Case Presentation: This patient is a 53-year-old woman with primary biliary cholangitis (PBC) complicated by decompensated cirrhosis, recent DVT, and multiple strokes secondary to paradoxical emboli who presented to an outside hospital with right-sided weakness and aphasia. This was the patient’s third presentation for stroke in the last month (most recently discharged 3 days prior on enoxaparin) and was being transferred to our center for a more thorough diagnostic workup.At the onset of her symptoms, the patient reports she was working with physical therapy doing a writing exercise when suddenly she felt her hand was no longer able to write the words. Her right arm and right leg began to weaken, and her right face began to droop. This event was witnessed by the physical therapists, who called EMS. Upon admission, she was stable but with labs notable for platelets of 45. Her neuro exam showed a slight right mouth droop, muscle strength 2/5 in the right bicep, tricep, deltoid, and right hand, and dysarthria. MRI brain revealed multi-focal left MCA infarcts, and CTA was negative at the time. During her first night, however, she developed right nasolabial flattening, right upper extremity drift, and worsening dysarthria. Repeat CTA revealed new distal left M1 occlusion. She was admitted to the ICU for closer neuro checks. Throughout her hospital course, she was treated carefully with enoxaparin given her chronically low platelets. She had no arrhythmias on exam, and her lipid panel was notable for HDL of 10. After an echocardiogram showing right to left shunting without evidence of a patent foramen ovale or ASD, she was thought to be having recurrent strokes due to hepatopulmonary syndrome (HPS). After discussion with our transplant team, her case was closed given concern for precipitation of stroke with hypotension that would make transplant too high risk. She was transferred to another institution for a second opinion regarding liver transplant.
Discussion: This patient has decompensated cirrhosis secondary to PBC. One of the possible sequelae of decompensated cirrhosis over time is HPS, which may have developed over the last 13 years since her original diagnosis of PBC. The pathophysiology of HPS is not very well understood. It is believed that nitric oxide and other vasodilatory compounds are not metabolized adequately by the liver. Thus, they persist in the bloodstream and cause vasodilation in pulmonary vasculature. Over time, this causes arteriovenous malformations (AVMs) in the lungs leading to right to left shunting. This enlarged pulmonary vasculature may lose its capability to catch emboli, leading to a potential passageway for emboli to progress to the brain. Other features of HPS include platypnea, which is difficulty breathing upon sitting up, with improvement of hypoxia upon lying flat.With recurrent embolic strokes, the patient’s heightened coagulable state from decompensated cirrhosis, and negative workup for other stroke etiologies, the presence of right-to-left shunting on echocardiogram without evidence for a patent foramen ovale or ASD makes HPS the most likely cause. At the moment, the only treatment for HPS is to stop progression of disease. For this patient, this includes ursodiol for treatment of PBC, cautious anticoagulation, supportive care, and hopefully a liver transplant soon.
Conclusions: – HPS is a possible sequela of decompensated cirrhosis.- In a patient with cirrhosis, platypnea is pathognomonic for HPS- HPS may cause recurrent embolic phenomena via AVMs in the lungs.