Xu Zhuoyuan, Gatzoulis Michael A, Dimopoulos Konstantinos, Li Qiangqiang, Zhang Chen, Keller Bradley B, Gu Hong
Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University Beijing Institute of Heart, Lung, and Blood Vessel Diseases, Beijing, China.
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College, London, United Kingdom.
CJC Open. 2021 Feb 17;3(7):872-879. doi: 10.1016/j.cjco.2021.02.010. eCollection 2021 Jul.
Pulmonary vascular changes in postoperative pulmonary artery hypertension (PAH) are similar to those seen in idiopathic PAH. Data are sparse on direct comparative midterm outcomes for these 2 high-risk populations.
Patients with idiopathic or postoperative PAH referred to a large tertiary hospital between June 2005 and July 2019 were retrospectively evaluated.
A total of 364 consecutive patients were studied, including 201 postoperative PAH patients and 163 patients with idiopathic PAH, with a median age of 18.7 (interquartile range 10.0, 31.5) and 7.3 (IQR: 2.9, 18.3) years, respectively. PAH-specific drugs were used in 77.7% of patients; 31.4% received combination therapy. Patients with idiopathic PAH had a shorter 6-mintue walk distance, lower percutaneous oxygen saturation, and higher B-type natriuretic peptide levels than those with postoperative PAH at diagnosis (all < 0.001), During a median follow-up time of 3.4 (interquartile range: 2.1, 5.8) years, 56 patients (15.4%) died, and one underwent bilateral lung transplantation. Patients with postoperative PAH had better survival than those with idiopathic PAH, according to age (hazard ratio [HR] 0.128, 95% confidence interval [CI]: 0.07-0.22, < 0.0001); Kaplan-Meier survival estimates at 5 years for idiopathic and postoperative PAH patients were 74.3% and 92.6%, respectively. Patients in New York Heart Association functional class III-IV had an over 4-fold increased risk of death (HR 4.85, 95% CI: 2.61-9.00, < 0.0001). Patients < 18 years of age at idiopathic PAH diagnosis had a worse survival compared to adult patients (HR 6.90, 95% CI: 4.19-15.56, = 0.040).
Postoperative-PAH patients had better midterm survival compared to patients with idiopathic PAH. Mortality was significant in both PAH groups, reinforcing the need for early diagnosis and optimal individualized management to improve outcomes.
术后肺动脉高压（PAH）的肺血管变化与特发性PAH相似。关于这两个高危人群直接比较的中期结局的数据很少。
对2005年6月至2019年7月转诊至一家大型三级医院的特发性或术后PAH患者进行回顾性评估。
共研究了364例连续患者，包括201例术后PAH患者和163例特发性PAH患者，中位年龄分别为18.7岁（四分位间距10.0，31.5）和7.3岁（四分位间距：2.9，18.3）。77.7%的患者使用了PAH特异性药物；31.4%接受了联合治疗。特发性PAH患者在诊断时的6分钟步行距离较短、经皮血氧饱和度较低且B型利钠肽水平较高，均低于术后PAH患者（均P<0.001）。在中位随访时间3.4年（四分位间距：2.1，5.8）期间，56例患者（15.4%）死亡，1例接受了双侧肺移植。根据年龄，术后PAH患者的生存率高于特发性PAH患者（风险比[HR]0.128，95%置信区间[CI]：0.07-0.22，P<0.0001）；特发性和术后PAH患者5年的Kaplan-Meier生存估计分别为74.3%和92.6%。纽约心脏协会功能分级为III-IV级的患者死亡风险增加超过4倍（HR 4.85，95%CI：2.61-9.00，P<0.0001）。特发性PAH诊断时年龄<18岁的患者与成年患者相比生存率更差（HR 6.90，95%CI：4.19-15.56，P=0.040）。
与特发性PAH患者相比，术后PAH患者的中期生存率更好。两个PAH组的死亡率都很高，这进一步强调了早期诊断和优化个体化管理以改善结局的必要性。
