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Pulmonary Circulation Apr 2024Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical...
Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross-sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital-based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow-up practices. Prescription of pulmonary arterial hypertension-specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center-specific experience and region-specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education.
PubMed: 38947169
DOI: 10.1002/pul2.12406 -
Circulation. Cardiovascular Quality and... Jul 2024Cardiovascular disease exacts a heavy toll on health and quality of life and is the leading cause of death among people ≥65 years of age. Although medical, surgical,... (Review)
Review
Cardiovascular disease exacts a heavy toll on health and quality of life and is the leading cause of death among people ≥65 years of age. Although medical, surgical, and device therapies can certainly prolong a life span, disease progression from chronic to advanced to end stage is temporally unpredictable, uncertain, and marked by worsening symptoms that result in recurrent hospitalizations and excessive health care use. Compared with other serious illnesses, medication management that incorporates a palliative approach is underused among individuals with cardiovascular disease. This scientific statement describes palliative pharmacotherapy inclusive of cardiovascular drugs and essential palliative medicines that work synergistically to control symptoms and enhance quality of life. We also summarize and clarify available evidence on the utility of guideline-directed and evidence-based medical therapies in individuals with end-stage heart failure, pulmonary arterial hypertension, coronary heart disease, and other cardiomyopathies while providing clinical considerations for de-escalating or deprescribing. Shared decision-making and goal-oriented care are emphasized and considered quintessential to the iterative process of patient-centered medication management across the spectrum of cardiovascular disease.
PubMed: 38946532
DOI: 10.1161/HCQ.0000000000000131 -
NeoReviews Jul 2024Bronchopulmonary dysplasia (BPD) is the heterogeneous chronic lung developmental disease of prematurity, which is often accompanied by multisystem comorbidities.... (Review)
Review
Bronchopulmonary dysplasia (BPD) is the heterogeneous chronic lung developmental disease of prematurity, which is often accompanied by multisystem comorbidities. Pulmonary vascular disease and pulmonary hypertension (PH) contribute significantly to the pathogenesis and pathophysiology of BPD and dramatically influence the outcomes of preterm infants with BPD. When caring for those patients, clinicians should consider the multitude of phenotypic presentations that fall under the "BPD-PH umbrella," reflecting the need for matching therapies to specific physiologies to improve short- and long-term outcomes. Individualized management based on the patient's prenatal and postnatal risk factors, clinical course, and cardiopulmonary phenotype needs to be identified and prioritized to provide optimal care for infants with BPD-PH.
Topics: Humans; Bronchopulmonary Dysplasia; Hypertension, Pulmonary; Infant, Newborn; Infant, Premature; Risk Factors
PubMed: 38945971
DOI: 10.1542/neo.25-7-e415 -
Circulation Journal : Official Journal... Jun 2024Identifying and understanding the microstructural changes within the wall of the pulmonary artery (PA) is crucial for elucidating disease mechanisms and guiding...
BACKGROUND
Identifying and understanding the microstructural changes within the wall of the pulmonary artery (PA) is crucial for elucidating disease mechanisms and guiding treatment strategies. We assessed the utility of optical coherence tomography (OCT) in identifying such changes within segmental/subsegmental PAs and compared the morphological variations in WHO group 4 pulmonary hypertension associated with Behcet Disease (BD), Takayasu arteritis (TA) and chronic thromboembolic pulmonary hypertension (CTEPH). Idiopathic pulmonary arterial hypertension (IPAH) patients served as controls.Methods and Results: A total of 197 cross-sectional images were analyzed from 20 consecutive patients. BD patients exhibited lower %wall area and mean wall thickness (MWT) compared with CTEPH, TA and, IPAH patients. TA patients showed a notably higher %wall area, which was significant in IPAH and BD patients. Variations in %wall area measurements were observed across distinct cross-sectional segments of the PA within individual patients (22% in CTEPH, 19% in BD, 16% in TA, 23% in IPAH patients). Intravascular webs, bands, and thrombi were observed in BD and CTEPH patients. OCT provided clear delineation of vascular wall calcifications and adventitial vasa vasorum. No procedure-related complications were observed.
CONCLUSIONS
PA involvement differs among the various etiologies of PH, with the PA being heterogeneously affected. OCT offers promise in elucidating microstructural vascular wall changes and providing insights into disease mechanisms and treatment effects.
PubMed: 38945862
DOI: 10.1253/circj.CJ-24-0254 -
Clinics in Liver Disease Aug 2024Portopulmonary hypertension (POPH), hepatopulmonary syndrome, and hepatic hydrothorax constitute significant complications of portal hypertension, with important... (Review)
Review
Portopulmonary hypertension (POPH), hepatopulmonary syndrome, and hepatic hydrothorax constitute significant complications of portal hypertension, with important implications for management and liver transplantation (LT) candidacy. POPH is characterized by obstruction and remodeling of the pulmonary resistance arterial bed. Hepatopulmonary syndrome is the most common pulmonary vascular disorder, characterized by intrapulmonary vascular dilatations causing impaired gas exchange. LT may improve prognosis in select patients with POPH. LT is the only effective treatment of hepatopulmonary syndrome. Hepatic hydrothorax is defined as transudative pleural fluid accumulation that is not explained by primary cardiopulmonary or pleural disease. LT is the definitive cure for hepatic hydrothorax.
