New York Presbyterian Hospital, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Prog Cardiovasc Dis. 2012 Sep-Oct;55(2):119-27. doi: 10.1016/j.pcad.2012.08.003.
Pulmonary hypertension in the setting of parenchymal lung disease and conditions associated with chronic hypoxemia is commonly encountered in clinical practice and may adversely affect patients' function and mortality. Diagnosis of this subgroup of pulmonary hypertension has evolved but still requires right heart catheterization for confirmation. The primary treatment goal is optimization of the underlying parenchymal lung or hypoxemia-associated condition prior to consideration of pharmacologic therapy. Limited published experience with pulmonary hypertension-specific medications for treatment of WHO Group 3 pulmonary hypertension suggests symptomatic and functional benefit in selected individuals. The potential for worsening ventilation-perfusion matching must be considered in these cases, however, since there is a paucity of data regarding the optimal approach to treatment selection. Ongoing medication trials and further investigation of mechanisms of hypoxic pulmonary vasoconstriction provide hope for these patients who in the past often had only lung transplantation as a potential treatment option.
肺实质疾病和与慢性低氧血症相关的情况下的肺动脉高压在临床实践中很常见，可能会对患者的功能和死亡率产生不利影响。该亚组肺动脉高压的诊断已经发展，但仍需要右心导管检查来确认。主要的治疗目标是在考虑药物治疗之前，优化潜在的肺实质疾病或与低氧血症相关的疾病。对于特发性肺动脉高压 3 组患者，有限的肺动脉高压特异性药物治疗的经验表明，在选择的个体中具有症状和功能获益。然而，在这些情况下，必须考虑到通气-灌注匹配恶化的可能性，因为关于治疗选择的最佳方法的数据很少。正在进行的药物试验和对低氧性肺血管收缩机制的进一步研究为这些患者提供了希望，过去这些患者通常只有肺移植作为一种潜在的治疗选择。
