Perioperative course in two cases of tetralogy of fallot with portal venous atresia.

We report here two rare surgical cases of tetralogy of Fallot with portal venous atresia. Both patients, previously diagnosed with tetralogy of Fallot, developed portal hypertension and showed hematemesis and/or melena. One patient needed endoscopic sclerotherapy. Intracardiac repair was performed under cardiopulmonary bypass (CPB) with moderate hypothermia. After surgery, one of the patients developed cardiogenic shock due to cardiac tamponade with accumulated chyle in the pericardial sac in the ninth postoperative day, and showed deteriorated liver function, suggesting that liver might have been vulnerable because of the absence of portal blood.

[Transportation for a pediatric heart transplant candidate to Germany].

A 8-year-old girl who underwent a total cavopulmonary connection as an one-staged repair for tricuspid atresia (Ic) had had a progressive dilated cardiomyopathy. Despite of any medical support, her left ventricular ejection fraction had been ranged from 10% to 20% since 4 months after Fontan operation. This patient was decided to apply for a heart transplantation in Germany 17 months after Fontan operation. An administration of the sufficient amount of inotropics and oxygen, volume resuscitation, reduction of the peripheral circulation at the time of take-off, and a meticulous monitoring may contribute to the successful transportation of the critically ill patient.

Atrioventricular groove patch plasty for anatomically corrected malposition of the great arteries.

OBJECTIVE: In anatomically corrected malposition of the great arteries, dextroposition of the posterior pulmonary artery and levoposition of the anterior aorta are associated with the leftward deviation of the proximal portion of the right coronary artery away from the right atrioventricular groove. This anatomic feature allows a transannular subpulmonary patch plasty of the right ventricular outflow tract along the right atrioventricular groove between the right coronary artery and the tricuspid anterior anulus (ie, atrioventricular groove patch plasty) for relief of subpulmonary stenosis without jeopardizing the right coronary artery. METHODS: This report describes the midterm results of a new surgical technique, atrioventricular groove patch plasty with a monocuspid transannular patch for subpulmonary stenosis, in 3 patients with anatomically corrected malposition of the great arteries, along with a concomitant closure of ventricular septal defects. RESULTS: Postoperative catheterization revealed adequate relief of pulmonary stenosis, with a pressure gradient of 8.0 +/- 3.5 mm Hg and with normalized right ventricular pressure (33 +/- 10 mm Hg), contributing to excellent midterm results with no late death and reoperation during a postoperative follow-up period of 70 +/- 47 months. CONCLUSION: This technique provides a promising alternative to Rastelli-type conduit repair for subpulmonary stenosis in anatomically corrected malposition of the great arteries.

[Midterm results of total aortic root replacement with pulmonary autograft (Ross operation)].

Between February 1995 and December 1999, 18 patients underwent Ross operation. Age at the operation ranged from 2 to 31 years. Diagnosis includes congenital aortic stenosis and/or regurgitation in 15, and adult aortic regurgitation in 3. In all cases autograft was implanted by the method of total aortic root replacement, associated with annuloplasty for the dilated aortic annulus in 2 and aortoventriculotomy by the Konno procedure in 3 (Ross-Konno). Right ventricular outflow tract was reconstructed by a pulmonary homograft in 12, a xenopericardial conduit in 3, or the other reconstructive procedures with autologous tissue and outflow patch in 3. There was no operative and late death. Reoperation was needed in 1 patient due to stenosis of pericardial conduit 4 years after the initial operation. Pressure gradient across implanted autograft valve was negligible (4.8 +/- 0.5 mmHg), and echocardiography revealed no aortic regurgitation in 12 cases and trivial to mild in 6, over a mean follow-up period of 23 +/- 18 months (range 2 to 60 months), signifying excellent durability of implanted autograft. Right ventricular outflow tract reconstruction with the homograft resulted in excellent mid-term performance as showing pressure gradient of 9.0 +/- 4.6 mmHg and no regurgitation in 11 of 12 cases, whereas pressure gradient was 17.9 +/- 13.1 mmHg in the patients underwent the other reconstructive procedures. We conclude that Ross procedure associated with the concomitant procedures to adjust the size discrepancy between the native aortic annulus and autograft has provided good midterm results with excellent autograft durability. And this procedure was thought to be a preferable method for children as well as young adults with congenital aortic stenosis.

Telomerase activity as an indicator of malignant potential in iodine-nonreactive lesions of the esophagus.

