1 Department of Radiology.
2 Department of Neurosurgery.
Ann Am Thorac Soc. 2016 Sep;13(9):1505-11. doi: 10.1513/AnnalsATS.201603-200OC.
There is sparse published literature on the causes and outcomes of hospitalization of patients with hereditary hemorrhagic telangiectasia (HHT).
To evaluate rates of various complications, comorbidities, and in-hospital outcomes of patients with HHT using a large, multihospital inpatient database.
We identified patients with HHT in the U.S. Nationwide Inpatient Sample between 2000 and 2012. Rates of hemorrhagic, neurological, hepatic, and cardiopulmonary complications among hospitalized patients with HHT were evaluated. We also studied procedure use rates for blood transfusion, endoscopy, and epistaxis treatment. Hospitalization outcomes, including in-hospital mortality, discharge status, charges, and length of stay, were evaluated.
We identified 10,293 patients with HHT. The mean age of the HHT population was 60.7 years. Sixty percent of patients were female. More than 75% of HHT hospitalizations occurred in those older than 50 years of age. Patients with HHT had high rates of bleeding-related complications, including anemia (53.3%), epistaxis (16.2%), and gastrointestinal bleeding (10.8%). Overall, bleeding complications accounted for 62.7% of HHT-related complications. Thirty-eight percent of hospitalized patients with HHT received one or more transfusions of a blood product. Cardiopulmonary complications were present in 41.0% of the cases. Congestive heart failure was the second most common individual complication among patients with HHT, affecting 19.9% of patients. The in-hospital mortality rate was 1.9%.
In this large, nationwide study, we found that nearly two-thirds of patients hospitalized with HHT experienced a bleeding-related complication. Nearly 40% of hospitalized patients with HHT required transfusion of blood products. Cardiopulmonary complications, including congestive heart failure, were the second most common complication. The high burden of bleeding-related complications points to a significant unmet clinical need for these patients.
遗传性出血性毛细血管扩张症（HHT）患者住院的原因和结局鲜有文献报道。
利用大型多医院住院患者数据库评估 HHT 患者各种并发症、合并症和住院结局的发生率。
我们在 2000 年至 2012 年期间在美国全国住院患者样本中确定了 HHT 患者。评估了住院 HHT 患者发生出血性、神经、肝和心肺并发症的发生率。我们还研究了输血、内镜和鼻出血治疗的程序使用率。评估了住院结局，包括院内死亡率、出院状态、费用和住院时间。
我们确定了 10293 名 HHT 患者。HHT 人群的平均年龄为 60.7 岁。60%的患者为女性。超过 75%的 HHT 住院发生在年龄大于 50 岁的患者中。HHT 患者有很高的出血相关并发症发生率，包括贫血（53.3%）、鼻出血（16.2%）和胃肠道出血（10.8%）。总体而言，出血并发症占 HHT 相关并发症的 62.7%。38%的住院 HHT 患者接受了一种或多种血液制品的输血。41.0%的病例存在心肺并发症。充血性心力衰竭是 HHT 患者的第二大常见并发症，影响 19.9%的患者。院内死亡率为 1.9%。
在这项大型全国性研究中，我们发现近三分之二的因 HHT 住院的患者发生了与出血相关的并发症。近 40%的住院 HHT 患者需要输血。心肺并发症，包括充血性心力衰竭，是第二大常见并发症。与出血相关的并发症负担高表明这些患者存在显著的未满足的临床需求。
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