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No standard treatments have been established for airway hemorrhage during cardiopulmonary bypass (CPB). Herein, we describe two cases of catheter-induced pulmonary hemorrhage during cardiac surgery. In each case, massive hemoptysis was observed during cardiopulmonary bypass (CPB) weaning. A bronchial blocker was inserted into the right bronchus, and extracorporeal membrane oxygenation (ECMO) was initiated to reduce pulmonary blood flow, and stabilize the hemodynamics. ECMO significantly reduced bleeding from the pulmonary artery (PA). Both cases had favorable outcomes following the implementation of extracorporeal membrane oxygenation (ECMO). ECMO is effective at controlling PA bleeding during and after cardiac surgery.

Objective To search the predictive factors of infliximab resistance in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients. Study design. Twenty-seven patients with KD who received infliximab after 4–5 g/kg of IVIG therapy from 2013 to 2015 were consecutively recruited in this study. They were divided into two groups: patients who responded to infliximab (infliximab-responsive group, n = 15) and patients who required additional therapy for the disease control (infliximab-resistant group, n = 12). We analyzed the clinical and laboratory parameters just before the infliximab treatment including serum levels of procalcitonin and cytokines with respect to the infliximab response. Results Serum procalcitonin concentration (P = 0.017), neutrophils to lymphocytes ratio (P = 0.013), and % neutrophils (P = 0.004) were higher, and serum sodium concentration (P = 0.017) was lower in infliximab-resistant group than those of infliximab-responsive group, respectively. Multivariate logistic regression analyses indicated that higher procalcitonin concentration (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.00–5.00, P = 0.046) and lower sodium levels (OR 0.64, 95% CI 0.32–1.00, P = 0.047), but not other variables, were associated with infliximab-resistance. Serum procalcitonin concentrations positively correlated with the serum levels of interleukin-6, soluble tumor necrosis factor receptor type 1 and type 2, respectively. Analyses of the receiver operating characteristic (ROC) curve showed that the cut-off value of procalcitonin 2.0 ng/ml had 58.3% of sensitivity and 93.3% of specificity. ROC analysis yielded an area under the curve (AUC) of 0.739 to predict infliximab-resistance. Conclusion Serum procalcitonin might be an effective biomarker to predict infliximab resistance in severe KD patients who are refractory to IVIG treatment.

Portosystemic venous shunt (PSVS) is a vascular anomaly between the portal and systemic veins, resulting in several critical complications. Although PSVS is often associated with congenital heart diseases, the clinical association between Fontan circulation and PSVS has not been elucidated. This study aimed to investigate the clinical features of Fontan patients with PSVS. Two hundred thirteen patients who underwent Fontan procedure are being followed up at Adult Congenital Heart Disease clinic in Kyushu University Hospital. Among them, 139 adult patients underwent cardiac catheterization between January 1, 2011 and September 30, 2019. Medical records were reviewed to investigate the laboratory, echocardiography, and cardiac catheterization findings, as well as clinical manifestations and outcomes. Eleven Fontan patients received the diagnosis of PSVS. The median age at cardiac catheterization was 25 (range 18–45) years. Fontan operation was performed using extracardiac conduit or lateral tunnel 22 (16–35) years previously. Ten patients presented with chronic heart failure [New York Heart Association class 2 (n = 5) and 3 (n = 5)]. The median level of peripheral oxygen saturation was 87 (70–95)%. Cardiac catheterization showed increased cardiac index [5.3 (2.72–14.3) L/min/m2] with or without high central venous pressure [18 (9–25) mmHg]. Although the pulmonary vascular resistance was within the normal range, the systemic vascular resistance was decreased [7.08 (1.74–18.6) Wood units]. Fontan patients complicated with PSVS had increased cardiac output. The presence of PSVS in Fontan circulation might be associated with unfavorable long-term outcome.