BACKGROUND: Iodine-nonreactive lesions of esophageal epithelium often are associated with dysplasia and carcinoma. The authors examined the usefulness of telomerase activity as an indicator for esophageal carcinogenesis in such lesions. METHODS: Telomerase activity was measured using the telomeric repeat amplification protocol assay in 18 samples of iodine-nonreactive lesions apart from the primary tumor in surgically resected specimens obtained from patients with esophageal squamous cell carcinoma (ESCC) and 55 endoscopic punch biopsies of iodine-nonreactive lesions obtained from 25 patients with ESCC and 30 patients who had undergone endoscopic examination for other reasons. RESULTS: Ten of 18 iodine-nonreactive samples (56%) obtained from surgically resected specimens showed telomerase activity. In all ten telomerase positive samples, carcinoma in situ (CIS) was observed in iodine-nonreactive mucosa by light microscopy. In eight telomerase negative samples, no tumor tissue was observed in iodine-nonreactive lesions. In a parallel study, telomerase activity was detected in 28 of 55 endoscopic punch biopsy specimens (51%). CIS was observed in 25 of 28 iodine-nonreactive lesions with positive telomerase activity (89%), and tumor tissue was not observed in the other 3 samples (11%), which included 2 cases of severe dysplasia and 1 case of moderate dysplasia. No tumor tissue was observed in any of the 27 telomerase negative samples. CONCLUSIONS: Positive and negative telomerase activity was found to be correlated with the presence and absence, respectively, of immortalized tumor cells in iodine-nonreactive lesions. The measurement of telomerase activity in iodine-nonreactive lesions independently contributes to the selection of an appropriate therapeutic strategy.

Protein tyrosine phosphatases from amphioxus, hagfish, and ray: divergence of tissue-specific isoform genes in the early evolution of vertebrates.

Since separation from fungi and plants, multicellular animals evolved a variety of gene families involved in cell-cell communication from a limited number of ancestral precursors by gene duplications in two separate periods of animal evolution. In the very early evolution of animals before the separation of parazoans and eumetazoans, animals underwent extensive gene duplications by which different subtypes (subfamilies) with distinct functions diverged. The multiplicity of members (isoforms) in the same subtype increased by further gene duplications (isoform duplications) in the first half of chordate evolution before the fish-tetrapod split; different isoforms are virtually identical in structure and function but differ in tissue distribution. From cloning and phylogenetic analyses of four subfamilies of the protein tyrosine kinase (PTK) family, we recently showed extensive isoform duplications in a limited period around or just before the cyclostome-gnathostome split. To obtain a reliable estimate for the divergence time of vertebrate isoforms, we have conducted isolation of cDNAs encoding the protein tyrosine phosphatases (PTPs) from Branchiostoma belcheri, an amphioxus, Eptatretus burgeri, a hagfish, and Potamotrygon motoro, a ray. We obtained 33 different cDNAs in total, most of which belong to known PTP subfamilies. The phylogenetic analyses of five subfamilies based on the maximum likelihood method revealed frequent isoform duplications in a period around or just before the gnathostome-cyclostome split. An evolutionary implication was discussed in relation to the Cambrian explosion.

Clinical significance of telomerase activity in the non-cancerous epithelial region of oesophageal squamous cell carcinoma.

BACKGROUND: The aim of this study was to examine telomerase activity in affected and adjacent tissue in patients with oesophageal squamous cell carcinoma (SCC). METHODS: Telomerase activity was measured in oesophageal SCC cells, normal oesophageal culture cells, primary cancer tissues and adjacent normal tissues from patients with oesophageal SCC using a polymerase chain reaction-based assay. RESULTS: All oesophageal SCC cells had telomerase activity, whereas normal cultured cells showed no activity. All 57 cancer tissues showed telomerase activity compared with only five (10 per cent) of 50 normal tissues. Cancer cells infiltrating the vessels of mucosal or submucosal tissues in non-cancerous regions were detected in four of five telomerase-positive normal tissues, whereas such infiltration was detected in only three of 45 telomerase-negative normal tissues. CONCLUSION: In patients with oesophageal SCC, measurement of telomerase activity in normal epithelium is a highly sensitive method of detecting the microinvasion of cancer cells.

[Telomerase assay for diagnosis of esophageal cancer].

Esophageal squamous cell carcinoma is one of the aggressive diseases that has poor outcome. Therefore it is appeared that early diagnosis is very important for improving its outcome. Iodine staining method is useful for detecting the abnormal squamous epithelium and unstaining lesions by iodine contain the early esophageal cancers. Recently, telomerase activity that provides an immortal capacity for the cells has been measured in many tissues. We measured the telomerase activity in the samples of unstaining lesion by iodine using a polymerase chain reaction-based assay and described the relation between telomerase activity and histopathological findings.

