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.

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.

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.

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.

We present a rare case of mitral valve prolapse associated with congenital bicuspid aortic valve, followed by abrupt left chordae tendineae rupture resulting in severe left heart failure and cardiac arrested. The patient, a 43-year-old man who had been admitted because of sudden orthopnea suffered cardiac arrest on arrival in the emergency unit. After successful cardiopulmonary resuscitation, emergency double-valve replacement (SJM 25mm for the aortic valve and Carbomedics 31mm for the mitral valve) was performed; his postoperative course was uneventful. Concerning the pathogenesis of the acute rupture of the chordae tendineae in this patient with no evidence of infective endocarditis, it was likely that chronic and progressive left ventricular volume overload due to aortic regurgitation caused by congenital bicuspid aortic valve was the causative factor of abrupt rupture of the chordae tendineae during the course of mild mitral valve prolapse.

Essential thrombocythemia is a rare disease belonging to the group of chronic myeloproliferative disorders. It displays both thrombogenic and bleeding tendencies due to increased platelet counts, as well as dysfunction. Aortic valve replacement with a 23mm Carpentier-Edwards bioprosthesis was performed for a 74-year-old man with aortic stenosis associated with essential thrombocythemia. No pre-treatment was performed before surgery, though the platelet count was 80×10⁴/μl. During the surgery, activated coagulation time was kept over 400 sec with heparin. There was no difficulty with hemostasis. Aspirin and warfarin were used as antiplatelet and anticoagulant agents after surgery, so the thrombin test results were controlled at around 30%. Since the platelet count reached 130×10⁴/μl, hydroxyurea as chemotherapy was given to suppress the platelet count below 100×10⁴/μl. The operation was completed without major problems and the postoperative course was uneventful. This patient remains in good condition.

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.