Therapeutic plasma exchange for refractory Kawasaki disease in children weighing less than 10 kg.

INTRODUCTION: Therapeutic plasma exchange (TPE) is used for treating refractory Kawasaki disease (KD); however, there are few reports on its use in small children. METHODS: Nine children with refractory KD who underwent TPE between January 2010 and December 2022 were retrospectively investigated. Data on patient demographics, inflammatory markers, coronary artery lesions (CALs), TPE settings and complications, and outcomes were examined. RESULTS: A total of 37 TPE sessions were performed on nine patients, with 3-6 sessions per patient. The median body weight was 8.9 kg. C-reactive protein, white blood cell (WBC), and interleukin-6 levels significantly decreased (p < 0.05). Of the 33 coronary arteries with CALs before TPE, 44% and 3% had CALs at 1 month and 1 year after TPE, respectively. Minor complications, such as mild hypocalcemia and naturally recovering coagulopathy, occurred without serious complications. CONCLUSIONS: TPE for refractory KD may be safe and effective in preventing CALs.

Pregnancy and delivery after percutaneous embolization with a combination of microvascular plugs and hydrogel-coated coils for unilateral diffuse pulmonary arteriovenous malformations: a case report.

Background: Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between the pulmonary arteries and veins; right-to-left shunts can cause hypoxaemia, emboli to systemic circulation, and brain abscesses. Cyanosis during pregnancy may increase the probability of premature birth or spontaneous abortion and may increase maternal cardiac complications. Case summary: We describe a case of a 24-year-old woman with diffuse multiple PAVMs localized to the left inferior lobe and chronic cyanosis. She had increased exertional fatigue and chronic headaches and was New York Heart Association class II, although her rest sitting peripheral oxygen saturation (SpO2) had remained unchanged at 83% over the past 20 years. She underwent percutaneous embolization with microvascular plugs and hydrogel-coated coils. A microvascular plug was placed as an anchor near the venous sac, followed by hydrogel-coated coil embolization of the proximal pulmonary artery. A total of six sessions of catheter intervention were performed. The embolization was successful, her hypoxaemia was relieved, and she was able to conceive and deliver. Three years have passed since the last session, and SpO2 97% has been maintained. Discussion: In the treatment of complex PAVMs, the combination of microvascular plugs and hydrogel-coated coils resulted in shorter procedure time, lower risk of migration of the embolus to the pulmonary veins, and less recanalization and revascularization. Percutaneous embolization of PAVMs resulted in safe delivery for the mother and child.

X-linked lymphoproliferative syndrome associated with Epstein-Barr virus encephalitis and lymphoproliferative disorder.

When treating patients with EBV encephalitis, the possibility of XLP should be considered. Once the diagnosis of XLP is made, aggressive treatment such as rituximab, and other immunosuppressive agents are desired for rapid transition to HSCT.

Anomalous origin of the left coronary artery from the right pulmonary artery.

ALCAPA should be considered in the differential diagnosis of myocarditis, and contrast-enhanced CT or catheterization should be considered even if coronary artery abnormalities are not detected on echocardiography.

An infant with acute decompensated heart failure caused by afterload mismatch due to tumour-induced secondary hypertension: a case report.

Background: Hypertensive crisis is a relatively rare condition among infants and usually occurs secondary to an underlying disease. If not managed promptly, it is life-threatening and can lead to irreversible damage to vital organs. While secondary hypertension due to tumours has been reported previously, acute decompensated heart failure is rare, especially in the paediatric population. Case summary: A 2-month-old female infant presented with poor feeding and poor body weight gain. She was extremely ill, and blood gas analysis showed prominent acidosis (pH 6.945). The patient was intubated and referred to our hospital for further care. Her arterial blood pressure (BP) was as high as 142/62 mmHg. Echocardiography showed decreased left ventricular function with an ejection fraction of 19.5% and a left ventricular end-diastolic diameter of 25.8 mm (Z score = 2.71). We promptly started treatment with antihypertensive drugs. She had no congenital heart disease or any lesions that may have caused an increased afterload. There was no palpable mass suggestive of the tumour; however, close examination with abdominal echo and subsequent contrast-enhanced computed tomography confirmed a left kidney mass. Blood tests suggested renin-dependent hypertension due to the tumour causing an excessive afterload. Laparoscopic left nephrectomy improved cardiac function improved as BP decreased. Discussion: Blood pressure measurement is often omitted in daily practice when examining infants because of difficulty in measurement. However, BP may be the only detectable sign in patients with secondary hypertension before decompensated heart failure, and BP should also be measured in infants.

