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Malignant lymphomas are a group of diseases in which epigenomic abnormalities are fundamental to the pathogenesis and pathophysiology and are characterized by a high frequency of abnormalities in DNA methylation regulators and histone modifiers. These epigenomic abnormalities directly amplify malignant clones. They also originated from a cell lineage differentiated from hematopoietic stem cells through epigenomic changes. These characteristics are associated with their high affinity for epigenomic therapies. Hematology has been a leader in the basic, clinical, and drug discovery areas of disease epigenetics. However, the epigenomic regulation is generally recognized as a complex system, and gaps are observed between basic and clinical studies. To overview the status and importance of "epigenomic abnormalities in malignant lymphoma," this review first summarizes the concept and essential importance of the epigenome and then outlines the current status and future perspective of epigenomic abnormalities in malignant lymphomas.

(Objective) Nocturia, an important male lower urinary tract symptom (LUTS), is often difficult to treat. Herein, we report our experience of the initial treatment of nocturia with the novel drug desmopressin. (Subjects and methods) Subjects included 25 patients with LUTS treated with desmopressin who had the chief complaint of nocturia. Before treatment, the frequency of nocturnal urination (≥2) and nocturnal polyuria index (≥0.33) were confirmed based on the urination diary for ≥ 72 h. Before sleep, 25 or 50 mg desmopressin (Minirin ® Melt OD tablets) was administered once daily. The frequency of nocturnal urination, volume of nocturnal urine, time from falling asleep to first urination, first urinary volume after falling asleep, nocturnal polyuria index, International Prostate Symptom Score (IPSS), quality of life index, Overactive Bladder Symptom Score, and residual urine volume were comparatively evaluated before and 4 weeks after treatment. Treatment effect was self-evaluated by patients 4 weeks after the treatment. Safety was evaluated by interview and blood testing 1 and 4 weeks after the treatment. (Results) Decrease in the frequency of nocturnal urination and improvement in IPSS were observed. According to self-evaluation of the treatment, 72.6% of the patients considered the treatment efficacious. Regarding safety, adverse events were observed in 28% of the patients, particularly hyponatremia (12% of the patients). (Conclusion) Desmopressin is a potential key drug for the treatment of nocturia caused by nocturnal polyuria.

Recently, number of adult congenital heart disease(ACHD) patients are rapidly increasing, but statistical evidences in adult congenital heart surgery are not sufficient because of its short history in addition to the complicated pathophysiology of ACHD. Therefore, a "heart team" including surgeons, pediatricians, and cardiologists are required, and blood flow imaging and 4-dimensional (4D) imaging plays an essential role in perioperative patient management. Enhanced 3-dimensional computed tomography( 3D-CT) scan is useful to determine cardiopulmonary bypass strategies in reoperation, and 4D flow magnetic resonance imaging( MRI) is useful in assessment of complicated hemodynamics. Diastolic function of systemic ventricle is very important in right side heart valve surgery or univentricular heart surgery, echocardiography is essential to examine the valve structure, and electrophysiological mapping is useful in concomitant arrhythmia surgery. This article describes the indications, procedures, and perioperative managements of surgery in the right ventricular outflow tract, tricuspid valve, aortic root, Fontan circulation, and systemic right ventricle.

Histone H3 lysine 27 tri-methylation (H3K27me3) -dependent transcription regulation is a fundamental process of gene control. Although EZH2 mutation is observed in certain lymphoma types, many other cancers show global H3K27me3 accumulation irrespective of mutation. However, the underlying mechanisms of gene silencing and therapeutic efficacies of epigenetic drugs remain unclear. In this study, we showed that globally-accumulated H3K27me3 is induced by both cis-bound EZH1 and EZH2 in mature lymphocyte-derived malignancies. Mutual interference and compensatory functions of co-expressed EZH1/2 rearrange the genome-wide distribution, establishing restricted chromatin and gene expression signatures. Using novel EZH1/2 dual inhibitors, we found that both EZH1 and EZH2 are required for the maintenance and induction of H3K27me3. The synthetic lethality of targeting EZH1 and EZH2 was observed in lymphoma models and primary adult T-cell leukemia/lymphoma (ATL) cells harboring H3K27me3 accumulation. This heritable EZH1/2 dysfunctional state was epigenetically imprinted at the virus-infected, immortalized phase. EZH1/2 dual inhibition could eliminate infected cell populations more effectively than EZH2 inhibition. Regarding the frequent observation of H3K27me3 accumulation in advanced-stage and early-phase malignant progenitors, the emerging EZH1- and EZH2-dependent epigenetic reprograming is an incipient process of fate decision within developmental pathways of cancers.