[Some techniques for the correction of the congenital heart disease with autologous flap].

14 living flaps in 8 children were used to repair for the congenital heart disease, because artificial material and xeno grafts were shrunk in growing children but living flaps were going to glow with children. It were possible to use the living flaps to reconstruct of pulmonary outflow in truncus arteriosus, for septoplasty in partial anomarous of pulmonary venous return and reconstruction of unloofed coronary sinus in endocardial defect with triatriatum and intraatrial tunnel in TCPC or Fontan type operation.

Case report: Metachronous early gastric carcinoma in a reconstructed gastric tube after radical operation for oesophageal carcinoma.

The incidence of double cancer has increased as a result of the significant advances in both diagnostic procedures and anti-cancer therapy, and as the outcome of oesophageal squamous cell carcinoma (ESCC) has improved, the number of cases of double cancer of the oesophagus and stomach has also increased. Two patients with metachronous early gastric carcinoma in a reconstructed gastric tube were successfully treated after subtotal oesophagectomy for ESCC. These cases have shown that early diagnosis of second cancer is very important for curative therapy.

[Comparison of left ventricular pressure-volume loops measured by two-dimensional echocardiography and a conductance catheter].

A left ventricular pressure volume loop (PV loop) is useful not only in evaluating cardiac function, but also in predicting the postoperative hemodynamic state. There are two methods available for the intraoperative measurement of a PV loop: a conductance catheter and two-dimensional echocardiography. Although the accuracy of conductance catheter is well established for the measurement of ventricular volume, however, the accuracy of echocardiography remains controversy. This paper describes the relationship of parameters including volumes and Emax calculated with each method. Six patients who underwent open heart surgery were included in this study. Comparison of the absolute volumes at four points at the corners of the PV loop showed a Pearson's correlation coefficient of 0.62 (p-value < 0.01). In comparison with the relative volumes at four points which reflect the preoperative to postoperative change ratio, the correlation coefficient was 0.74 (p < 0.01). The correlation coefficients were 0.76 (p = 0.16) and 0.63 (p = 0.29) for the ratio of the end-diastolic and end-systolic volume respectively. Each preload-varying "Emax: out" (r = 0.84, p < 0.01) were highly correlated. As a predictor of cardiac oxygen consumption, each PVA (r = 0.84, p < 0.01) was highly correlated. We conclude that the measurement of left ventricular volume using two-dimensional echocardiography is as reliable as the conductance catheter.

Altered p16/MTS1/CDKN2 and cyclin D1/PRAD-1 gene expression is associated with the prognosis of squamous cell carcinoma of the esophagus.

The p16/MTS1/CDKN2 gene and the cyclin D1/PRAD-1 gene cooperatively regulate cyclin-dependent kinase 4-mediated phosphorylation of pRB in the cell cycle of normal cells. p16/CDKN2 gene and cyclin D1/PRAD-1 gene alterations have been detected in squamous cell carcinoma cell lines and in several primary squamous cell carcinomas of the esophagus. We immunohistochemically assessed p16 and cyclin D1 expression in 111 squamous cell carcinomas of the esophagus after evaluation of the antibodies against p16 and cyclin D1 protein using four squamous cell carcinoma cell lines. Loss of p16 expression was detected in 56 of 111 cases (50%). The mean number of metastatic lymph nodes without p16 expression was significantly higher than the number of nodes with p16 expression (P = 0.04). The postoperative survival rate for patients without p16 expression was significantly lower than that of patients with p16 expression (P = 0.04). Cyclin D1 overexpression was found in 28 of the 111 cases (25%) and correlated with distant organ metastasis after curative surgery (P = 0.05). The survival rate of patients with cyclin D1 overexpression was significantly lower than that of patients without cyclin D1 overexpression (P = 0.01). A positive correlation between the loss of p16 expression and cyclin D1 overexpression was observed (P = 0. 03). The loss of p16 expression and overexpression of cyclin D1 may be useful prognostic indicators in patients with squamous cell carcinomas of the esophagus. It may be possible to select more suitable treatment for patients with squamous cell carcinomas of the esophagus by evaluating the status of p16 and cyclin D1 expression.

[Successful direct embolectomy for acute massive pulmonary thromboembolism].

