Cantu Jose, Wang Degang, Safdar Zeenat
Division of Pulmonary-Critical Care Medicine, Baylor College of Medicine, Houston, TX, USA.
Int J Clin Pract Suppl. 2012 Oct(177):5-12. doi: 10.1111/ijcp.12004.
Pulmonary arterial hypertension (PAH) is a disabling disease that may result in haemoptysis. Patients with congenital heart disease associated PAH (CHD-APAH) may have a survival advantage when compared with patients with other types of PAH presenting with haemoptysis. The effects of aetiology and subsequent management choice of haemoptysis in PAH patients is not well-defined.
We conducted outcome analysis in CHD-APAH vs. all other subtypes of PAH patients presenting with haemoptysis to The Methodist Hospital. Twenty-one patients were identified, 13 patients in the CHD-APAH group and eight patients in the non-CHD group. We evaluated outcomes related to treatment (bronchial artery embolisation (BAE) vs. conservative management), hospital length of stay, mortality rates and survival in this cohort.
The CHD-APAH and non-CHD groups had similar baseline demographic, haemodynamic and laboratory values except BMI was higher in the non-CHD group and haematocrit was higher in the CHD-APAH group. Twenty-eight-day mortality (0% vs. 31%) and 1-year mortality (0% vs. 54%) was lower in the CHD-APAH patients as compared with non-CHD group. A statistically significant difference was found in the survival rate in favour of CHD-APAH group for the total follow-up period (p = 0.02). Although not statistically significant, patients treated with BAE had shorter length of stay (4.0 days ± 4.0 vs. 13.7 days ± 22.5; p = 0.26). There was recurrent haemoptysis in 43% of patients treated with BAE.
Haemoptysis in PAH patients is a serious event with a high mortality rate. CHD-APAH seems to confer a survival advantage, independent of therapy utilised. Termination of haemoptysis with BAE is rapid with relatively few complications except for frequent re-bleeding episodes. Further studies are needed to determine the risk factors that may predispose PAH patients to excessive mortality from haemoptysis and to identify an optimal therapeutic modality.
肺动脉高压（PAH）是一种致残性疾病，可能导致咯血。与其他类型出现咯血的PAH患者相比，先天性心脏病相关性PAH（CHD-APAH）患者可能具有生存优势。PAH患者咯血的病因及后续治疗选择的影响尚不明确。
我们对在卫理公会医院出现咯血的CHD-APAH患者与所有其他PAH亚型患者进行了结局分析。共确定了21例患者，其中CHD-APAH组13例，非CHD组8例。我们评估了该队列中与治疗（支气管动脉栓塞术（BAE）与保守治疗）、住院时间、死亡率和生存率相关的结局。
CHD-APAH组和非CHD组的基线人口统计学、血流动力学和实验室值相似，但非CHD组的BMI较高，CHD-APAH组的血细胞比容较高。与非CHD组相比，CHD-APAH患者的28天死亡率（0%对31%）和1年死亡率（0%对54%）较低。在整个随访期内，CHD-APAH组的生存率有统计学显著差异（p = 0.02）。尽管无统计学意义，但接受BAE治疗的患者住院时间较短（4.0天±4.0对13.7天±22.5；p = 0.26）。接受BAE治疗的患者中有43%出现反复咯血。
PAH患者咯血是一个严重事件，死亡率高。CHD-APAH似乎具有生存优势，与所采用的治疗方法无关。BAE终止咯血迅速，除了频繁再出血发作外，并发症相对较少。需要进一步研究以确定可能使PAH患者因咯血导致过高死亡率的危险因素，并确定最佳治疗方式。
