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Science Advances May 2024Biomechanical forces, and their molecular transducers, including key mechanosensitive transcription factor genes, such as , are required for cardiac valve morphogenesis....
Biomechanical forces, and their molecular transducers, including key mechanosensitive transcription factor genes, such as , are required for cardiac valve morphogenesis. However, mutants fail to completely recapitulate the valveless phenotype observed under no-flow conditions. Here, we identify the transcription factor EGR3 as a conserved biomechanical force transducer critical for cardiac valve formation. We first show that null zebrafish display a complete and highly penetrant loss of valve leaflets, leading to severe blood regurgitation. Using tissue-specific loss- and gain-of-function tools, we find that during cardiac valve formation, Egr3 functions cell-autonomously in endothelial cells, and identify one of its effectors, the nuclear receptor Nr4a2b. We further find that mechanical forces up-regulate / expression in the developing zebrafish heart and in porcine valvular endothelial cells, as well as during human aortic valve remodeling. Altogether, these findings reveal that EGR3 is necessary to transduce the biomechanical cues required for zebrafish cardiac valve morphogenesis, and potentially for pathological aortic valve remodeling in humans.
Topics: Animals; Zebrafish; Heart Valves; Zebrafish Proteins; Morphogenesis; Humans; Early Growth Response Protein 3; Gene Expression Regulation, Developmental; Endothelial Cells; Mechanotransduction, Cellular; Swine
PubMed: 38748804
DOI: 10.1126/sciadv.adl0633 -
Texas Heart Institute Journal May 2024Turner syndrome is a genetic disorder that occurs in female individuals and is characterized by the absence of 1 of the X chromosomes. This study examined the risk of...
BACKGROUND
Turner syndrome is a genetic disorder that occurs in female individuals and is characterized by the absence of 1 of the X chromosomes. This study examined the risk of cardiovascular disease and inpatient clinical outcomes in patients with Turner syndrome.
METHODS
Data were extracted from the Nationwide Inpatient Sample 2016 database. Propensity score analysis was used to match women with Turner syndrome and women without Turner syndrome admitted to a hospital in the same year to evaluate the risk of cardiovascular disease and inpatient clinical outcomes in patients with Turner syndrome.
RESULTS
After 1:1 matching, 710 women with Turner syndrome and 710 women without Turner syndrome were included in the final analysis. Compared with women without Turner syndrome, women with Turner syndrome were more likely to have a bicuspid aortic valve (9.4% vs 0.01%; P < .01), coarctation of the aorta (5.8% vs 0.3%; P < .01), atrial septal defect (6.1% vs 0.8%; P < .01), and patent ductus arteriosus (4.6% vs 0.6%; P < .01). Patients with Turner syndrome were more likely to have an aortic aneurysm (odds ratio [OR], 2.46 [95% CI, 1.02-5.98]; P = .046), ischemic heart disease (OR, 1.66 [95% CI, 1.10-2.5]; P = .02), heart failure (OR, 3.15 [95% CI, 1.99-4.99]; P < .01), and atrial fibrillation or flutter (OR, 2.48 [95% CI, 1.42-4.34]; P < .01). Patients with Turner syndrome were more likely to have pulmonary arterial hypertension (OR, 2.12 [95% CI, 1.08-4.14]; P = .03) and acute kidney injury (OR, 1.60 [95% CI, 1.06-2.42]; P = .03) and to require mechanical ventilation (OR, 1.66 [95% CI, 1.04-2.68]; P = .04).
CONCLUSION
Turner syndrome is associated with an increased rate of cardiovascular disease and inpatient complications. These findings suggest that patients with Turner syndrome should be screened and monitored closely for cardiovascular disease and inpatient complications.
Topics: Humans; Turner Syndrome; Female; Propensity Score; Cardiovascular Diseases; Adult; Retrospective Studies; United States; Middle Aged; Risk Factors; Inpatients; Risk Assessment; Incidence; Follow-Up Studies; Young Adult
PubMed: 38748548
DOI: 10.14503/THIJ-23-8245 -
Journal of Cardiothoracic Surgery May 2024Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and... (Review)
Review
Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.
