Robalino B D, Moodie D S
Department of Cardiology, Cleveland Clinic Foundation, Ohio 44195.
J Am Coll Cardiol. 1991 Feb;17(2):492-8. doi: 10.1016/s0735-1097(10)80121-4.
To determine the clinical, laboratory and hemodynamic profile in patients with primary pulmonary hypertension and associated portal hypertension, 7 new cases and 71 previously reported cases were analyzed. There was no gender predilection and the average age at diagnosis was 41 years. Liver cirrhosis was the most frequent cause of hypertension (82%) and a surgical portosystemic shunt was present in 29%. Almost invariably, portal hypertension either preceded or was diagnosed concurrently with pulmonary hypertension, favoring the hypothesis that in portal hypertension, the pulmonary vasculature may be exposed to vasoactive substances normally metabolized or produced by the diseased liver, possibly inducing vasoconstriction or direct toxic damage to the pulmonary arteries. Clinically, exertional dyspnea was the most frequent presenting symptom (81%); other symptoms, such as syncope, chest pain and fatigue, were present in less than 33%. An accentuated pulmonary component of the second heart sound (82%) and a systolic murmur (69%) were the most common physical findings. At least 75% of these patients had evidence of pulmonary hypertension on electrocardiography (right ventricular hypertrophy) or roentgenography (cardiomegaly or dilated main pulmonary arteries, or both). Hemodynamic findings included severe pulmonary hypertension (mean pulmonary artery pressure 59 +/- 19 mm Hg) with normal pulmonary capillary wedge pressure and cardiac output. Treatment was basically palliative and the mean and median survival times were 15 and 6 months, respectively. In brief, on the basis of clinical presentation and laboratory features, patients with combined primary pulmonary hypertension and portal hypertension seldom represent a diagnostic challenge. Further research is needed on treatment, which remains palliative. The survival rate is poor and worse than that seen in isolated primary pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)
为确定原发性肺动脉高压合并门静脉高压患者的临床、实验室及血流动力学特征，分析了7例新病例及71例既往报道病例。无性别倾向，诊断时平均年龄为41岁。肝硬化是高血压最常见的病因（82%），29%的患者存在外科门体分流。几乎无一例外，门静脉高压要么先于肺动脉高压出现，要么与肺动脉高压同时被诊断，这支持了以下假说：在门静脉高压中，肺血管系统可能暴露于通常由病变肝脏代谢或产生的血管活性物质中，可能导致血管收缩或对肺动脉的直接毒性损害。临床上，劳力性呼吸困难是最常见的症状（81%）；其他症状，如晕厥、胸痛和疲劳，发生率不到33%。第二心音肺动脉成分亢进（82%）和收缩期杂音（69%）是最常见的体格检查发现。这些患者中至少75%在心电图（右心室肥厚）或X线检查（心脏扩大或主肺动脉扩张，或两者皆有）中有肺动脉高压的证据。血流动力学结果包括严重肺动脉高压（平均肺动脉压59±19 mmHg），肺毛细血管楔压和心输出量正常。治疗基本为姑息性，平均生存时间和中位生存时间分别为15个月和6个月。简而言之，基于临床表现和实验室特征，原发性肺动脉高压合并门静脉高压患者很少构成诊断挑战。治疗方面仍需进一步研究，目前仍为姑息性治疗。生存率较低，且比孤立性原发性肺动脉高压更差。（摘要截短于250字）