(Objective) Enzalutamide is an oral androgen-receptor inhibitor that prolongs survival in men with castration-resistant prostate cancer (CRPC). We retrospectively evaluated clinical efficacy and safety of enzalutamide in CRPC. (Patients and methods) We reviewed clinical records of 73 patients who had received enzalutamide for the CRPC at Showa University and affiliated 7 hospitals. Enzalutamide was given at a dose of 160 mg/day, but some patients were treated at lower dose because of there age or poor performance status. Prostrate-specific antigen (PSA) response, prior docetaxel use and the previously administered agents were evaluated retrospectively. (Results) The median patients age was 77 years, the median Gleason score was 9 and the median PSA level at baseline was 26.9 ng/ml. The patients who had prior docetaxel use were 29 (39.7%) and the median of total docetaxel dose was 460 mg/body. The median number of total prior treatments (anti-androgens, Estramustine and steroid) was 3. Twenty seven (61.4%) patients with docetaxel-naïve achieved over 50% reduction of PSA level from baseline, but only 7 (24.1%) in patients previously treated with docetaxel. The most common adverse events included fatigue (24.7%), anorexia (24.7%) and the nausea (16.4%). We found a small proportion of responders to enzalutamide experienced a PSA flare. (Conclusion) Our results of the use of Enzaltamide for CRPC were similar with previous reports. PSA flare was found in some patients with CRPC who responded to enzaltamide. It should be noted that this possible PSA flare phenomenon.

In the recent years, the outcomes of treatment for hypoplastic left heart syndrome (HLHS) is improved because of perioperative management and procedure. We adopt a strategy of the primary Norwood procedure basically for neonates with HLHS, however bilateral pulmonary artery banding precede in patients with risk factors. In the Norwood procedure, aortic arch is reconstructed without artificial prostheses and pulmonary blood flow is supplied from the Blalock-Taussig shunt or the right ventricle-pulmonary artery shunt by case. We administer high dose vasodilators and nitric oxide gas with low resistance strategy after the Norwood procedure. The survival rate of patients with HLHS after 2005 is 84.2% in this strategy.

With the increase of adult patients who need repeated surgery long after definitive repair of congenital heart defects in their childhood, right ventricular outflow tract reconstruction (RVOTR) is gaining more importance. From the viewpoint of timing and indication of surgical intervention, early primary repair and annulus preserving procedure have been the 2 current standards of RVOTR;however, long term outcome of both procedures has to be determined by further study. Although a wide variety for materials are used for pulmonary valve replacement in RVOTR, deficient longevity is one of the most common problems, especially when used for young patients mainly due to immunological rejection. In our institute, we originally developed and manufactured expanded polytetrafluoroethylene (ePTFE) valved patches/conduits, which showed excellent clinical results in a former multicenter study in Japan. This improved outcome may partly be attributable to the biocompatibility and low antigenicity of ePTFE and also to the fluid dynamical properties arising from the 2 characteristic structures:bulging sinus and fan-shaped valve. However, to achieve better patients prognosis with good quality of life,there still remains a lot of problems to be solved concerning RVOTR, such as indication and timing of definitive repair and choice of materials for pulmonary valve replacement. This review describes recent surgical strategies and remaining problems of RVOTR.

The timing and indication of re-right ventricular outflow tract reconstruction (reRVOTR) remains controversial. The main cause for reRVOTR is pulmonary stenosis and pulmonary regurgitation. A pressure gradient of more than 50 mmHg between right ventricle and pulmonary artery or a right ventricular systolic pressure/left ventricular systolic pressure ratio higher than 0.7~0.8 is the threshold for recommending reoperation. Pulmonary regurgitation is difficult to quantify and is not an indication for reoperation until there was evidence of increasing right ventricular dilatation. That is, reoperation is recommended when there are mild to moderate pulmonary regurgitation with mild to moderate right ventricular dilatation and severe pulmonary regurgitation with or without right ventricular dilatation. ReRVOTR should be undertaken in maintaining normal right ventricular function, that is,under remaining free of symptoms. Therefore the right ventricular function needs to be assessed over time.

