Perioperative changes in platelet count in patients with atrial septal defect.

BACKGROUND: Platelet count and function change following atrial septal defect (ASD) closure with the Amplatzer septal occluder (ASO). However, the clinical significance of these changes remains unclear. We examined changes in platelet count and mean platelet volume (MPV) before and after ASO placement, and the association between platelet count, MPV and various factors. We discussed the mechanism and clinical significance of changes in platelet count and MPV. METHODS: We evaluated 109 patients with ASD who underwent ASO placement, and we performed various analyses of platelet count and MPV. RESULTS: Younger patients typically had higher platelet counts and lower MPV on admission. They also had large ASDs relative to their body constitution; larger devices were therefore used. Rates of change in platelet count were higher in younger patients. There were no significant associations between platelet count or MPV before placement and mean pulmonary artery pressure, and Qp/Qs, and between the number of thrombocytopenia and presence or absence of headache or residual ASD. Platelet counts decreased on average by 21.3% for the first 3 days after ASO placement. One month after placement, platelet counts were slightly improved but remained lower than before placement. Conversely, MPV increased significantly after ASO placement and remained high a month after placement. The ASO size was the most influential factor in platelet count reduction after ASD closure by ASO. CONCLUSIONS: One month after ASO placement, platelet counts decreased and MPVs continued to increase, suggesting that platelet consumption and new production were still occurring a month after placement.

A Case of Kawasaki Disease Accompanied by Encephalitis and Several Kinds of Arrhythmia during the Acute Phase.

Although central nervous system complications occasionally accompany during the acute phase of Kawasaki disease, clinically problematic arrhythmia is quite rare. We report a case accompanied by encephalitis and several kinds of problematic arrhythmia. Following the diagnosis of Taussig-Bing anomaly and coarctation of the aorta, the patient underwent aortic arch reconstruction, an arterial switch operation, and ventricular septal defect closure. No significant arrhythmias were observed. At the age of 5 years, the patient presented with a fever, rash, conjunctival hyperemia, and redness of the lips and fingertips. He was subsequently diagnosed with Kawasaki disease. The patient also presented with disorientation, and electroencephalography revealed overall slow-wave activity, indicating encephalitis. The patient received high-dose immunoglobulin and steroid pulse therapy. Sinus arrest was detected on day 10, and an atrial flutter with a 2 : 1 to 4 : 1 atrioventricular conduction block occurred on day 20. Although cardioversion succeeded in alleviating the atrial flatter, the patient experienced significant sinus arrest. The sinus arrest was alleviated 3 days later. Kawasaki disease-induced vasculitis and the arterial switch operation may both have influenced the sinus node dysfunction. Although sinus node function recovered, the possibility of progression into the sinus node dysfunction in the future should be considered.

Double vascular ring: a case report of double aortic arch and concurrent pulmonary artery sling.

BACKGROUND: Double aortic arch (DAA) and pulmonary artery sling (PAS) are vascular ring formations that present in neonates and infants with symptoms of respiratory stenosis. CASE SUMMARY: The patient was a girl with suspected ventricular septal defect (VSD), right aortic arch (AA), left patent ductus arteriosus, and bilateral superior vena cava (SVC) on foetal echography in the first day of life. The girl was delivered at 40 weeks and 4 days of gestation. Ventricular septal defect, DAA, coarctation of the left AA, and bilateral SVC were diagnosed. Contrast-enhanced computed tomography at Day 16 revealed PAS with concurrent anomalous tracheal branching in addition to DAA. The right A2 segmental artery, which supplies the right upper pulmonary artery, showed abnormal branching from the left pulmonary artery (LPA). At 3 months of age, VSD patching, left AA resection distal to the root of the left subclavian artery, arterial ligament dissection, and LPA replacement were performed. DISCUSSION: Pulmonary artery sling coexists with anomalous branching of the trachea and abnormal branching of the right pulmonary artery (RPA). Our patient had an extremely rare case of DAA concurrent with PAS and presented with anomalous tracheal and RPA branching. We were concerned that increased pulmonary blood flow caused by the VSD would exacerbate tracheal displacement. Radical surgery at 3 months of age resulted in good postoperative progress.

Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial.

