Department of Cardiovascular Medicine, Duke University Health System, Durham, NC.
Department of Cardiovascular Medicine, Duke University Health System, Durham, NC.
Prog Cardiovasc Dis. 2018 Sep-Oct;61(3-4):320-327. doi: 10.1016/j.pcad.2018.07.017. Epub 2018 Jul 18.
Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Management can be challenging, but the therapeutic armamentarium is continually expanding and now includes surgical, transcatheter and medical options. Timely correction of defects along with early treatment with advanced medical therapies appears to improve quality of life and possible even improve survival. Interestingly most studies of PAH-CHD have focused on its most severely afflicted patients, those with Eisenmenger Syndrome, making it less certain how to manage PAH-CHD of milder degrees. This review summarizes our current understanding of PAH-CHD and emphasizes the need for close follow-up in specialized centers of care where close collaboration is common practice.
儿童先天性心脏病（CHD）管理的进步导致越来越多的成年人患有 CHD。与 CHD 相关的肺动脉高压（PAH-CHD）是一种常见的并发症，影响多达 10%的患者；即使在成功和完全修复缺陷后，也可能发生，具有严重且潜在致命的后果。对这些患者进行仔细的检查至关重要，特别是血流动力学评估，可以帮助确定最合适的治疗方法。管理可能具有挑战性，但治疗手段不断扩大，现在包括手术、经导管和药物治疗。及时纠正缺陷，早期采用先进的医疗治疗方法，似乎可以提高生活质量，甚至可能提高生存率。有趣的是，大多数关于 PAH-CHD 的研究都集中在病情最严重的患者，即 Eisenmenger 综合征患者，因此，对于病情较轻的 PAH-CHD，如何进行管理就不太确定。这篇综述总结了我们目前对 PAH-CHD 的认识，并强调了在专门的护理中心进行密切随访的必要性，在这些中心，密切合作是常见的做法。
