Goz Mustafa, Hazar Abdussemet, Mordeniz Cengiz, Kocarslan Aydemir, Demirkol Abbas Heval, Koc Ahmet
Department of Cardiovascular Surgery, Medical Faculty, Harran University, Sanliurfa, Turkey.
J Pak Med Assoc. 2010 Aug;60(8):690-2.
In severe haemophilia A, patients, start from the first years of life, with spontaneous bleeding and require transfusion. However, cardiac tamponade due to spontaneous pericardial bleeding is rare. An 11-year-old boy receiving haemophilia A treatment was referred to the Department of Paediatric Haematology with pneumonia, fever, dyspnoea, and palpitation. In his PA chest radiograph, pneumonic infiltration in the right lung and enlargement in the pericardial area were found. On his echocardiograph, pericardial effusion reaching 3.9 cm and other findings of tamponade were detected. APTT was outside the measurable range. It was deranged to > 120 seconds. The patient received 1000 U of factor VIII intravenously. A pericardial window was made via left anterior mini thoracotomy due to fluid drained. In his control echocardiograph taken after one month, no pathology was found. At 50th day, the patient showed left pleural serohaemorrhagic effusion, which was treated with tube thoracostomy. In haemophilia A patients, either pericardiocentesis or subxiphoid pericardial drainage or pericardial window creation via thoracotomy may be applied, depending on the primary pathology. In paediatric cases, pericardial window creation via mini thoracotomy can be an alternative treatment of choice considering complications such as recurring bleeding and effusion during pericardiocentesis.
在重度甲型血友病患者中，从生命的最初几年起就会出现自发性出血并需要输血。然而，自发性心包出血导致的心包填塞很少见。一名接受甲型血友病治疗的11岁男孩因肺炎、发热、呼吸困难和心悸被转诊至儿科血液科。在他的胸部后前位X线片上，发现右肺有肺炎浸润以及心包区域增大。在他的超声心动图检查中，检测到心包积液达3.9厘米以及其他心包填塞的表现。活化部分凝血活酶时间（APTT）超出可测量范围，紊乱至>120秒。患者静脉注射了1000单位的凝血因子VIII。由于有液体引出，通过左前小切口开胸术做了心包开窗引流。在一个月后进行的超声心动图复查中，未发现异常。在第50天时，患者出现左侧胸膜浆液血性积液，通过胸腔闭式引流术进行了治疗。对于甲型血友病患者，可根据主要病情采用心包穿刺术、剑突下心包引流术或开胸心包开窗术。在儿科病例中，考虑到心包穿刺术期间反复出血和积液等并发症，通过小切口开胸术创建心包开窗可能是一种可供选择的治疗方法。
