Redneck pulmonologist submits this question:
Would you do anything for secondary pulmonary hypertension (estimated PAP of 80-90 mmHG on echo) due to COPD before undergoing renal transplant? Her COPD is 2 to AAT deficiency and stable on AAT replacement. FEV1 of 40% predicted. She has documented nocturnal desat on sleep study without significant sleep apnea. But she is non-compliant with home O2. Daytime sat on RA is OK. She is not a smoker. She is on dialysis and her ESRD is due to pauci-immune vasculitis and has been treated with steroids and Cellcept. Work up for other causes of secondary pulmonary HTN is negative.
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Is the DLCO equally low? In addition to the chronic hypoxemia, is she a CO2 retainer?
ECHOs like to overestimate RVSPs but this is still pretty high. I might consider a R heart cath too.
I think trying to get a VO2Max (either measured or a MET-equivalent with exercise) might further improve my comfort level with surgery.
I thought this was a really good question. I f I could expand on it:
We know people with COPD have higher PA pressures (hence cor pulmonale). But how high is too high that you might add a specific Tx? With renewed interest on PDE inhibitors in COPD, are you treating more secondary PHTN?
Thank you for the comments. DLCO is 45% pred. No CO2 retention.
I am also considering rt heart cath or repeating echo.
If PAP is really that high, I am considering nitric oxide to get her through surgery based on some case reports after consulting anesthesia.
First, I think that as more treatment options have become available (Bosentan, Remodulin, Revatio), we're trying to treat secondary pulmonary hypertension more aggresively. I don't think there is much data on efficacy in these setting.
Next: for this patient, I would start with a right heart cath and vasodilator study. I would then evaluate for chronic VTE disease, as these pressures seem quite high for secondary pul htn. Additionally, I would be very aggresive in treating her nocturnal desaturations with positive pressure and supplemental oxygen.
Given the (presumed) severity of her pulmonary hypertension, I really wonder if she is having the wrong organ transplanted... and I would definitely be worried about perioperative complications due to her pulmonary hypertension.
I would consider getting an ABG as if she is a CO2 retainer, I would argue she does have nocturnal hypoventilation (whether or not you have documented apneic epidodes). The
What was her AHI on the sleep study?
I also agree with an exercise study. If her VO2 is < 15-20, I would consider her high risk for an operation.
Also, pulmonary rehab and an exercise program may be helpful.
Oh, and why will she not wear oxygen? She obviously needs it.
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