Topics: Humans; Male; Middle Aged; Pulmonary Artery; Sarcoma; Pulmonary Valve; Vascular Neoplasms; Diagnosis, Differential; Vasculitis; Diagnostic Errors
PubMed: 38745263
DOI: 10.1186/s13019-024-02700-3 -
International Journal of Chronic... 2024There is an assumption that because EBLVR requires less use of hospital resources, offsetting the higher cost of endobronchial valves, it should therefore be the... (Comparative Study)
Comparative Study
Bronchoscopic Lung Volume Reduction as the Treatment of Choice versus Robotic-Assisted Lung Volume Reduction Surgery in Similar Patients with Emphysema - An Initial Experience of the Benefits and Complications.
OBJECTIVE
There is an assumption that because EBLVR requires less use of hospital resources, offsetting the higher cost of endobronchial valves, it should therefore be the treatment of choice wherever possible. We have tested this hypothesis in a retrospective analysis of the two in similar groups of patients.
METHODS
In a 4-year experience, we performed 177 consecutive LVR procedures: 83 patients underwent Robot Assisted Thoracoscopic (RATS) LVRS and 94 EBLVR. EBLVR was intentionally precluded by evidence of incomplete fissure integrity or intra-operative assessment of collateral ventilation. Unilateral RATS LVRS was performed in these cases together with those with unsuitable targets for EBLVR.
RESULTS
EBLVR was uncomplicated in 37 (39%) cases; complicated by post-procedure spontaneous pneumothorax (SP) in 28(30%) and required revision in 29 (31%). In the LVRS group, 7 (8%) patients were readmitted with treatment-related complications, but no revisional procedure was needed. When compared with uncomplicated EBLVR, LVRS had a significantly longer operating time: 85 (14-82) vs 40 (15-151) minutes (p<0.001) and hospital stay: 7.5 (2-80) vs 2 (1-14) days (p<0.01). However, LVRS had a similar total operating time to both EBLVR requiring revision: 78 (38-292) minutes and hospital stay to EBLVR complicated by pneumothorax of 11.5 (6.5-24.25) days. Use of critical care was significantly longer in RATS group, and it was also significantly longer in EBV with SP group than in uncomplicated EBV group.
CONCLUSION
Endobronchial LVR does use less hospital resources than RATS LVRS in comparable groups if the recovery is uncomplicated. However, this advantage is lost if one includes the resources needed for the treatment of complications and revisional procedures. Any decision to favour EBLVR over LVRS should not be based on the assumption of a smoother, faster perioperative course.
Topics: Humans; Retrospective Studies; Pneumonectomy; Male; Middle Aged; Bronchoscopy; Pulmonary Emphysema; Aged; Female; Treatment Outcome; Robotic Surgical Procedures; Time Factors; Lung; Length of Stay; Postoperative Complications; Operative Time; Risk Factors; Pneumothorax; Clinical Decision-Making; Patient Readmission
PubMed: 38741941
DOI: 10.2147/COPD.S442380 -
Journal of Thoracic Disease Apr 2024Mitral valve (MV) regurgitation (MR) is the second most frequent indication for valvular surgery in Europe. Right ventricular (RV) dysfunction is a common finding after...
BACKGROUND
Mitral valve (MV) regurgitation (MR) is the second most frequent indication for valvular surgery in Europe. Right ventricular (RV) dysfunction is a common finding after cardiac surgery and might persist for years. The RV-function after MV surgery has been controversially discussed. We therefore aimed to evaluate early RV-performance in patients undergoing MV surgery.
METHODS
Between 09/2020 and 06/2022, ninety-two patients presenting with MR undergoing MV surgery were consented and prospectively included for evaluation. Echocardiographic evaluation was performed one day before surgery, one week after surgery and three months later. Primary endpoints reported RV-function changes including tricuspid annular plane systolic excursion (TAPSE), RV systolic prime (S') and fractional area change (FAC). Secondary endpoints included stability of MV repair, changes in left ventricular functions and early mortality.