Analysis of Coronary Arterial Aneurysm Regression in Patients With Kawasaki Disease by Aneurysm Severity: Factors Associated With Regression.

Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed. Methods and Results The cohort of the Z-Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving 44 institutions in Japan including 1006 patients with KD, was examined. CAAs were classified by the z score of their internal diameter in the acute phase: small (z<5), medium (5≤z<10), and large (z≥10). The lower limit of small CAA was based on the Japanese Ministry of Health, Labour and Welfare criteria. In the right coronary artery, the CAA regression rates 10 years after diagnosis were 95.5% for small, 83.2% for medium, and 36.3% for large. In the proximal left anterior descending artery, the regression rates 10 years after diagnosis were 95.3% for small, 80.1% for medium, and 28.8% for large. Cox regression analysis showed that diagnosis under the age of 1 year and onset of KD in 2010 to 2012 for the right coronary artery and the left anterior descending artery, and female for the right coronary artery were significantly associated with a high regression rate, whereas large CAAs for the right coronary artery and the left anterior descending artery were significantly associated with a low regression rate. Conclusions The current study, the largest Japanese study of its kind, found that small aneurysm, recent onset, and diagnosis under the age of 1 year predict regression, and that even giant aneurysms could regress. These data may contribute to long-term management of coronary aneurysms. Registration URL: https://www.umin.ac.jp/ctr/; Unique identifier: UMIN000010606.

Incidence of non-benign arrhythmia in neonatal intensive care unit: 18 years experience from a single center.

Background: Non-benign arrhythmias, which require urgent recognition and care in neonatal intensive care unit (NICU) settings, are rare but can severely impact neonates. We aimed to clarify the epidemiology and characteristics of non-benign arrhythmias and their influence on neonates. Methods: This single-center retrospective study included patients admitted to the NICU at Kurashiki Central Hospital between January 2001 and December 2019. Only patients with structurally normal hearts were included. The use of direct cardioversion (DC), antiarrhythmic agents, and the presence of risk factors was reviewed from medical records. Results: Of the 8082 admissions, 2919 patients (36.1%) were low birth weight infants (LBWI) weighing less than 1500 g. There were 23 patients with arrhythmias (nine of them were LBWIs) with an incidence of 0.28%. There were 16 patients with tachyarrhythmia (eight supraventricular tachycardia [SVT] cases, three atrial flutters [AFL] cases, three ventricular tachycardia cases, two junctional ectopic tachycardia cases), and seven with bradyarrhythmia (all with complete atrioventricular [AV] block). For tachyarrhythmia cases, seven patients required DC, and eight were on antiarrhythmic agents at the time of discharge. Two patients (28.5%) with complete AV block required pacemaker implantation before discharge. The treatment strategy was dependent on the type of arrhythmia. All patients were discharged without significant morbidities. Conclusions: The incidence of non-benign arrhythmias was as low as 0.28%. Arrhythmias can be managed successfully in neonates, yet risk factors related to mortality warrant further study.

Hypoplastic coronary artery disease and hypertension in a child: a case report.

BACKGROUND: Hypoplastic coronary artery disease (HCAD) is an extremely rare disease associated with a risk of sudden cardiac death. It is rarely recognized in a live paediatric patient. CASE SUMMARY: We report a case of HCAD in a patient who first presented with vomiting and poor feeding, suggestive of acute heart failure due to cardiomyopathy or acute myocarditis in infancy. Hypertension and signs of ischaemia became evident on electrocardiography and scintigraphy after his cardiac function fully recovered, and he was diagnosed with HCAD by angiography performed at the age of 8 years. He has remained under close observation with anti-hypertensives, aspirin, and exercise restriction. DISCUSSION: Although HCAD is a rare disease, it may not only cause ischaemia but may also result in heart failure and sudden cardiac death. It should be considered in any paediatric patient with heart failure. Mid-term follow-up visits might be necessary to detect signs of ischaemia in paediatric patients presenting with features of heart failure.

