肺動脈高壓:概述及兒科近期成就

肺動脈高壓是一種慢性肺血管系統疾病,其特徵是肺血管重構和肺動脈壓力升 高。臨床表現、實驗室檢查和心肺運動試驗以及 6 分鐘步行距離有助於正確診斷。 確診依賴於右心導管插入術。CT 肺血管造影的肺灌注成像與肺血管造影的效果相 似,顯示出重要的診斷價值。常規生化、血液學和甲狀腺功能測試以及抗核抗體 可能會有所幫助。常規藥物治療、姑息性房間隔切開術和肺移植是首選治療方法。 microRNA 、間充質幹細胞、遠程後處理等新技術已取得初步治療效果。最近的研 究表明一氧化氮吸入、米力農、磷酸二酯酶 5 抑制劑和前列環素類似物對治療兒 科患者的潛在有效性。  Pulmonary arterial hypertension is a chronic pulmonary vasculature disorder characterized by pulmonary vascular remodeling and elevated pulmonary artery pressures. Clinical manifestations, laboratory work-ups, cardiopulmonary exercise testing and 6-minute walk distance are helpful in reaching the correct diagnosis. The definite diagnosis relies on right heart catheterization. Pulmonary perfusion imaging with computed tomographic pulmonary angiography is similar to the effect of pulmonary angiography, showing important diagnostic values. Routine biochemistry, hematology and thyroid function tests as well as antinuclear antibodies can be helpful. Conventional medication, palliative septostomy and lung transplantation are treatments of choice. Novel techniques including microRNA, mesenchymal stem cell and remote postconditioning treatments have achieved initial therapeutic effects. Recent studies have demonstrated the potential effectiveness of inhaled nitric oxide, milrinone, phosphodiesterase-5 inhibitors and prostacyclin analogues for the treatment of PAH in pediatric patients.