Bilateral Phrenic Nerve Palsy

My wife is 42 years old and mother of four. She had a 1 kg mediastinal mass removed last August, diagnosed as a pleomorphic liposarcoma, through a right thoracotomy. At the time the R. phrenic nerve was damaged resulting in paralysis of the right hemidiaphragm, though it was not formally picked up at the time.

After 3 rounds of a high dose Adriamycin/Ifosphamide chemo regime, she was recently operated on for a re-excision of the tumour since the original surgery did not get it all. This was through a median sternotomy and at this operation the L. phrenic nerve was found to be involved with tumour and had to be sacrificed.

Postoperatively she has been unable to lie flat otherwise she becomes breathless, so she need assistance with CPAP/IPAP. With this assistance her oxygen saturations are 98-100%, but without it when lying flat they fall to about 90%. In addition she has exhibited a sinus tachycardia (120) which on echo showed reduced motility of the septum. X-rays do not show any fluid in the lungs. The working diagnosis of the cardiologist is that the cardiac dysfunction is a side effect of the adriamycin.

What is the usual clinical outcome of bilateral phrenic nerve palsy?
Do people need ventilatory assistance for life?
Is plication of the diaphragm warranted in these cases and is it effective?
If so how long after surgery should it be performed?
Is the cardiotoxicity of the Adriamycin permanent or is it reversible?

Sorry to ask so many questions, but the answers to these questions and any other info would be very helpful.

Thanks very much!!

Hanif

[National Jewish]

Through the phrenic nerve the brain tells the diaphragm to move down so that a breath is taken in.  When the phrenic nerve is weak on both the right and left sides, the diaphragm does not work as well and it is common to have breathlessness and a decrease in oxygen level, especially when lying flat and when active.  Ventilatory assistance machines, such as continuous positive airway pressure (CPAP) and/or intermittent positive airway pressure (IPAP), do some of the work of the diaphragm and will be needed as long as the nerve is weak.
Surgery with plication to help the diaphragm work better can be effective.  This surgery should be done as soon as your wife has recovered from her previous surgery.
Adriamycin may cause heart problems, such as an increase in heart rate, weeks, months or years after the last dose and generally does not go away.