Quantification of end diastolic forward flow in two cases with pulmonary atresia with intact ventricular septum.

Similar to patients with repaired tetralogy of Fallot, patients with repaired pulmonary atresia with intact ventricular septum may need a reintervention at a later stage. Although the role of MRI in the long-term follow-up of patients with repaired tetralogy of Fallot has been established, the same has not been established for patients with repaired pulmonary atresia with intact ventricular septum. To define this role, we quantified the end-diastolic forward flow by fractioning it by the total flow of the main pulmonary artery in two cases during their long-term follow up after biventricular repair. In case 1, a male patient had hepatic congestion and a high end-diastolic forward flow fraction and underwent surgical take down to one and one-half ventricle repair at the age of 18 years. In case 2, a female patient, currently 13 years old, has an increasing end-diastolic forward flow fraction. She is under close observation as a potential candidate for one and one-half ventricle repair in the near future. Both patients had a high end-diastolic forward flow fraction of the total right ventricle output, suggesting that end-diastolic forward flow fraction may become a possible become a possible indicator of the adequacy of biventricular repair and the optimal timing for re-intervention.

Congenitally Corrected Transposition of the Great Arteries at Age 88 Years.

Most patients with congenital heart disease are diagnosed shortly after birth; however, some patients are overlooked and diagnosed in adulthood. We present the case of a man in whom congenitally corrected transposition of the great arteries (CCTGA) was first diagnosed at the age of 88 years. CCTGA is a rare disease; patients without frequently associated cardiac anomalies are considered to have long life expectancies; however, their prognosis remains unclear. This case is the oldest patient at the time of CCTGA diagnosis in the literature, which may provide new insights for CCTGA without frequently associated cardiac anomalies.

La plupart des cardiopathies congénitales sont diagnostiquées peu après la naissance; certains cas passent toutefois inaperçus et sont diagnostiqués une fois le patient arrivé à l'âge adulte. Nous présentons le cas d'un homme chez qui une transposition congénitalement corrigée des gros vaisseaux (TCCGV) a été diagnostiquée alors qu'il avait 88 ans. La TCCGV est une maladie rare; les patients qui ne présentent pas aussi l'une ou l'autre des anomalies cardiaques qui y sont fréquemment associées ont généralement une bonne espérance de vie, bien que le pronostic demeure obscur. De tous les cas rapportés dans la littérature médicale, le patient qui est présenté ici est le plus âgé qui ait jamais reçu un diagnostic de TCCGV, ce qui pourrait éclairer sous un jour nouveau la TCCGV non accompagnée des autres anomalies cardiaques qui y sont fréquemment associées.

Pregnancy and Delivery in Patients With Repaired Congenital Heart Disease　- A Retrospective Japanese Multicenter Study.

BACKGROUND: Although advances in cardiac surgery have led to an increased number of survivors with congenital heart disease (CHD), epidemiological data regarding the pregnancies and deliveries of patients with repaired CHD are scarce.Methods and Results:In this study, we retrospectively reviewed the clinical outcomes of pregnancies and deliveries of women with repaired CHD. Overall, 131 women with repaired CHD were enrolled and there were 269 gestations. All patients were classified as New York Heart Association (NYHA) Class I or II. The prevalence of cesarean sections was higher in patients with (CyCHD) than without (AcyCHD) a past history of cyanosis (51% vs. 19%, respectively; P<0.01). There were 228 offspring from 269 gestations and the most prevalent neonatal complication was premature birth (10%), which was more frequent in the CyCHD than AcyCHD group (15.7% vs. 5.6%, respectively; P<0.01). Five maternal cardiac complications during delivery were observed only in the CyCHD group (8%); these were classified as NYHA Class II and none was fatal. CONCLUSIONS: Delivery was successful in most women with repaired CHD who were classified as NYHA Class I or II, although some with CyCHD and NYHA Class II required more attention. Cesarean sections were more common in the CyCHD than AcyCHD group, and CyCHD may be a potential risk for preterm deliveries.

Chemoradiotherapy for Unresectable INI1-negative Chordoma in a Child.

