-
JACC. Case Reports Apr 2024Pulmonary vein stenosis might be caused by mediastinal migration into the vacated pleural space after pneumonectomy. In a patient complaining of worsening dyspnea in the...
Pulmonary vein stenosis might be caused by mediastinal migration into the vacated pleural space after pneumonectomy. In a patient complaining of worsening dyspnea in the left lateral decubitus position after left pneumonectomy, transthoracic echocardiography during different postures revealed pulmonary vein stenosis that worsened in the left lateral position.
PubMed: 38645288
DOI: 10.1016/j.jaccas.2024.102284 -
BMC Pulmonary Medicine Apr 2024Stenosis and obliteration of the pulmonary vein can be developed by multiple diseases and might cause hemoptysis. Traditional therapy including surgical procedure and...
BACKGROUND
Stenosis and obliteration of the pulmonary vein can be developed by multiple diseases and might cause hemoptysis. Traditional therapy including surgical procedure and conservative treatments might be inappropriate choices to manage massive hemoptysis.
CASE PRESENTATION
A 64-year-old man, diagnosed with advanced stage IVA lung squamous cell carcinoma, presented with dyspnea and recurrent, massive hemoptysis. An initial contrast-enhanced computed tomography revealed a giant tumor in the left lung hilus and occlusion of the left superior pulmonary vein. Despite immediate selective bronchial artery embolization and simultaneous embolization of an anomalous branch of the internal thoracic artery, the massive hemoptysis continued. Subsequently, embolization of the left superior pulmonary artery was performed, achieving functional pulmonary lobectomy, which successfully treated the hemoptysis without relapse during a six-month follow-up. The patient continues to undergo cancer therapy and remains stable.
CONCLUSIONS
This case successfully managed massive hemoptysis associated with lung cancer invasion into the pulmonary vein through functional pulmonary lobectomy via embolization of the corresponding pulmonary artery.
Topics: Humans; Hemoptysis; Male; Middle Aged; Embolization, Therapeutic; Lung Neoplasms; Pulmonary Artery; Carcinoma, Squamous Cell; Tomography, X-Ray Computed; Pulmonary Veins; Pneumonectomy
PubMed: 38644505
DOI: 10.1186/s12890-024-02968-0 -
Journal of the American Heart... May 2024Peripheral pulmonary stenosis (PPS) is a condition characterized by the narrowing of the pulmonary arteries, which impairs blood flow to the lung. The mechanisms...
BACKGROUND
Peripheral pulmonary stenosis (PPS) is a condition characterized by the narrowing of the pulmonary arteries, which impairs blood flow to the lung. The mechanisms underlying PPS pathogenesis remain unclear. Thus, the aim of this study was to investigate the genetic background of patients with severe PPS to elucidate the pathogenesis of this condition.
METHODS AND RESULTS
We performed genetic testing and functional analyses on a pediatric patient with PPS and Williams syndrome (WS), followed by genetic testing on 12 patients with WS and mild-to-severe PPS, 50 patients with WS but not PPS, and 21 patients with severe PPS but not WS. Whole-exome sequencing identified a rare nonsense variant (p.E314X) in a patient with WS and severe PPS. Prostaglandin I synthase (PTGIS) expression was significantly downregulated and cell proliferation and migration rates were significantly increased in cells transfected with the p.E314X variant-encoding construct when compared with that in cells transfected with the wild-type -encoding construct. p.E314X reduced the tube formation ability in human pulmonary artery endothelial cells and caspase 3/7 activity in both human pulmonary artery endothelial cells and human pulmonary artery smooth muscle cells. Compared with healthy controls, patients with PPS exhibited downregulated pulmonary artery endothelial prostaglandin I synthase levels and urinary prostaglandin I metabolite levels. We identified another rare splice-site variant (c.1358+2T>C) in another pediatric patient with WS and severe PPS.
CONCLUSIONS
In total, 2 rare nonsense/splice-site variants were identified in 2 pediatric patients with WS and severe PPS. variants may be involved in PPS pathogenesis, and PTGIS represents an effective therapeutic target.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Male; Cell Movement; Cell Proliferation; Cells, Cultured; Codon, Nonsense; Cytochrome P-450 Enzyme System; Endothelial Cells; Exome Sequencing; Genetic Predisposition to Disease; Intramolecular Oxidoreductases; Phenotype; Pulmonary Artery; Pulmonary Valve Stenosis; Severity of Illness Index; Williams Syndrome
PubMed: 38639351
DOI: 10.1161/JAHA.123.032872 -
The American Journal of Case Reports Apr 2024BACKGROUND Congenital heart diseases (CHDs) are the most common form of birth defects, affecting the structure and function of neonatal hearts. Pulmonary valve stenosis...
