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European Heart Journal. Case Reports Jun 2024There is limited evidence for the use of an intra-aortic balloon pump (IABP) in adult patients with a total cavopulmonary, or Fontan circulation.
BACKGROUND
There is limited evidence for the use of an intra-aortic balloon pump (IABP) in adult patients with a total cavopulmonary, or Fontan circulation.
CASE SUMMARY
A patient in his twenties with a Fontan circulation presented with sepsis, pneumonia, and pulmonary oedema. He was born with a hypoplastic left ventricle, atrioventricular septal defect, and hypoplastic aortic arch, and a total cavopulmonary circulation had been established within his first years of life. Standard of care treatment with antibiotics, non-invasive ventilatory support, loop diuretics, and vasopressors was initiated. Due to persistent pulmonary congestion and increasing general fatigue, an IABP was placed via a femoral artery to offload the failing systemic ventricle. Secondary to IABP treatment, mean arterial pressure rose, and vasodilatory nitroprusside could be introduced. Over 4 days of IABP treatment, the patient's general condition and ventricular systolic function improved significantly.
DISCUSSION
This case suggests that IABP treatment was important in the recovery of our patient with a Fontan circulation, pneumonia, and heart failure. We propose that during IABP treatment, an increase in stroke volume and a reduction in ventricular filling pressure is achieved, thereby increasing the transpulmonary pressure gradient that is central to pulmonary blood flow in Fontan patients. More definitive evidence is necessary to confirm our hypotheses.
PubMed: 38912116
DOI: 10.1093/ehjcr/ytae289 -
Pulmonary Circulation Apr 2024Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease resulting from impaired patency of the pulmonary arteries by a clot, and the treatment method of...
Nonsurgical treatment of a patient with decompensated right ventricular failure due to chronic thromboembolic pulmonary hypertension with proximal clot location-A case report.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease resulting from impaired patency of the pulmonary arteries by a clot, and the treatment method of choice is pulmonary endarterectomy (PEA). In inoperable patients, balloon pulmonary angioplasty (BPA) is recommended, but we need to implement pharmacological bridge therapy to BPA in some cases. We report a case of a 38-year-old male diagnosed with CTEPH, disqualified from PEA due to comorbidity, who developed right ventricular (RV) failure. The case shows a complex pharmacological treatment method that can be successfully used as an effective bridge therapy to BPA in patients with CTEPH and severe RV dysfunction, disqualified from surgery.
PubMed: 38911185
DOI: 10.1002/pul2.12400 -
Pulmonary Circulation Apr 2024Pulmonary hypertension (PH) adds a substantial disease burden, including higher mortality, when associated with interstitial lung disease (ILD), a severe, chronic,...
Pulmonary hypertension (PH) adds a substantial disease burden, including higher mortality, when associated with interstitial lung disease (ILD), a severe, chronic, progressive condition. Yet little is known of the lived experiences, perspectives, priorities, and viewpoints of patients and carers living with PH-ILD. The Voice of the Patient meeting at the center of this qualitative research study aims to provide these difficult-to-obtain insights from a European perspective for the first time. The multistakeholder approach brought together four PH-ILD patients, three primary caregivers, two patient associations, clinical experts, sponsor representatives, and a facilitator. Of the six major themes identified in the thematic analysis, symptoms, and physical limitations were the most impactful. Shortness of breath was the most bothersome symptom affecting patients daily. Further symptoms included fatigue, cough, dizziness, syncope, edema, and palpitations. Physical limitations focused on reduced mobility, impacting patients' ability to perform daily tasks, hobbies, sports, and to enjoy travel. Existing antifibrotic and pulmonary arterial hypertension-targeted treatments were perceived as beneficial. However, despite advances in treatment, severe disease burdens and high unmet medical needs persist from the perspectives of patients. Most meaningful to patients' daily wellbeing was supplemental oxygen, enabling greater mobility. Patients and carers reported difficulties and barriers in navigating the healthcare system and obtaining adequate information to reduce their considerable uncertainties, documenting the substantial challenges that rare and complex conditions such as PH-ILD pose for routine clinical practice beyond PH expert centers and indicating an urgent need for high-quality patient- and clinician-directed information to support patient-centered care.
