[Ruptured ascending colonic varices in a patient with alcoholic liver cirrhosis treated using topical skin adhesive:a case study].

A 59-year-old man had been admitted to our hospital because of general malaise and abdominal fullness caused by massive ascites. He was diagnosed with alcoholic liver cirrhosis. Following the removal of ascites, he was referred to our department because of hematochezia. Emergent colonoscopy revealed the rupture of ascending colonic varices. Endoscopic variceal sclerotherapy using topical skin adhesive (75% Histoacryl) was performed to treat the colonic varices, which proved to be an effective treatment. Enhanced computerized tomography performed 5 days after the treatment of ascending colonic varices showed complete obstruction of the ileocolic varices without complication. It is important to consider the possibility of ectopic varices when a patient with liver cirrhosis reports bloody stool.

Multicenter and retrospective case study of warfarin and aspirin combination therapy in patients with giant coronary aneurysms caused by Kawasaki disease.

BACKGROUND: To determine the prognosis of patients with giant coronary aneurysms (GA) caused by Kawasaki disease (KD) treated with combined oral warfarin and aspirin. METHODS AND RESULTS: A multicenter follow-up study of 83 patients (65 males, 18 females) with GA who had been treated for > or =3 months with warfarin. Most patients were placed on the combination therapy as soon as the GA was detected and remained on it for 6.0 +/-5.3 years, giving a total of 482 patient-years. Target international normalized ratio of prothrombin time ranged from 1.5 to > or =2.5. During this observational period, 5 patients suffered from 8 episodes of acute myocardial infarction and 1 died. Coronary thrombus formation enforced 6 courses of intracoronary thrombolysis in 3 patients (1-4 times). Consequently, freedom of cardiac events was 92.5% at 1 year and 91% at 10 years and the linearized cardiac event rate was 2.9% patient-year. Hemorrhagic complications occurred on 8 occasions (1 subdural hematoma) in 5 patients, giving 1.7% patient-year. CONCLUSIONS: The combination of warfarin and aspirin has an acceptably high cardiac-event-free survival in patients with GA caused by KD, though it has a certain risk of hemorrhagic complications.

[Case of cardial varices rupture due to danaparoid sodium with portal venous thrombosis].

A 54-year-old man had been admitted to Nara city hospital because of hematemesis and dyspnea caused by physical exertion, and was given a diagnosis of esophago-cardial varices and portal venous thrombosis. He was transferred to our hospital for further examinations and treatments. Ultrasonography (US) and computed tomography (CT) revealed the progression of portal venous thrombosis. Danaparoid sodium was administered to treat the portal vein thrombus. 5 days later, the patient was found to have hematemesis resulting from a cardial varices rupture. After endoscopic variceal ligation (EVL) and endoscopic injection sclerotherapy (EIS) was performed, danaparoid sodium was administered for 2 weeks. After the treatment, portal vein thrombus had almost disappeared. Due to an increased risk of bleeding, cases of esophago-cardial varices with portal venous thrombosis must be treated with care. This is the first report of upper gastrointestinal bleeding due to danaparoid sodium. Danaparoid sodium must be carefully administered when patients have portal venous thrombosis with delicate varices.

Influence of living style and situation on the compliance of taking antihypertensive agents in patients with essential hypertension.

BACKGROUND: As the reason for insufficient control of blood pressure (BP), poor compliance of taking antihypertensive agents is an important issue. In Japan, no prospective study on the compliance of antihypertensive agents has been done. In this study we prospectively investigated the details of the relationship between the compliance of taking antihypertensive agents and living style and situation in hypertensive patients. METHODS: We prospectively examined 121 outpatients with essential hypertension treated with antihypertensive agents for 12 months. Using an oral interview based on the questionnaire sheet, the factors about living style and situations which worsen the compliance were assessed. Also we evaluated the relationship of BP control and season, which was compared between patients with poor compliance and those with good compliance. RESULTS: As for the background of the poor compliance, many factors related to the patients' living style and situation were clarified. On average for 12 months the levels of the compliance and BP showed a significant negative correlation and BP was significantly higher in patients with poor compliance than those with good compliance. However, in the summer season BP did not differ between patients with poor and good compliance, CONCLUSION: Many factors regarding the patients' living style and situation were related to poor compliance. The development of strategy that changes these factors is a future task for improving compliance. This study may also imply that unnecessary antihypertensive agents are prescribed in some patients with poor compliance in the summer season. Determination of the suitable prescription by which to attain optimal individual patients' compliance is important in the treatment of hypertensive patients.

Cryptogenic cirrhosis in the region where obesity is not prevalent.

