Charcot-Marie-Tooth News reports the findings of a study where patients with Charcot-Marie-Tooth disease (CMT) who undergo Fontan surgery to correct a heart defect in childhood may later develop difficulties with the diaphragm — the muscle that allows us to breath — that can cause the earlier heart surgery to fail while leaving them ineligible for a heart transplant, a case study of a CMT1A patient reports.
Its researchers stress the importance of identifying CMT in patients recommended for Fontan surgery, as some disease types — like CMT1A — can result in damage to the nerve controlling breathing and make the procedure fail later in life.
The report, “Fontan Failure Secondary to Charcot-Marie-Tooth–Induced Phrenic Neuropathy,” appeared in the journal Texas Heart Institute Journal.