A 35-year-old woman was bedridden because of a fall and developed pulmonary embolism after venous thromboembolism of lower extremity. Although anticoagulation therapy was immediately carried out, hemodynamics deteriorated gradually into the state of class IV in Green-field's classification. As a result, an urgent surgical procedure was performed. Under moderate hypothermic cardio-pulmonary bypass, the aorta was cross-clamped and the pulmonary artery was opened. An 18 gram clot was taken out directly and other small pieces of clot were removed with a fiberscope, a Fogarty catheter and an aspirating tube. After surgery the patient recovered from symptoms efficiently. Some cases of acute pulmonary thromboembolism have been reported, however, the generalized classification and surgical indication for this disease have not been established yet. But, as all of reports mentions, surgical procedure is recommended positively to these cases that resist anticoagulation therapy.

[Clinicopathological study on primary lung cancer--immunohistochemical expression of p53 suppressor gene and bcl-2 oncogene in relation to prognosis].

Two hundred and eleven surgically resected primary lung tumors were studied immunohistochemically. According to histologic type, they were 129 adenocarcinomas, 56 squamous cell carcinomas, 4 small cell carcinomas, 8 large cell carcinomas, 8 adenosquamous cell carcinomas, 5 so-called carcinosarcomas and 2 other tumors. Immunohistochemical expression of p53 and bcl-2 was studied in relation to the disease-free survival. Among the 211 patients with lung cancer, 109 were positive for p53 expression, and there was no significant relationship between p53 expression and sex, or clinicopathological stage and size of the tumor, although the patients with squamous cell carcinoma had a significantly higher frequency of p53 expression than those with adenocarcinomas. The frequency of p53 expression was significantly higher in the patients with poorly differentiated adenocarcinomas than in those with other histologic types. Seventy four of the 211 patients were positive for bcl-2 expression and bcl-2 expression was higher in the stage I patients and patients with small lung tumors 2cm or less in diameter than in the other patients. The patients with adenocarcinoma had a higher frequency of expression than those with squamous cell carcinoma but no difference was found in the histological differentiation of the tumor. The 5-year survival of patients positive for p53 expression was poorer than that of those with negative expression and the survival rate was higher in the patients positive for bcl-2 expression than in those with negative expression. These findings suggested that the expression of p53 and bcl-2 is a useful marker of follow-up and prognosis, but will require more data concerning the mechanism of carcinogenesis. Seven cases of primary lung cancer were examined for genetic abnormality of the p53 gene. cDNA was synthesized from total RNA of primary tissues of lung cancer using oligo (dT) primer and reverse transcriptase and polymerase chain reaction (RT-PCR), and PCR-single strand conformation polymorphism (SSCP) analysis were performed. Five patients gave a positive result upon PCR-SSCP analysis of the p53 gene. To confirm the results of PCR-SSCP analysis, their nucleotide sequences were further analyzed and four of them had point mutations at different codons (154, 176, 207, 236) and one had deletion of one nucleotide (245) in exon 5 and 8. Fifteen percent of 26 patients with small peripheral lung adenocarcinomas less than 2cm in diameter were already advanced in stage and various factors such as vascular invasion, pleural involvement and degree of scar grade were higher than in patients with clinicopathological stage I. In advanced cases, the frequencies of p53 expression was higher than in stage I cases. Concerning the relationship of the degree of scar grade to PDGF-B expression, we demonstrated the production of PDGF-B protein immunohistochemically and the expression of PDGF-B-mRNA by In situ hybridization in the adenocarcinoma cells and macrophages of the lung tumors. However, no significant correlation was observed between the degree of PDGF-B expression and collagen production in the fibrotic focus.

Total cavopulmonary connection without the use of prosthetic material: technical considerations and hemodynamic consequences.

Total cavopulmonary connection with use of an autogenous intraatrial tunnel to create a straight tube between the inferior vena cava and the pulmonary artery was attempted in several types of cardiac anomaly in eight consecutive candidates for the Fontan operation. A small right atrium with an extraordinary location of the inferior vena cava and a short superior vena cava prevented the use of this procedure in two cases. By preserving the crista terminalis and the sinus node and its arteries we prevented the development of postoperative atrial arrhythmias in the short follow-up period, and the P trigger-signal averaged P waves were not different from those of other cardiac anomalies. The proximal stump of the superior vena cava was not incised in any case to enlarge the anastomosis, even when size mismatch between the superior and inferior venae cavae existed, as in a case of bilateral superior venae cavae. Stretching the vessels by approximately 150% was possible and permitted an adequate anastomosis. Cavopulmonary connections via the intraatrial tunnel ensured smooth, nonturbulent, somewhat pulsatile flow without a pressure gradient. We concluded that the creation of an autogenous intraatrial tunnel was possible in many cases without serious complications and that this procedure has potential benefit for the pulmonary circulation in the aspect of pulsatility.