AIM: Recent studies have demonstrated that obesity is the common feature of cryptogenic cirrhosis (CC) and non-alcoholic steatohepatitis. However, there is little information on CC in the region where obesity is not prevalent. METHODS: The clinical features, and the liver-related morbidity and mortality of CC were analyzed in Japan where the prevalence of obesity is low. Among 652 cirrhotic patients, we identified 29 patients (4.4%) with CC. Of these, 24 CC patients who were followed up for more than 6 months were compared in a case-control study with age-, sex-, and Child-Pugh score-matched controls having cirrhosis of viral etiology. RESULTS: Obesity (BMI>or=25 kg/m(2)), diabetes mellitus, and hypertriglyceridemia were more frequent, and the visceral fat area was larger in the CC patients than in the controls. The indices of insulin resistance were higher and the serum aminotransferase levels were lower in the CC patients than in the controls. Logistic regression analysis identified the elevated hemoglobin A1c, BMI>or=25 kg/m(2), and normal aminotransferase levels as independent predictors of CC. Kaplan-Meier analysis demonstrated lower occurrence of hepatocellular carcinoma and higher survival rate in the CC than in the controls in contrast to the similar cumulative probability of liver-related morbidity between those groups. CONCLUSION: CC more frequently presents with the clinical features suggestive of non-alcoholic steatohepatitis compared with controls even in the region where obesity is not prevalent. The lower occurrence of hepatocellular carcinoma and higher survival rate may indicate an indolent clinical course in CC as compared with viral cirrhosis.

Comparison of efficacy of medium-sized cutting balloons versus standard balloons for dilation of peripheral pulmonary stenosis.

The efficacy of medium-sized cutting balloons (CBs) 5 to 8 mm in diameter was compared with that of standard balloons (SBs) in the dilation of peripheral pulmonary stenosis (PPS). Nine vascular lesions in 7 patients were primarily dilated by CBs, and 13 vascular lesions in 11 patients were dilated by SBs alone. The efficacy of balloon dilation was evaluated by angiography and intravascular ultrasound (IVUS). There was no significant difference between the 2 groups in the success rate (66% vs 62%), the percentage increase in minimal lesion diameter (MLD; median 71% vs 52%) on angiography, and the number of new tears (2 vs 1) on IVUS. However, in the subgroup of vascular lesions with MLDs of <4.0 mm, for which CBs with diameters >200% of the MLD could have been applied, the percentage increase in MLD (95% vs 53%, p <0.05) and the number of tears (2 vs 1, p <0.05) with CBs were significantly greater than with SBs, requiring significantly smaller maximum balloon pressure (10 vs 13 atm, p <0.05). CBs are as effective as conventional balloons in the dilation of PPS and may be more effective when larger balloons are available.

Sclerotherapy for gastric fundal variceal bleeding: is complete obliteration possible without cyanoacrylate?

BACKGROUND: Many studies have suggested that endoscopic obliteration using cyanoacrylate for bleeding gastric fundal varices is effective. However, serious complications by injection of cyanoacrylate into varices have also been reported. METHODS: Thirty patients with bleeding gastric fundal varices underwent endoscopic injection sclerotherapy using 5% ethanolamine oleate under fluoroscopic guidance plus infusion of vasopressin and a transdermal nitroglycerin patch. The injection of 5% ethanolamine oleate was continued until it filled the varices and their feeder veins under fluoroscopic guidance. The injection needle was removed while thrombin glue was sprayed at the puncture site through the side hole of the injector needle to prevent bleeding from the puncture site. RESULTS: Complete hemostasis was achieved in 28/30 patients (93.3%). The cumulative rebleeding rate after 1, 3 and 5 years was 13%, 19% and 19%, respectively. The 1-, 3-, and 5-year cumulative mortality rates were 31%, 54% and 59%, respectively. There was no complication related to infusion of vasopressin and sclerotherapy procedure. CONCLUSION: The sclerotherapy method carried out using 5% ethanolamine oleate combined with infusion of vasopressin under fluoroscopic guidance might be a feasible method for obliteration of gastric fundal varices as an alternative to cyanoacrylate.

Alleviation of hemolysis after the stent implantation into an obstructed extracardiac conduit.

An 11-year-old boy who had undergone extracardiac conduit repair for the corrected transposition of the great arteries at 1 year of age showed hemolytic anemia due to a significant stenosis of the conduit placed between the right ventricle and pulmonary artery. There was a pressure gradient of 157 mmHg across the stenosis, giving 1.6:1 of right to left ventricular pressure ratio. Hemoglobin was 93 g/L with increased serum lactate dehydrogenase of 1382 IU/L. Stent implantation into this obstructed conduit has decreased a pressure gradient to 45 mmHg, leaving 0.74 of right to left ventricular pressure ratio. At 3 months after the stent implantation, hemoglobin has normalized. This case illustrate that an extremely severe stenosis of the right ventricular extracardiac conduit can induce hemolytic anemia and stent implantation into the conduit can be one of the alternative treatments to alleviate conduit stenosis and stop hemolysis.

