Moll Matthew, Payne Julie G, Tukey Melissa H, Farber Harrison W
Boston Medical Center, Boston, Massachusetts, USA.
Pulmonary Center, Boston University School of Medicine, Boston, Massachusetts, USA.
Pulm Circ. 2015 Dec;5(4):730-3. doi: 10.1086/683689.
Pulmonary arterial hypertension (PAH) is a progressive disease marked by the irreversible pulmonary vascular changes of vasoconstriction, thrombosis, and proliferation of smooth muscle and endothelial cells. The untreated clinical course is characterized by progressive dyspnea and a median survival of less than 3 years. Many of these patients are of child-bearing age; however, pregnancy leads to physiologic changes that are particularly poorly tolerated in PAH, conferring a 30%-56% mortality. We present a case of PAH that spontaneously resolved after termination of pregnancy and recurred during each of two subsequent pregnancies. To our knowledge, this case is unique, because no cases of spontaneous resolution of idiopathic PAH have been reported in adults, nor have there been any reports of pulmonary hypertension that is isolated to the gestational period.
肺动脉高压(PAH)是一种进行性疾病,其特征是出现血管收缩、血栓形成以及平滑肌和内皮细胞增殖等不可逆的肺血管变化。未经治疗的临床病程表现为进行性呼吸困难,中位生存期不到3年。这些患者中有许多处于育龄期;然而,怀孕会导致生理变化,而PAH患者对这些变化的耐受性特别差,死亡率为30%-56%。我们报告一例PAH患者,该患者在终止妊娠后自发缓解,并在随后的两次妊娠中每次都复发。据我们所知,此病例独一无二,因为在成人中尚未有特发性PAH自发缓解的病例报告,也没有仅在妊娠期出现的肺动脉高压的报告。