Introduction. Plastic bronchitis is a rare and life threatening entity. A patient develops a rare complication after a Fontan procedure - plastic bronchitis. It is thought to be caused by hampered lymph flow into venous system.
Methods: case report and a short literature review.
Case report. Patient was born after normal pregnancy. During the fifth day tachycardia and cyanosis became apparent. After diagnosing congenital heart disease - transposition of the great arteries and double inlet left ventricle a Damus-Kaye-Stansel procedure and later bidirectional Glenn procedure were performed. When patient was three years old extracardiac conduit fenestrated Fontan procedure was formed. Shortly after a bilateral chylothorax and hypoproteinemia developed, which was treated with bilateral pleural drainage, octreotide iniusion and a low-fat diet. Without adequate rer sponse to conservaiive treatment a bii ati eral pleurodesis was performed. Few months after patient started coughing with expectoration of fibrinous bronchial casts. The diagnosis of plastic bronchitis was made and a novel treatment with dornasis alfa was started, allhough because of insufficient response to treatment it was discontinued afier 6 days. The cough remained however expectoration of bronchial casts ceased. This is the first case report of plastic bronchitis development after a Fontan procedure. Treatment with bosentan, diuretics, and individual low-fat diet remains, but patient still has hypoxemia, chronic cardiac failure and dependency on oxygen.
Conclusions. Even modern treatment methods cannot ensure favourable prognosis, but we should not yield, because every day there are new ways of therapy to be found.