Elevated levels of high-sensitivity C-reactive protein and serum amyloid-A late after Kawasaki disease: association between inflammation and late coronary sequelae in Kawasaki disease.

BACKGROUND: Coronary sequelae that persist after Kawasaki disease (KD) have been associated with obstructive changes of the lesions and coronary vascular events in adolescents and young adults. However, little is known about the association between sequelae late after KD and inflammatory markers, which are potential mediators and markers for atherogenesis. METHODS AND RESULTS: Cross-sectional study was performed to test the hypothesis that coronary sequelae are associated with elevated levels of inflammatory markers in patients late after KD (mean time interval after the onset, 10 years, 10 months). Levels of high-sensitivity C-reactive protein (CRP), serum amyloid-A (SAA), interleukin-6, and soluble intercellular adhesion molecule-1 were measured in the 4 groups (n=80): the referent group (n=15) and KD subgroups with normal coronary arteries from the onset (n=27); with regressed aneurysms (n=18); and with coronary artery lesions, such as persistent aneurysms, stenosis, and occlusion (n=20). CRP levels were significantly elevated in a KD subgroup with coronary artery lesions compared with the referent or other KD subgroups, as analyzed by ANOVA and ANCOVA after adjustment for a confounding factor body mass index. Levels of CRP, SAA, and interleukin-6 were positively correlated. Stepwise regression and logistic regression analyses support the association between the persistence of coronary artery lesions and the levels of CRP and SAA. CONCLUSIONS: Results demonstrate that the persistence of coronary lesions late after KD was independently associated with levels of CRP and SAA, suggesting that inflammation may be a novel functional aspect of coronary artery diseases late after KD.

Stent implantation into vertical arterial duct in adult patient with cyanotic congenital heart disease.

A 26-year-old patient, who has double inlet left ventricle, pulmonary atresia, and a patent vertical arterial duct, presented with progressive cyanosis with an arterial oxygen saturation of 71%. He had had three shunt operations and only the last aortopulmonary shunt was patent. Angiography revealed a stenotic aortopulmonary shunt, stenotic vertical arterial duct, and a branch stenosis of the left pulmonary artery. He underwent successful stent implantation into both the aortopulmonary shunt and vertical arterial duct and his arterial oxygen saturation increased to 88%. This case report illustrates that stent implantation into a vertical arterial duct is feasible and can be an alternative to shunt operation.

Hemoptysis from collateral arteries 12 years after a fontan-type operation.

A 20-year-old patient who had a single ventricle and pulmonary stenosis presented with recurrent hemoptysis 12 years after a Fontan-type operation. He was referred to us because of unsuccessful treatment with a tentative diagnosis of lung tuberculosis for 3 months. He had been relatively well for the prior 12 years, although he had attacks of paroxysmal supraventricular tachycardia and had underwent successful catheter ablation 3 years ago. Aortography revealed multiple collateral arteries as a cause of hemoptysis and coil embolization of these collateral arteries successfully stopped the hemoptysis. This case illustrates that collateral arteries may stay open or develop, and these collateral arteries can become a source of hemoptysis long after a Fontan-type operation.

Increased vascular endothelial growth factor in patients with cyanotic congenital heart diseases may not be normalized after a Fontan type operation.

BACKGROUND: To determine the change of serum concentration of vascular endothelial growth factor (VEGF) in patients with cyanotic congenital heart disease (C-CHD). METHODS: Patients comprised four groups: group A, 19 patients without cyanosis; group B, 24 patients with C-CHD; group C, 17 patients who had C-CHD and underwent biventricular repair; and group D, 15 patients who had single ventricle and underwent a Fontan type operation. Blood samples were obtained from upper arm veins and serum VEGF was determined. We determined correlation between serum VEGF and arterial oxygen saturation and compared levels of serum VEGF among groups. In addition, age and hemodynamic variables derived from cardiac catheterization were analyzed in terms of correlation with serum VEGF. RESULTS: Serum VEGF significantly negatively correlated with arterial oxygen saturation (r = -0.62, p < 0.0001). Serum VEGF in B and D were significantly higher than those in A and C (median = 443 pg/mL in B and 302 pg/mL in D vs 180 pg/mL in A and 216 pg/mL in C, respectively). Serum VEGF was significantly negatively correlated with patient's age (p = 0.54, p < 0.04) in D, but significantly positively correlated with systemic venous pressure (p = 0.45, p < 0.01) in pooled data. CONCLUSIONS: Patients with C-CHD have increased serum VEGF in parallel with the degree of cyanosis. With biventricular repair, cyanosis and serum VEGF are normalized. However, with a Fontan type operation, cyanosis disappears but serum VEGF may not be normalized because of elevated venous pressure in association with younger age.

