A novel sodium channel mutation causing a hyperkalemic paralytic and paramyotonic syndrome with variable clinical expressivity

BACKGROUND: The CarboMedics valve is a relatively new, low-profile, bileaflet, mechanical prosthesis. The results of a prospective follow-up study after valve replacement with this prosthesis in a university hospital are presented. METHODS: We implanted 640 CarboMedics prostheses in 583 patients in the aortic (n = 359), mitral (n = 167), or aortic and mitral positions (double valve replacement; n = 57). Patient ages ranged from 11 to 81 years (mean age, 58 +/- 12.3 years). RESULTS: Overall hospital mortality was 9.0%; however, when high-risk urgent cases were removed from the calculation, the operative mortality fell to 4.5%. Follow-up was 98% complete, comprising 2,027 patient-years for a mean follow-up of 44 months (range, 6 to 72 months). Actuarial freedom from complications (linearized rates in parentheses) was as follows: late mortality, 85% +/- 2.0% (2.3%/patient-year); thromboembolism, 92% +/- 1.1% (1.6%/patient-year); anticoagulation-related hemorrhage, 87% +/- 1.2% (2.8%/patient-year); prosthetic valve endocarditis, 98% +/- 0.5% (0.1%/patient-year); and overall valve-related morbidity and mortality, 76% +/- 2.1% (4.3%/ patient-year). CONCLUSIONS: The CarboMedics valve shows a low rate of valve-related complications comparable with other new mechanical heart valve prostheses.