Duijnhouwer A L, Lemmers J, Smit J, van Haren-Willems J, Knaapen-Hans H, Ten Cate T, Hagmolen Of Ten Have W, de Boer M-J, Roos-Hesselink J, Vonk M, van Dijk A
Department of Cardiology and Radboudumc Centre for Pulmonary Hypertension, Radboud University Medical Centre, Nijmegen, The Netherlands.
Department of Rheumatic Diseases and Radboudumc Centre for Pulmonary Hypertension, Radboud University Medical Centre, Nijmegen, The Netherlands.
Neth Heart J. 2020 Dec;28(12):645-655. doi: 10.1007/s12471-020-01467-1.
Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. The PA dilates over time and therefore may reflect disease severity and duration. Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality.
Patients visiting an outpatient clinic of a tertiary centre between 2004 and 2018 with a cardiac catheterisation confirmed diagnosis of PAH or CTEPH and a CT scan available for PA diameter measurement were included. PA diameter and established predictors of survival were collected (New York Heart Association (NYHA) class, N‑terminal pro-brain natriuretic peptide (NT-proBNP) level and 6‑min walking distance (6MWD)).
In total 217 patients were included (69% female, 71% NYHA class ≥III). During a median follow-up of 50 (22-92) months, 54% of the patients died. Overall survival was 87% at 1 year, 70% at 3 years and 58% at 5 years. The mean PA diameter was 34.2 ± 6.2 mm and was not significantly different among all the diagnosis groups. We found a weak correlation between PA diameter and mean PA pressure ( r = 0.23, p < 0.001). Male sex, higher age, shorter 6MWD and higher NT-proBNP level were independently associated with mortality, but PA diameter was not.
The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients.
肺动脉（PA）扩张在肺动脉高压（PH）中很常见，被认为是压力升高的长期后果。随着时间的推移，肺动脉会扩张，因此可能反映疾病的严重程度和病程。生存率与诊断时的疾病阶段相关，因此肺动脉直径可用于预测预后。本研究评估了肺动脉高压（PAH）和慢性血栓栓塞性肺动脉高压（CTEPH）患者的预后，并调查诊断时的肺动脉直径是否与死亡率相关。
纳入2004年至2018年间在三级中心门诊就诊、经心导管检查确诊为PAH或CTEPH且有CT扫描可测量肺动脉直径的患者。收集肺动脉直径和已确定的生存预测指标（纽约心脏协会（NYHA）分级、N末端脑钠肽前体（NT-proBNP）水平和6分钟步行距离（6MWD））。
共纳入217例患者（69%为女性，71%的NYHA分级≥III级）。在中位随访50（22 - 92）个月期间，54%的患者死亡。1年时总生存率为87%，3年时为70%，5年时为58%。平均肺动脉直径为34.2±6.2mm，在所有诊断组中无显著差异。我们发现肺动脉直径与平均肺动脉压力之间存在弱相关性（r = 0.23，p < 0.001）。男性、高龄、较短的6MWD和较高的NT-proBNP水平与死亡率独立相关，但肺动脉直径与死亡率无关。
PAH和CTEPH的预后仍然很差。已确认了已知的生存预测指标，但诊断时的肺动脉直径与PAH或CTEPH患者的生存无关。
