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5. 1, 2 and 3
A high resolution CT scan (HRCT) is important to further access the fibrotic changes noted on chest x-ray. It can be very specific in identifying certain radiological patterns of disease such as usual interstitial pneumonia (UIP) which is highly suggestive of idiopathic pulmonary fibrosis (IPF).
A right heart catheterization is also reasonable to obtain in evaluating a patient with severe pulmonary hypertension seen on echocardiogram.
Obtaining a macro-aggregated albumin nuclear scan is warranted to evaluate for hepato-pulmonary syndrome in this patient with decompensated cirrhosis and symptoms consistent with platypnea.
Results
The repeat chest CT scan demonstrates regions of inter and intralobular septal thickening, peribronchial thickening with mild ground-glass and centrilobular reticulations. There is increased atelectasis in the lung bases. An area of air trapping is identified in the lingula. No significant pleural effusion is present. No significant areas of honey combing or bronchiectasis.
A right heart catheterization demonstrated normal hemodynamics with the following findings:
The macroaggregated albumin nuclear study obtained to better characterize the possible presence of hepatopulmonary syndrome demonstrated no evidence of right to left shunt.
His supplemental oxygen requirement continued to increase and on hospital day 8, he was transferred to the ICU in respiratory distress saturating 80% on 6L face mask.
What is the best choice as the next diagnostic step in accurately diagnosing his ILD? (click on correct answer to move to next panel)