Aortic Valve Replacement in Anomalous Aortic Origin of Right Coronary Artery

A 75-year-old man with severe aortic regurgitation was referred to our department for surgery. Coronary angiography revealed 75% stenosis at left coronary artery( LAD) and a normally positioned left coronary artery. However, the location of the right coronary artery was anomalous. The right coronary artery ostium originated from the ascending aorta above the left coronary cusp. Emergency surgery proceeded to address severe dyspnea due to congestive heart failure. The higher portion of the right coronary artery was incised in sigmoid fashion, the aortic valve was replaced with a 23-mm stented bioprosthesis and coronary bypass grafting proceeded. The postoperative course was uneventful

Gene-based genome-wide association studies and meta-analyses of conotruncal heart defects.

Conotruncal heart defects (CTDs) are among the most common and severe groups of congenital heart defects. Despite evidence of an inherited genetic contribution to CTDs, little is known about the specific genes that contribute to the development of CTDs. We performed gene-based genome-wide analyses using microarray-genotyped and imputed common and rare variants data from two large studies of CTDs in the United States. We performed two case-parent trio analyses (N = 640 and 317 trios), using an extension of the family-based multi-marker association test, and two case-control analyses (N = 482 and 406 patients and comparable numbers of controls), using a sequence kernel association test. We also undertook two meta-analyses to combine the results from the analyses that used the same approach (i.e. family-based or case-control). To our knowledge, these analyses are the first reported gene-based, genome-wide association studies of CTDs. Based on our findings, we propose eight CTD candidate genes (ARF5, EIF4E, KPNA1, MAP4K3, MBNL1, NCAPG, NDFUS1 and PSMG3). Four of these genes (ARF5, KPNA1, NDUFS1 and PSMG3) have not been previously associated with normal or abnormal heart development. In addition, our analyses provide additional evidence that genes involved in chromatin-modification and in ribonucleic acid splicing are associated with congenital heart defects

Modified Bentall operation with bioprosthetic valve and Valsalva graft conduit:the "slit skirt" technique

We elucidated the efficacy of the slit skirt technique to prevent bleeding from the proximal anastomosis between the graft and aortic annulus. Between September 2008 and September 2014, 15 patients underwent a modified Bentall operation with the slit skirt technique at our institution. No patients had bleeding from the proximal anastomosis. No re-thoracotomy for bleeding was required. During midterm follow-up (median period, 21 months), no patient had pseudoaneurysms at the proximal suture line. We conclude that the slit skirt technique is useful to prevent bleeding from the proximal anastomosis after the Bentall operation

Hemolytic Anemia soon after Replacement of Ascending Aorta for acute Aortic Dissection

A 61-year-old female underwent replacement of the ascending aorta for Type A aortic dissection, then gradually developed severe hemolytic anemia over 1 month. The cause of hemolysis was found to be mechanical damage of red blood cells caused by an inverted felt strip at the proximal anastomosis. A reoperation for resection of the felt and repair of the proximal anastomosis successfully resolved the problem. Here, we report a rare case of hemolytic anemia at the site of an inverted felt strip that occurred after replacement of the ascending aorta

Impaired cardiac autonomic nervous control after cardiac bypass surgery for congenital heart disease

We undertook a study to describe changes in heart rate variability (HRV) postoperatively in children undergoing cardiac bypass surgery for congenital heart disease (CHD). HRV was recorded for a 1-h period preoperatively and a 24-h period postoperatively in 20 children with CHD. We found a highly significant reduction in HRV in both time and frequency domain indices compared to preoperative values, which was sustained throughout the 24-h study period. There was a negative correlation between both time and frequency domain HRV measurements and length of cardiac bypass. HRV is reduced postoperatively and correlates with cardiac bypass time. Length of cardiac bypass time may be one mechanism whereby HRV is reduced following surgery