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Pediatric Hospital Bambino Ges├?┬╣, Italy
Posters-Accepted Abstracts: Pediat Therapeut
Plastic bronchitis (PB) is a rare and severe respiratory disorder, characterized by formation of gelatinous plugs bronchial casts in the large airways that take the shape of bronchial ├ó┬?┬?casts.├ó┬?┬Ł Plastic bronchitis generally occurs in patients suffering from asthma, respiratory infections, cystic fibrosis, and lymphatic abnormalities; it is rarely potentially life-threatening complication of the Fontan operation, the final palliative procedure for various forms of single-ventricle heart diseases. PB is characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and in few reports, aerosolized fibrinolytic such as inhaled plasminogenon activator (rTPA) and pulmonary vasodilators. Hemodynamic alterations (elevated central venous pressure and low cardiac output) likely contribute to the formation of tracheobronchial casts composed of inflammatory debris, mucin, and fibrin. We treated 6 cases with PB, 5 Fontan and 1 Glenn procedures, two died, one underwent heart transplantation with no further episodes of PB, one successfully suspended rTPA after the implantation of a dual pace maker, one is successfully weaning rTPA with a progressive reduction of central venous pressure at the cardiac catheterization and in the Glenn patient is still administrated rTPA with no recurrences. The exact causes of PB in these series of patients remain unknown. The optimization of hemodynamics, aggressive pulmonary vasodilatation, and inhaled t-PA is an effective treatment strategy for patients with PB after cavopulmonary connection. When the Fontan circulation fails or symptoms are unmanageable, PB may resolve after cardiac transplantation.
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