RESULTS
Mean patients' age was 59.1±11.4 years. Fifty-five (59.7%) patients were male. Most of patients presented with severe (n=88; 95.7%) MR. Mean systolic pulmonary artery pressure was 35.6±15.7 mmHg. Moderate or severe pulmonary arterial hypertension (PAH) was present in 60 (65.2%) patients. Patients underwent either isolated MV surgery (n=67; 72.8%) or combined with tricuspid valve surgery (n=25; 27.2%). Minimal invasive surgery was performed in 26.1% (n=24) of the patients. Postoperative short-term follow-up at 3 months reported RV-dysfunction in 44.5% (n=41) of the patients as indicated by reductions in TAPSE & RV S' from 21.2±4.7 to 14±3.3 mm (P<0.001) and from 14.7±4.3 to 9.7±2.8 cm/s (P<0.001) respectively. The FAC reduction from 42.9%±9.6% to 42.2%±9.9% was non-significant (P=0.593) and no need for redo mitral or tricuspid valve surgery was reported. Finally, the presence and severity of preoperative PAH played significant roles for the incidence of RV dysfunction, P=0.021 and P=0.047, respectively. Minimal invasive surgical procedure significantly reduced the incidence of postoperative RV-dysfunction (P=0.013).
CONCLUSIONS
Study early results report a significant reduction of RV-function after MV surgery as measured by TAPSE, & RV S', even when the FAC remains unchanged. Even though, this finding has limited prognostic implications during an uneventful surgical course.
PubMed: 38738229
DOI: 10.21037/jtd-23-1727 -
Cureus Apr 2024Infective endocarditis, a fatal infection with rising morbidity and mortality rates among infants and children, is characterized by microbial infection within the...
Infective endocarditis, a fatal infection with rising morbidity and mortality rates among infants and children, is characterized by microbial infection within the endocardium, the inner lining of the heart including heart valves. The heightened susceptibility to infection in children is attributed to pre-existing pathologies, structural defects, and comorbidities. This report details a case of a one-year-old child with tetralogy of Fallot, showcasing isolated pulmonary valve vegetations as a distinctive manifestation of infective endocarditis.
PubMed: 38738063
DOI: 10.7759/cureus.58013 -
Therapeutic Advances in Pulmonary and... 2024Bullous lung disease caused by tuberculosis is rare, and complications have a poor prognosis with uncertain pathophysiologic mechanisms. We describe a 29-year-old male...
Bullous lung disease caused by tuberculosis is rare, and complications have a poor prognosis with uncertain pathophysiologic mechanisms. We describe a 29-year-old male patient who was admitted to the emergency department due to bilateral tension pneumothorax, which was complicated by bronchopleural fistula. This was managed with the placement of chest tubes, continuity of anti-TB drug treatment, and Heimlich valve placement.
PubMed: 38736690
DOI: 10.1177/29768675241249652 -
BMC Veterinary Research May 2024Hydrops fetalis (HF) is fluid accumulation in fetus body cavities and subcutaneous tissue. The condition has been described in various farm and companion animal species,...
BACKGROUND
Hydrops fetalis (HF) is fluid accumulation in fetus body cavities and subcutaneous tissue. The condition has been described in various farm and companion animal species, including dogs. Most of cases result from a heart defect. Exact nature of this defect is rarely clarified.
CASE PRESENTATION
A newborn, male French bulldog puppy with severe HF underwent a full anatomopathological examination to diagnose the primary cause of HF. Based on the anatomopathological examination, fetal ultrasound, and micro-computed tomography, transposition of the great arteries with hypoplasia of the ascending aorta, aortic arch interruption, ostium secundum atrial septal defect, severe tricuspid valve dysplasia, as well as hypoplasia of pulmonary vessels and lungs were diagnosed.
CONCLUSIONS
This is the first report of HF caused by severe, complex congenital heart defects with concurrent pulmonary vessel and lung hypoplasia.
