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Background: Degos disease, also known as malignant atrophic papulosis, is characterised by cutaneous manifestations due to chronic thrombo-obliterative vasculopathy. There have been reports of the rare late-onset Degos disease complicated by constrictive pericarditis (CP). This study reports a case of CP caused by Degos disease that developed 20 years after diagnosis.Case Presentation: A 62-year-old woman who had been taking aspirin for 20 years for Degos disease was hospitalised for worsening of heart failure. The patient was diagnosed with CP and underwent pericardiectomy. Pathological findings suggested the involvement of Degos disease. The postoperative course was uneventful, and her heart failure and Degos disease did not worsen.Conclusions: The study findings suggests that Degos disease can cause long-term CP. Aspirin effectively inhibited the progression of Degos disease, and surgical treatment was necessary when heart failure due to CP was refractory to treatment. [ABSTRACT FROM AUTHOR]

Objectives: Postoperative pleural effusion (PE) is common after pediatric cardiac surgery, and if prolonged can lead to the deterioration of the general condition due to malnutrition and result in death. This study aims at identifying the prognostic factors of prolonged PE after pediatric cardiac surgery. Design and settings: Patients were divided into the effective (with chest tube removal within 10 days after medical therapy) and ineffective (with chest tube in place for more than 10 days) groups. The factors were compared between the two groups retrospectively. Participants: Participants included patients who had prolonged PE after cardiac surgery in national center for child and health development between October 2014 and October 2017. Main outcome measures: Baseline characteristics and procedure details were compared between the two groups to determine the predictor of prolonged PE. White blood cell count, platelet count, neutrophil-to-lymphocyte ratio, hemoglobin level, serum total protein level, serum albumin level, blood fibrinogen level, serum creatinine level, etc. were examined. Results: Twenty patients were included. Between the two groups, no significant differences in baseline characteristics, such as age, weight, and sex were found, and significant differences were observed only in the NLR change ratio (effective group, 5.1 [4.1–8.0] versus ineffective group, 11.9 [9.9–14.1]; P = 0.01). Conclusions: NLR change ratio is a potential prognostic factor of prolonged PE, including chylothorax, after pediatric cardiac surgery. [ABSTRACT FROM AUTHOR]

Cardiopulmonary bypass ( CPB) elicits a systemic inflammatory response. Our previous reports revealed that prophylactic sivelestat administration at CPB initiation suppresses the postoperative acute inflammatory response due to CPB in pediatric cardiac surgery. The purpose of this study was to compare the effects of sivelestat administration before CPB and at CPB initiation in patients undergoing pediatric open-heart surgery. Twenty consecutive patients weighing 5-10 kg and undergoing ventricular septal defect closure with CPB were divided into pre- CPB ( n = 10) and control ( n = 10) groups. Patients in the pre- CPB group received a 24 h continuous intravenous infusion of 0.2 mg/kg/h sivelestat starting at the induction of anesthesia and an additional 0.1 mg/100 mL during CPB priming. Patients in the control group received a 24-h continuous intravenous infusion of 0.2 mg/kg/h sivelestat starting at the commencement of CPB. Blood samples were tested. Clinical variables including blood loss, water balance, systemic vascular resistance index, and the ratio between partial pressure of oxygen and fraction of inspired oxygen ( P/ F ratio) were assessed. White blood cell count and neutrophil count as well as C-reactive protein levels were significantly lower in the pre- CPB group according to repeated two-way analysis of variance, whereas platelet count was significantly higher. During CPB, mixed venous oxygen saturation remained significantly higher and lactate levels lower in the pre- CPB group. Postoperative alanine aminotransferase and blood urea nitrogen levels were significantly lower in the pre- CPB group than in the control group. The P/ F ratio was significantly higher in the pre- CPB group than in the control group. Fluid load requirement was significantly lower in the pre- CPB group.Administration of sivelestat before CPB initiation is more effective than administration at initiation for the suppression of inflammatory responses due to CPB in pediatric open-heart surgery, with this effect being confirmed by clinical evidence. [ABSTRACT FROM AUTHOR]

