先天性心脏病相关肺动脉高压患者的管理

Management of pulmonary arterial hypertension associated with congenital heart disease

摘要: 先天性心脏病相关肺动脉高压的发病机制复杂，本病对患者的生活质量有相当大的影响。虽然目前对这类疾病尚无有效的治疗措施，但通过合理的治疗干预可以延长患者的生存时间，改善其临床症状和生活质量。因此，有必要通过多学科小组的交流对这类患者进行适当的管理，以改善患者的生活质量和预后。

Abstract: The mechanism of congenital heart diseases (CHD) associated with pulmonary arterial hypertension (PAH) is complicated and poor quality of life (QOL) is usually reported in patients with CHDPAH. Although there have been no effective therapies for CHDPAH, patients’ lifetime can be lengthened, QOL improved and clinical syndrome alleviated using reasonable treatment. Multidisciplinary treatment can improve QOL and prognosis in CHDPAH patients.