BACKGROUND: Genetic studies have indicated possible involvement of the upregulated calcium-nuclear factor of activated T cells pathway in the pathogenesis of Kawasaki disease. We aimed to assess safety and efficacy of ciclosporin, an immunosuppressant targeting this pathway, for protection of patients with Kawasaki disease against coronary artery abnormalities. METHODS: We did a randomised, open-label, blinded endpoints trial involving 22 hospitals in Japan between May 29, 2014, and Dec 27, 2016. Eligible patients predicted to be at higher risk for intravenous immunoglobulin (IVIG) resistance were randomly assigned to IVIG plus ciclosporin (5 mg/kg per day for 5 days; study treatment) or IVIG (conventional treatment) groups, stratified by risk score, age, and sex. The primary endpoint was incidence of coronary artery abnormalities using Japanese criteria during the 12-week trial, assessed in participants who received at least one dose of study drug and who visited the study institution at least once during treatment. This trial is registered to Center for Clinical Trials, Japan Medical Association, number JMA-IIA00174. FINDINGS: We enrolled 175 participants. One patient withdrew consent after enrolment and was excluded and one patient (in the study treatment group) was excluded from analysis because of lost echocardiography data. Incidence of coronary artery abnormalities was lower in the study treatment group than in the conventional treatment group (12 [14%] of 86 patients vs 27 [31%] of 87 patients; risk ratio 0·46; 95% CI 0·25-0·86; p=0·010). No difference was found in the incidence of adverse events between the groups (9% vs 7%; p=0·78). INTERPRETATION: Combined primary therapy with IVIG and ciclosporin was safe and effective for favourable coronary artery outcomes in Kawasaki disease patients who were predicted to be unresponsive to IVIG. FUNDING: Japan Agency for Medical Research and Development (grant CCT-B-2503).

Intractable Back Pain After Coil Embolization of Giant Veno-Venous Collaterals in a Patient With Fontan Circulation.

Veno-venous collaterals are sometimes seen in patients after the Fontan procedure. A 28-year-old female with tricuspid atresia who underwent the Fontan procedure had oxygen desaturation due to a giant veno-venous collateral. Coil embolization was performed for the collateral. After the procedure, she complained of severe back pain. Anti-inflammatory analgesics and steroids were not effective, although carbamazepine promptly relieved the intractable pain. Treatment-related pain after coil embolization for veno-venous collaterals in patients with Fontan physiology is quite rare, although cardiologists must recognize a critical condition to be differentiated from vascular occlusion.

Siblings with Idiopathic Left Atrial Appendage Ostial Stenosis and Cor Triatriatum.

Isolated left atrial appendage (LAA) ostial stenosis is a very rare entity found coincidentally in adults by transesophageal echocardiography. A 3-month-old healthy infant was suspected as having cor triatriatum. His brother had a history of surgical treatment of cor triatriatum. A cardiac catheterization revealed a narrowed ostium of the LAA and confirmed the echocardiographic diagnosis of isolated LAA ostial stenosis. This is the first pediatric case of idiopathic LAA ostial stenosis. The siblings called our attention to the differential diagnosis and the etiopathogenesis between LAA ostial stenosis and cor triatriatum.

Cellulitis-Like Rash Associated with Kawasaki Disease.

We present the case of a 5-year-old-boy who developed a fever and cellulitis-like groin rash 5 days before developing conjunctivitis and 6 to 7 days before other typical signs of Kawasaki disease (KD) appeared. The cellulitis failed to respond to antibiotics and no pathogens were isolated. His fever and clinical signs resolved with intravenous immunoglobulin and high-dose aspirin after discontinuation of antibiotics. Nonbacterial cellulitis is a rare presenting sign of KD, but in the appropriate clinical setting and population, a diagnosis of KD should be considered when cellulitis and fever fail to respond to an appropriate antibiotic regimen and no pathogen can be isolated.

A Logical Method of Selecting an Approach for Amplatzer Septal Occluder Implantation: Using Transesophageal Echocardiography to Reduce Procedure Durations and Avoid Complications.

Percutaneous closure of atrial septal defects using an Amplatzer Septal Occluder (ASO) has recently become the procedure of first choice. However, when ASO deployment is difficult, procedures may be prolonged and complications may occur. We investigated a method for identifying cases in which ASO deployment would be difficult. After retrospectively identifying 70 patients (age: 4.1-70.4 years; body weight: 15.6-77.3 kg) who underwent atrial septal defect closure using an ASO in 2007 or later, we classified them into three groups: Group A, implantation by a conventional approach; Group B, implantation by a right upper pulmonary vein approach, and Group C,. change from a conventional to a right upper pulmonary vein approach. Characteristics of the groups were compared. Individually, none of the investigated characteristics was suitable for identifying difficult cases. Furthermore, we observed no consistent trends between aortic rim deficiency and ASO diameter, or between SG/IAS angle, which is the angle formed by the super stiff guidewire (SG) and the intra-atrial septum (IAS). However, the ASO diameter divided by the diameter of the left atrium (ASO/LA) correlated with the SG/IAS angle in Group C. Using this correlation, ASO implantation is predicted to be difficult in patients with an ASO/LA (%) ratio exceeding the (SG/IAS angle) x 1.44 + 48.1, which represents the 95th percentile of Group C. Graphing the SG/IAS angle and the ASO/LA ratio can identify cases in which ASO implantation may be difficult. We consider this method of selecting an approach to be extremely useful for avoiding various risks.