PubMed: 38911184
DOI: 10.1002/pul2.12405 -
BMC Cardiovascular Disorders Jun 2024Fibrosing mediastinitis (FM) is a rare disease characterized by excessive proliferation of fibrous tissue in the mediastinum and can cause bronchial stenosis, superior...
INTRODUCTION
Fibrosing mediastinitis (FM) is a rare disease characterized by excessive proliferation of fibrous tissue in the mediastinum and can cause bronchial stenosis, superior vena cava obstruction, pulmonary artery and vein stenosis, etc. CASE PRESENTATION: An aging patient with intermittent chest tightness and shortness of breath was diagnosed with FM associated pulmonary hypertension (FM-PH) by echocardiography and enhanced CT of the chest, and CT pulmonary artery (PA)/ pulmonary vein (PV) imaging revealed PA and PV stenosis. Selective angiography revealed complete occlusion of the right upper PV, and we performed endovascular intervention of the total occluded PV. After failure of the antegrade approach, the angiogram revealed well-developed collaterals of the occluded RSPV-V2b, so we chose to proceed via the retrograde approach. We successfully opened the occluded right upper PV and implanted a stent.
CONCLUSIONS
This report may provide new management ideas for the interventional treatment of PV occlusion.
Topics: Humans; Treatment Outcome; Stents; Pulmonary Veins; Chronic Disease; Pulmonary Veno-Occlusive Disease; Stenosis, Pulmonary Vein; Mediastinitis; Male; Phlebography; Angioplasty, Balloon; Aged; Hypertension, Pulmonary; Fibrosis; Collateral Circulation; Pulmonary Circulation; Female
PubMed: 38909188
DOI: 10.1186/s12872-024-03984-y -
Ear, Nose, & Throat Journal Jun 2024Superimposed high-frequency jet ventilation (SHFJV) is a new type of jet ventilation, but its safety and effectiveness in rigid bronchoscopy have not been fully...
Superimposed high-frequency jet ventilation (SHFJV) is a new type of jet ventilation, but its safety and effectiveness in rigid bronchoscopy have not been fully verified, especially in patients with airway stenosis and preoperative cardiovascular disease. This study is intended to retrospectively analyze the effectiveness and safety of SHFJV in the endobronchial treatment under rigid bronchoscopy. A total of 363 patients were included in this study. They were divided into 2 groups: Group A (n = 176)-presence of airway stenosis; Group B (n = 187)-absence of airway stenosis. Mean arterial pressure, heart rate, and pulse oxygen saturation were recorded before anesthesia and during the procedure. Arterial blood gases was recorded before anesthesia, at the end of the procedure and second-day postoperation respectively. The duration of procedure, extubation time, length of stay in the postanesthesia care unit (PACU), length of postoperative hospitalization, incidence of intraoperative and postoperative complications as well as 30 day mortality were also recorded. All the patients had stable circulation during the procedure, including that with preoperative cardiovascular and pulmonary diseases. There were no substantial differences observed in terms of extubation time, PACU stay, and postoperative hospital days. Patients with severe preoperative airway stenosis exhibited longer procedure duration compared to those with mild to moderate stenosis, but there was no difference noted in terms of the extubation and PACU time. SHFJV is effective and safe in the endobronchial treatment for patients with airway stenosis and preoperative cardiovascular disease. It can serve as an ideal airway management strategy for rigid bronchoscopy.
PubMed: 38907650
DOI: 10.1177/01455613241261594 -
BMJ Open Jun 2024To compare the patterns of multimorbidity between people with and without rheumatic and musculoskeletal diseases (RMDs) and to describe how these patterns change by age... (Observational Study)
Observational Study
10-year multimorbidity patterns among people with and without rheumatic and musculoskeletal diseases: an observational cohort study using linked electronic health records from Wales, UK.
