The combined superior transsseptal approach (CSTA) has been used for 12 mitral or left atrial myxoma operations. This approach provided excellent exposure of the mitral valve or myxoma. This approach was compared with the transseptal and left atrial approaches in 1 and 3 cases, respectively. There were no differences in operative time, cardiopulmonary bypass time, anoxic time, bleeding volume, blood transfusion volume and postoperative arrhythmic complications. We use CSTA for cases with tricuspid valve disease, small left atrium, reoperation and left atrial myxomas.

In the past 9 years, 37 patients with infective endocarditis underwent valve replacement. The aortic valve was involved in 17 patients, the mitral valve in 10, and both valves in 10, respectively. 35 patients had native valve and 2 had prosthetic valve endocarditis. Bacterial findings were Streptococcus in 20 patients (54%), Staphylococcus in 5 (13.5%), gram-negative in 3 (8%), and undetected in 10 (27%). 10 patients developed aortic annular abscess. After aggressive debridement of all apparently infected tissue of annular abscess, the defects left in the left ventricular outflow tract were repaired by interrupted mattress sutures with pledgets in 4 patients, by autologous pericardial patch in 4, and by valved conduit in 2 PVE patients, respectively. Retrograde cardioplegic infusion from the coronary sinus not only facilitated operative manipulation but also provided superior myocardial protection in such patients. Operative mortality was 11% (4/37). Reoperation was necessary in 2 patients; one for periprosthetic leak, and the other for newly developed severe left coronary ostial stenosis after the first operation, but both died eventually. Late mortality was 8% (3/37). Mean follow-up of 31 months was achieved in all 30 survivors, in whom there was no recurrence of infection and clinical improvement was excellent.

BACKGROUNDIn an earlier study, we identified a locus for Moyamoya disease (MMD) on 17q25.3.

METHODSLinkage analysis and fine mapping were conducted for two new families in additional to the previously studied 15 families. Three genes, CARD14, Raptor, and AATK, were selected based on key words, namely, "inflammation", "apoptosis", "proliferation", and "vascular system", for further sequencing. A segregation analysis of 34 pedigrees was performed, followed by a case-control study in Japanese (90 cases vs. 384 controls), Korean (41 cases vs. 223 controls), Chinese (23 cases and 100 controls), and Caucasian (25 cases and 164 controls) populations.

RESULTSLinkage analysis increased the LOD score from 8.07 to 9.67 on 17q25.3. Fine mapping narrowed the linkage signal to a 2.1-Mb region. Sequencing revealed that only one newly identified polymorphism, ss161110142, which was located at position -1480 from the transcription site of the Raptor gene, was common to all four unrelated sequenced familial affected individuals. ss161110142 was then shown to segregate in the 34 pedigrees studied, resulting in a two-point LOD score of 14.2 (P = 3.89 × 10(-8)). Its penetrance was estimated to be 74.0%. Among the Asian populations tested (Japanese, Korean, and Chinese), the rare allele was much more frequent in cases (26, 33, and 4%, respectively) than in controls (1, 1, and 0%, respectively) and was associated with an increased odds ratio of 52.2 (95% confidence interval 27.2-100.2) (P = 2.5 × 10(-49)). This allele was, however, not detected in the Caucasian samples. Its population attributable risk was estimated to be 49% in the Japanese population, 66% in the Korean population, and 9% in the Chinese population.

CONCLUSIONss161110142 may confer susceptibility to MMD among East Asian populations.

ELECTRONIC SUPPLEMENTARY MATERIALThe online version of this article (doi:10.1007/s12199-009-0116-7) contains supplementary material, which is available to authorized users.