BACKGROUND Congenital heart diseases (CHDs) are the most common form of birth defects, affecting the structure and function of neonatal hearts. Pulmonary valve stenosis (PVS) and ventricular septal defects (VSD) are 2 of the more prevalent forms, both of which can lead to significant morbidity if left untreated. The emergence of transcatheter techniques has revolutionized the therapeutic landscape, presenting minimally invasive yet effective alternatives to open-heart surgery and significantly reducing associated patient morbidity and recovery time. CASE REPORT The presented case details the management of a 19-year-old man with complex CHDs, highlighting the nuanced decision-making process that led to a transcatheter approach. The patient's clinical presentation, marked by symptoms reflective of significant cardiac compromise, demanded a tailored approach that utilized the latest advancements in non-surgical intervention. The successful closure of the VSD with an Amplatzer device and the resolution of PVS via balloon valvuloplasty were achieved without complications, showcasing the potential of these techniques in managing similar cases. The post-intervention period was marked by a noteworthy recovery, confirming the procedural efficacy and enhancing the patient's quality of life. CONCLUSIONS The favorable outcome of this case highlights the pivotal role of transcatheter interventions in treating complex CHDs and suggests a shift towards less invasive approaches in cardiac care. This case contributes valuable insights to the existing body of evidence, reinforcing the potential of transcatheter techniques to become the preferred treatment modality. With promising immediate and short-term results, these techniques highlight the need for continued research into their long-term efficacy and application across diverse patient demographics.
Topics: Humans; Male; Young Adult; Cardiac Catheterization; Cardiac Surgical Procedures; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Pulmonary Valve Stenosis; Quality of Life; Treatment Outcome
PubMed: 38637976
DOI: 10.12659/AJCR.942032 -
Heliyon Apr 2024The ultrasonic cardiac output monitor (USCOM), an instrument that monitors the evolution of a patient's hemodynamic status and determines the type of shock, has become...
OBJECTIVE
The ultrasonic cardiac output monitor (USCOM), an instrument that monitors the evolution of a patient's hemodynamic status and determines the type of shock, has become an important tool for assessing cardiac pathology and predicting changes in disease, but there are some variations in the instrumental findings for different physical conditions of patients. This article examines whether there are differences in the quality of USCOM waveforms measured in different types of critically ill patients based on clinical characteristics and test parameters.
METHODS
Baseline data, diagnoses, echocardiograms, ventilation patterns, and USCOM results were retrospectively collected from patients in the emergency intensive care unit. Waveform quality was quantified using the Fremantle score to determine the extent to which age, body mass index (BMI), chronic obstructive pulmonary disease (COPD), respiratory failure, cardiac enlargement, valvular heart disease, and ventilation pattern influenced USCOM waveform quality.
RESULTS
Age, body mass index, chronic obstructive pulmonary disease, respiratory failure, right and left heart enlargement, aortic valve disease (excluding aortic stenosis), and ventilation mode did not have a significant effect on USCOM waveform quality in critically ill patients ( > 0.05).
CONCLUSIONS
Various physical conditions of critically ill patients may have limited effect on the quality of the USCOM waveform, potentially rendering USCOM suitable for early assessment of hemodynamic status during ICU admission.
PubMed: 38623198
DOI: 10.1016/j.heliyon.2024.e29242 -
Journal of Clinical Medicine Mar 2024Echocardiography has long been established as the primary noninvasive method for diagnosing pulmonary hypertension (PH) prior to transcatheter aortic valve replacement...
Echocardiography has long been established as the primary noninvasive method for diagnosing pulmonary hypertension (PH) prior to transcatheter aortic valve replacement (TAVR) in patients with severe aortic valve stenosis (AS). In recent years, radiological methods for diagnosing PH have been investigated. Measurements such as the computed tomography angiography (CTA)-derived pulmonary artery (PA) diameter and PA diameter/body surface area (PA/BSA) have shown promising results regarding their diagnostic strength. However, it has yet to be determined if a patient's sex has any impact on the effectiveness of these diagnostic measurements. In all, 271 patients (51.3% male, mean age 82.6 ± 4.8 years) with severe AS undergoing TAVR were separated into male and female groups. The cut-off values for the diagnosis of PH were calculated for the CTA-derived PA diameter and PA/BSA based on different systolic pulmonal artery pressure values (40-45-50 mmHg). Patients were then subclassified according to measurements above or below these PA diameters and PA/BSA cut-off values. A PA diameter ≥29.5 mm and PA/BSA ≥ 15.7 mm/m qualified for PH. The 1-5 year survival rate in these cohorts was further analyzed. Patients with a PA diameter ≥29.5 mm showed a significantly higher 1 year mortality rate ( = 0.014). This observation could only be confirmed for the male sex ( = 0.018) and not for the female sex ( = 0.492). As for the PA/BSA, in patients over the cut-off value, no significant increase in mortality was noted in the overall cohort. However, the male patients showed increased 3 year ( = 0.048) and 5 year mortality rates ( = 0.033). The CTA-obtained PA diameter and PA/BSA are both useful in the diagnosis of PH and mortality risk stratification in patients with severe AS undergoing TAVR, especially in males. Male patients with PA ≥ 29.5 mm or PA/BSA ≥ 15.7 mm/m seem to be at a higher risk of death during follow-up after undergoing TAVR. In females, no such correlation was observed.
PubMed: 38610764
DOI: 10.3390/jcm13071999 -
Journal of Science and Medicine in Sport Jul 2024Pre-participation screening and management of congenital cardiac valvulopathy in competitive athletes can be challenging, particularly within the context of...