OBJECTIVES
To compare the patterns of multimorbidity between people with and without rheumatic and musculoskeletal diseases (RMDs) and to describe how these patterns change by age and sex over time, between 2010 and 2019.
PARTICIPANTS
103 426 people with RMDs and 2.9 million comparators registered in 395 Wales general practices (GPs). Each patient with an RMD aged 0-100 years between January 2010 and December 2019 registered in Clinical Practice Research Welsh practices was matched with up to five comparators without an RMD, based on age, gender and GP code.
PRIMARY OUTCOME MEASURES
The prevalence of 29 Elixhauser-defined comorbidities in people with RMDs and comparators categorised by age, gender and GP practices. Conditional logistic regression models were fitted to calculate differences (OR, 95% CI) in associations with comorbidities between cohorts.
RESULTS
The most prevalent comorbidities were cardiovascular risk factors, hypertension and diabetes. Having an RMD diagnosis was associated with a significantly higher odds for many conditions including deficiency anaemia (OR 1.39, 95% CI (1.32 to 1.46)), hypothyroidism (OR 1.34, 95% CI (1.19 to 1.50)), pulmonary circulation disorders (OR 1.39, 95% CI 1.12 to 1.73) diabetes (OR 1.17, 95% CI (1.11 to 1.23)) and fluid and electrolyte disorders (OR 1.27, 95% CI (1.17 to 1.38)). RMDs have a higher proportion of multimorbidity (two or more conditions in addition to the RMD) compared with non-RMD group (81% and 73%, respectively in 2019) and the mean number of comorbidities was higher in women from the age of 25 and 50 in men than in non-RMDs group.
CONCLUSION
People with RMDs are approximately 1.5 times as likely to have multimorbidity as the general population and provide a high-risk group for targeted intervention studies. The individuals with RMDs experience a greater load of coexisting health conditions, which tend to manifest at earlier ages. This phenomenon is particularly pronounced among women. Additionally, there is an under-reporting of comorbidities in individuals with RMDs.
Topics: Humans; Female; Male; Musculoskeletal Diseases; Multimorbidity; Middle Aged; Wales; Adult; Aged; Rheumatic Diseases; Electronic Health Records; Adolescent; Young Adult; Child; Aged, 80 and over; Child, Preschool; Infant; Prevalence; Infant, Newborn; Cohort Studies; Risk Factors
PubMed: 38904124
DOI: 10.1136/bmjopen-2023-079169 -
Pulmonary Circulation Apr 2024This retrospective study was conducted to evaluate all-cause healthcare resource utilization (HCRU) and costs in commercially insured patients living with pulmonary...
This retrospective study was conducted to evaluate all-cause healthcare resource utilization (HCRU) and costs in commercially insured patients living with pulmonary arterial hypertension (PAH) and explore end-of-life (EOL)-related HCRU and costs. Data from the IQVIA PharMetrics® Plus database (October 2014 to May 2020) were analyzed to identify adults (≥18 years) with PAH (PAH cohort) and those without PH (non-PH cohort). Patients were required to have data for ≥12 months before (baseline) and ≥6 months after (follow-up) the first observed PH diagnosis (index date) for PAH cohort or pseudo index date for non-PH cohort. A PAH EOL cohort was similarly constructed using a broader data window (October 2014 to March 2022) and ≥1 month of follow-up. Annualized all-cause HCRU and costs during follow-up were compared between PAH and non-PH cohorts after 1:1 matching on propensity scores derived from patient characteristics. EOL-related HCRU and costs were explored within 30 days and 6 months before the death date and estimated by a claims-based algorithm in PAH EOL cohort. The annual all-cause total ($183,616 vs. $20,212) and pharmacy ($115,926 vs. $7862; both < 0.001) costs were 8 and 14 times higher, respectively, in the PAH cohort versus matched non-PH cohort ( = 386 for each). In PAH EOL cohort ( = 28), the mean EOL-related costs were $48,846 and $167,524 per patient within 30 days and 6 months before the estimated death, respectively. Hospitalizations contributed 58.8%-70.8% of the EOL-related costs. The study findings indicate substantial HCRU and costs for PAH. While pharmacy costs were one of the major sources, hospitalization was the primary driver for EOL-related costs.
