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目的提高对门脉高压相关性肺动脉高压(POPH)的临床认识,减少漏诊、误诊。方法回顾性分析1999年1月至2009年1月北京协和医院近10年来住院诊治的7例POPH患者的临床资料。结果7例POPH患者中男2例,女5例。确诊时中位年龄51岁。门脉高压的基础病包括:乙肝后肝硬化(3例)、自身免疫性肝炎(2例)、免疫缺陷病(1例)和门静脉血栓形成(1例)。肺动脉高压(PH)症状5例患者,以活动后呼吸困难起病,2例无症状。全部患者经胸超声心动图(TTE)证实肺动脉收缩压均>55mmHg,其中1例还行右心导管术(RHC)检查发现平均肺动脉压为62mmHg、肺血管阻力8.64mN/(s.cm5)。6例患者存在免疫异常。肝脏储备功能和PH严重度并不相关。随访的3例死亡患者中,2例为心源性(右心力衰竭和猝死)。结论PH是门脉高压少见但严重的并发症,POPH的根本病因是门脉高压,而不是潜在肝病。TTE是重要的筛查手段,RHC获得血流动力学资料是诊断的金标准。临床医师需要提高对POPH的认识,早期治疗,改善预后。
Objective To improve the clinical understanding of portal hypertension-related pulmonary hypertension (POPH) and reduce the missed diagnosis and misdiagnosis. Methods The clinical data of 7 POPH patients admitted to Peking Union Medical College Hospital from January 1999 to January 2009 in the past 10 years were analyzed retrospectively. Results There were 2 males and 5 females in 7 POPH patients. The median age at diagnosis was 51 years. The underlying diseases of portal hypertension include cirrhosis of the liver (3 cases), autoimmune hepatitis (2 cases), immunodeficiency disease (1 case) and portal vein thrombosis (1 case). Pulmonary hypertension (PH) symptoms in 5 patients, onset of dyspnea after activity, 2 asymptomatic. All patients underwent transthoracic echocardiography (TTE) confirmed pulmonary artery systolic pressure were> 55mmHg, of which 1 case also underwent right heart catheterization (RHC) examination found that the average pulmonary artery pressure was 62mmHg, pulmonary vascular resistance 8.64mN / (s.cm5). Six patients had an immune disorder. Liver reserve function is not related to PH severity. Of the 3 deaths followed up, 2 were cardiogenic (right heart failure and sudden death). Conclusion PH is a rare but serious complication of portal hypertension. The underlying cause of POPH is portal hypertension rather than underlying liver disease. TTE is an important screening tool, RHC hemodynamic data obtained is the gold standard for diagnosis. Clinicians need to improve awareness of POPH, early treatment, and improve prognosis.