Topics: Animals; Hydrops Fetalis; Male; Lung; Dog Diseases; Dogs; Heart Defects, Congenital; X-Ray Microtomography; Animals, Newborn
PubMed: 38734649
DOI: 10.1186/s12917-024-04060-5 -
Sensors (Basel, Switzerland) May 2024Heart failure (HF) is a complex clinical syndrome associated with significant morbidity, mortality, and healthcare costs. It is characterized by various structural... (Review)
Review
Heart failure (HF) is a complex clinical syndrome associated with significant morbidity, mortality, and healthcare costs. It is characterized by various structural and/or functional abnormalities of the heart, resulting in elevated intracardiac pressure and/or inadequate cardiac output at rest and/or during exercise. These dysfunctions can originate from a variety of conditions, including coronary artery disease, hypertension, cardiomyopathies, heart valve disorders, arrhythmias, and other lifestyle or systemic factors. Identifying the underlying cause is crucial for detecting reversible or treatable forms of HF. Recent epidemiological studies indicate that there has not been an increase in the incidence of the disease. Instead, patients seem to experience a chronic trajectory marked by frequent hospitalizations and stagnant mortality rates. Managing these patients requires a multidisciplinary approach that focuses on preventing disease progression, controlling symptoms, and preventing acute decompensations. In the outpatient setting, patient self-care plays a vital role in achieving these goals. This involves implementing necessary lifestyle changes and promptly recognizing symptoms/signs such as dyspnea, lower limb edema, or unexpected weight gain over a few days, to alert the healthcare team for evaluation of medication adjustments. Traditional methods of HF monitoring, such as symptom assessment and periodic clinic visits, may not capture subtle changes in hemodynamics. Sensor-based technologies offer a promising solution for remote monitoring of HF patients, enabling early detection of fluid overload and optimization of medical therapy. In this review, we provide an overview of the CardioMEMS device, a novel sensor-based system for pulmonary artery pressure monitoring in HF patients. We discuss the technical aspects, clinical evidence, and future directions of CardioMEMS in HF management.
Topics: Humans; Heart Failure; Cardiology; Monitoring, Physiologic; Disease Management; Hemodynamics
PubMed: 38733027
DOI: 10.3390/s24092922 -
Journal of Clinical Medicine May 2024: Conflicting data exist on the occurrence and outcome of infective endocarditis (IE) after pulmonary valve implantation. Objectives: This study sought to assess the...
: Conflicting data exist on the occurrence and outcome of infective endocarditis (IE) after pulmonary valve implantation. Objectives: This study sought to assess the differences between transcatheter pulmonary valve implantation (TPVI) and surgical pulmonary valve replacement (SPVR). : All patients ≥ 4 years who underwent isolated pulmonary valve replacement between 2005 and 2018 were analyzed based on the data of a major German health insurer (≈9.2 million insured subjects representative of the German population). The primary endpoint was a composite of IE occurrence and all-cause death. : Of 461 interventions (cases) in 413 patients (58.4% male, median age 18.9 years [IQR 12.3-33.4]), 34.4% underwent TPVI and 65.5% SPVR. IE was diagnosed in 8.0% of cases during a median follow-up of 3.5 years. Risk for IE and all-cause death was increased in patients with prior IE ( < 0.001), but not associated with age ( = 0.50), sex ( = 0.67) or complexity of disease ( = 0.59). While there was no difference in events over the entire observational time period ( = 0.22), the time dynamics varied between TPVI and SPVR: Within the first year, the risk for IE and all-cause death was lower after TPVI (Hazard Ratio (HR) 95% CI 0.19 (0.06-0.63; = 0.006) but increased over time and exceeded that of SPVR in the long term (HR 10.07 (95% CI 3.41-29.76; < 0.001). : Patients with TPVI appear to be at lower risk for early but higher risk for late IE, resulting in no significant difference in the overall event rate compared to SPVR. The results highlight the importance of long-term specialized care and preventive measures after both interventions.
PubMed: 38731212
DOI: 10.3390/jcm13092683