Long-term follow up of the Biocor porcine bioprosthesis in the mitral position.

BACKGROUND AND AIM OF THE STUDY: The study aim was to review the long-term results of mitral valve replacement (MVR) with the Biocor porcine bioprosthesis, in order to investigate patient survival and valve-related complications. METHODS: Data were presented for 546 patients (320 females, 226 males; mean age 49.2 +/- 17.1 years) who underwent MVR with the Biocor porcine bioprosthesis between March 1983 and December 2000. Preoperatively, rheumatic fever was present in 42.3% of patients. Associated procedures were performed in 179 patients (32.7%), and myocardial revascularization in 54 (9.9%). Preoperatively, 41.9% of patients were in NYHA class IV. Postoperative follow up was conducted by telephone interviews, questionnaires, or examination of hospital records. RESULTS: The hospital mortality was 9.5% (n=52); of these patients, 30 died after their first MVR and 22 after mitral reoperation. Mortality after isolated MVR was 7.6%. The total follow up was 2,148.9 patient-years, and actuarial survival was 45.0 +/- 15.8% at 15 years. Freedom from structural valve deterioration (SVD) was 51.8 +/- 13.8% for patients aged <50 years, 88.7 +/- 5.1% for those aged 51-60 years, and 84.0 +/- 9.8% for those aged 61-80 years. The incidence of prosthetic valve endocarditis was low, with 88.8 +/- 6.1% non-recurrence in 15 years. Freedom from reoperation was 33.9 +/- 10.4%, and from thromboembolism was 82.3 +/- 15.0%. Freedom from valve-related mortality was 80.7 +/- 12.5% during a 15-year period. CONCLUSION: The 15-year clinical results with the Biocor porcine bioprosthesis in the mitral position were excellent in a young, predominantly rheumatic, patient population which, preoperatively, was in a grave clinical condition. The incidence of valve-related complications was low, as was that of SVD in patients aged >50 years.

Holt-Oram syndrome presenting as agenesis of the left pericardium.

We report on a case of a 60-year-old man with progressive heart failure, mitral and aortic valve insufficiency and bilateral asymmetrical skeletal upper-limb deformities. Central to the suspicion of Holt-Oram syndrome in this patient was the surgical finding of agenesis of the left pericardium. A Holt-Oram syndrome diagnostic was confirmed through molecular analysis of the TBX5 gene. A new amino acid substitution at position 61 of the TBX5 gene was identified and confirmed the clinical diagnosis of Holt-Oram syndrome. The clinical presentation of the present case broadens the clinical spectrum of Holt-Oram syndrome and point out the importance of Tbx 5 in pericardium development. It is still an unstudied issue whether TBX5 mutations may also be present in other clinical presentations where absence of the pericardium is a feature.