Numerous dark-brown-coloured small spots called “Wischnewski spots” are often observed in the gastric mucosa in the patients dying of hypothermia, but the molecular mechanisms through which they develop remain unclear. We hypothesised that hypothermia may activate the secretion of gastric acid and pepsin, leading to the development of the spots. To investigate this, we performed experiments using organotypic rat gastric tissue slices cultured at 37 °C (control) or 32 °C (cold). Cold loading for 6 h lowered the extracellular pH in the culture medium. The mRNA expression of gastrin, which regulates gastric acid secretion, increased after cold loading for 3 h. Cold loading increased the expression of gastric H+,K+-ATPase pump protein in the apical canalicular membrane and resulted in dynamic morphological changes in parietal cells. Cold loading resulted in an increased abundance of pepsin C protein and an elevated mRNA expression of its precursor progastricsin. Collectively, our findings clarified that cold stress induces acidification by activating gastric H+,K+-ATPase pumps and promoting pepsin C release through inducing progastricsin expression on the gastric mucosa, leading to tiny haemorrhages or erosions of the gastric mucosa that manifest as Wischnewski spots in fatal hypothermia.

Persistent sciatic artery (PSA) is a rare anomaly that may cause various symptoms, such as aneurysm, rupture, thromboembolism, and sciatica. Direct surgery can be performed to treat PSA aneurysm (PSAA), but is associated with complications; e.g., anatomical problems such as sciatic nerve injury. Herein we report a case of a 74-year-old woman with acute limb ischemia that developed from a distal embolism caused by a thrombus in the left PSAA; favorable results were obtained for her by treatment with a stent-graft after rapid anticoagulation therapy for limb salvage.

Congestive hepatopathy and hepatocellular carcinoma is a serious complication after Fontan procedure. Liver fibrosis due to hepatic congestion could occur also in adult patients after repair of tetralogy of Fallot (rTOF). However, the incidence and severity remain unclear.A total of 111 patients with adult congenital heart disease between 2009 and 2016 were enrolled. Liver fibrosis markers and hemodynamic parameters assessed by cardiac magnetic resonance imaging and catheterization were analyzed in 50 rTOF patients having significant pulmonary regurgitation and/or stenosis, 50 Fontan patients and 11 controls.Liver fibrosis markers in patients with rTOF were significantly higher than controls, and tended to be lower than Fontan patients (median, hyaluronic acid: 25.8 vs. 15.9 vs. 40.8, type IV collagen: 129 vs. 113 vs. 166, ng/mL, p 0.05, respectively). Patients with rTOF showed abnormal hyaluronic acid levels more frequently than controls, and less frequently than Fontan patients (22% vs. 0% vs. 38%, respectively, p 0.05). Multivariate analyses indicated a positive association of right atrial pressure with type IV-collagen or hyaluronic acid levels (each, p 0.001, p = 0.003). Abdominal ultrasonography revealed hepatic congestion in 50% of rTOF patients tested. Liver biopsy of the two rTOF patients with highest hyaluronic acid levels showed pathological evidence of moderate and severe (F2 and F3) liver fibrosis and one had combined hepatocellular and cholangiocarcinoma.We first demonstrated elevated liver fibrosis markers in adult patients with rTOF. These levels may help to predict the progressive liver disease as well as consider the timing of pulmonary valve replacement.

Case We report a 2-year-old boy with duodenal ulcer with active bleeding that occurred as a result of norovirus gastroenteritis. On admission, the patient presented with shock accompanied with vomiting and melena. Abdominal contrast enhanced computed tomography scan showed signs of duodenal bleeding. Outcome He was successfully treated with emergent transcatheter arterial embolization. After the treatment, endoscopic examination revealed duodenal ulcer and the stool norovirus antigen test was found to be positive. The patient recovered completely without any sequelae. Conclusion Life-threatening duodenal ulcer bleeding in children can be caused by viral gastroenteritis. When endoscopic therapy is unsuccessful or difficult, in cases of small children, angiographic intervention can be a safe alternative treatment option of gastrointestinal bleeding.