Although human T cell leukemia virus type I (HTLV-I) is undoubtedly involved in the immortalization and leukemogenesis of infected cells, mechanistic underpinnings of its molecular pathophysiology in long latent period of Adult T-cell leukemia (ATL) remain to be elucidated. One of the most significant recent advances in biomedical research has been the discovery of small noncoding RNAs designated microRNA (miRNA), which affect the field of virology including HTLV-1 research. Mounting evidence indicates that viruses use these miRNAs to manipulate both cellular and viral gene expression. Viral infection also can exert a profound impact on the cellular miRNA expression profile. Some studies have demonstrated that some deregulations of miRNA are involved in the pathogenesis of HTLV-1. Furthermore, global analyses of ATL patient samples have provided a conceptual progress that Polycomb family induces miR-31 silencing, resulting in overexpression of NF- kappaB inducing kinase (NIK) following NF-kappaB activation. Given that miRNAs act as pleiotropic molecules essential in all cellular events, deregulation of miRNA signature caused by HTLV-1 infection strongly involves the imbalance of molecular network of lymphocytes. Recognition and understanding of the widespread molecular applicability of miRNAs will increasingly have much effect on the development of novel strategies to treat the HTLV-1-associated diseases. Here we discuss our current knowledge of viral miRNAs and virally influenced cellular miRNAs and their relationship to ATL.

Invasive diagnostic imaging technique of coronary atherosclerosis has rapidly developed. For example, intravascular ultrasound(IVUS) is recognized as an essential device for percutaneous coronary intervention to evaluate the vessel wall, vascular lumen and coronary plaque morphologies because of its accuracy for quantitative analysis capability. Recently new imaging modalities such as radio-frequency signal analysis, elastography and contrast harmonic echography have been developed for the evaluation of histological characteristics. Also, optical coherence tomography(OCT), which provides approximately ten-times higher-resolutional cross-section images of the coronary arterial wall in comparison with IVUS, became available in clinical setting. In this article, we review the latest progress of the invasive diagnostic imaging of coronary atherosclerosis.

Acute myocardial infarction(AMI) and unstable angina(UA) are part of a spectrum of clinical disease collectively identified as acute coronary syndrome (ACS) which is the most common proximate cause of sudden cardiac death. In a diagnosis of ACS, electrocardiogram(ECG) is still important. But, it is somewhat difficult to find out abnormal findings of ECG when it is taken in early phase of ACS. Therefore, it is necessary to record ECG on several times and to follow up even if there is no ECG abnormalities at first recording. The effective interventions for patients with ACS, particularly ST-elevation MI(STEMI), are extremely time-sensitive. It is imperative that we evaluate efficient risk stratification, and effective treatment of patients with ACS as quickly as possible.

The success of arterial switch operations for transposition of the great arteries largely depends on faultless coronary translocation and subsequent sufficient myocardial perfusion. Occurrence of late coronary obstructions after arterial switch operation was often reported. We developed the bay window technique as a useful adjunct to prevent coronary complications. Late results of this technique are described. No coronary events occurred (i.e. inclusive of coronary stenosis, myocardial infarction, and coronary death). The bay window technique is an innovative and simple surgical adjunct for translocating complex coronary arteries.

We administered gefitinib to a patient after considering his request to be treated with the drug. Fortunately, he responded favorably to the treatment and did not show signs of serious adverse effects or deterioration of renal functions. The patient was a 69-year-old male who visited an outpatient clinic because of chronic renal failure and was diagnosed with primary lung cancer. He then underwent an operation for lung cancer, but because it had progressed to stage IV the lesion was not completely resected. The patient was unable to receive effective chemotherapy due to the prior chronic renal failure. During best support care, the patient suffered from respiratory failure due to the tumor growth, and his quality of life (QOL) deteriorated. The patient was administered 250 mg of gefitinib orally, which was effective in reducing the tumor, improving his QOL, and prolonging his survival time. With the lack of literature on administering gefitinib to patients with chronic renal failure and evidence supporting the effectiveness of this treatment, the physician in charge should obtain the patient's informed consent before initiating treatment using this anticancer drug.

In 25 patients operated on for idiopathic interstitial pneumonia (IIP) associated with primary lung cancer, we clinically examined the predictive factors related to the acute exacerbation of IIP preoperatively and during operation. Most were male heavy smokers. Ages ranged 57 to 78 years. Standard surgery was performed in 11 patients, extended resection in 4 patients and limited resection in 10 patients. The incidence of postoperative acute exacerbation of IIP was 40% (10 patients). These patients were treated with steroid pulse therapy, 3 patients died due to acute exacerbation but 7 patients recovered. It seemed difficult to anticipate postoperative exacerbation of IIP based on preoperative patients evaluation and the degree of surgical invasiveness. Seven patients were alleviated with erythromycin before and after the operation and 4 patients were alleviated with high-dose steroid during or after surgery, with these patients not developing exacerbation of IIP. From these results, it was suggested that high-dose steroid administration during or after surgery and erythromycin before and after the operation were effective to avoid postoperative exacerbation of IIP.

