A 57-year-old man with known liver cirrhosis comes to the physician because of progressive shortness of breath and fatigue for the last few months that has recently worsened. He now becomes short of breath even at rest, particularly in the morning when getting out of bed. He was diagnosed with chronic hepatitis C ten years ago and liver cirrhosis three years ago. He quit using alcohol and IV drugs upon being diagnosed with hepatitis C. He is currently disabled and usually comes to the emergency department for abdominal paracentesis once every 6 to 8 weeks. He has been non-compliant with his outpatient medications. He had a large volume paracentesis 3 days ago.
His temperature is 36.7 C (98 F), blood pressure is 100/70 mm Hg, pulse is 94/min, and respirations are 16/min. The patient's pulse oximetry shows 88% saturation on room air while sitting. After resting supine in a quiet room for 15 min, his blood pressure is 95/68 mm Hg, pulse is 88/min, and oxygen saturation is 93% on room air. He is fully alert and has no asterixis. Breath sounds are mildly decreased at the right base. A moderate amount of ascites is present on abdominal examination. There is no rebound tenderness or rigidity. He has 1+ peripheral edema, a few spider nevi on his chest, and a slight bluish discoloration of his toes and digits.
His platelet count is 50,000 cells/µL and serum creatinine is 1.4 mg/dL. Chest x-ray shows normal heart size and a small right-sided pleural effusion, but is otherwise unremarkable.
Which of the following is most likely to diagnose the cause of this patient's shortness of breath?

Question

A 57-year-old man with known liver cirrhosis comes to the physician because of progressive shortness of breath and fatigue for the last few months that has recently worsened.  He now becomes short of breath even at rest, particularly in the morning when getting out of bed.  He was diagnosed with chronic hepatitis C ten years ago and liver cirrhosis three years ago.  He quit using alcohol and IV drugs upon being diagnosed with hepatitis C.  He is currently disabled and usually comes to the emergency department for abdominal paracentesis once every 6 to 8 weeks.  He has been non-compliant with his outpatient medications.  He had a large volume paracentesis 3 days ago.
His temperature is 36.7 C (98 F), blood pressure is 100/70 mm Hg, pulse is 94/min, and respirations are 16/min.  The patient's pulse oximetry shows 88% saturation on room air while sitting.  After resting supine in a quiet room for 15 min, his blood pressure is 95/68 mm Hg, pulse is 88/min, and oxygen saturation is 93% on room air.  He is fully alert and has no asterixis.  Breath sounds are mildly decreased at the right base.  A moderate amount of ascites is present on abdominal examination.  There is no rebound tenderness or rigidity.  He has 1+ peripheral edema, a few spider nevi on his chest, and a slight bluish discoloration of his toes and digits.
His platelet count is 50,000 cells/µL and serum creatinine is 1.4 mg/dL.  Chest x-ray shows normal heart size and a small right-sided pleural effusion, but is otherwise unremarkable.
Which of the following is most likely to diagnose the cause of this patient's shortness of breath?

Quizplus · Accepted Answer

11ec193a_9607_9692_853d_417a636004bb_MD0002_00 Dyspnea in the setting of cirrhosis is non-specific and can be due to hepatic hydrothorax, ascites, anemia, and muscle wasting. However, the presence of orthodeoxia and platypnea are quite specific for hepatopulmonary syndrome (HPS). Hepatopulmonary syndrome is seen in 5%-40% of chronic liver disease patients. It is associated with portal hypertension, and usually presents years after the onset of chronic liver disease of any etiology. Patients typically present with the classic triad of liver disease, hypoxemia, and intrapulmonary vascular dilatations (IPVDs). IPVDs cause right-to-left shunting, which results in cyanosis and hypoxemia. The IPVDs can be demonstrated by contrast enhanced echocardiography (best method), nuclear scanning (more expensive, less sensitive), or pulmonary angiography (invasive, seldom preferred). Contrast echocardiography (eg, with agitated saline; "bubble study") confirms intrapulmonary right-to-left shunt if the microbubbles passing through the dilated pulmonary vessels cause left heart opacification 3-6 heart beats after right heart opacification. There is no effective medical therapy for HPS, and liver transplant is recommended for patients with severe hypoxemia. (Choice A) AFP and liver ultrasound can evaluate for hepatocellular carcinoma (HCC), which can result in rapidly reaccumulating ascitic fluid. However, patients with massive ascites have more shortness of breath in the supine position. (Choice C) Chest CT may show evidence of IPVDs (e.g., dilated pulmonary vessels, increased pulmonary artery to bronchus ratios), but it is not as sensitive or specific as contrast enhanced echocardiography. (Choice D) Right heart catheterization would confirm portopulmonary hypertension, another important cause of dyspnea in these patients. Portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodelling, and eventually leads to right-heart failure. However, portopulmonary hypertension is not associated with platypnea and orthodeoxia. (Choice E) Ventilation-perfusion (V/Q) scan would likely detect the ventilation-perfusion mismatch seen in HPS, but it is fairly nonspecific. Also, this patient's positional hypoxia is not consistent with pulmonary embolism. Educational objective: Hepatopulmonary syndrome (HPS) is a complication of chronic liver disease that is associated with hypoxemia and intrapulmonary vascular dilatations (IPVDs). Patients typically present with symptoms/signs of liver disease, dyspnea, platypnea, and orthodeoxia. Contrast echocardiography is preferred to diagnose IPVDs and confirm likely HPS. __________ References: Portopulmonary hypertension and hepatopulmonary syndrome (http://www.ncbi.nlm.nih.gov/pubmed/15121411)

A 57-Year-Old Man with Known Liver Cirrhosis Comes to the Physician