Cullivan Sarah, McCormack Ciara, O'Callaghan Marissa, Kevane Barry, NiAinle Fionnuala, McCullagh Brian, Gaine Sean P
National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.
Department of Haematology, Mater Misericordiae University Hospital, Dublin, Ireland.
Pulm Circ. 2021 Oct 8;11(4):20458940211048703. doi: 10.1177/20458940211048703. eCollection 2021 Oct-Dec.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and under-recognised complication of acute pulmonary embolism. Information regarding the characteristics of CTEPH in Ireland is limited, and the aim of this retrospective cohort study was to address this knowledge gap. Seventy-two cases of CTEPH were diagnosed in the National Pulmonary Hypertension Unit (NPHU) in Ireland between 2010 and 2020. This accounted for 6% of all referrals to the unit and translates to an estimated annual incidence of 1.39 per million population (95% confidence interval, 0.33-2.46). The prevalence of diagnosed CTEPH in Ireland in 2020 was estimated at 12.05 per million population (95% CI 9.00-15.10). The average duration of symptoms prior to CTEPH diagnosis was 23 (±22) months. Patients with CTEPH were more likely to be male (n = 40, 56%), older (60 ± 17 years) and have identifiable risk factors for CTEPH (n = 61, 85%) at diagnosis. Regarding treatment, pulmonary hypertension (PH) vasodilator therapy was prescribed in 75% (n = 54) within 12 months of diagnosis, inferior vena cava filters were placed in 24% (n = 17) and 97% (n = 70) of cases were anticoagulated. Pulmonary endarterectomy was performed in 35% (n = 25), balloon pulmonary angioplasty in 6% (n = 4). One-, three- and five-year survival was 93%, 80% and 65% from the time of diagnosis, and this was significantly better in patients who underwent pulmonary endarterectomy (p = 0.01). This is the first study describing the characteristics of CTEPH in Ireland and highlights suboptimal disease recognition and referral for the assessment for pulmonary endarterectomy.
慢性血栓栓塞性肺动脉高压（CTEPH）是急性肺栓塞一种罕见且未得到充分认识的并发症。关于爱尔兰CTEPH特征的信息有限，这项回顾性队列研究的目的就是填补这一知识空白。2010年至2020年间，爱尔兰国家肺动脉高压科（NPHU）诊断出72例CTEPH病例。这占该科室所有转诊病例的6%，换算后估计年发病率为每百万人口1.39例（95%置信区间，0.33 - 2.46）。2020年爱尔兰确诊CTEPH的患病率估计为每百万人口12.05例（95% CI 9.00 - 15.10）。CTEPH诊断前症状的平均持续时间为23（±22）个月。CTEPH患者在诊断时更可能为男性（n = 40，56%）、年龄较大（60 ± 17岁）且具有CTEPH的可识别危险因素（n = 61，85%）。在治疗方面，75%（n = 54）的患者在诊断后12个月内接受了肺动脉高压（PH）血管扩张剂治疗，24%（n = 17）的患者植入了下腔静脉滤器，97%（n = 70）的病例接受了抗凝治疗。35%（n = 25）的患者接受了肺动脉内膜剥脱术，6%（n = 4）的患者接受了球囊肺动脉成形术。从诊断时起，1年、3年和5年生存率分别为93%、80%和65%，接受肺动脉内膜剥脱术的患者生存率显著更高（p = 0.01）。这是第一项描述爱尔兰CTEPH特征并强调疾病识别欠佳以及肺动脉内膜剥脱术评估转诊不足的研究。