A case of solitary hemangioma which occurred in the rib. Tumor located in the right seventh rib, incidentally taken X-ray film demonstrated bone tumor, but she had no symptoms. Four years after, chest pain occurred and taken X-ray film, tumor size was inceeced. Computed tomography (CT) showed an expansile, well demarcated lesion, with thin corices and fine trabeclae. Chest wall, from the sixth rib to the eighth rib resection was performed and surgical margin was 4 cm. This case were diagnosed hemangioma of the rib. Hemangiomas of the bone are rare benign vascular tumors that account for less than 1% of all bony neoplasms. These lesions are most often occurring in the vertebral column or in the skull. The localization to the ribs is even more rare, with only sporadic case reports in the literature.

Objectives: Left ventricular dysfunction is known in patients with mitral stenosis, but the incidence and cause remain unclear. The incidence and the factors related to left ventricular dysfunction were investigated in strictly selected patients with isolated mitral stenosis., Methods: This study investigated 33 patients (5 males, 28 females) with isolated mitral stenosis aged 56 +/- 9 years. Left atrial dimension, left ventricular diastolic and systolic dimensions, mitral valve area, and mean transmitral pressure gradient were measured by echocardiography. Left ventricular ejection fraction was measured by Simpson's method. Patients were divided into two groups according to the ejection fraction (< 50%, > or = 50%)., Results: Seven patients (21%) had decreased left ventricular contraction and 26(79%) had normal contraction. The incidence of patients with atrial fibrillation in the low ejection fraction group was significantly higher than in the normal ejection fraction group(86% vs 31%, p < 0.01). There were no significant differences in the severity of mitral stenosis or other echocardiographic indices between the two groups., Conclusions: Low ejection fraction was present in 21% of patients with mitral stenosis. Since atrial fibrillation was more common in patients with low ejection fraction than those with normal ejection fraction, the rhythm disturbance may be related to the decreased left ventricular contraction.

A 64-year-old woman with hypertension presented with a left atrial giant mass during the treatment of congestive heart failure. She was admitted to our hospital for intensive treatment. Transesophageal echocardiography demonstrated a cauliflower-like, large (3 x 2 cm), mobile echogenic mass attached to the left atrial wall. There were no signs of systemic embolism. Anticoagulant therapy was started. Repeated echocardiography showed the mass was reduced gradually and had diminished on the 10th day. She remained asymptomatic during the anticoagulant therapy. The diagnosis was thrombus based on the response to treatment. Surgical removal should be considered for such a large thrombus, but the present case of giant thrombus was successfully treated by anticoagulants without systemic complication.

A 17-year-old man with supravalvular aortic stenosis associated with Williams syndrome was admitted to our hospital for intensive treatment for intractable infective endocarditis. The patient had a history of percutaneous balloon valvuloplasty for aortic stenosis in 1992. He was well until late in 1999, when he had a high temperature after dental work-up. The diagnosis was infective endocarditis but antibiotic therapy was not effective. He was transferred to our clinic. Transthoracic echocardiography demonstrated bicuspid aortic valve, supraaortic stenosis, mitral valve prolapse with severe regurgitation and scattered vegetations on the anterior mitral and aortic valves. In addition, transesophageal echocardiography showed innumerable mobile vegetations located from Valsalva's sinus to the descending aorta. Aortic root and arch replacement with a homograft and mitral valve replacement with an artificial valve were successfully performed to eliminate the infective endocarditis. In the present patient, the flow jet across the supraaortic stenosis seemed to cause a predisposition to severe endocarditis.

A 23-month-old female infant with situs solitus, atrioventricular and ventriculoarterial discordance, pulmonary atresia, ventricular septal defect, who had received modfied right Blalock-Taussig shunt (4 mm Gore-Tex tube) at the age of 2 months, was carried out double switch operation with left atrial augumentation by in situ autologous pericardium. The spatial position of the heart was mesocardia. The systemic right ventricular end-diastolic volume was 100% of normal, and the pulmonic left ventricular end-diastolic volume was 105% of normal. Atrioventricular valvular regurgitation was not found. Postoperative course was uneventful. On the 28th postoperative day,she discharged from hospital. The disadvantage of double switch operation is difficulty of atrial switch procedure, because most patients have a small atrium due to dextrocardia or mesocardia. For the purpose of atrial augumentation without artificial materials, our modified Senning procedure is useful. But longer follow-up is necessary to determine whether this technique is indeed beneficial.

We experienced 2 cases of septic children after open heart surgery. Both of them recovered by using exchange blood transfusion technique. We use irradiated and dialysed fresh blood for exchange blood transfusion. After this procedure, they recovered from sepsis, as the datas improving, white blood cell reduced from 19,700 +/- 3,710 to 8,200 +/- 2,360, CRP reduced from 5.46 +/- 1.65 to 1.89 +/- 0.70, T-Bil reduced from 7.61 +/- 2.66 to 3.02 +/- 0.89, and BUN reduced from 525.92 +/- 6.64 to 19.76 +/- 5.34. Furthermore, blood pressure and urine volume were stable between exchange blood transfusion, although after open heart surgery. Therefore this procedure has benefits for the compromised, septic patients, performed open heart surgery, because of its stability of the circulating circumstances. And using the irradiated and dialysed fresh blood provides stable condition eventhough under high dose catecholamine use.

