Carney complex (CNC) is a familial multiple neoplasia syndrome characterized by cardiac and extracardiac myxomas in the setting of spotty skin pigmentation and endocrinopathy. We previously identified PRKAR1A (regulatory subunit 1α of protein kinase A) mutations in CNC. Mutational analyses of the PRKAR1A gene in 51 unrelated CNC probands now detect mutations in 65%. All mutations, except for one unique missense mutation, lead to PRKAR1A haploinsufficiency. Therefore, we studied the consequences of prkar1a haploinsufficiency in mice. Although we did not observe cardiac myxomas or altered pigmentation in prkar1a +/– mice, we did observe some phenotypes similar to CNC, including altered heart rate variability. Moreover, prkar1a +/– mice exhibited a marked propensity for extracardiac tumorigenesis. They developed sarcomas and hepatocellular carcinomas. Sarcomas were frequently associated with myxomatous differentiation. Tumors from prkar1a +/– mice did not exhibit prkar1a loss of heterozygosity. Thus, we conclude that although PRKAR1A haploinsufficiency does predispose to tumorigenesis, distinct secondary genetic events are required for tumor formation.

Irreversible atrioventricular block requiring a permanent pacemaker implantation is an uncommon complication after AVR. Risk factors are annular calcification, bicuspid aorta, female sex, presence of RBBB or LBBB, prolonged total perfusion time, and hypertension.

No abstract

Surgical treatment for RSVA carries an acceptably low operative risk and long-term freedom from death and reoperation. Surgical approach must be chosen according to the ruptured chamber and associated lesions. Patch repair of RSVA must be preferred.

Surgical management of cardiac myxoma gives excellent results. In selected cases, a conservative approach may be adequate. Despite the scarcity of the neoplastic properties, careful follow-up is necessary.

IntroductionOver the years, operability criterias for cardiac surgery has been enhanced. Therefore, averege age of operated patients and co-morbidities have increased. 1) Cerebral injury is one of the most important complications of cardiac surgery which may cause mortality, morbidity, increased hospital costs and impaired quality of life. Perioperative cerebral injury includes stroke that occurs in 1.5% to 5.2% of patients, encephalopathy affecting 8.4% to 32% of patients. 2) Cognitive dysfunction has been reported in approximately 50% of patients at discharge, 36% at 6 weeks, 26%-33% at one year, and 42% at five years. 3,4) Pre-exisiting atherosclerotic plaque, thrombogenesis from the foreign surfaces of cardiopulmonary bypass, air from the cardiopulmonary bypass circuit, and pericardial fat globules are tought to be primary predictors of cognitive decline by creating microemboli during Purpose: The purpose of this study was to research the use of near-infrared spectroscopy (NIRS) on the neurocognitive functions in the patients undergoing coronary artery bypass grafting (CABG) with asymptomatic carotid artery disease. Methods: The study design was carried out with the participation of 79 patients in a prospective, randomized and double blind control method. The patients were separated into two groups as NIRS (n = 43) and no NIRS (n = 36). A neurocognitive test was applied preoperatively and postoperatively to all patients before discharge. Cognitive functions were evaluated by applying the Montreal Cognitive Assessment test (MoCA). Results: The decrease in the postoperative score of mean MoCA in no NIRS group was statistically significant when compared to preoperatively (p <0.001). Postoperative mean MoCA score was found to be significantly higher in NIRS group (NIRS: 26.8 ± 1.9 vs. no NIRS: 23.6 ± 2.5, p <0.001). It has been determined that there was a moderately positive significant correlation between the increase in the NIRS used patients (%) and increase in the MoCA score of the patients (r = 0.59, p <0.001). Conclusion: Intraoperative NIRS usage in the patients undergoing CABG with carotid artery disease might be useful due to its postoperative positive effects on the cognitive functions.

Melatonin has been shown to diminish ischemia-reperfusion (I/R) injury in many tissues. The main aim of this study was to evaluate the protective antioxidant effect of melatonin in skeletal muscle during I/R injury. Wistar albino rats were randomly divided into three groups. Hindlimb ischemia was achieved by clamping the common femoral artery in two groups but not in control group. Limbs were rendered ischemic for 1.5 hr; at the end of the reperfusion period of 1.5 hr muscle tissue samples were taken for the histological evaluation and biochemical analysis. Melatonin (10 mg/kg) was injected i.p. in the I/R + Mel group at the onset of ischemia whereas the vehicle solution was injected in the I/R group. In I/R + Mel group histological damage was significantly less than in the I/R group (P < 0.001). In the I/R + Mel group, the mean malonedialdehyde level was lower than in the I/R group (P < 0.01) and was quite near to the levels in the control group (P > 0.05). Glutathione levels were found to be reduced in the I/R group compared with the control (P < 0.01) and I/R + Mel group (P < 0.01). Melatonin has a protective effect against I/R injury in skeletal muscle and may reduce the incidence of compartment syndrome, especially after acute or chronic peripheral arterial occlusions.

Aortic root abscess is the most severe sequela of infective endocarditis, and its surgical management is a complicated procedure because of the high risk of morbidity and death. Twenty-seven patients were included in this 15-year retrospective study: 21 (77.8%) with native- and 6 (22.2%) with prosthetic-valve endocarditis. The surgical reconstruction of the aortic root consisted of aortic valve replacement in 19 patients (70.4%) with (11) or without (8) a pericardial patch, or total aortic root replacement in 7 patients (25.9%); 5 of the 27 (18.5%) underwent the modified Bentall procedure with the flanged conduit. Only one patient (3.7%) underwent subaortic pericardial patch reconstruction without valve replacement. A total of 7 patients (25.9%) underwent reoperation: 6 with prior valve surgery, and 1 with prior isolated sinus of Valsalva repair. The mean follow-up period was 6.8 ± 3.7 years. There were 6 (22.2%) in-hospital deaths, 3 (11.1%) of which were perioperative, among patients who underwent emergent surgery. Five patients (23.8%) died during follow-up, and the overall survival rates at 1, 5, and 10 years were 70.3% ± 5.8%, 62.9% ± 6.4%, and 59.2% ± 7.2%, respectively. Two of 21 patients (9.5%) underwent reoperation because of paravalvular leakage and early recurrence of infection during follow-up. After complete resection of the perianular abscess, replacement of the aortic root can be implemented for reconstruction of the aortic root, with or without left ventricular outflow tract injuries. Replacing the aortic root with a flanged composite graft might provide the best anatomic fit.