Isolated unilateral absence of the pulmonary artery without any intracardiac anomaly is a rare congenital cardiovascular disorder. We performed a successful anatomical repair after systemic-to-pulmonary shunt. The patient was a 1-day-old boy who was transferred to our institution because of continuous murmur. Cardiac echography revealed anomalous origin of the right pulmonary artery from the ascending aorta (AORPA). This aorto-right pulmonary arterial blood flow, however, disappeared in the following 2 days. We altered the diagnosis to right unilateral absence of pulmonary artery (UAPA) from AORPA. A right systemic-to-pulmonary shunt using a 3 mm polytetrafluoroethylene (PTFE) graft was placed, aiming for growth of the right pulmonary artery at the age of 8 days. Forty days after the initial surgery, he underwent a definitive procedure. The right pulmonary artery was anatomically reconstructed with an 8-mm PTFE graft. He was discharged in excellent condition on postoperative day 41. Cardiac catheterization, 1 year later, showed the surgically created right pulmonary artery was patent. A pulmonary perfusion scintigraphy showed satisfactory blood distribution in the right lung.

Giant left atrium (LA) in association with mitral valve disease is a significant risk factor for mortality and morbidity. It may cause obstruction of the bronchus, pulmonary artery, inferior vena caval orifice, or left ventricle, and has also required prolonged hospital stay. We describe our experience of left atrial reduction by means of circular LA resection concomitant with mitral valve plasty in 2 children with severe mitral regurgitation (MR) and giant LA. Case 1 was a 1-year-old boy, who had severe MR and giant LA, presenting with left bronchial obstruction. He underwent mitral valve plasty and circular LA resection. His LA volume, which was calculated using enhanced chest CT examination, was decreased by 23% postoperatively, comparing that before the operation. The cardiothoracic ratio on chest X-ray changed from 60% to 49%, and his sequential symptoms from giant LA disappeared. His postoperative course was uneventful and he was discharged on postoperative day 21. Case 2 was a 12-year-old girl with severe MR and giant LA. Her LA together with the sternum and spine compressed the right atrium and caused right heart failure. She underwent mitral valve plasty and circular LA resection. Her LA volume was decreased by preoperative 22%. Cardiothoracic ratio on chest X-ray changed from 63% to 57%. Her sequential symptoms from giant LA disappeared. Her postoperative course was uneventful and she was discharged 28 days after the operation. The circular LA resection technique reduced LA volume effectively, and the patients became free of clinical symptoms. Even in children, this procedure should be indicated in selective cases who presented clinical symptoms caused by the expanded LA.

A 42-year-old woman with antiphospholipid syndrome (APLS) secondary to systemic lupus erythematosus (SLE) complicated with thrombocytopenic purpura was successfully treated by mitral valve replacement with a mechanical prosthesis and tricuspid valve annuloplasty for mitral valve stenosis and regurgitation due to Libman-Sacks endocarditis. Intraoperative hemorrhagic oozing due to thrombocytopenia was effectively managed with platelet transfusion. Negative microbial culture and pathological examination of the resected mitral valve demonstrated an atypical sterile verrucose lesion, the findings of which were typically characteristic of Libman-Sacks endocarditis in SLE. She was successfully discharged 31 days after the operation without any hemorrhagic or thromboembolic events. However, 100 days after surgery, she suffered from fatal cerebral infarction caused by poor Coumadin compliance. Regarding the prosthetic valve selection, it is reasonable to select the mechanical valve because 1) anticoagulation therapy is necessary for APLS, 2) the risk of the dialysis induction due to the lupus-induced renal failure leading to a high calcium turnover, which results in accelerated bioprosthetic valve calcification. In case of SLE with APLS, in which anticoagulation and antiplatelet therapy is required to prevent the thromboembolic event and thrombocytopenic purpura, after valve replacement, strict management of anticoagulation plays an essential role to prevent thromboembolic complication.

We report a case of methicillin-resistant Staphylococcus aureus (MRSA) infectious endocarditis (IE) which was successfully treated with linezolid (LZD). The patient was a 44-year old woman. She was referred to our hospital because of fever of unknown origin. MRSA was detected from blood cultures and echocardiography revealed vegetation on the right coronary cusp of the aortic valve. She was diagnosed with MRSA endocarditis according to the Duke criteria, and was immediately give vancomycin (VCM) and isepamicin. Sixteen days after administration of VCM, she had a progressively increasing skin rash. It was considered a side effect of antibiotics and VCM was replaced with teicoplanin (TEIC). Eventually, LZD was given to her at 22 days after hospitalization because TEIC was not effective. LZD alleviated the fever and diminished the signs of vasculitis due to endocarditis within a week. LZD was continued for 4 weeks with cardiac failure medically controlled, and she underwent aortic valve replacement using a mechanical prosthetic valve. LZD was injected just before the operation and continued for 15 days postoperatively, followed by oral administration of levofloxacin. She was discharged 35 POD and no recurrence of the infection had been observed at 1 year after the surgery. LZD could be an alternative therapy for MRSA endocarditis, but further examinations are warranted to determine the most appropriate regimen.

A 79-year old man presented with hoarseness and we diagnosed an aortic arch aneurysm, 60 mm in diameter. The aortic arch was right-sided and traversed posterior to the esophagus and trachea, and the arch vessels were mirror-imaged. Total arch replacement was performed under hypothermic circulation arrest using selective cerebral perfusion through a median sternotomy. Three cervical vessels were reconstructed, a 24-mm Hemashield was passed anterior to the trachea and esophagus, and an additional right thoracotomy was not necessary. The patient was uneventfully discharged on the 26th postoperative day.

A 52-year-old man had a history that included aortic valve replacement due to infectious endocarditis in 1987. Chest X-ray showed slight enlargement of the superior mediastinum in 1998, but the enlargement was very mild and there had not been any significant change since 1998. However, chest X-ray demonstrated an extremely protruding mass on the right side of the superior mediastinum in May 2004 and a pseudoaneurysm located in the ascending aorta was demonstrated by computed tomography. We considered this aneurysm had been caused by ascending aortic cannulation for blood return from cardiopulmonary bypass (CPB) during the previous surgery. On re-operation, CPB was established by femoro-femoral bypass and median sternotomy was performed. The pseudoaneurysm measured 60mm in diameter and there was a felt-pledget on top of the aneurysm. Under deep hypothermic cardiac arrest, we incised the aneurysm and closed the orifice of the pseudoaneurysm using a patch (Hemashield Woven Fabrics). On pathological examination, the wall of the pseudoaneurysm showed a structural loss of the blood vessel and the felt-pledget had been exposed to the inferior of the aneurysm breaking through the wall. We considered this a non-mycotic pseudoaneurysm because of this patient's clinical course, surgical and pathological findings. We encountered a pseudoaneurysm in the ascending aorta that was detected and treated surgically about 20 years after aortic valve replacement.