A male infant with right isomerism, single ventricle, severe pulmonary stenosis (PS), and total anomalous pulmonary venous return (TAPVR) underwent successful staged Fontan operation. Pulmonary vein drained into right superior vena cava without a stenotic lesion. Common pulmonary venous chamber was also connected with the atrium by a thin bridging vein. Blalock-Taussig shunt was performed at 2-month-old. Because of progressive pulmonary congestion, an anastomosis between common pulmonary venous chamber and the atrium followed 4 days after the first shunt. Bidirectional cavopulmonary shunt was performed 7 months after the second operation. Because of progressive atrioventricular valve regurgitation, repeated bandings of the shunt were required to regulate the pulmonary flow. Total cavopulmonary connection was completed 9 months after the BCPS. Staged operation is a useful strategy to acquire an appropriate pulmonary blood flow for the isomeric heart with TAPVR and severe PS.

With the development of prosthetic valves with excellent hemodynamics and durability, aortic valve replacement has become the first choice in the surgical treatment of valve disease. The significant improvement in mortality rates after open-heart surgery has resulted in a demand not only for good hemodynamics but also for a better postoperative quality of life. Because mechanical prosthetic valves are associated with postoperative complications and patients receiving them must remain on anticoagulant therapy for life, and bioprosthetic valves are not sufficiently durable, valve repair techniques are being reevaluated and new ones developed. The indications for valve repair have been extended in mitral valve disease due to the decreased incidence of rheumatic valve disease and increase in degenerative tissue valve disease. Reports indicate that both acute and long-term postoperative results are superior after valve repair compared with valve replacement. Similarly, although some reports on the results of aortic valve repair indicate that the standard techniques do not yield satisfactory long-term results, others suggest that newer techniques may improve the results in both the acute and long-term, chronic phase.

Objectives: Recent advances in ultrasound technology allow reconstruction of images from stored radiofrequency information and creating of M-mode echocardiograms along an M-mode cursor of any direction (anatomical M-mode echocardiography)., Methods: The accuracy of the measurements obtained by anatomical M-mode echocardiography was evaluated by comparing the measurements with those by B-mode echocardiography with or without harmonic imaging in 8 normal subjects and 14 patients with cardiac disorders. Measurements used the left ventricular short-axis image in 4 different directions (0 to 6, 3 to 9, 1 to 7 and 5 to 11 o'clock)., Results: Anatomical M-mode and B-mode measurements showed good linear relationships with correlation coefficients of 0.90 to 0.99 in any of the 4 directions. However, measurements in the lateral direction (3 to 9 o'clock) showed larger errors than those in the other directions (p < 0.05). With the use of harmonic imaging, the errors tended to become smaller, although it did not reach statistical significance., Conclusions: Anatomical M-mode echocardiography accurately measures the left ventricular internal diameter in any direction except the lateral direction. Harmonic imaging is useful to decrease the errors.

We report a method for estimation of age from teeth using the racemization of amino acids (racemization method). This method is based on the characteristics of the constant age-related increase in the amount of D-aspartic acid in dentin. We estimated age by measuring the ratio of D-aspartic acid to L-aspartic acid, i.e. the ratio of racemization ¿ratio of D/L, ln[(1 + D/L)/(1 - D/L)]¿. Because different D/L ratios have been obtained from different teeth in the same individuals and from different sites of dentin in the same tooth, we usually prepare bucco-lingual longitudinal sections at the central part of each tooth, and prepare samples of powdered whole dentin. This powder is then mixed and used to measure the D/L ratio in the dentin. To accurately estimate age from forensic specimens, we simultaneously measured the D/L ratios in more than four control teeth of the same type obtained from subjects of known age. Use of control teeth is necessary because it is sometimes difficult to maintain constant running conditions for gas chromatography to obtain reproducible values in different runs. Therefore, for every measurement, we determined an equation for calculating age from the D/L ratios of control teeth, and estimated the age of the specimen tooth by substituting in its D/L ratio. The most reliable results were obtained using samples of lower incisors or premolars, which are single-rooted teeth with a relatively small volume of dentin. Thus sampling of the dentin is easier than for other teeth. It is better to keep control teeth desiccated because racemization does not proceed readily under such conditions. The deviation from the actual age in the cases we examined was less than 3 years. Thus, racemization of amino acids can be used for accurate estimation of age from teeth.

