AI Article Synopsis

  • A 55-year-old woman with hereditary hemorrhagic telangiectasia (HHT) had been experiencing worsening respiratory issues and weight gain due to volume overload, leading to her admission to the pulmonary hypertension unit.
  • Prior hospital visits revealed mild pulmonary hypertension and diuretic treatment improved her symptoms temporarily, but they reoccurred quickly after discharge despite her adherence to medications.
  • The patient also faced concerns over elevated liver enzymes indicative of potential cirrhosis, with an ongoing evaluation to determine the cause as she had no significant alcohol history or known liver disease.

Article Abstract

A 55-year-old woman with a medical history of hereditary hemorrhagic telangiectasia (HHT) complicated by recurrent nosebleeds, severe blood loss anemia, hepatic arterial-venous malformation (AVM), pulmonary hypertension, and severe tricuspid regurgitation presented to the HHT specialty clinic with acute hypoxic respiratory failure (new 3-L O requirement), weight gain, and volume overload. She was directly admitted to the pulmonary hypertension unit of our hospital. She had two recent admissions for similar symptoms thought to be due to worsening pulmonary arterial hypertension. In prior admissions, she had undergone right heart catheterization demonstrating mild pulmonary hypertension (pulmonary arterial pressure, 29 mm Hg, cardiac output by Fick 5.76, and cardiac index 3.22, mildly elevated pulmonary vascular resistance to 5.5 woods units). She would undergo diuresis with symptomatic improvement; however, after discharge she would rapidly develop recurrent heart failure symptoms. She reported compliance with guideline-directed medications, diuretics, and dietary restrictions and was still suffering severe symptoms. Notably she had previously elevated liver enzymes concerning for cirrhosis and had begun a workup to evaluate for causes of cirrhosis; she had a history of mild alcohol use, negative hepatitis viral serology, and no known history of liver disease.

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http://dx.doi.org/10.1016/j.chest.2021.07.2180DOI Listing

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