Pre-participation screening and management of congenital cardiac valvulopathy in competitive athletes can be challenging, particularly within the context of ultra-endurance disciplines. A 55-year-old female athlete without a reported history of cardiac disease exhibited clinical signs of cardiogenic pulmonary edema during a 156 km ultra-trail race. The echocardiographic assessment revealed the presence of a parachute mitral valve, with no evidence of mitral stenosis or regurgitation at rest, but it demonstrated severe dynamic mitral stenosis during exercise. In competitive athletes, the detection of rare valvulopathy should prompt a comprehensive cardiac evaluation aimed at assessing the potential for dynamic valvular dysfunction.
Topics: Humans; Female; Middle Aged; Mitral Valve Stenosis; Mitral Valve; Echocardiography; Pulmonary Edema; Athletes
PubMed: 38609817
DOI: 10.1016/j.jsams.2024.03.002 -
Proceedings of the National Academy of... Apr 2024Pulmonary arterial hypertension (PAH) is characterized by stenosis and occlusions of small pulmonary arteries, leading to elevated pulmonary arterial pressure and right...
Pulmonary arterial hypertension (PAH) is characterized by stenosis and occlusions of small pulmonary arteries, leading to elevated pulmonary arterial pressure and right heart failure. Although accumulating evidence shows the importance of interleukin (IL)-6 in the pathogenesis of PAH, the target cells of IL-6 are poorly understood. Using mice harboring the allele of , a subunit of the IL-6 receptor, we found substantial Cre recombination in all hematopoietic cell lineages from the primitive hematopoietic stem cell level in mice. We also revealed that a CD4 cell-specific gp130 deletion ameliorated the phenotype of hypoxia-induced pulmonary hypertension in mice. Disruption of IL-6 signaling via deletion of in CD4 T cells inhibited phosphorylation of signal transducer and activator of transcription 3 (STAT3) and suppressed the hypoxia-induced increase in T helper 17 cells. To further examine the role of IL-6/gp130 signaling in more severe PH models, we developed knockout (KO) rats using the CRISPR/Cas9 system and showed that IL-6 deficiency could improve the pathophysiology in hypoxia-, monocrotaline-, and Sugen5416/hypoxia (SuHx)-induced rat PH models. Phosphorylation of STAT3 in CD4 cells was also observed around the vascular lesions in the lungs of the SuHx rat model, but not in KO rats. Blockade of IL-6 signaling had an additive effect on conventional PAH therapeutics, such as endothelin receptor antagonist (macitentan) and soluble guanylyl cyclase stimulator (BAY41-2272). These findings suggest that IL-6/gp130 signaling in CD4 cells plays a critical role in the pathogenesis of PAH.
Topics: Animals; Mice; Rats; CD4-Positive T-Lymphocytes; Cytokine Receptor gp130; Hypertension, Pulmonary; Hypoxia; Interleukin-6; Pulmonary Artery
PubMed: 38602915
DOI: 10.1073/pnas.2315123121 -
Vascular Health and Risk Management 2024Despite the fact that patients with rheumatic heart disease (RHD) need early medical attention and follow-up, most patients in developing countries tend to present with...
BACKGROUND
Despite the fact that patients with rheumatic heart disease (RHD) need early medical attention and follow-up, most patients in developing countries tend to present with debilitating complications. The objective of this study was to evaluate the echocardiographic features of adult individuals diagnosed with RHD and examine the associated complications among patients who started follow-up at Jimma Medical Center's (JMC) cardiac follow-up clinic.
METHODS
A prospective cross-sectional study was conducted at JMC between January 5 and April 15, 2023. Echocardiographic patterns were taken by senior cardiologists; socio-demographic variables, anthropometric measurements, and behavioral factors were collected through a structured questioner.
RESULTS
The study recruited a total of 115 participants, of whom 86 (74.8%) were female and 29 (25.2%) were male. The mean age of the patients was 32.31 (SD± 12.16) years. The mitral valve was affected in 98.26% of cases, while the aortic and tricuspid valve abnormalities were diagnosed in 49.5% and 21.7%, respectively. The most frequent combinations of valve lesions were mitral regurgitation (MR) + mitral stenosis (MS) + aortic regurgitation (AR) (15.7%), followed by MR + AR + TR (8.7%). The occurrence of MR+MS+AR was higher in females (17.4%) compared to males (10.3%), whereas the occurrence of MS+MR was higher in males (24.1%) compared to females (20.9%). Females have a severely reduced ejection fraction compared to males (84.8% vs 15.2%, P = 0.044). Nearly two-thirds (63.5%) of individuals experienced RHD-related complications; the most commonly encountered complications were pulmonary hypertension (26.1%) and atrial fibrillation (19.1%).
CONCLUSION
RHD predominantly affects individuals in their active and productive years, particularly females. Most patients have multiple-valve lesions.
Topics: Adult; Humans; Male; Female; Rheumatic Heart Disease; Cross-Sectional Studies; Prospective Studies; Echocardiography; Mitral Valve; Constriction, Pathologic
PubMed: 38595828
DOI: 10.2147/VHRM.S451957