A 60 year old gentleman presents with shortness of breath. Interestingly, his dyspnea is worse while upright and relieved while lying down. He has been out of care for a long time and does not know his past medical history. He takes no medications, has no known allergies, and does not know his family history. His social history is significant for being homeless, having a 40 year heavy drinking history, smoking 50 pack-years, and trying pretty much "any recreational drug invented."
Physical exam shows an O2 sat of 85%. This worsens while he is sitting and improves while he is lying down. You also notice these findings on his skin:
An arterial blood gas on room air shows a PaO2 of 60mmHg. Your attending, after evaluating the patient, says, "I bet at rest, he has an elevated cardiac output, perhaps even up to 7L/min.
Challenge: What is this syndrome called?
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An arterial blood gas on room air shows a PaO2 of 60mmHg. Your attending, after evaluating the patient, says, "I bet at rest, he has an elevated cardiac output, perhaps even up to 7L/min.
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2 comments:
Two Organ Systems
This is hepatopulmonary syndrome with a triad of liver disease, increased alveolar-arterial gradient, and evidence for intrapulmonary vascular dilatations. Hepatic findings include a hyperdynamic circulation with increased cardiac output, decreased systemic and pulmonary vascular resistance, and narrowed arterial-venous O2 content difference. The image shows spider nevi which may be a marker of intrapulmonary vascular dilatations. Platypnea is dyspnea while upright relieved by recumbency; orthodeoxia is desaturation while upright which improves with recumbency. The pulmonary vascular dilatations lead to a right-left shunt, worse while upright. Diagnosis can be made with echocardiography, nuclear scanning, or pulmonary angiography. Treatment is limited; liver transplant may be beneficial.
Sources: UpToDate; Wikipedia.
Nice case Doc! I didnt know much abt Hepatopulmonary syndrome..
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