Department of Pediatric Cardiology, Center for Congenital Heart Diseases, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands; National Referral Center for Children with Pulmonary Hypertension, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Am J Cardiol. 2013 Nov 1;112(9):1505-9. doi: 10.1016/j.amjcard.2013.06.034. Epub 2013 Aug 22.
Data concerning the prevalence, risk factors, and prognostic significance of hemoptysis in pediatric pulmonary arterial hypertension (PAH) are scarce. A Dutch national cohort of 74 children with either idiopathic or heritable PAH (IPAH/HPAH, n = 43) or PAH associated with congenital heart disease (PAH-CHD, n = 31) were followed from 1993 to 2012. During a median follow-up of 3.5 years (range 0.1 to 19.2), hemoptysis occurred in 13 children (17.6%). The hemoptysis event rate was 9.9 per 100 patient-years, equally divided between IPAH/HPAH and PAH-CHD (p = 0.824). The median age at first hemoptysis was 12.5 years, and the median time since PAH diagnosis to first hemoptysis was 6.1 years. Patients with hemoptysis had longer time since PAH diagnosis (p = 0.001) and more frequently used anticoagulant therapy (p = 0.006). Univariate Cox regression analysis indicated that older age (hazard ratio [HR] 1.15, 95% confidence interval [CI] 1.01 to 1.30, p = 0.031), World Health Organization functional class IV (HR 0.28, 95% CI 0.08 to 0.95, p = 0.042), higher mean pulmonary arterial pressure (HR 1.04, 95% CI 1.00 to 1.07, p = 0.028), and higher indexed pulmonary vascular resistance (HR 1.08, 95% CI 1.02 to 1.15, p = 0.009), all at the time of PAH diagnosis, were associated with increased risk of hemoptysis during follow-up. Ten of 13 patients with hemoptysis died or underwent (heart-) lung transplantation; in 6 patients, this was directly related to hemoptysis. In conclusion, the occurrence of hemoptysis in pediatric IPAH/HPAH and PAH-CHD increases with time since diagnosis, is a serious condition, and is, in case of life-threatening hemoptysis, associated with poor outcome.
有关儿童肺动脉高压（PAH）中咯血的患病率、风险因素和预后意义的数据很少。对 1993 年至 2012 年间的 74 名儿童进行了一项荷兰全国队列研究，这些儿童患有特发性或遗传性 PAH（IPAH/HPAH，n=43）或与先天性心脏病相关的 PAH（PAH-CHD，n=31）。在中位数为 3.5 年（范围为 0.1 至 19.2 年）的随访期间，有 13 名儿童（17.6%）发生了咯血。咯血事件发生率为每 100 患者年 9.9 例，在 IPAH/HPAH 和 PAH-CHD 中相等（p=0.824）。首次咯血的中位年龄为 12.5 岁，从 PAH 诊断到首次咯血的中位时间为 6.1 年。有咯血的患者从 PAH 诊断到首次咯血的时间更长（p=0.001），更常使用抗凝治疗（p=0.006）。单因素 Cox 回归分析表明，年龄较大（风险比[HR]1.15，95%置信区间[CI]1.01 至 1.30，p=0.031）、世界卫生组织功能分类 IV（HR 0.28，95%CI 0.08 至 0.95，p=0.042）、平均肺动脉压较高（HR 1.04，95%CI 1.00 至 1.07，p=0.028）和较高的指数化肺血管阻力（HR 1.08，95%CI 1.02 至 1.15，p=0.009），均与随访期间咯血风险增加相关。13 例咯血患者中有 10 例死亡或接受（心肺）移植；在 6 例患者中，这直接与咯血有关。总之，儿童特发性/遗传性 IPAH/HPAH 和 PAH-CHD 中咯血的发生随着诊断后时间的推移而增加，是一种严重的疾病，如果发生危及生命的咯血，则预后不良。
