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The Patient with Non-Group 2 Pulmonary Hypertension
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
Sophia Anastasia Mouratoglou, George Giannakoulas
Although linked with pulmonary embolism, the pathophysiologic development of CTEPH extends beyond thrombosis. Recently, the role of small vessel abnormalities in the lungs of patients with CTEPH has received a major update, as the scope of small vessel involvement of CTEPH is found to extend beyond the precapillary small arteries. Histopathologic changes in the pulmonary venous and capillary systems, similar to that seen in pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis, as well as systemic to pulmonary anastomoses and hypertrophied bronchial collaterals, have been reported in lungs of CTEPH patients.14
Malignant Tumors and the Microcirculation
Published in John H. Barker, Gary L. Anderson, Michael D. Menger, Clinically Applied Microcirculation Research, 2019
Bernhard Endrich, Peter Vaupel
(2) A number of studies suggest that components of the basal lamina such as fibronectin, collagen type IV, and laminin undergo a more rapid turnover in growing capillaries. Consequently, agents that interfere with the synthesis of these components or accelerate their degradation favor a dissolution of the basement membrane, thus inhibiting angiogenesis. In particular, interferon-alpha has received marked attention during recent years. This agent was used to successfully treat at least 20 children suffering from pulmonary capillary hemangiomatosis as well as systemic hemangiomatosis where steroid therapy had already failed.42 Beyond this rare disease with its excessive growth of capillaries, however, interferon-alpha has not been established as a potent agent in medical oncology.43
Lung transplantation for pulmonary hypertension
Published in Wickii T. Vigneswaran, Edward R. Garrity, John A. Odell, LUNG Transplantation, 2016
Stéphane Collaud, Marc de Perrot
Patients with group 1 PH who have pulmonary venoocclusive disease and pulmonary capillary hemangiomatosis should be referred for transplantation assessment as soon as the diagnosis is established because no other viable medical therapy is available.1 Patients with group 3 PH (PH resulting from lung disease, hypoxia, or both) or group 5 PH (PH with unclear multifactorial mechanisms) are discussed elsewhere in this book. Timing for referral and indications for transplantation follow the criteria for the primary underlying disease. In principle, patients with group 4 PH (chronic thromboembolic PH) can also be candidates for transplantation; however, thanks to the current success of pulmonary endarterectomy, transplantation is now indicated for very few such patients.6 Hence, all patients with a new diagnosis of PH should have a ventilation-perfusion scan performed and in the presence of mismatched perfusion defects be seen by an experienced surgical center to determine the feasibility of pulmonary endarterectomy.
Bronchoscopy during the COVID-19 pandemic: A Canadian Thoracic Society Position Statement update
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2022
Simon A. Houston, Yusing Gu, Thomas Vandemoortele, Elaine Dumoulin, Ashley-Mae E. Gillson, Chung-Chun Tyan, Lama Sakr, Glenda N. Bendiak, Anne V. Gonzalez, Marc Fortin
The histologic characteristics of persistent radiographic findings have been characterized in detail using transbronchial cryobiopsy. Ravaglia et al. described three clusters of combined clinical, radiographic, and pathological findings: patients with probable underlying chronic fibrosis, those with acute/subacute changes including organizing pneumonia, and patients with vascular changes associated with minimal radiographic abnormalities felt to be akin to pulmonary capillary hemangiomatosis.61
Efficacy and safety of novel-targeted drugs in the treatment of pulmonary arterial hypertension: a Bayesian network meta-analysis
Published in Drug Delivery, 2021
Wenhai Fu, Wenjun He, Yuexin Li, Yangxiao Chen, Jingyi Liang, Hui Lei, Lin Fu, Yanghang Chen, Ni Ren, Qian Jiang, Yi Shen, Ran Ma, Tao Wang, Xinni Wang, Nuofu Zhang, Dakai Xiao, Chunli Liu
In terms of outcomes, we found that ERA and PDE5i combined treatments, such as Bosentan combined with Sildenafil, Bosentan combined with Tadalafil, and ERA and ProsA combined treatments, such as Bosentan combined with Iloprost, have a higher probability of more improve 6MWD and cardiac function. Although patients were prone to edema, headache, diarrhea, dizziness, and other adverse reactions (Galiè et al., 2008), most patients could tolerate them. Consequently, we considered that ERA combined with PDE5i or ERA combined with ProsA of PAH in the early stage could prevent irreversible remodeling of pulmonary vessels (O'Connell et al., 2013), to get more significant benefits. However, continuous monitoring of blood concentration of patients is needed to judge the progress of PAH (Coghlan et al., 2018) in patients and timely respond to possible AEs when combined treatment regimens are used. Besides, the interaction between drugs should also be considered. For example, the pharmacokinetic interaction between Bosentan and Sildenafil may cause insufficient sildenafil drug plasma concentrations (Grünig et al., 2017). Therefore, the routine monitoring of Sildenafil-Bosentan plasma concentrations is necessary. If patients with inadequate treatment response, the switch from Bosentan to other alternative ERA (Ambrisentan or Macitentan) should be considered (Apitz and Schranz, 2018). However, expect for Sildenafil + Bosentan, Sildenafil + Tadalafil, Ambrisentan + Tadalafil, different ERA + PDE5i still no RCTs to confirm the efficacy and safety. It is necessary to be cautious in clinical treatment. For patients receiving long-term single-agent therapy (>5–10 years) with stable and low-risk symptoms, Age >75, Suspected pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH), and other patients not recommended to use combined therapy. Although Sildenafil may cause headache, epistaxis, and muscle pain, the benefit of Sildenafil may be more significant. It is crucial to watch the dosage during treatment (Spradley, 2012).