A 23-year-old man had had a fever of unknown origin for a month. Aggravation of shortness of breath brought him to our hospital. After a close inspection, transthoracic and esophageal echocardiography (TEE) showed severe aortic valve regurgitation (AR) with vegetation extending for 25mm. The valve was bicuspid and the vegetation was on the left side valve. TEE also revealed a streak of mitral valve regurgitation (MR). In spite of continuous antibiotic therapy, congestive heart failure developed with progressive MR, so we performed an emergency operation. The aortic valve was bicuspid composed of an agglutinated left and non-coronary cusp, and 15×30mm vegetation was attached on the left. Checking the mitral valve after resection of aortic valve, we found a perforation 3mm in size at the center of the anterior mitral leaflet. After resection of the infected area, we repaired it with a Xenomedica patch 10mm in size through the aortic orifice. Two abscesses located beneath both leaflets were eradicated and finally aortic valve replacement was done with an SJM 23.

We present a very rare case of abdominal aortic aneurysm associated with paraplegia. A 68-year-old man developed paraplegia following resection of a infrarenal abdominal aortic aneurysm. The aorta was clamped just below the renal arteries. In this case interruption of the radicular artery magna (RAM; Adamkiewicz artery) might have caused serious ischemia of the spinal cord. Spinal cord ischemia is a very rare and unpredictable complication in surgery of infrarenal abdominal aortic aneurysms because the spinal cord is generally protected from irreversible ischemia during infrarenal aortic occlusion by the presence of the RAM which arises above the renal artery (Even if RAM interruption might arise, the lower renal artery, and other radicular arteries are usually present above the renal arteries). We feel that reducing aortic cross-clamping time as short as possible and avoiding intra- and postoperative hypotensive episodes to keep adequate blood flow of collaterals seem to be the most important factors to prevent spinal cord ischemia.

A 6-month-old baby boy had undergone the Jatene procedure at 4 days. Four months later, catheter intervention (balloon angioplasty) was performed because of severe stenosis at the bifurcation of the pulmonary arteries. Twenty days later, several episodes of cyanosis occurred and he was readmitted. The existence of shunt flow between the sinus of valsalva and the pulmonary bifurcation was detected by echocardiography and examination by 16-row MDCT revealed 2 holes at this site. Under a diagnosis of aortopulmonary (AP) window, the patient was placed on cardiopulmonary bypass and the pulmonary artery was opened after aortic clamping. There was a ridge between the bifurcation of the pulmonary arteries. After removing it, 2 holes were visualized that resembled the findings on 16-row MDCT. These holes were closed with Xenomedica patches and the main pulmonary artery was also extended with a Xenomedica patch. AP window is a rare complication after balloon angioplasty for pulmonary stenosis, but we must take great care to prevent this complication.

The patient was a 71-year-old man who had been treated for Parkinson's disease for 21 years. He was admitted because nocturnal dyspnea occurred several times. Echocardiography revealed congestive heart failure because of combined mitral and aortic regurgitation. Double valve replacement was planned. There was a risk of the occurrence of neuroleptic malignant syndrome (NMS) if his drugs for Parkinson's disease were stopped suddenly, so careful control of drug doses was required. Although the patient developed aggravation of his Parkinson's symptoms, careful observation and adjustment of medications prevented the occurrence of NMS.

A 29-year-old woman, who had been diagnosed with Ebstein's anomaly associated with paroxysmal supraventricular tachycardia due to Wolff-Parkinson-White (WPW) syndrome, was referred to our hospital for treatment of congestive heart failure and tachycardia. She had undergone a catheter ablation for WPW syndrome at the age of 28 years. Subsequently, surgical treatment for Ebstein's anomaly was indicated because of persistent symptoms of heart failure due to tricuspid regurgitation (TR). The echocardiogram and pathologic findings corresponded to Ebstein's anomaly of the Carpentier type B classification, with severe displacement of the septal and posterior leaflets resulting in moderate TR. A mobile anterior leaflet of sufficient size without a cleft enabled us to successfully perform Hetzer's procedure. In this procedure, the large mobile anterior leaflet was approximated to the opposing true tricuspid annulus with a mattress suture of 3-0 polypropylene passed from the anterior leaflet annulus to the true tricuspid annulus at the site of atrialized right ventricle near the coronary sinus. The postoperative course was uneventful, and the cardiothoracic ratio reduced from 56% to 48% with mild TR. In this adult case of Carpentier's type B adult Ebstein's anomaly, Hetzer's procedure allowed reconstruction of the tricuspid valve mechanism of “leaflet-to-septum” coaptation at the level of the true annulus by approximating the anterior leaflet. This was, effective in reducing the patient's moderate TR. We conclude that this procedure is a simple and reproducible method for repairing the tricuspid valve in Ebstein's anomaly, especially for cases with a large mobile anterior leaflet.

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.

Perivalvular leakage (PVL) is one of the serious complications of mitral valve replacement. Between 1991 and 2006, 9 patients with mitral PVL underwent reoperation. All of them had severe hemolytic anemia before surgery. The serum lactate dehydrogenase (LDH) level decreased from 2,366±780 IU/l to 599±426 IU/l after surgery. The site of PVL was accurately defined in 7 patients by echocardiography. PVL occurred around the posterior annulus in 3 patients, anterior annulus in 2, anterolateral commissure in 1, and posteromedial commissure in 1. The most frequent cause of PVL was annular calcification in 5 patients. Infection was only noted in 1 patient. In 4 patients, the prosthesis was replaced, while the leak was repaired in 5 patients. There was one operative death, due to multiple organ failure, and 4 late deaths. The cause of late death was cerebral infarction in 1 patient, subarachnoid hemorrhage in 1, sudden death in 1, and congestive heart failure (due to persistent PVL) in 1. Reoperation for PVL due to extensive annular calcification is associated with a high mortality rate and high recurrence rate, making this procedure both challenging and frustrating for surgeons.

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.

Chronic disseminated intravascular coagulation (DIC) complicates 5.7% of thoracic aortic aneurysm. DIC with thoracic aortic aneurysm is characterized by hyperfibrinolysis, but usually shows a stable condition in a state of compensated non-overt DIC with limited hemorrhagic symptoms. However, in some cases, hemorrhage caused by external factors may induce uncompensated overt DIC and lead to serious hemorrhagic tendencies. In the present study, we report a patient with a thoracic aortic aneurysm complicated by DIC who exhibited marked hemorrhagic tendencies. DIC remarkably improved following administration of recombinant human soluble thrombomodulin.

Renal damage caused by hemolysis during cardiopulmonary bypass (CPB) was investigated, and the preventive effects of haptoglobin in regard to this condition was also evaluated. Nineteen patients who underwent open heart surgery were divided into two groups: a control group (n=11) and a haptoglobin group (n=8). In the control group, the level of plasma-free hemoglobin increased significantly after CPB (p<0.01), and this level was strongly correlated with renal tubular leaking enzymes: NAG (r=0.76) and γ-GTP (r=0.81), in the Intensive Care Unit or on the first day after surgery. On the contrary, in the haptoglobin group, in which 4, 000 units of haptoglobin was added in the priming solution of CPB, no increased level of plasma free hemoglobin was observed. Furthermore, leak age of renal tubular enzymes were statistically less (p<0.05). It was concluded that free hemoglobin was a cause of renal damage during CPB and the damage was preventable by the administration of haptoglobin.