Topics: Humans; Hypertension, Portal; Hepatopulmonary Syndrome; Hydrothorax; Hypertension, Pulmonary; Liver Transplantation
PubMed: 38945638
DOI: 10.1016/j.cld.2024.03.005 -
Revista Portuguesa de Cardiologia :... Jun 2024Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of patients referred to PH centers. It is a three-compartment disease involving proximal (lobar-to-segmental) and distal (subsegmental) pulmonary arteries that are obstructed by persistent fibrothrombotic material, and precapillary pulmonary arteries that can be affected as in pulmonary arterial hypertension. It is a rare complication of pulmonary embolism (PE), with an incidence of around 3% in PE survivors. The observed incidence of CTEPH in the general population is around six cases per million but could be three times higher than this, as estimated from PE incidence. However, a previous venous thromboembolic episode is not always documented. With advances in multimodality imaging and therapeutic management, survival for CTEPH has improved for both operable and inoperable patients. Advanced imaging with pulmonary angiography helps distinguish proximal from distal obstructive disease. However, right heart catheterization is of utmost importance to establish the diagnosis and hemodynamic severity of PH. The therapeutic strategy relies on a stepwise approach, starting with an operability assessment. Pulmonary endarterectomy (PEA), also known as pulmonary thromboendarterectomy, is the first-line treatment for operable patients. Growing experience and advances in surgical technique have enabled expansion of the distal limits of PEA and significant improvements in perioperative and mid- to long-term mortality. In patients who are inoperable or who have persistent/recurrent PH after PEA, medical therapy and/or balloon pulmonary angioplasty (BPA) are effective treatment options with favorable outcomes that are increasingly used. All treatment decisions should be made with a multidisciplinary team that includes a PEA surgeon, a BPA expert, and a chest radiologist.
PubMed: 38945473
DOI: 10.1016/j.repc.2024.04.006 -
Clinica Chimica Acta; International... Jun 2024Treprostinil (Remodulin®) is a Food and Drug Administration (FDA) approved prostacyclin analog to treat pulmonary arterial hypertension. Recently, treprostinil has been...
Quantification of treprostinil concentration in rat and human using a novel validated and rapid liquid chromatography-tandem mass spectrometry method: Experimental and clinical applications in ischemia-reperfusion injury.
Treprostinil (Remodulin®) is a Food and Drug Administration (FDA) approved prostacyclin analog to treat pulmonary arterial hypertension. Recently, treprostinil has been investigated to reduce ischemia-reperfusion injury (IRI) during transplantation, which currently has no approved treatment. A validated analytical method is necessary to measure treprostinil concentrations in biological specimens. Here, a novel, sensitive, and specific method to measure treprostinil concentrations in rat serum, human serum, and human plasma has been developed using liquid chromatography with tandem mass spectrometry (LC-MS/MS). Biological samples were processed by protein precipitation before chromatography and 6-keto Prostaglandin F1α-d4 was used as an internal standard. A gradient method was established with a total run time of 4 min. The assay was linear over the range of 0.25-75.0 ng/ml with accuracy (92.97-107.87 %), intra-assay precision (1.16-3.34 %), and inter-assay precision (1.11-4.58 %) in all biological matrices, which are within FDA acceptance criteria. No significant variation in treprostinil or 6-keto Prostaglandin F1α-d4 concentrations were observed under the investigated storage conditions. This novel, sensitive, and specific LC/MS-MS method is cost-effective and suitable for measuring treprostinil concentrations in animal studies and human biological samples for clinical applications.
PubMed: 38945284
DOI: 10.1016/j.cca.2024.119837 -
Mymensingh Medical Journal : MMJ Jul 2024Now a days, chronic obstructive pulmonary disease (COPD) is a global health problem. This study was done to evaluate the changes of body mass index (BMI) and blood...