PubMed: 38903484
DOI: 10.1002/pul2.12390 -
Cureus May 2024A coronary artery fistula (CAF) is an abnormal, direct connection between the coronary arteries and cardiac chambers, systemic circulation, or pulmonary circulation...
A coronary artery fistula (CAF) is an abnormal, direct connection between the coronary arteries and cardiac chambers, systemic circulation, or pulmonary circulation without an intervening capillary network. We report a case of a CAF observed in an elderly female. Congenital CAFs are indeed relatively rare, with an incidence of 0.002% to 0.3% in the general population. Over the past few decades, coronary angiography and multi-detector computed tomography have become more widely available, leading to an increased detection of asymptomatic patients with CAFs. By accurately characterizing the CAF's anatomy and understanding the risk factors for complications, clinicians can make more informed decisions about the most appropriate treatment strategy.
PubMed: 38903329
DOI: 10.7759/cureus.60753 -
Annals of Medicine Dec 2024The lung is an important site of extramedullary platelet formation, and megakaryocytes in the lung participate in immune responses in addition to platelet production. In... (Review)
Review
The lung is an important site of extramedullary platelet formation, and megakaryocytes in the lung participate in immune responses in addition to platelet production. In acute lung injury and chronic lung injury, megakaryocytes and platelets play a promoting or protective role through different mechanisms. The authors reviewed the role of megakaryocytes and platelets in common clinical lung injuries with different course of disease and different pathogenic factors in order to provide new thinking for the diagnosis and treatment of lung injuries.
Topics: Megakaryocytes; Humans; Blood Platelets; Acute Lung Injury; Lung Injury; Lung; Animals; Respiratory Distress Syndrome
PubMed: 38902986
DOI: 10.1080/07853890.2024.2362871 -
Journal of Cellular and Molecular... Jun 2024This study explores the impact of senescence on autocrine C-C motif chemokine ligand 5 (CCL5) in human endothelial progenitor cell (EPCs), addressing the poorly...
This study explores the impact of senescence on autocrine C-C motif chemokine ligand 5 (CCL5) in human endothelial progenitor cell (EPCs), addressing the poorly understood decline in number and function of EPCs during ageing. We examined the effects of replication-induced senescence on CCL5/CCL5 receptor (CCR5) signalling and angiogenic activity of EPCs in vitro and in vivo. We also explored microRNAs controlling CCL5 secretion in senescent EPCs, its impact on EPC angiogenic activity, and validated our findings in humans. CCL5 secretion and CCR5 levels in senescent EPCs were reduced, leading to attenuated angiogenic activity. CCL5 enhanced EPC proliferation via the CCR5/AKT/P70S6K axis and increased vascular endothelial growth factor (VEGF) secretion. Up-regulation of miR-409 in senescent EPCs resulted in decreased CCL5 secretion, inhibiting the angiogenic activity, though these negative effects were counteracted by the addition of CCL5 and VEGF. In a mouse hind limb ischemia model, CCL5 improved the angiogenic activity of senescent EPCs. Analysis involving 62 healthy donors revealed a negative association between CCL5 levels, age and Framingham Risk Score. These findings propose CCL5 as a potential biomarker for detection of EPC senescence and cardiovascular risk assessment, suggesting its therapeutic potential for age-related cardiovascular disorders.
Topics: Chemokine CCL5; Endothelial Progenitor Cells; Humans; MicroRNAs; Animals; Cellular Senescence; Neovascularization, Physiologic; Mice; Cell Proliferation; Male; Receptors, CCR5; Vascular Endothelial Growth Factor A; Down-Regulation; Ischemia; Signal Transduction; Angiogenesis
PubMed: 38899522
DOI: 10.1111/jcmm.18489