A 57-year-old man who underwent aortic root replacement developed continued to have highgrade fever and rhabdomyolysis after administration of haloperidol on the 10th postoperative day, resulted in deterioration of respiratory and hemodynamic status. We established a diagnosis of neuroleptic malignant syndrome (NMS) incomplete type with difficulty after examination by a psychiatrist, and started administration of dantrolen on postoperative day 17. The serum level of CK rapidly decreased. Because NMS causes severe worsening of general status, early diagnosis and immediate treatment is important particularly after cardiac surgery. Care should be paid to the recognition of causative drugs and status, and symptoms of the early stag, such as muscular rigidity, psychiatric symptoms. It is necessary to investigate medical treatments immediately together with a psychiatrist or neurologist. On the other hand, NMS after heart valve replacements should be distinguished from prosthetic valve endocarditis, and the type of fever and inflammatory reaction can help in differential diagnosis.

A 1-year-old girl with patent ductus arteriosus (PDA) was admitted for cardiac catheter examination which identified a 7.8 mm Krichenko D type PDA. An Amplatzer duct occluder (ADO) was used but fluoroscopy showed the device at an oblique angle and residual shunt. The girl underwent surgical removal of the device 2 days after deployment because of progression of residual PDA shunt and left pulmonary artery encroachment, suggesting device dislodgement. Median sternotomy was performed, cardiopulmonary bypass was established and dissection was carried out around the PDA. Marked protrusion of the PDA wall made by the ADO retention disc was noted. The main pulmonary artery was incised under cardioplegic arrest. The device was incarcerated in PDA and attempts to remove the device failed. Therefore delivery cable through sheath was reconnected to the device by its microscrew, and the pulmonary end of the device was recaptured into sheath. The incarceration was dissolved and the device was removed. PDA was ligated.

Primary repair of the tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is associated with high mortality rates of 17-33%, especially in neonates. Our standard strategy involves a staged repair with a first palliation, performed during the neonatal period, that includes main pulmonary septation with an ePTFE patch, pulmonary arterioplasty for reduction of vascular dilation, and a modified Blalock-Taussig shunt. We performed successful repairs on two neonates with TOF/APV, one symptomatic and the other non-symptomatic, with this strategy. Case 1 : A 7-day-old boy had TOF/APV, with progressively worsening respiratory distress. His left bronchi, superior vena cava and left atrium were compressed by a dilated pulmonary artery, which was repaired by emergency surgery. Decreasing the diameter of the pulmonary artery (PA index from 2,550 to 525) relieved the compressed organs. Case 2 : A 16-day-old boy with TOF/APV with a main pulmonary artery that increased in diameter from 8 to 17 mm in the course of a single day. He was treated in the same fashion as Case 1. At 1 year of age, an intracardiac repair with tricuspid anuuloplasty was performed successfully. This strategy is much safer than a primary repair and is a good choice for neonatal repair of TOF/APV.

Isolated unilateral absence of the right pulmonary artery without any intracardiac anomaly is a rare congenital cardiovascular disorder. We performed a successful surgical reconstruction with autologous tissue of this anomaly. The patient was a 1-month-old boy who had been transferred to our center at 3 days of age because of tachypnea and heart murmur. Multi-detector CT and radial angiography imaging revealed isolated unilateral absence of the right pulmonary artery and left patent ductus arteriosus. Conservative treatment did not help his progressive heart failure and pulmonary hypertension due to an acute increase of pulmonary blood flow. Therefore surgical correction was determined to avoid the worsening of those symptoms. Under cardiopulmonary bypass, the right pulmonary artery branching off from the brachiocephalic artery was removed and anastomosed to the main pulmonary artery with an autologous pericardium roll. Symptoms improved postoperatively and he was discharged in good condition on the 21st of postoperative day. Cardiac catheterization, 3 months later, showed excellent results.

Coronary artery fistula is an unusual congenital anomaly, particularly in association with coronary aneurysm. In the present case, a right coronary fistula leading to the right atrium was associated with a giant coronary aneurysm. There have only been 3 such cases reported in the literature. Since both the aneurysm and the fistula were completely thrombosed, no heart murmur was detected and the patient was initially diagnosed as having an intracardiac tumor by echocardiography.

We present a case of Marfan's syndrome with acute aortic dissection during the trimester of her pregnancy, who underwent a Bentall operation 2 days after emergency cesarean section. A 24-year-old woman during the 31st week of pregnancy visited our emergency room due to sudden onset of chest and back pain, though she had no abnormality until this event. Because of her tall height, spider fingers, positive wrist sign, visual disorder and scoliosis, she was given a diagnosis of Marfan's syndrome. Enhanced CT and cardiac ultrasonography revealed that she was suffering from acute aortic dissection with annulo-aortic ectasia. Since it was difficult for her to continue with her pregnancy, she underwent emergency cesarean section and gave birth to a male baby weighted 1, 706g. Although there was little likelifood of early thrombus formation in the false lumen or significant aortic regurgitation indicating an emergency operation, fear of massive bleeding from her uterus and the exfoliated surface of the placenta after cesarean section required an observation period of 2 days. We performed a Bentall operation successfully after careful sedation, ventilation and blood pressure control for 2 days.

