Souza Rogério, Fernandes Caio Júlio César dos Santos, Jardim Carlos Viana Poyares
Pulmonary Department-Heart Institute, University of São Paulo Medical School, 44 Sao Paulo, Brazil 05403-000.
Semin Respir Crit Care Med. 2009 Aug;30(4):448-57. doi: 10.1055/s-0029-1233314. Epub 2009 Jul 24.
Recent years have witnessed a significant increase in the knowledge about the pathophysiology of pulmonary arterial hypertension (PAH) and the availability of multiple drugs specifically aimed at pulmonary circulation. Although most of this is related to the idiopathic form of PAH, this development has also turned attention to other forms of pulmonary hypertension such as schistosomiasis-associated PAH (Sch-PAH), portopulmonary hypertension (POPH), and hemolysis-associated pulmonary hypertension. The importance of these different forms of pulmonary hypertension spans their epidemiology and the multiple pathophysiological mechanisms associated with their development and complications. Taken together, Sch-PAH and hemolysis-associated pulmonary hypertension may represent the most prevalent forms of PAH worldwide. Portopulmonary hypertension is particularly important if the morbidity and mortality that it adds to patients with liver disease is considered. Although clear progress has been reached in these various forms of PAH, there are many different aspects yet to be addressed that may contribute to the basis of specific treatment applied to these subgroups of patients.
近年来，人们对肺动脉高压（PAH）病理生理学的认识显著增加，并且有多种专门针对肺循环的药物可供使用。尽管其中大部分与特发性PAH有关，但这一进展也将注意力转向了其他形式的肺动脉高压，如血吸虫病相关性PAH（Sch-PAH）、门脉性肺动脉高压（POPH）和溶血相关性肺动脉高压。这些不同形式的肺动脉高压的重要性体现在其流行病学以及与其发生和并发症相关的多种病理生理机制上。总体而言，Sch-PAH和溶血相关性肺动脉高压可能是全球最普遍的PAH形式。如果考虑到门脉性肺动脉高压给肝病患者增加的发病率和死亡率，那么它就显得尤为重要。尽管在这些不同形式的PAH方面已经取得了明显进展，但仍有许多不同方面有待解决，这些方面可能有助于为这些亚组患者应用特定治疗奠定基础。
