[Unplanned Ascending Aortic Graft Replacement at Valvuler Surgery in a Patient with Systemic Lupus Erythematosus;Report of a Case].

A 65-year-old woman with severe mitral stenosis was admitted to our hospital. She had been previously diagnosed with systemic lupus erythematosus (SLE) and had been taking prednisolone (5 mg/day) for 19 years. As SLE patients with prolonged steroid use are known to be at risk of an aortic dissection and aneurysm, femoral artery was chosen for arterial perfusion to reduce the risk of a dissection of the ascending aorta. However, hemostasis was difficult at the insertion point of the catheter to infuse the antegrade cardioplegic solution. An ascending aortic graft replacement was therefore performed. Histopathological examination of the aortic wall showed the presence of intimal hypertrophy and a decrease in elastic fiber content but without any evidence of vasculitis. Because prolonged steroids use is a risk factor of atherosclerotic change in the aortic wall, the aorta should be treated carefully during cardiovascular surgery in such patients.

[Hemorrhagic Shock due to an Aortoesophageal Fistula in an Infant with a Double Aortic Arch；Report of a Case].

We experienced a case of a female infant with a double aortic arch (DAA) which formed an aortoesophageal fistula, leading to hemorrhagic shock. The patient had severe dyspnea at birth, and was intubated and tube-feeding was started through a nasogastric tube immediately after birth. A DAA was diagnosed by contrast-enhanced computed tomography. Due to abdominal organ malformation, we proceeded with abdominal surgery. Forty-nine days after birth, she suddenly developed massive hematemesis and went into hemorrhagic shock. The bleeding was stopped using an endoscope and was shown to have originated from the esophagus membrane. Compression of the esophageal wall by both the inserted nasogastric tube and vascular ring led to the development of ulceration, resulting in a fistula associated with massive hematemesis. An operation for a DAA was performed on the 53rd day after birth. The inferior side of the DAA was cut, to decompress the bronchus and esophagus and close the fistula. The patient's postoperative course was good and there was no further bleeding. In severe cases of a DAA who require respiratory intubation and tube feeding from a nasogastric tube it is important to carry out surgery as soon as possible.

Protein kinase A-dependent phosphorylation of ryanodine receptors increases Ca2+ leak in mouse heart.

In heart failure, chronic catecholaminergic stimulation increases diastolic Ca(2+) leak from ryanodine receptors (RyRs) of sarcoplasmic reticulum (SR), possibly due to the phosphorylation of RyRs through the activation of protein kinase A (PKA) or Ca(2+)/calmodulin-dependent protein kinase II (CaMKII). In the present study, we attempted to identify which activated kinase is responsible for the enhanced Ca(2+) leak caused by beta-adrenergic stimulation. Trabeculae obtained from the hearts of adult male C57BL/6J mice were treated with isoproterenol and then permeabilized with saponin. To examine SR functions, Ca(2+) in SR was released with caffeine and measured with fluo-3. The Ca(2+) leak in isoproterenol-treated preparations was significantly increased when the PKA-dependent phosphorylation of RyR was increased without the involvement of CaMKII-dependent phosphorylation. Both the increase in Ca(2+) leak and the phosphorylation of RyR were blocked by a PKA inhibitor. Our results show that beta-adrenergic stimulation increases Ca(2+) leak from SR through PKA-dependent phosphorylation of RyR.