Protein C (PC) deficiency is an inherited thrombotic disorder with a prevalence of 0.19% among the general population. PC deficiency is associated with an increased risk of thrombosis when other risk factors are present, such as trauma, surgery, or infection, and is an important cause of mechanical valve thrombosis. We performed tricuspid valve replacement with a 29mm Carpentier-Edwards Perimount valve in a 20-year-old man with PC deficiency. The patient had corrected transposition of the great vessels with severe tricuspid insufficiency, as well as a history of cerebral infarction. In the perioperative period, we used only heparin sodium as the anticoagulant. When we restarted administration of warfarin, changing over from heparin, transient increases of serum plasmin inhibitor-plasmin complex (PIC) and thrombin antithrombin complex (TAT) levels were observed. Despite an increased dose of heparin, an appropriate activated partial thromboplastin time (APTT) was not obtained. This suggested a hypercoagulatory state, but the postoperative course was uneventful. Management of perioperative anticoagulation, prevention of late thrombotic events, and prosthetic valve selection in this particular situation are discussed.

Infected aortic aneurysm is very difficult to treat and is associated with a high mortality rate. A 78-year-old man had been scheduled to undergo selective endovascular repair for distal aortic arch aneurysm. While standby, however, he was admitted to our emergency room because of hemoptysis. Rapid dilatation of the aneurysm shown on serial CT and elevated of inflammatory reactions yielded a diagnosis of infected aortic aneurysm. Because the aneurysm had ruptured into the left lung, emergency surgery was performed. Six days after the first operation, critical bleeding due to anastomotic disruption of the distal aorta caused by infection and subsequent cardiac arrest occurred. We immediately started open chest massage and controlled the bleeding manually in the ICU, while an operating room was prepared. In the redo operation, anastomotic disruption was repaired using the visceral pleura under deep hypothermic circulatory arrest. Anastomotic bleeding is a potentially life-threatening condition, therefore extremely prompt measures are vital. Appropriate management based on the assumption of anastomotic bleeding was very important in the postoperative course of this case of infectious aortic aneurysm.

A 56-year-old woman suffering from mitral stenosis had underwent PTMC (percutaneous transvenous mitral commissurotomy) at age 46. After she developed congestive heart failure, mitral valve replacement (MVR) with Carbomedics 29M and tricuspid annuloplasty (TAP) was carried out. Four hours after admission to the ICU, massive bleeding was noticed. Cardiopulmonary bypass was restarted in the operating room. Laceration and hematoma were found at the posterolateral wall of the left ventricle. Under cardiac arrest with removal of the prosthetic valve, an internal tear was detected about 2cm below the anterolateral commissure (Miller Type III). The tear was covered with a horse pericardial patch (2×3cm) using 6-0 running sutures with reinforcement with gelatin-resorcine-formaline (GRF) glue between the laceration and the patch. MVR sutures in the annulus above the ventricular tear were first passed through the annulus, the pericardial patch and then the prosthetic cuff. Additionally, an epicardial tear was covered and reinforced with the fibrin sheet, GRF glue and pericardial patch in turn. Cardiopulmonary bypass was weaned easily without bleeding. The patient was intentionally on respiratory support with sedation for 3 days. The subsequent postoperative course was uneventful.

We report a successful conversion of anti-coagulant therapy from warfarin to heparin for a case of mechanical heart valve and warfarin-induced skin ulcer. A 57-year-old female who was diagnosed with Ebstein disease and underwent mechanical valve replacement suffered from a recurring and resistant leg skin ulcer. Nine years after the induction of warfarin, her right leg skin ulcer occurred suddenly and worsened gradually. No dermatological treatment could cure it and three auto-skin transplantations were performed in the past four years. The definitive cause of the ulcer could not be diagnosed by any laboratory study or image inspection. Further, after every transplant surgery, the resumption of warfarin had made her ulcer recur and worsen. Therefore, we finally came to think of this disease as the warfarin-induced skin ulcer. To avoid warfarin, subcutaneous self-injection therapy of Heparin-Ca was applied as her anti-coagulation after the 4th transplant surgery. The patient was discharged from hospital on post-operative day 78 with a good condition of the transplanted skin graft and right leg. An adequate level of APTT could be maintained with injections of 10,000 units of Heparin-Ca twice a day and data on D Dimer and cardiac echography show no thromboembolism at the out-patient visits. The result of auto-skin transplantation is also a good course under this therapy. As we have very few reports concerning heparin self-injection therapy for artificial valves, it is very important that careful observation should be continued.