Khalid Sakib K, Pershbacher James, Makan Majesh, Barzilai Benico, Goodenberger Daniel
Department of Internal Medicine, Washington University School of Medicine, St. Louis, MO, USA.
Am J Med. 2009 Aug;122(8):779.e1-9. doi: 10.1016/j.amjmed.2009.01.037.
Hereditary hemorrhagic telangiectasia is characterized by arteriovenous malformations or telangiectasias in multiple organs. Nose bleeding is the most common manifestation of hereditary hemorrhagic telangiectasia and can be debilitating. Cardiac involvement in the form of high-output cardiac failure is a poorly studied complication of hereditary hemorrhagic telangiectasia. The objective of this study is to describe the natural history of high-output cardiac failure in hereditary hemorrhagic telangiectasia and define the relation between bleeding complications and high-output cardiac failure.
In this case-control study at the Washington University hereditary hemorrhagic telangiectasia center, all patients evaluated for hereditary hemorrhagic telangiectasia and found to have high cardiac output between 1999 and 2006 were enrolled as cases (n = 17) and compared with 17 hereditary hemorrhagic telangiectasia controls without high-output cardiac failure.
During lifetime, cases needed more transfusions, emergency department visits, and hospital admission for nose bleeding. Cases experienced significant worsening of nose bleeding immediately before heart failure diagnosis. During the 2 years before study-related evaluation, 16 (94%) cases needed transfusions every month as compared with 2 (12%) controls (P <.001). The number of transfusions required during these 2 years also was higher in cases (median 3, range 0-12) as compared with controls (median 0, range 0-1.5, P <.001).
Hereditary hemorrhagic telangiectasia patients with high-output cardiac failure have significantly greater nose bleeding as compared with patients without high-output cardiac failure. In addition, nose bleeding worsens around the time of development of high-output cardiac failure. Early recognition of the relationship between severe nose bleeding and high-output cardiac failure can lead to earlier diagnosis and provide an opportunity for earlier institution of therapy for high-output cardiac failure.
遗传性出血性毛细血管扩张症的特征是多个器官出现动静脉畸形或毛细血管扩张。鼻出血是遗传性出血性毛细血管扩张症最常见的表现，且可能使人衰弱。以高输出量心力衰竭形式出现的心脏受累是遗传性出血性毛细血管扩张症一种研究较少的并发症。本研究的目的是描述遗传性出血性毛细血管扩张症中高输出量心力衰竭的自然病程，并确定出血并发症与高输出量心力衰竭之间的关系。
在华盛顿大学遗传性出血性毛细血管扩张症中心进行的这项病例对照研究中，所有在1999年至2006年间接受遗传性出血性毛细血管扩张症评估且发现心输出量高的患者被纳入病例组（n = 17），并与17名无高输出量心力衰竭的遗传性出血性毛细血管扩张症对照组进行比较。
在一生中，病例组因鼻出血需要更多的输血、急诊就诊和住院治疗。病例组在心力衰竭诊断前鼻出血明显加重。在与研究相关评估前的2年中，16例（94%）病例每月需要输血，而对照组为2例（12%）（P <.001）。与对照组（中位数为0，范围为0 - 1.5，P <.001）相比，病例组在这2年中所需的输血量也更高（中位数为3，范围为0 - 12）。
与无高输出量心力衰竭的患者相比，患有高输出量心力衰竭的遗传性出血性毛细血管扩张症患者鼻出血明显更多。此外，在高输出量心力衰竭发生时鼻出血会加重。早期认识到严重鼻出血与高输出量心力衰竭之间的关系可导致更早的诊断，并为高输出量心力衰竭的更早治疗提供机会。
