Pulmonary Vascular Disease Unit, Royal Papworth Hospital, Cambridge, UK.
Pulmonary Vascular Disease Unit, Royal Papworth Hospital, Cambridge, UK.
J Heart Lung Transplant. 2023 Sep;42(9):1275-1285. doi: 10.1016/j.healun.2023.04.016. Epub 2023 May 16.
We describe baseline characteristics, disease progression and mortality in chronic thromboembolic pulmonary disease patients as a function of mean pulmonary artery pressure (mPAP) according to new and previous definitions of pulmonary hypertension.
All patients diagnosed with chronic thromboembolic pulmonary disease between January, 2015 and December, 2019 were dichotomized according to initial mPAP: ≤ 20 mmHg ('normal') vs 21-24 mmHg ('mildly-elevated'). Baseline features were compared between the groups, and pairwise analysis performed to determine changes in clinical endpoints at 1-year, excluding those who underwent pulmonary endarterectomy or did not attend follow-up. Mortality was assessed for the whole cohort over the entire study period.
One hundred thirteen patients were included; 57 had mPAP ≤ 20 mmHg and 56 had mPAP 21-24 mmHg. Normal mPAP patients had lower pulmonary vascular resistance (1.6 vs 2.5WU, p < 0.01) and right ventricular end-diastolic pressure (5.9 vs 7.8 mmHg, p < 0.01) at presentation. At 3 years, no major deterioration was seen in either group. No patients were treated with pulmonary artery vasodilators. Eight had undergone pulmonary endarterectomy. Over 37 months median follow-up, mortality was 7.0% in the normal mPAP group and 8.9% in the mildly-elevated mPAP group. Cause of death was malignancy in 62.5% of cases.
Chronic thromboembolic pulmonary disease patients with mild pulmonary hypertension have statistically higher right ventricular end-diastolic pressure and pulmonary vascular resistance than those with mPAP ≤ 20 mmHg. Baseline characteristics were otherwise similar. Neither group displayed disease progression on non-invasive tests up to 3 years. Mortality over 37 months follow-up is 8%, and mainly attributable to malignancy. Further prospective study is required to validate these findings.
我们根据肺动脉高压的新定义和旧定义，描述了慢性血栓栓塞性肺动脉高压患者的平均肺动脉压（mPAP）的基线特征、疾病进展和死亡率。
所有在 2015 年 1 月至 2019 年 12 月期间被诊断为慢性血栓栓塞性肺动脉高压的患者，根据初始 mPAP 分为两类：≤20mmHg（“正常”）和 21-24mmHg（“轻度升高”）。比较两组之间的基线特征，并进行两两分析，以确定在 1 年内临床终点的变化，排除那些接受肺动脉内膜切除术或未参加随访的患者。在整个研究期间，对整个队列进行了死亡率评估。
共纳入 113 例患者，其中 57 例 mPAP≤20mmHg，56 例 mPAP 21-24mmHg。正常 mPAP 患者的肺血管阻力（1.6 对 2.5WU，p<0.01）和右心室舒张末期压（5.9 对 7.8mmHg，p<0.01）较低。在 3 年时，两组均未出现明显恶化。没有患者接受肺动脉血管扩张剂治疗。8 例患者接受了肺动脉内膜切除术。在 37 个月的中位随访期间，正常 mPAP 组的死亡率为 7.0%，轻度升高 mPAP 组为 8.9%。62.5%的死亡原因为恶性肿瘤。
与 mPAP≤20mmHg 的患者相比，轻度肺动脉高压的慢性血栓栓塞性肺动脉高压患者的右心室舒张末期压和肺血管阻力统计学上更高。其他基线特征则相似。两组在 3 年内的非侵入性检查中均未出现疾病进展。37 个月的随访期间死亡率为 8%，主要归因于恶性肿瘤。需要进一步的前瞻性研究来验证这些发现。
