[An unexpected cause of pulmonary hypertension]

Dtsch Med Wochenschr. 2016 Jul;141(15):1102. doi: 10.1055/s-0041-109874. Epub 2016 Jul 27.
[Article in German]

Abstract

History and admission findings: We report on a 48-year-old man presenting with progressive hepatopathy and encephalopathy for two weeks based on a chronic hepatitis C. He takes ledipasvir and sofosbuvir (Harvoni) and ribavirin for almost 24 weeks. After admission to hospital his state deteriorated rapidly. He is directly transferred to the medical intensive care unit, where he died on day 3.

Investigations: During the physical examination, a pronounced jaundice and significant peripheral edema were found. Laboratory tests showed anemia, an increased C-reactive proteine and bilirubin, a limited coagulation and renal insufficiency with elevated creatinine. Quantitative HCV-PCR was negative. Echocardiographically a severe tricuspid- and mitral-valve regurgitation was found in a massively increased pulmonary artery pressure and pulmonary heart disease. The gastroscopy revealed a Forrest IIb situation with corresponding clip supply.

Diagnosis, treatment and course: In the autopsy we find signs of portal hypertension in presence of progressive liver cirrhosis. In addition, portopulmonary hypertension is diagnosed. The patient died on right ventricular failure resulting from a massively increased pulmonary pressure Conclusion: Advanced liver disease and an increased pulmonary pressure are often associated. Therefore, an early as possible diagnosis and classification are essential for adequate therapy in these patients.

Publication types

  • Case Reports

MeSH terms

  • Blood Pressure Determination
  • Diagnosis, Differential
  • Fatal Outcome
  • Hepatic Encephalopathy / complications*
  • Hepatic Encephalopathy / diagnosis*
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / etiology*
  • Male
  • Metabolism, Inborn Errors / complications*
  • Metabolism, Inborn Errors / diagnosis*
  • Middle Aged

Supplementary concepts

  • Combined Oxidative Phosphorylation Deficiency 1