Portopulmonary hypertension history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Overview

History and Symptoms

Once patients are symptomatic, they present with right heart dysfunction secondary to pulmonary hypertension and its consequent dyspnea, fatigue, chest pain and syncope.[1] 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,[2] while in another it was equally distributed throughout the diagnoses.[3].

References

  1. Martinex-Palli et al. Liver Transplant in High Risk Patients. Transplant Proceedings 2005;37:3861-3864
  2. Kawut SM et al. Clinical Risk Factors for Portopulmonary Hypertension. Hepatology 2008;48
  3. 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.

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