目的 了解肝肺綜合征( HPS) Ⅱ型肺血管異常( 肺動(dòng)靜脈畸形) 合并血胸的臨床特征和處理原則。方法 對(duì)1 例HPS Ⅱ型肺血管異常合并反復(fù)血胸患者的臨床資料進(jìn)行分析, 并結(jié)合國內(nèi)外相關(guān)文獻(xiàn)進(jìn)行復(fù)習(xí)。結(jié)果 患者男性,72 歲, 以反復(fù)加重的喘憋和包裹性胸腔積液就診。先后4次胸穿, 總共抽取血樣胸液2510 mL, 胸液常規(guī)紅細(xì)胞計(jì)數(shù)在100 ×109 /L 以上, 細(xì)胞學(xué)檢查和腫瘤標(biāo)記物均陰性。后經(jīng)CTPA 和肺動(dòng)脈造影證實(shí)為HPS Ⅱ型肺血管異常合并血胸?；颊吡⑴P位PaO2 分別為58. 3 mmHg 和66. 3 mmHg。對(duì)血管畸形行栓塞治療后, 低氧血癥改善, 血胸得到控制。總結(jié)文獻(xiàn), HPS Ⅱ型肺血管異常合并血胸既往未見報(bào)道。HPS 最突出的臨床表現(xiàn)是呼吸困難和發(fā)紺。直立位低氧血癥或臥位呼吸是HPS 較為特異的表現(xiàn)。P( A-a) O2 是篩查HPS 低氧血癥的最敏感指標(biāo)。HPS胸部CT 特點(diǎn)為被強(qiáng)化的團(tuán)片狀密度增高影或多發(fā)小結(jié)節(jié)影, 以雙下肺野多見。肺動(dòng)脈造影可對(duì)肺
血管異常予以分型。HPS肺血管病變Ⅱ型患者對(duì)吸氧反應(yīng)不佳, 血管畸形栓塞治療有助于緩解癥狀,改善低氧血癥。結(jié)論 HPS Ⅱ型肺血管異常破裂是血胸的少見病因。肺血管畸形栓塞治療可有效改善低氧血癥, 控制血胸。對(duì)于具有肝臟病史者, 出現(xiàn)肺血管擴(kuò)張和低氧血癥應(yīng)警惕HPS 的存在。
引用本文： 金建敏,孫永昌,陳東寧,劉曉芳,梁熙虹. 肝肺綜合征Ⅱ 型肺血管異常合并血胸病例報(bào)道并文獻(xiàn)復(fù)習(xí). 中國呼吸與危重監(jiān)護(hù)雜志, 2010, 9(1): 61-65. doi: 復(fù)制
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| 13. | Stratakos G,Malagari K,Broutzos E,et al.Dyspnoea and cyanosis in a cirrhotic patient.Eur Respir J,2002,19:780-783. |
| 14. | Manson D,Traubici J,Mei-Zahav M,et al.Pulmonary nodular opacities in children with hereditary hemorrhagic telangiectasia.Pediatr Radiol,2007,37:264-268. |
| 15. | Hirai N,Toyota N,Kakizawa H,et al.Endovascular therapy of pulmonary arteriovenous malformations: Utility of 16-detector-row computed tomography pulmonary angiography as a pre-embolization survey.J Comput Assist Tomogr,2006,30:238-243. |
- 1. Alizadeh AH,Fatemi SR,Mirzaee V,et al.Clinical features of hepatopulmonary syndrome in cirrhotic patients.World J Gastroenterol,2006,12:1954-1956.
- 2. Yilmaz S,Dursum M,Canoruç F,et al.A severe (type II) hepatopulmonary syndrome in a patient with idiopathic portal hypertension and treatment with paroxetine.Neth J Med,2005,63:448-452..
- 3. 薛雁山,王峻,陳麥林，等.肝肺綜合征的CT表現(xiàn)及臨床意義.中國醫(yī)學(xué)影像學(xué)雜志，2004，12:246-248.
- 4. 平紅霞,姚保珍,王瑞英.肝肺綜合征一例誤診分析.臨床誤診誤治,2003,16:419．.
- 5. Groves AM,See TC,Appleton DS,Win T,et al.Transpleural ultrasound diagnosis of a pulmonary arteriovenous malformation.Br J Radiol,2004,77:620-622.
- 6. Thomas R,Christopher DJ,Chacko J,et al.Pulmonary arteriovenous malformation in a patient with tuberculosis--an association? Eur J Cardiothorac Surg,2006,30:405-407.
- 7. Ho V.Current concepts in the management of hepatopulmonary syndrome.Vasc Health Risk Manag,2008,4:1035-1041.
- 8. Ference BA,Shannon TM,White RI Jr,et al.Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia.Chest,1994,106:1387-1390.
- 9. Khandaker MH,Knoll BM,Arora AS.63-year-old man with cryptogenic cirrhosis and dyspnea.Mayo Clin Proc,2008,83:580-583.
- 10. Rodríguez-Roisin R,Krowka MJ.Hepatopulmonary syndrome--a liver-induced lung vascular disorder.N Engl J Med,2008,358:2378-2387.
- 11. Leung AN.Case 63:hepatopulmonary syndrome.Radiology,2003,229:64-67.
- 12. McAdams HP,Erasmus J,Crockett R,et al.The hepatopulmonary syndrome: radiologic findings in 10 patients.AJR Am J Roentgenol,1996,166:1379-1385.
- 13. Stratakos G,Malagari K,Broutzos E,et al.Dyspnoea and cyanosis in a cirrhotic patient.Eur Respir J,2002,19:780-783.
- 14. Manson D,Traubici J,Mei-Zahav M,et al.Pulmonary nodular opacities in children with hereditary hemorrhagic telangiectasia.Pediatr Radiol,2007,37:264-268.
- 15. Hirai N,Toyota N,Kakizawa H,et al.Endovascular therapy of pulmonary arteriovenous malformations: Utility of 16-detector-row computed tomography pulmonary angiography as a pre-embolization survey.J Comput Assist Tomogr,2006,30:238-243.