The characteristics of chordomas in children are distinct from those in adults. In particular, the prognosis of patients with INI1-negative chordoma is dismal. The standard treatment for localized chordoma, complete surgical resection with a wide margin, is seldom feasible for chordomas arising at the clivus in children, mainly due to associated complications. Therefore, other treatments for unresectable chordomas in children, including chemoradiotherapy, must be explored. Here, we report a 7-year-old girl with an INI1-negative chordoma of the clivus, who responded to conventional chemotherapy plus radiotherapy. Without surgical resection, she remains alive after 1 year and 7 months of the initial diagnosis.

Emergence and Characterization of Acute Coronary Syndrome in Adults After Confirmed or Missed History of Kawasaki Disease in Japan: A Japanese Nationwide Survey.

Background: Acute coronary syndrome (ACS), which is emerging in adults long after confirmed (followed-up or lost-to-follow), or missed Kawasaki disease (KD), is poorly characterized. Methods and Results: A Japanese retrospective nationwide hospital-based questionnaire survey of ACS during 2000-09 was conducted to characterize such patients. Among a total of 67 patients (median age 35, male 76%) recruited, low conventional coronary risks (≤1/6) was noted in 75%, a diagnosis of ST-elevation and myocardial infarction or cardiac arrest in 66%, medication before ACS in 22% (warfarin in 4%), and no prior history of acute myocardial infarction in 94%. One-month mortality was 19%. KD diagnosis was made in 32 during acute illness (Group A), in which 17 were lost to follow, and retrospectively in the other 35 from coronary imaging at ACS (Group B). Group A developed ACS at lower coronary risks (≤2/5 in 87 vs. 65% in group B, p = 0.043) at a younger age (26.5 vs. 40 yo, p < 0.001). In group A, followed-up patients developed ACS under medication before ACS (87 vs. 0% in lost-to-follow patients, p < 0.001) for giant aneurysm in culprit lesions (69 vs. 29%, p = 0.030). One-month mortality was comparable between groups A and B, and between patients followed-up and lost-to-follow in group A. The culprit lesion in group A was characterized by the association of an aneurysm ≥6 mm in acute KD (100%), lack of significant stenosis (61%) or giant aneurysm (50%) in the long-term (median interval 16 y), and the presence of intravascular ultrasound-derived calcification at ACS (86%). Conclusions: The present retrospective nationwide questionnaire survey demonstrated nationwide emergence of initial ACS in young adults at low coronary risks, who are followed-up or lost-to-follow after confirmed KD and initial coronary aneurysms ≥6 mm.

Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study.

BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ±â€¯7 years old, follow-up: 18 ±â€¯6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ±â€¯1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years. CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.

[Recurrent thrombosed external hemorrhoids due to L-asparaginase administration in a Philadelphia chromosome-positive acute lymphoblastic leukemia patient].

A 13-year-old female developed L-asparaginase (L-ASP) -associated thrombosed external hemorrhoids (TEH) during chemotherapy for Philadelphia chromosome-positive acute lymphoblastic leukemia. While undergoing induction therapy combined with imatinib, she experienced intense anal pain a day after the four-time administration of L-ASP. The anal verge contained painful bluish hemorrhoids, which reportedly were absent before the therapy commencement. Hemorrhoids occurred 5-9 days after every L-ASP treatment, which was eventually diagnosed as L-ASP-associated TEH. After the failure of conservative treatment, opioid therapy was initiated. During myeloid reconstitution, she underwent divided ligation of hemorrhoids; however, the hemorrhoids became necrotic and formed an ulcerated tissue bed. This case suggests that while undertaking L-ASP therapy in adolescents and young adults with acute lymphoblastic leukemia, physicians should monitor signs of hemorrhoids and consider divided ligation when appropriate.

Recurrent Meningitis in an 11-year-old Girl with a Petrous Apex Cystic Lesion.

Cases of recurrent meningitis in elderly patients with a spontaneous cerebrospinal fistula have been reported, and in some of these patients, cystic lesions were thought to be the underlying cause. We report a case of recurrent meningitis in an 11-year-old Japanese girl with an arachnoid cyst in the petrous apex. Pulsation of the cystic lesion was thought to cause bone erosion, leading to the formation of a fistula. Magnetic resonance imaging was useful in evaluating the arachnoid cyst and fistula. During 2 years of follow-up, the osteolytic lesion enlarged and the rate of bone erosion was higher than expected.