RÉSUMÉ
Heart transplantation is the most effective therapy for children with end-stage heart disease; however, its use is limited by the number of donor organs available. This shortage may be further compounded by concerns about organ quality, leading to refusal of potential donor organ offers. We report on the successful transplantation and 5-year follow-up of a heart from a donor with Ullrich congenital muscular dystrophy (UCMD). The candidate was critically ill at the time of the transplant and the donor organ was declined repeatedly on the match run list due to concerns about organ quality, despite having normal cardiac function by echocardiography on minimal inotropic support. We believe the diagnosis of "muscular dystrophy" in the donor combined with a lack of understanding about the specifics of the diagnosis of UCMD enabled our candidate to receive a primary offer for this organ. We are unaware of any previous reports of the use of a heart from a donor with UCMD for orthotopic heart transplantation in adults or children.
Sujet(s)
Cardiomyopathie dilatée/chirurgie , Transplantation cardiaque/méthodes , Dystrophies musculaires/chirurgie , Sclérose/chirurgie , Donneurs de tissus , Acquisition d'organes et de tissus/méthodes , Cardiomyopathie dilatée/diagnostic , Enfant , Enfant d'âge préscolaire , Échocardiographie , Études de suivi , Survie du greffon , Humains , Mâle , Facteurs tempsRÉSUMÉ
Anomalous origin of the left main coronary artery from the pulmonary artery is rarely associated with other conditions. We report the case of an infant born with tetralogy of Fallot and aortopulmonary window who at the time of surgical repair was found to have an anomalous left main coronary artery originating from the right pulmonary artery.
Sujet(s)
Anomalies congénitales des vaisseaux coronaires/complications , Artère pulmonaire/malformations , Tétralogie de Fallot/complications , Cathétérisme cardiaque , Procédures de chirurgie cardiaque/méthodes , Anomalies congénitales des vaisseaux coronaires/chirurgie , Humains , Nouveau-né , Mâle , Tétralogie de Fallot/chirurgieRÉSUMÉ
Forty-six patients, 24 male and 22 females, with a mean age of 78.4 years (range 75 to 88) underwent aortic valve replacement (AVR) for severe calcific aortic stenosis during a five year period. Twenty-six patients (56.5%) had combined aortic valve replacement/coronary artery bypass (AVR/CABG) procedures. Bovine pericardial or porcine bioprostheses were used in 47.8% of cases. The mean length of stay in the intensive care unit was 2.9 days (range 2-13) with a mean hospital stay of 12.6 days (range 6-41). Operative mortality rate was 6.5% (3 patients), all in the AVR/CABG group. Complications included pneumonia (8.7%), stroke (6.5%), and complete heart block requiring pacemaker insertion (6.5%). Follow-up of survivors from 11-69 months (mean 34.6) shows 92.9% survival, with 87.2% in New York Heart Association (NYHA) Class I and II. Aortic valve replacement in the elderly population has an acceptable mortality rate and is associated with significantly improved quality of life over the intermediate term.