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Portopulmonary hypertension (PPH)〔Adapted from: Tapper EB: http://wikidoc.org/index.php/Portopulmonary_hypertension〕 is defined by the coexistence of portal and pulmonary hypertension. PPH is a serious complication of liver disease, present in 0.25 to 4% of all patients suffering from cirrhosis. Once an absolute contraindication to liver transplantation, it is no longer, thanks to rapid advances in the treatment of this condition.〔Kuo PC et al. Portopulmonary Hypertension and the Liver Transplant Candidate. Transplantation 1999;67(8):1087-1093〕 Today, PPH is comorbid in 4-6% of those referred for a liver transplant.〔Torregrosa M et al. Role of Doppler echocardiography in the assessment of portopulmonary hypertension in liver transplant candidates. Transplantation 2001;71:572-574〕〔Tapper EB, Knowles D, Heffron T, Lawrence EC, Csete M. Portopulmonary hypertension: imatinib as a novel treatment and the Emory experience with this condition. Transplant Proc. 2009 Jun;41(5):1969-71.〕 ==Presentation== PPH presents roughly equally in male and female cirrhotics; 71% female in an American series and 57% male in a larger French series.〔Le Pavec et al. Portopulmonary Hypertension: Survival and Prognostic Factors. Am J Respir Crit Care Med Vol 178. pp 637–643, 2008〕〔Kawut SM et al. Clinical Risk Factors for Portopulmonary Hypertension. Hepatology 2008;48〕 Typically, patients present in their fifth decade, aged 49 +/- 11 years on average.〔〔Bejaminov et al. Portopulmonary hypertension in decompensated cirrhosis with refractory ascites. Gut 2003; 52:1355-1362〕 In general, PPH is diagnosed 4–7 years after the patient is diagnosed with portal hypertension〔Hadengue et al. PH complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991;100:520-528〕 and in roughly 65% of cases, the diagnosis is actually made at the time of invasive hemodynamic monitoring following anesthesia induction prior to liver transplantation.〔Hadengue et al. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991;100:520-528〕 Once patients are symptomatic, they present with right heart dysfunction secondary to pulmonary hypertension and its consequent dyspnea, fatigue, chest pain and syncope.〔Martinex-Palli et al. Liver Transplant in High Risk Patients. Transplant Proceedings 2005;37:3861-3864〕 Patients tend to have a poor cardiac status, with 60% having stage III-IV NYHA heart failure.〔 PPH is actually independent of the severity of cirrhosis but may be more common in specific types of cirrhosis, in one series more so in Autoimmune Hepatitis and less in Hepatitis C cirrhosis,〔 while in another it was equally distributed throughout the diagnoses.〔Tapper EB, Knowles D, Heffron T, Lawrence EC, Csete M. Portopulmonary hypertension: imatinib as a novel treatment and the Emory experience with this condition. Transplant Proc. 2009 Jun;41(5):1969-71.〕 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Portopulmonary hypertension」の詳細全文を読む スポンサード リンク
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