Now a days, chronic obstructive pulmonary disease (COPD) is a global health problem. This study was done to evaluate the changes of body mass index (BMI) and blood pressure (BP) in COPD patients in comparison to healthy person. This analytical type of cross sectional study was carried out in the Department of Physiology, Mymensingh Medical College, Mymensingh between the periods from July 2018 to June 2019. Total 160 male subjects, age ranged from 30-70 years was included in this study. Among them, in study group (Group II) eighty (80) male COPD subjects and eighty (80) age matched male healthy subjects were taken as control group (Group I). BMI was calculated as weight in kilogram divided by the height in meter square. Blood pressure was measured with an aneroid sphygmomanometer. Data were expressed as mean±SD and statistical significance of difference among the group was calculated by unpaired students' 't' test. The mean±SD of BMI of Group I and Group II were 24.52±1.35kg/m² and 21.22±1.30kg/m² respectively. The mean±SD of systolic blood pressure of Group I and Group II were 118.75±7.73 mm of Hg and 134.56±15.24 mm of Hg respectively. The mean±SD diastolic blood pressure of Group I and Group II were 77.63±6.70 mm of Hg and 84.69±8.05 mm of Hg respectively. The mean±SD of BMI was significantly lower in study group and the mean±SD of systolic and diastolic blood pressure was significantly higher in study group than control group. Low BMI and hypertension in subjects with COPD are associated with a high risk of exacerbations and mortality. So assessment of this parameter is important for prevention of complication related to COPD for leading a healthy life.
Topics: Humans; Male; Middle Aged; Body Mass Index; Pulmonary Disease, Chronic Obstructive; Cross-Sectional Studies; Blood Pressure; Adult; Aged; Case-Control Studies; Hypertension
PubMed: 38944707
DOI: No ID Found -
BMC Pulmonary Medicine Jun 2024For patients with congenital heart disease-related pulmonary arterial hypertension (CHD-PAH), cardiopulmonary exercise testing (CPET) can reflect cardiopulmonary reserve...
BACKGROUND
For patients with congenital heart disease-related pulmonary arterial hypertension (CHD-PAH), cardiopulmonary exercise testing (CPET) can reflect cardiopulmonary reserve function. However, CPET may not be readily accessible for patients with high-risk conditions or limited mobility due to disability. Echocardiography, on the other hand, serves as a widely available diagnostic tool for all CHD-PAH patients. This study was aimed to identify the parameters of echocardiography that could serve as indicators of cardiopulmonary function and exercise capacity.
METHODS
A cohort of 70 patients contributed a total of 110 paired echocardiogram and CPET results to this study, with 1 year interval for repeated examinations. Echocardiography and exercise testing were conducted following standardized procedures, and the data were collected together with clinically relevant indicators for subsequent statistical analysis. Demographic comparisons were performed using t-tests and chi-square tests. Univariate and multivariate analyses were conducted to identify potential predictors of peak oxygen uptake (peak VO) and the carbon dioxide ventilation equivalent slope (VE/VCO slope). Receiver operating characteristic (ROC) analysis was used to assess the performance of the parameters.
RESULTS
The ratio of tricuspid annular plane systolic excursion to pulmonary artery systolic pressure (TAPSE/PASP) was found to be the only independent indicator significantly associated with both peak VO and VE/VCO slope (both p < 0.05). Additionally, left ventricular ejection fraction (LVEF) and right ventricular fractional area change (FAC) were independently correlated with the VE/VCO slope (both p < 0.05). TAPSE/PASP showed the highest area under the ROC curve (AUC) for predicting both a peak VO ≤ 15 mL/kg/min and a VE/VCO slope ≥ 36 (AUC = 0.91, AUC = 0.90, respectively). The sensitivity and specificity of TAPSE/PASP at the optimal threshold exceeded 0.85 for both parameters.
CONCLUSIONS
TAPSE/PASP may be a feasible echocardiographic indicator for evaluating exercise tolerance.
Topics: Humans; Female; Male; Exercise Test; Heart Defects, Congenital; Echocardiography; Adult; ROC Curve; Exercise Tolerance; Pulmonary Arterial Hypertension; Oxygen Consumption; Middle Aged; Young Adult; Hypertension, Pulmonary; Pulmonary Artery
PubMed: 38944669
DOI: 10.1186/s12890-024-03113-7 -
Mathematical Biosciences Jun 2024Ventricular ventricular interaction (VVI) affects blood volume and pressure in the right and left ventricles of the heart due to the location and balance of forces on...
Ventricular ventricular interaction (VVI) affects blood volume and pressure in the right and left ventricles of the heart due to the location and balance of forces on the septal wall separating the ventricles. In healthy patients, the pressure of the left ventricle is considerably higher than the right, resulting in a septal wall that bows into the right ventricle. However, in patients with pulmonary hypertension, the pressure in the right ventricle increases significantly to a point where the pressure is similar to or surpasses that of the left ventricle during portions of the cardiac cycle. For these patients, the septal wall deviates towards the left ventricle, impacting its function. It is possible to study this effect using mathematical modeling, but existing models are nonlinear, leading to a system of algebraic differential equations that can be challenging to solve in patient-specific optimizations of clinical data. This study demonstrates that a simplified linearized model is sufficient to account for the effect of VVI and that, as expected, the impact is significantly more pronounced in patients with pulmonary hypertension.
PubMed: 38944112
DOI: 10.1016/j.mbs.2024.109242