I have been involved in the care of a few patients with COPD/emphyesema with profound hypoxia out of proportion to their FEV1 (> 60% predicted), HRCT chest which do not demonstrate severe emphysema, and Type 1 Respiratory Failure. Apart from a very high alveolar/arterial oxygen gradient, they don't seem to have much else going on, after the other culprits like shunting, CTEPH etc have been ruled out. They also tend to have a disparity between their Echocardiogram and right heart cath RVSP values. They are usually classified as Group 3 Pulm HTN.

2 questions here:

1. Any links to articles or publications looking at explant diagnoses for a series of patients in this category who were fortunate enough to have lung transplants ? References please

2.If we are able to obtain fully-consented post-mortem lung biopsy samples in the few unfortunate patients, is there a centre in the UK can process them to look at the pathology of the lung in these patients, in case the answers may be useful to others.

Thanks in advance.

- Lawrence