Dilated cardiomyopathy (DCM) is often accompanied by severe mitral regurgitation (MR) which deteriorates the clinical course. Mitral reconstruction for severe MR may improve the symptoms and prognosis. Five patients with DCM and one patient with dilated phase of hypertrophic cardiomyopathy underwent mitral reconstruction for severe MR (4 males, 2 females, mean age 50 +/- 17 years) from 1983 to 1995. Their New York Heart Association (NYHA) functional class and findings of echocardiography and cardiac catheterization were compared before and after surgery. Five patients underwent annuloplasty and one patient underwent mechanical valve replacement. There was no operative or in-hospital death. NYHA class improved from 3.2 to 1.8 (p < 0.05). The degree of MR was reduced from 3.5 to 1.2 (p < 0.05), and left ventricular end-diastolic pressure decreased from 18 +/- 7 to 13 +/- 8 mmHg (p < 0.05). Left ventricular fractional shortening, ejection fraction and cardiac index (2.4 +/- 0.4 to 2.7 +/- 1.1 l/min/m2) did not change significantly. Two patients died within one year due to exacerbation of congestive heart failure (2.5 month later) or sudden death (6 months later). Three patients died at 21 months, 5 and 8 years after the operation (renal insufficiency, heart failure, sudden death, respectively). Mitral reconstruction improved the symptoms in patients with DCM and severe MR in the short term. However, 5 of 6 patients died in the intermediate or long-term. Mitral reconstruction may be a therapeutic option for early outcome in patients with refractory congestive heart failure due to DCM and severe MR.

A 21-year-old man with dilated cardiomyopathy recovered from terminal heart failure with the long-term use of the left ventricular assist system (LVAS). His heart failure was refractory to maximum medical therapy including beta-blockade and intravenous catecholamines. Application of LVAS restored renal and hepatic function and even cardiac function. Left ventricular size decreased from 79 to 57 mm, and cardiac index increased from 1.6 to 2.2 l/min/m2. After 6 weeks of LVAS application, he could walk in the ward and could start exercise on a bicycle ergometer 3 months after. Ninety-five days after implantation, the LVAS could be removed. His general condition remained good and stable for more than a year after the removal of the LVAS. Timely application of LVAS seems to be one of the most important factors for successful circulatory support. Detailed criteria for LVAS application are not established yet.

This study estimates the perioperative left ventricular function of VSD and TOF by using Pressure-Volume (PV) loop. The most characteristic hemodynamic change of VSD is a decreased volume load. Although ejection fraction significantly decreased after the operation, this change did not mean a deteriorated left ventricular contractility. Emax, which is an index of left ventricular contractility on PV loop, improved and pressure-volume area (PVA) which is correlated with 02 consumption of left ventricule was reduced. Mechanical efficiency of left ventricular energetic state did not significantly change. Left ventricular volume and ejection fraction in TOF slightly increased after the operation. Increased ejection fraction reflected a compensation to volume overload after surgery. Emax slightly decreased and PVA increased. Meanwhile mechanical efficiency increased after the operation indicating well preserved ventricular contractility. In particular, the shape of PV loop of both VSD and TOF eventually became usual square after operation due to recover of the isovolumic contraction and relaxation phase. These characteristics had better to be concerned for improvement of surgical techniques as in conotruncal repair, particularly in TOF in the setting of better quality of life with low atrial pressure.

The efficacy of both preliminary and continuous hemodialysis during extracorporeal circulation (ECC) was evaluated in the pediatric population. Preliminary hemodialysis of the priming solution was initiated in eight children (HD-I group), while both preliminary and continuous hemodialysis during ECC was performed in another eight children (HD-II group). The control group which consisted of eight children did not undergo hemodialysis. Urine volume during ECC, perfusion pressure, and water balance were measured and compared among the three groups. In the HD-I and HD-II groups, the total urine volume during ECC was greater than in control group, but there was no significant difference. In the control group, the perfusion pressure temporally decreased after the pump was operating for five minutes, but the perfusion pressures remained stable in the HD-I and HD-II groups. Therefore, preliminary hemodialysis appears to prevent the "initial drop" seen in perfusion pressures. Water balance in the control group tended to be in positive balance, but both the HD-I and HD-II groups appeared to be in negative balance. Significant differences between HD-I and HD-II in contrast to the control group (p < 0.05) and p < 0.04, respectively) were observed. Preliminary hemodialysis is useful during ECC in infants and children because by maintaining perfusion pressure urine output remains adequate while a net negative water balance is achieved. Preliminary hemodialysis will increase the continuous hemodialysis in infants who are subjected to prolonged extracorporeal circulation.

