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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.

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.

SummaryVeno-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. (Int Heart J 2017; 58: 298-301) Key words: Cardiac catheterization, Complication, Neuropathic pain, Total cavopulmonary connection T he complete caval-pulmonary repair of single ventricle lesions has created a whole new spectrum of systemic artery to pulmonary artery and systemic vein to pulmonary vein collaterals/fistulae. 1) These collaterals provide competition to the already precarious forward pulmonary blood flow, produce extra volume on the single ventricle, and/ or create a right to left shunt with systemic desaturation of the patient. 1) Therefore, when "Fontan" type patients undergo catheterization, cardiologists should check these lesions carefully and occlude if they exist. 1,2)Occlusion of vital structures/organs, embolization of unwanted areas, migration of devices, and hemolysis are wellknown complications of vascular occlusions.1) To the best of our knowledge, there are no previous reports regarding the refractory pain associated with the procedures of coil embolization for veno-venous collaterals in patients who underwent the Fontan operation.Here, we describe a 28-year-old female with Fontan physiology who developed intractable severe back pain after undergoing coil embolization for giant veno-venous collaterals. Carbamazepine successfully controlled the intractable pain although many other analgesic drugs failed to relieve it. Case ReportA female patient with tricuspid atresia (type Ic) initially underwent banding of the pulmonary arterial trunk at 6 months of age. The second operation had not been carried out until she was 5 years old, when the total cavopulmonary connection (lateral tunnel) was completed. She received coil embolization of a veno-venous collateral from the innominate vein to the left atrium for the first time when she was 12 years old. At that time, her peripheral oxygen saturation was at around 90%. She was referred to our hospital when she moved to our city at 26 years of age. Two years after the first visit to our hospital, a follow-up enhanced computed tomography (CT) revealed many veno-venous collaterals ( Figure 1A). Her peripheral oxygen saturation was 86% under room air. Hence, she was admitted to our hospital for embolization of the collaterals. On admission, she was well and her vital signs were as follows; blood pressure 110/70 mmHg; pulse rate 78/min; body temperature 36.5°C; an...

Background:Patients with adult congenital heart disease (ACHD) who do not attend return-to-clinic (RTC) visits may experience, worsening of the condition and require advanced management. Purpose: To review the problems and countermeasures among ACHD who dropout (DO) during treatment. Subjects and Methods: Group A included 26 patients who made 27 RTC visits after DO, and Group B included 34 patients who did not attend 34 RTC visits after DO. We examined the diagnoses, surgical histories, DO age, New York Heart Association (NYHA) classification, DO reason, RTC reason, referral history, post-RTC treatments, and outcomes.Results: In Group A, DO age was < 15 years in 3, 16-19 years in 12, and adults in 12. The most common DO reasons were impossible diagnosis/treatment during childhood, treatment refusal, lack of subjective symptoms, and change of residence during adolescence and adulthood. Development of subjective symptoms was a commonest RTC reason, and symptomatic patients had worse conditions. Elucidating the clinical history was difficult in 18 patients (67%) at the RTC visit. Intervention was required in all cases with RTC visit, and the NYHA classification levels were similar/improved, except for 1 case of mortality. In Group B, 28 NYHA class I patients had minor conditions, but 4 of 6 class II patients had a DO history. Conclusion: Because DO is an important problem in ACHD treatment, pediatric cardiologist should educate patients from childhood to prevent DO during or after transition to adulthood.

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.