Cardiopulmonary bypass ( CPB) elicits a systemic inflammatory response. The neutrophil elastase inhibitor sivelestat is known to suppress this systemic inflammatory response, which can eventually result in acute organ failure. The prophylactic effect of sivelestat on acute lung injury, especially in pediatric cardiac surgery, remains unclear. This prospective double-blind, randomized study evaluated the perioperative prophylactic effect of sivelestat in patients undergoing elective pediatric open heart surgery with CPB. Thirty consecutive patients, weighing 5-10 kg and undergoing open heart surgery with CPB, were assigned to sivelestat ( n = 15) or control ( n = 15) groups. From CPB initiation to 24 h after surgery, patients in the sivelestat group received a continuous intravenous infusion of 0.2 mg/kg/h sivelestat, whereas patients in the control group received the same volume of 0.9% saline. Blood samples were collected, and levels of interleukin ( IL)-6, IL-8, tumor necrosis factor alpha, polymorphonuclear elastase ( PMN- E), C-reactive protein ( CRP), as well as the white blood cell ( WBC) count, platelet count, and neutrophil count ( NC) were measured. PMN- E levels, IL-8 levels, WBC count, NC, and CRP levels were significantly lower, and platelet count was significantly higher in the sivelestat group, according to repeated two-way analysis of variance. The activated coagulation time was significantly shorter in the sivelestat group, similarly, blood loss was significantly less in the sivelestat group. In conclusion, Sivelestat attenuates perioperative inflammatory response and clinical outcomes in patients undergoing pediatric heart surgery with CPB. [ABSTRACT FROM AUTHOR]

At our institution, the strategy for patients with bicuspid aortic valve, aortic valve stenosis (<5 mm), and aortic hypoplasia [hypoplastic aortic arch, coarctation of the aorta (CoA), or interrupted aortic arch (IAA)] with ventricular septal defects (VSDs) as well as normal left ventricular (LV) volume and mitral valve size consists of two parts. The Norwood operation is applied as the first palliation for this group of patients. Second, the decision whether the patients are to undergo the Rastelli operation or a univentricular repair is made depending on the size of the right ventricle after the Norwood operation. This study aimed to examine whether the aforementioned surgical strategy for this group of patients is adequate or not. Seven patients undergoing the Norwood operation as the first palliation for bicuspid aortic valve, aortic valve stenosis (<5 mm), and aortic hypoplasia with VSDs as well as normal LV volume and mitral valve size between February 2005 and March 2010 at Kitasato University Hospital and the Gunma Children's Medical Center were reviewed. Postoperative serum B-type natriuretic peptide (BNP) and central venous pressure (CVP) were measured in the patients undergoing the staged Norwood-Rastelli operation to assess whether the authors' right ventricular end-diastolic volume index (RVEDVI) cutoff (80 % of normal) is adequate. At this writing, all seven patients are alive after a mean follow-up period of 58.8 ± 17.8 months. They all had aortic valve stenosis of <5 mm and a bicuspid aortic valve. Four patients had a diagnosis of CoA with VSD, and three patients had IAA with VSD. Six patients underwent biventricular repair, and one patient had univentricular repair due to the small RVEDVI (74 % of normal). The patients with 80-90 % of normal RVEDVI had higher BNP and higher CVP than those with more than 90 % of normal RVEDVI after the Rastelli operation, whereas the patient undergoing the Fontan operation had a low BNP level. In conclusion, the described strategy for patients with severe aortic hypoplasia and aortic stenosis with VSD as well as normal LV and mitral valve size is reasonable. [ABSTRACT FROM AUTHOR]