Anomalous origin of the left main coronary artery from the pulmonary artery (Bland-White-Garland syndrome) is a rare congenital anomaly. Intramyocardial coronary flow dynamics by pulsed Doppler echocardiography were studied in three patients with this syndrome who underwent surgical repair by Hamilton's method. Before surgery, the intramyocardial flow at the ventricular septum showed a retrograde velocity pattern which had two peaks in systole and diastole in all patients. After surgery, two patients with successful repair showed a biphasic intramyocardial flow pattern which consisted of retrograde and antegrade flows in systole and diastole, respectively. In contrast, one patient who had a residual shunt between the left coronary artery and the pulmonary artery showed a biphasic pattern which had antegrade flow in systole and retrograde flow in diastole. These results may suggest that the evaluation of postoperative intramyocardial coronary flow velocity pattern by pulsed Doppler echocardiography is useful for detecting a residual shunt after surgical repair of Bland-White-Garland syndrome.

Long-term conventional pulmonary artery banding deteriorates ventricular function in patients who have univentricular atrioventricular connection with subaortic obstruction. Protection of the pulmonary vascular bed and early relief of subaortic stenosis is essential to improve the outcome after Fontan operation. From January 1995 through January 1996, three infants underwent open heart palliation because of univentricular atrioventricular connection with subaortic stenosis. All infants had discordant ventriculoarterial connection. One infant underwent the Norwood procedure (patient 1). Two infants underwent palliative arterial switch operation, one with repair of aortic arch and a Blalock-Taussig shunt (patient 2) and the other with endoluminal pulmonary artery banding (patient 3). Patient 3 required a subsequent conventional pulmonary arterial banding. The postoperative recovery period were smooth in the all infants. All infants kept sufficient PO2 ranging from 34 to 37 mmHg postoperatively. On follow-up after 16 months pulmonary artery index decreased in patient 1. On the other hand, angiogram demonstrated satisfactory pulmonary arterial growth in patient 2. There were two late death, occurring in patient 1 (sudden death) and patient 3 (pneumonia). Patient 2 awaits a Fontan type procedure. It is difficult to adjust appropriate blood flow through a Blalock-Taussing shunt and a surgically isolated pulmonary artery is capable of inducing pulmonary distortion after Damus-Norwoood type operation. Whereas natural regulation of the pulmonary arterial blood flow by a restrictive ventricular outflow tract is come up after a palliative arterial switch operation. Palliative arterial switch operation is an useful alternative open heart palliation for neonates and early infants who had univentricular atrioventricular connection with subaortic stenosis.

An outer surface of a 4 mm polytetrafluoroethylene (PTFE) prosthesis, Gore-Tex thin-walled stretch graft, was treated by the fibrin glue spray with a 1.0 kg/m2 compressed air. The distance between the PTFE graft and a spray-nozzle was within 0.5 cm. After the treatment, an outer surface and a cross-section of the PTFE graft were examined under a scanning electron microscope. Fibrin glue mended the gap of the PTFE fibril of an outer surface and a fibrin glue layer adhered firmly to the graft. The fibrin glue spray treatment may prevent postoperative serous fluid leakage.

A radical correction involving Vargas's method and a direct anastomosis between the left pulmonary vein (PV) and left atrium (LA) was performed in a 29 day-old infant with supracardiac type Ib total anomalous pulmonary venous return (TAPVR) and an unusual form of the left PV. the left upper and lower PVs drained into a left "common" PV that was just behind the LA, and then into the right pleural cavity. The left common PV was located cephalad to the normal course and received blood from the right lower and upper PVs and drained into the supra-vena cava (SVC). The junction of the SVC and the right PV was slightly stenotic. Vargas's method is a useful technique for Darling's classification Ib TAPVR even in cases without the common PV situated behind the LA. But this patient had a left common PV and it was possible either to anastomose the common PV and LA directly or to perform Vargas's technique. We performed both procedures to prevent left PV obstruction (PVO). Cineangiography performed 2 months after surgery showed that a large amount of blood from the right and left PV drained into the LA through the common PV-LA route. These procedures, which create a dual PV channel, reduce the risk of PVO, so they are useful for radical correction of Darling's Ib type TAPVR.

A successful palliative arterial switch operation with arch repair in a 30-day-old infant with tricuspid atresia, ventriculoarterial discordance, subaortic stenosis, coarctation of the aorta, and aortic arch hypoplasia is reported. A concomitant Blalock-Taussig shunt was required because of insufficient pulmonary blood flow through the restrictive bulboventricular foramen. Postoperative arterial oxygen saturation was maintained around 90% and the pressure gradient between the left ventricle and the pulmonary artery was 45 mmHg according to a Doppler echocardiogram. The infant becomes free of subaortic stenosis and is awaiting the subsequent Fontan operation. This alternative palliative operation for neonates and early infants with double inlet ventricle, subaortic stenosis, and arch hypoplasia offers some advantages on the Fontan operation for avoiding pulmonary arterial distortion and subaortic stenosis followed by ventricular hypertrophy.