BackgroundDuctal patency is mandatory to manage patients with ductal-dependent pulmonary circulation. The aim of this study is to elucidate the morphological and haemodynamic features of ductus arteriosus with right ventricular outflow tract obstruction, and investigate the appropriate perinatal management.Patients and methodsPatients with prenatal diagnosis of right ventricular outflow tract obstruction at our institution between 2010 and 2015 were included in the study. Reverse orientation of the ductus arteriosus is defined as an inferior angle of 90°. We retrospectively reviewed the shape and flow pattern of ductus arteriosus and the clinical characteristics of the cases.ResultsA total of 39 patients were enrolled. The shape was divided into normal orientation (n=15) and reverse orientation (n=24) of the ductus arteriosus. There was no significant difference in the type of oxygen saturation at birth and age at shunt operation between both the groups. However, the median narrowest diameter of ductus arteriosus in the normal orientation group was significantly smaller than that in the reverse orientation group (2.0 [1.0–5.4] versus 3.0 [1.3–4.4] mm, pConclusionsNormal orientation pattern might have high incidence of an early narrowing or closure of ductus arteriosus at birth. The critical patients need careful evaluation by repeated foetal echocardiography and further maternal interventions.

Background Dilatation of the ascending aorta affects those patients with bicuspid aortic valve (BAV), even after valvular surgery, possibly due to tissue fragility. The goal of the study was the molecular characterization of aorta with BAV compared to that with normal tricuspid aortic valve (TAV). Methods and Results: The subjects were patients who underwent surgery for aortic valve stenosis in 2013 and 2014. Nine patients with BAV and 13 with TAV were examined. There was no difference in the clinical characteristics or grade of aortic valve stenosis, but the diameters of the ascending aorta were significantly higher in the BAV group. The ascending aortic specimens were subjected to transcriptome analyses, which revealed the changes in receptor tyrosine kinase (RTK) pathway-related genes between TAV and BAV samples. Immunohistochemical study revealed higher staining of phosphorylated AKT (pAKT) in the media of the ascending aorta in the BAV group, regardless of the size of ascending aorta, whereas total AKT did not show such a difference. Immunofluorescence staining revealed the AKT activation was mainly in the medial vascular smooth muscle cells. Conclusions The results showed that the RTK-AKT pathway in the medial layer of the ascending aorta is activated in aortae with BAV. Activation of this pathway may be associated with fragility and dilatation of the ascending aorta with BAV.

An 8-year-old girl presenting with fulminant myocarditis and cardiogenic shock underwent peripheral extracorporeal membrane oxygenation. She was switched to central extracorporeal membrane oxygenation because of inadequate venous drainage, and an echocardiogram showed left ventricular (LV) thrombus. She underwent removal of the LV thrombus with LV venting. An echocardiogram showed LV thrombus again 2 days later. The LV thrombus was again removed, and a biventricular assist device support was initiated to avoid further thrombus formation. The patient was completely weaned from mechanical circulatory support after 7 days. She recovered fully without any neurologic deficit.

There is limited information available regarding the role of infliximab (IFX) following the acute phase of Kawasaki disease (KD). We aimed to evaluate whether IFX is associated with coronary artery aneurysm (CAA) regression.Between 2005 and 2016, we identified 971 consecutive patients with KD from 3 tertiary institutions, and 49 (5%) with CAAs were enrolled in our study. Patients were divided into 2 groups: 27 who received IFX and 22 who did not. The persistence rate of CAAs was compared between the groups.Age, sex, and duration of the febrile period did not significantly differ between the groups. The maximum value of C-reactive protein was higher in the IFX- than in the non-IFX group. The maximum z-score of CAAs did not differ between the groups. The 2-, 4- and 6-year cumulative persistence rate of CAA was 24%, 24% and 24% in IFX-group, whereas 67%, 52% and 33% in non-IFX group, respectively (P = 0.03). The median duration of CAA regression was 1.1 vs. 4.6 years. Among those who developed medium- or large-sized CAAs, the 2-, 4- and 6-year cumulative persistence rate of CAA was 33%, 33% and 33% in IFX group, whereas 77%, 51% and 48% in non-IFX group, respectively (P = 0.047). Multivariate logistic regression analysis indicated that the maximum z-score (hazard ratio 0.72, p 0.001) and response to IFX (hazard ratio 4.56, p = 0.017) were independently related to regression.IFX therapy was observed to be effective for the early improvement of CAAs in patients with intravenous immunoglobulin-resistant KD.

