an antiapoptotic, proliferative phenotype in arterial ECs, whereas capillary ECs showed a propensity for cell death. These distinct responses were prevented in hypoxic mice or after silencing, yet replicated by autophagy stimulation. In lung tissue from mice, rats, or patients with PH, the abundance of PAECs in precapillary arterioles was increased, and that of MVECs reduced relative to controls, indicating replacement of microvascular by macrovascular ECs. EC replacement was prevented by genetic or pharmacological inhibition of autophagy in vivo. Conditioned medium from hypoxic PAECs yet not MVECs promoted pulmonary artery smooth muscle cell proliferation and migration in a platelet-derived growth factor-dependent manner. Autophagy inhibition attenuated PH development and distal vessel muscularization in preclinical models.
CONCLUSIONS
Autophagic activation by hypoxia induces in parallel PAEC proliferation and MVEC apoptosis. These differential responses cause a progressive replacement of MVECs by PAECs in precapillary pulmonary arterioles, thus providing a macrovascular context that in turn promotes pulmonary artery smooth muscle cell proliferation and migration, ultimately driving distal vessel muscularization and the development of PH.
PubMed: 38873770
DOI: 10.1161/CIRCULATIONAHA.124.068726 -
International Journal of Cardiology.... Sep 2024Limited data exists on upfront combination therapy for portopulmonary hypertension. We evaluated the clinical efficacy, long-term outcomes, and safety of upfront...
BACKGROUND
Limited data exists on upfront combination therapy for portopulmonary hypertension. We evaluated the clinical efficacy, long-term outcomes, and safety of upfront combination therapy in patients with portopulmonary hypertension.
METHODS
We performed a retrospective, single-center cohort study involving a final analysis of 33 consecutive patients diagnosed with portopulmonary hypertension who were taking pulmonary arterial hypertension-specific medication. We compared hemodynamic parameters, risk profiles, composite clinical worsening events, and safety between monotherapy (n = 23) and upfront combination therapy (n = 10).
RESULTS
Twenty-seven patients (82 %) were classified into the Child-Pugh A stage. The change ratios of pulmonary vascular resistance (-32 % vs. -57 %, P = 0.006) were significantly better with upfront combination therapy. Upfront combination therapy also showed significant improvement in risk profiles. Kaplan-Meier analysis showed that the composite event-free rate was significantly lower in patients who received upfront combination therapy than in those who received monotherapy (P = 0.016), although no statistical differences were observed in all-cause death. In the univariate Cox proportional hazards analysis, upfront combination therapy was a factor for decreasing composite clinical worsening outcomes (hazard ratio 0.190, 95 % confidence interval 0.042-0.854; P = 0.030). No significant hepatic impairments were observed over 2 years of follow-up in the upfront combination group.
CONCLUSIONS
In patients with portopulmonary hypertension, upfront combination therapy significantly improved symptoms and short-term hemodynamics, and reduced long-term clinical worsening events without serious adverse effects. This study's findings suggest that patients with portopulmonary hypertension presenting with mild hepatic impairment benefit from upfront combination therapy.
PubMed: 38872733
DOI: 10.1016/j.ijcrp.2024.200294 -
Echocardiography (Mount Kisco, N.Y.) Jun 2024
Topics: Humans; Pulmonary Disease, Chronic Obstructive; Pulse Wave Analysis; Hemodynamics; Pulmonary Artery; Male; Female
PubMed: 38872394
DOI: 10.1111/echo.15863 -
Pulmonary Circulation Apr 2024A blood test identifying patients at increased risk of pulmonary hypertension (PH) could streamline the investigative pathway. The prospective, multicenter CIPHER study...
A blood test identifying patients at increased risk of pulmonary hypertension (PH) could streamline the investigative pathway. The prospective, multicenter CIPHER study aimed to develop a microRNA-based signature for detecting PH in breathless patients and enrolled adults with a high suspicion of PH who had undergone right heart catheterization (RHC). The CIPHER-MRI study was added to assess the performance of this CIPHER signature in a population with low probability of having PH who underwent cardiac magnetic resonance imaging (cMRI) instead of RHC. The microRNA signature was developed using a penalized linear regression (LASSO) model. Data were modeled both with and without N-terminal pro-brain natriuretic peptide (NT-proBNP). Signature performance was assessed against predefined thresholds (lower 98.7% CI bound of ≥0.73 for sensitivity and ≥0.53 for specificity, based on a meta-analysis of echocardiographic data), using RHC as the true diagnosis. Overall, 926 CIPHER participants were screened and 888 were included in the analysis. Of 688 RHC-confirmed PH cases, approximately 40% were already receiving PH treatment. Fifty microRNA (from 311 investigated) were algorithmically selected to be included in the signature. Sensitivity [97.5% CI] of the signature was 0.85 [0.80-0.89] for microRNA-alone and 0.90 [0.86-0.93] for microRNA+NT-proBNP, and the corresponding specificities were 0.33 [0.24-0.44] and 0.28 [0.20-0.39]. Of 80 CIPHER-MRI participants with evaluable data, 7 were considered PH-positive by cMRI whereas 52 were considered PH-positive by the microRNA signature. Due to low specificity, the CIPHER miRNA-based signature for PH (either with or without NT-proBNP in model) did not meet the prespecified diagnostic threshold for the primary analysis.
PubMed: 38868397
DOI: 10.1002/pul2.12386 -
European Heart Journal Supplements :... Apr 2024Patients with advanced heart failure, due to the instability of their clinical conditions, need close surveillance to avoid dangerous exacerbations or sudden events....
Patients with advanced heart failure, due to the instability of their clinical conditions, need close surveillance to avoid dangerous exacerbations or sudden events. Digital technology can be of great help in this contest, thanks to remote monitoring, made possible with the use of wearable or implantable instruments. The latter are currently generally inserted inside defibrillators or resynchronization systems, or inserted inside the pulmonary circulation for monitoring pulmonary pressure. Parameters such as thoracic impedance, physical activity, heart rate variability, atrial and ventricular arrhythmias, blood pressure, and O saturation can be controlled remotely. The data relating to the actual benefit in terms of avoidable events (death and hospitalizations) are not definitive, but certainly from an organizational point of view, the benefit is evident, both on the part of the patient and of the organization of care. The latter, provided in the form of televisits, requires a re-modulation of the system, making use of trained personnel, a well-structured network, and digital technologies (platforms, electronic health records) that are not yet perfectly developed. The evolution of the solutions offered by artificial intelligence guarantees a rapid and progressive refinement of telemedicine in this sector.
PubMed: 38867862
DOI: 10.1093/eurheartjsupp/suae026