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Query: UMLS:C0037315 (sleep apnea)
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Pulmonary artery hypertension secondary to chronic lung diseases is a clinical entity with no specific symptoms that can develop as a result of parenchymal lung disorders (COPD-emphysema, sleep apnea syndrome, diffuse parenchymal lung diseases, etc.) and pulmonary vascular disorders (vasculitis, sarcoidosis, etc.). In the clinical history of these chronic and invalidating diseases, pulmonary vasculature goes through various degenerative and/or proliferative changes, responsible of the pulmonary arterial hypertension appearance. The rise in pulmonary artery pressure can be subtle and the progression from an asymptomatic disease to a more severe syndrome is often common in all forms of secondary pulmonary arterial hypertension. Etiopathology of pulmonary artery hypertension secondary to chronic lung diseases is based on one or more of the following mechanisms: hypoxic vasoconstriction, decreased area of pulmonary vascular bed, volume/pressure overload. In these forms, the above three mechanisms show common mediators, all responsible of disease progression but singularly potential reversible. Therapies for secondary pulmonary artery hypertension consist primarily on the treatment of the underlying disease. Therapy is most effective when initiated prior to the onset of irreversible pulmonary vascular damage. In the last two decades, new medical treatments (prostacyclins, endothelin receptor antagonists, phosphodiesterase inhibitors) for pulmonary arterial hypertension have been available for the sporadic and the secondary to systemic sclerosis forms. The role of these drugs in the other forms of pulmonary arterial hypertension has not been well studied yet. This review will go through the pathogenesis and the several therapeutic approaches for pulmonary artery hypertension secondary to chronic pulmonary diseases or pulmonary vasculature disorders.
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PMID:Pulmonary arterial hypertension secondary to chronic lung diseases: pathogenesis and medical treatment. 1803 16

High-altitude pulmonary hypertension (HAPH) affects individuals residing at altitudes of 2,500 meters and higher. Numerous pathogenic variables play a role in disease inception and progression and include low oxygen concentration in inspired air, vasculopathy, and metabolic abnormalities. Since HAPH affects only some people living at high altitude genetic factors play a significant role in its pathogenesis. The clinical presentation of HAPH is nonspecific and includes fatigue, shortness of breath, cognitive deficits, cough, and in advanced cases hepatosplenomegaly and overt right-sided heart failure. A thorough history is important and should include a search for additional risk factors for lung disease and pulmonary hypertension (PH) such as smoking, indoor air pollution, left-sided cardiac disease and sleep disordered breathing. Twelve-lead electrocardiogram, chest X-ray and echocardiography can be used as screening tools. A definitive diagnosis should be made with right-sided heart catheterization using a modified mean pulmonary artery pressure of at least 30 mm Hg, differing from the 25 mm Hg used for other types of PH. Treatment of HAPH includes descent to a lower altitude whenever possible, oxygen therapy and the use of medications such as endothelin receptor antagonists, phosphodiesterase 5 blockers, fasudil and acetazolamide. Some recent evidence suggests that iron supplementation may also be beneficial. However, it is important to note that the scientific literature lacks long-term randomized controlled data on the pharmacologic treatment of HAPH. Thus, an individualized approach to treatment and informing the patients regarding the benefits and risks of the selected treatment regimen are essential.
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PMID:High-altitude Pulmonary Hypertension: an Update on Disease Pathogenesis and Management. 2701 74