In most patients with aortic regurgitation (AR), aortic valve replacement (AVR) improves left ventricular (LV) function, but some patients will not have favorable remodeling. Our objectives were to review long term clinical results of AVR for AR and to examine what factors affect the normalization of LV function after AVR for chronic AR.Between 1989 and 2010, 177 patients underwent isolated AVR for chronic pure AR. The patients were divided into 2 groups based on indexed end-systolic LV diameter (iESD): Group L (iESD) ≥25 mm/m(2)) (130 patients) and Group S (iESD25 mm/m(2)) (47 patients).There was no significant difference between groups in late mortality, freedom from cardiac-related death and rehospitalization for heart failure at late follow up after operation. At postoperative follow-up, 16% of patients had not recovered normal LV systolic function. By means of multivariate analysis, iESD and cardiac index (CI) were independent predictors of recovery of LV function and iESD26.7 mm/m(2) and CI2.71 l/min/m(2) were the best cut-off values.Early and late surgical results of AVR for chronic AR were good, but for the preservation of postoperative normal LV function, AVR for AR patients should be performed before iESD reaches 26.7 mm/m(2).

OBJECTIVE Congenital portosystemic venous shunt (CPSVS) is a rare vascular malformation with a high risk of mortality from pulmonary arterial hypertension (PAH), but the treatment outcome of CPSVS closure remains elusive. Our aim was to investigate the clinical features and establish the optimal management of CPSVS with or without PAH. METHODS Twenty-four patients with CPSVS treated in Kyushu University Hospital between 1990 and 2015 were enrolled in this study. The patients were divided into a PAH group (n = 9) and a non-PAH group (n = 15). Clinical characteristics and outcomes were evaluated. RESULTS The first manifestation of CPSVS at diagnosis (28.5 [1-216] months) was hypergalactosemia in 13 (54%) or PAH in six (25%) patients. PAH was the cause of all three deaths. The PAH group had higher levels of serum total bile acid, manganese, and total bilirubin, along with higher pulmonary vascular resistance index (PVRI) than the non-PAH group (7.2 [5.1-38.1] vs 1.2 [0.5-3.3] unit/m2 , P

OBJECTIVE Chronic inflammation plays a key role in the development of atherosclerosis. Early progression of atherosclerosis has been reported in patients with RA. Cryopyrin-associated periodic syndromes (CAPS) are autosomal dominant autoinflammatory disorders caused by heterozygous NLRP3 gene mutations. Chronic infantile neurological cutaneous and articular (CINCA) syndrome is the most severe form of CAPS and patients display early onset of rash, fever, uveitis and joint manifestations. However, there has been no previous report on atherosclerosis in patients with CAPS. The objective of this study is to assess the development of atherosclerosis in patients with CINCA syndrome. METHODS Intima-media thickness (IMT) of the carotid arteries, stiffness parameter β, ankle brachial index (ABI) and pressure wave velocity (PWV) were evaluated by ultrasonography in 3 patients with CINCA syndrome [mean age 9.0 years (S.D. 5.3)] and 19 age-matched healthy controls [9.3 years (S.D. 4.3)]. RESULTS The levels of carotid IMT, stiffness parameter β and PWV in CINCA syndrome patients were significantly higher than those in healthy controls [0.51 mm (S.D. 0.05) vs 0.44 (0.04), P = 0.0021; 6.1 (S.D. 1.7) vs 3.9 (1.0), P = 0.0018; 1203 cm/s (S.D. 328) vs 855 (114), P = 0.017, respectively]. CONCLUSION Patients with CINCA syndrome showed signs of atherosclerosis from their early childhood. The results of this study emphasize the importance of chronic inflammation in the development of atherosclerosis. Further analysis on atherosclerosis in young patients with CINCA syndrome may provide more insights into the pathogenesis of cardiovascular disease.