Anomalous origin of the left coronary artery (ALCA) arising from the pulmonary artery is a rare anomary, and causes myocardial infarction and death in children. Direct implantation of ALCA to the aorta was performed in the 6-year-old female patient. A large button of pulmonary arterial wall resected with ALCA could spare the shortage of the distance between the aorta and the ALCA. Postoperative catheterization displayed non-stenotic left coronary artery. Left ventricular function assessed by echocardiography showed improvement. Cold area in early stage postoperative myocardial imaging disappeared one year after the surgery, which suggested the hibernation of the preoperative myocardium.

To eliminate the cytokines and leukocytes-induced proteases which could cause the multisystem organ failure postoperatively, we performed a preliminary hemodialysis to the priming solution and a continuous hemodialysis during extracorporeal circulation (ECC) in 5 children (HD group), and neither hemodialysis in another 5 children (Control group). We measured the plasma level of Interleukin-1 (IL-1), Interleukin-6 (IL-6) and leukocyte elastase (Ease) to evaluate the efficiency of these hemodialysis. Urine level of alpha 1-microglobulin (MG), beta 2-MG, urine volume, water balance and perfusion pressures during ECC were also measured to evaluate its protective effect for the renal function. IL-1 level significantly decreased in HD group 1 and 12 hours after operation. Not only IL-6 and Ease during ECC but also alpha 1- and beta 2-MG 1 hour after operation decreased in HD group so hemodialysis could be useful to eliminate the cytokines. Ease and could protect the renal function. Water balance and perfusion pressures also obtained good results with these hemodialysis. Plasma osmolality and glucose level changed within the normal range in HD group, conversely over the normal range in control group. We conclude this method is useful for neonatal, ECC.

The distribution of centrilobular emphysematous lesions in the transverse plane was studied with high-resolution CT (HRCT) images of inflated and fixed lungs obtained from 49 patients with centrilobular emphysema (CLE). The transaxial distribution of low attenuation areas (LAA) was assessed quantitatively on HRCT images obtained from 21 patients. LAA in the outer layer accounted for a significantly lower ratio of the lung parenchyma than did those in the inner layer. Macroscopic analysis of 31 lobes obtained from 28 patients with CLE was done by point counting. The inner layer had many more LAA than the outer layer. Bronchiolar lesions were examined under the microscope for their relation to the observed variations in distribution in CLE. No significant differences were found in the bronchiolar lesions between the inner and outer layers. These data show a characteristic distribution pattern of CLE in the transverse plane. They also suggest that the disparity in distribution of LAA between the inner and outer layers is not influenced by the grade of bronchiolar pathological lesions.

Transplantation of the coronary arteries is an essential and critical part of the arterial switch operation, which is considered to be the treatment for most neonate with transposition of the great arteries. The presence of an intramural segment of coronary arteries, close coronary ostia adjacent to each other increase the risk of coronary twisting and kinking after transplantation. This case has an unusual coronary anatomy characterized by two coronary ostia arising from the same aortic cusp, intramural segment in a proximal part of the left anterior descending coronary artery (LAD) and slit-like narrow ostium of LAD. This neonate was successfully operated on using Aubert modification which is the arterial switch operation without coronary transplantation. In this method, the reconstruction of the new pulmonary trunk must be accomplished by direct anastomosis of the posterior wall in order to allow for a growth of pulmonary artery. Because of a patch retraction which was used to create its anterior wall, pulmonary stenosis may occur. In case of reoperation for pulmonary stenosis, we have only to enlarge the anterior part of the pulmonary artery to take down the stenosis. Coronary transplantation should be avoided in TGA neonate with an unusual coronary anatomy including intramural coronary artery and closed coronary ostia adjacent to each other.

One and one half ventricle repair consisting of the Glenn operation and the right ventricular outflow tract reconstruction was performed in a 4-year-old boy. The diagnosis of this patient was pure pulmonary atresia. He had undergone Brock's operation, Blalock-Taussig shunt and a central shunt at neonatal period. At the time of one and one half ventricle repair, the connection between the superior vena cava and the right atrium was left open. Although the RVEDV was 34.3% of normal, the topology of the right ventricle was tripartite. At three months after one and one half ventricle repair, RVEDV increased up to 52.7% of normal. This data suggests that a further definitive surgery, i.e., complete biventricular repair could be applicable in the future.