Review ArticlesCellular pathophysiology and therapy of pulmonary hypertension☆
Section snippets
Risk factors for pulmonary hypertension
Although the primary pathophysiologic mechanisms may be substantially different in different types of pulmonary hypertension, after severe pulmonary hypertension occurs, the consequences for the right heart and the pulmonary arteries are quite similar (Fig 1).
Therapy of pulmonary hypertension
On the basis of our knowledge of molecular and cellular mechanisms involved in the pathogenesis of pulmonary hypertension, therapeutic approaches could address inhibition of cell proliferation or increase of apoptosis; vasodilatation or inhibition of vasoconstric-tion; inhibition of inflammatory, prothrombotic, and proproliferative mediators; and increase of their antagonists.
Indeed, during the last decade, considerable progress in medical therapy has contributed to improved prognosis of severe
Acknowledgements
We thank Sylvia Weissmann, PhD, Ralph Wiedemann, Thomas Schmehl, PhD, and Tobias Gessler, MD, for stimulating discussions and Mary-Kay Steen-Mueller, MD, for carefully reviewing the manu-script.
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Cited by (55)
Serum levels of tumor necrosis factor-related apoptosis-inducing ligand correlate with the severity of pulmonary hypertension
2015, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :Pulmonary hypertension (PH) is a malignant disease that is associated with progressive deterioration and poor prognosis [1].
Endothelin-1 driven proliferation of pulmonary arterial smooth muscle cells is c-fos dependent
2014, International Journal of Biochemistry and Cell BiologyCitation Excerpt :Increased intrapulmonary pressure leading to right heart hypertrophy, pressure overload and eventually to right heart failure are the hallmarks of pulmonary hypertension (PH) (Olschewski et al., 2001).
56-year-old man with pulmonary hypertension
2013, Air Medical JournalCitation Excerpt :Endothelial dysfunction can result in a decreased production of nitric oxide, leading to pulmonary vasoconstriction, which, in turn, potentiates the formation of thrombi.1 Diseases such as chronic thromboembolism, a large post-tricuspid left-to-right shunt, congestive heart failure, scleroderma, chronic hypoxia, and interstitial lung disease do not appear to have similar identified genetic pathways.4 In this case, the patient had a history of scleroderma and COPD.
Preserved pulmonary vasodilative properties of aerosolized brain natriuretic peptide
2009, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :More importantly, we demonstrate a maintained vasodilative capability of BNP during its application as an aerosol. Inhalation of vasodilators has been suggested before in pulmonary hypertension in order to reach preferential distribution into ventilated areas of the lung, to gain pulmonary and intrapulmonary selectivity and consequently to reduce systemic side effects and to avoid further gas exchange disturbance [19,24]. However, it is not self-evident that a peptide which is exposed to a nebulizer-technique and proteolytic enzymes on the lung surface preserves its biologic activity.
Chronic thromboembolic and pulmonary arterial hypertension share acute vasoreactivity properties
2006, ChestCitation Excerpt :The primary objective of acute vasodilator testing in patients with PAH is to identify the subset of patients who might be treated effectively with oral calcium-channel blockers. However, this drug class will probably never be a valuable therapeutic option in patients with CTEPH due to its capacity to increase ventilation-perfusion mismatch and thus intrapulmonary shunting.13,33,34,35,36 In patients with PAH, a pronounced response to short-acting pulmonary vasodilators has been shown37,38 to be associated with a better prognosis, at least in some patients.
Clinical utility of inhaled iloprost in pulmonary arterial hypertension
2014, Archivos de Cardiologia de Mexico
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Reprint requests: Horst Olschewski, MD, Medical Clinic II, Justus-Liebig-University, Klinikstr. 36, 35392 Giessen, Germany.