Pathologic studies of the heart in patients with Kawasaki disease (KD) revealed vasculitis, valvulitis, myocarditis, and pericarditis. However, there have been no studies on the quantitative determination of multi-site echogenicity of the heart in KD patients. It is also undetermined whether the degree of echogenicity of each site of the heart in patients with KD might be related to the response to intravenous immunoglobulin (IVIG) treatment. In 81 KD patients and 30 control subjects, we prospectively analyzed echogenicity of the heart. Echogenicity was measured in four sites: coronary artery wall (CAW), mitral valve (MV), papillary muscle (PM), and ascending aortic wall (AAo wall) by the calibrated integrated backscatters (cIBs). The cIB values of all measurement sites at acute phase in KD patients were significantly higher than those in control subjects (KD patients vs control subjects; CAW, 19.8 ± 6.2 dB vs 14.5 ± 2.0 dB, p < 0.05; MV, 23.3 ± 5.3 dB vs 16.0 ± 3.3 dB, p < 0.05; PM, 22.4 ± 5.1 dB vs 12.7 ± 1.9 dB, p < 0.05; AAo wall, 25.3 ± 5.6 dB vs 18.3 ± 3.4 dB, p < 0.05). The cIB values of CAW at the acute phase in IVIG nonresponders were significantly higher than those in responders. Conclusion: Echogenicity of the heart in KD patients at the acute phase increased not only in the coronary artery wall but also in other parts of the heart. Echogenicity of CAW might be helpful in determining the unresponsiveness of IVIG treatment.

Purpose: Pulmonary valve replacement (PVR) improves right ventricular (RV) function in patients with tetralogy of Fallot (TOF). However, the impact of PVR on left ventricular (LV) function and the mechanism of improvement are still unclear. The purpose of this study is to clarify the impact of PVR on LV mechanical efficiency in adult patients with TOF. Methods: Fourteen patients with TOF (age 30.1±11.5 years) who underwent PVR for significant pulmonary insufficiency were enrolled in this study. We measured left ventricular end-diastolic volume index (LVEDVI), left ventricular end-systolic volume index (LVESVI), contractility (end-systolic elastance; Ees), afterload (effective arterial elastance; Ea), and mechanical efficiency (ventriculoarterial coupling; Ea/Ees and the rate of stroke work and pressure-volume area; SW/PVA) on the basis of the echocardiographic data before, after and late after PVR. Ees, Ea and SW/PVA were approximated as follows: Ees=mean arterial pressure/minimal ventricular volume, Ea = maximal ventricular pressure / (maximal ventricular volume-minimal ventricular volume), and SW/PVA=1 / (1 + 0.5 Ea/Ees). Results: LVEDVI, stroke volume and ejection fraction increased significantly after PVR. Contractility (Ees) did not change but afterload (Ea) decreased, and LV mechanical efficiency (Ea/Ees and SW/PVA) improved significantly. These changes were observed just after PVR, and further improvement was not noted in the late period. ![Figure][1] Hemodynamic changes after PVR Conclusions: LV efficiency improves after PVR. Increased pulmonary forward flow and relief of the compression from the RV increase LVEDVI and stroke volume. LV afterload decreases to adapt the increase of stroke volume without the change of contractility. These changes result in the improvement of LV mechanical efficiency. [1]: pending:yes