Objective: We have established a low–priming volume cardiopulmonary bypass system for pediatric heart surgery to avoid homologous blood transfusion. The priming volume of our system is down to 140 mL for patients weighing less than 7 kg. We can prime the bypass circuits without blood products for patients weighing more than 4 kg. Methods: Seventy consecutive patients weighing 4 to 7 kg underwent heart surgery with a bloodless prime from October 2003 to September 2006. The type of procedures (Risk Adjustment in Congenital Heart Surgery category) included the following: category 1: atrial septal defect (n = 3); category 2: ventricular septal defect, tetralogy of Fallot, bidirectional Glenn shunt, and others (n = 55); category 3: atrioventricular septal defect, double-outlet right ventricle, and others (n = 8); category 4: Rastelli procedure for transposition of the great arteries (n = 3); and category 6, Damus–Kaye–Stansel procedure (n = 1). Transfusion criteria were hematocrit less than 20%, mixed venous oxygen saturation less than 70%, regional cerebral oxygenation less than 50%, and plasma lactate level greater than 4.0 mmol/L during bypass. Results: The mean age and body weight were 7.3 ± 5.4 months and 5.4 ± 0.8 kg, respectively. Forty-five patients (64%) underwent transfusion-free procedures. Preoperative hematocrit, age, body weight, complexity of procedure and cardiopulmonary bypass time were compared between patients with and without transfusion. Bypass time and Risk Adjustment in Congenital Heart Surgery risk category in patients with transfusion were significantly greater than those in patients without (P < .0001, and P < .05, respectively). Body weight in patients without transfusion was significantly greater than that in patients with (P < .01). In multiple regression analysis, the determinants of blood transfusion were the bypass time and body weight (odds ratio 1.026, 95% confidence interval 1.011–.040, P < .0001, and odds ratio 0.366, 95% confidence interval 0.171–0.785, P < .01). Conclusions: It is possible to do complex transfusion-free procedures safely for patients weighing more than 4 kg by using the low–priming volume circuit. The limiting factors of bloodless heart surgery are not preoperative hematocrit and complexity of procedure but the cardiopulmonary bypass time and the patient’s body weight. [Copyright &y& Elsevier]

: BackgroundPoly(2-methoxyethylacrylate) (PMEA) is a new coating material, and several experimental studies have revealed excellent biocompatibility of PMEA-coated cardiopulmonary bypass circuits. The clinical utility of the PMEA-coated circuits was compared with that of uncoated circuits, focusing on perioperative inflammatory response.: MethodsTwenty-two patients were randomized to PMEA-coated (group P; Capiox RX25; n = 11) or uncoated (group U; Capiox SX10; n = 11) circuit group, and underwent coronary artery bypass grafting and/or valve operations. The following markers, as well as clinical outcomes, were analyzed perioperatively: (a) complement activation by C3a (including C3a-desArg) concentrations; (b) leukocyte activation by polymorphonuclear-elastase concentrations; (c) acute phase inflammatory response by interleukin-6 concentrations; and (d) platelet preservation by number of platelets.: ResultsThe maximal values of C3a and polymorphonuclear-elastase were significantly lower in group P than in group U. The intergroup difference of interleukin-6 was not significant. Although preservation of platelets was significantly better in group P until 1 hour after initiating cardiopulmonary bypass, no significant intergroup difference was observed thereafter. The duration of postoperative mechanical ventilation revealed no significant intergroup difference.: ConclusionsThe PMEA-coated circuits exhibited better suppression of perioperative complement and leukocyte activation than the uncoated circuits. In addition, the price of the PMEA-coated circuits is the same as that of the uncoated circuits. Therefore, we judged that the clinical utility of the PMEA-coated circuits is superior to those of the uncoated circuits. [Copyright &y& Elsevier]

A bridging bronchus (BB) is a rare congenital heart anomaly. Frequently, BB also is complicated with congenital cardiac malformation, especially with sling (retrotracheal) left pulmonary artery (SLPA). This report presents a patient who underwent a Norwood procedure for a complex congenital heart disease with BB and SLPA. [ABSTRACT FROM AUTHOR]