Myotonic dystrophy presenting as atrial flutter in childhood.

A 12-year-old boy presented with tachycardia and cardiomegaly. An electrocardiogram showed atrial flutter with a ventricular rate of 170 beats/min. The echocardiogram showed left ventricular dilation, with decreased contractility. Electromyography confirmed the diagnosis of myotonic dystrophy. The atrial flutter was converted to sinus rhythm by electroversion, leading to restoration of normal cardiac function. Our case illustrates that atrial flutter can be an early sign of the cardiac complications of myotonic dystrophy in childhood.

Disappearance of hepatic encephalopathy and improvement of liver function after surgical treatment of portal-systemic shunt in a patient with liver cirrhosis.

Large portal-systemic shunts in cirrhotic patients often cause recurrent hepatic encephalopathy and might promote liver dysfunction because of the reduced portal blood flow. We report a case of liver cirrhosis in which hepatic encephalopathy disappeared and liver function improved together with an increase of hepatopetal portal blood flow and liver volume after shunt resection. A 70-year-old woman with liver cirrhosis was admitted because of recurrent disorientation. Serum ammonia levels ranged from 174 to 321 micrograms/dL. Computed tomography demonstrated an atrophic liver and a large shunt. Portography disclosed that this shunt originated from the superior mesenteric vein and flowed into the inferior vena cava, common iliac vein and ovarian vein. Portal blood flow was poor because of the deviation into this shunt. After the surgical resection of the shunt, ammonia levels were normalized and hepatic encephalopathy no longer occurred. Portography and computed tomography after surgery demonstrated that hepatopetal portal blood flow evidently improved and the liver volume increased (before 369; after 574 cm3). Two years after surgery, hepaplastin test and serum albumin level improved from 41 to 76% and from 2.7 to 3.4 g/dL, respectively. This case supports the effectiveness of shunt resection for hepatic encephalopathy and the deteriorated liver function in cirrhotic patients with large portal-systemic shunt.

Clinical implication of plasma natriuretic peptides in children with ventricular septal defect.

BACKGROUND: There is little information available concerning plasma concentrations of B-type natriuretic peptide (BNP) in children with a ventricular septal defect. The aim of the present study was to determine hemodynamic factors that control plasma concentrations of BNP and the clinical implications of BNP compared with atrial natriuretic peptide (ANP) in children with ventricular septal defect. METHODS: Fifty-nine patients with ventricular septal defect (28 boys and 31 girls) without pulmonary vascular disease were enrolled. The patients' ages ranged from 3 months to 13 years (mean 3.1 years). Plasma BNP and ANP were determined by immunoradiometric assay. Hemodynamic variables derived from cardiac catheterization were analyzed in terms of correlation with BNP and ANP. RESULTS: It was found that plasma BNP significantly positively correlated with ANP (ANP = 2.1 x BNP + 25 pg/mL; r = 0.81, P < 0.0001) and BNP never exceeded ANP in the present patient series. Plasma BNP as well as ANP significantly positively correlated with pulmonary to systemic flow ratio (r = 0.65 and r = 0.59, respectively) and mean pulmonary artery pressure (r = 0.72 and r = 0.68, respectively). In addition, plasma BNP of > or =20 pg/mL and ANP of > or =50 pg/mL identified children with mean pulmonary artery pressure of > or =20 mmHg with a sensitivity of 82% and 97%, respectively, and a specificity of 89% and 84%, respectively. CONCLUSION: Plasma BNP and ANP reflect pressure and volume loads to the pulmonary artery and right ventricle and may help to identify children with ventricular septal defect complicated by pulmonary hypertension that demands early intervention.

Kawasaki disease complicated by cerebral infarction.

An 8-month-old boy presented with right hemiplegia of sudden onset after 20 days of Kawasaki disease, which was not initially treated by gamma globulin. Cranial X-ray computed tomography confirmed cerebral infarction as the cause of the right hemiplegia. In subsequent weeks, he developed multiple thromboses in coronary aneurysms. He successfully underwent intracoronary thrombolysis using tissue plasminogen activator without haemorrhagic complications. Cerebral infarction as a complication of Kawasaki disease is rare, and is a difficult clinical situation to manage.