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.

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.

Arrhythmia risk and ß-blocker therapy in pregnant women with long QT syndrome.

BACKGROUND: Pregnancy is one of the biggest concerns for women with long QT syndrome (LQTS). OBJECTIVES: This study investigated pregnancy-related arrhythmic risk and the efficacy and safety of ß-blocker therapy for lethal ventricular arrhythmias in pregnant women with LQTS (LQT-P) and their babies. METHODS: 136 pregnancies in 76 LQT-P (29±5 years old; 22 LQT1, 36 LQT2, one LQT3, and 17 genotype-unknown) were enrolled. We retrospectively analysed their clinical and electrophysiological characteristics and pregnancy outcomes in the presence (BB group: n=42) or absence of ß-blocker therapy (non-BB group: n=94). RESULTS: All of the BB group had been diagnosed with LQTS with previous events, whereas 65% of the non-BB group had not been diagnosed at pregnancy. Pregnancy increased heart rate in the non-BB group; however, no significant difference was observed in QT and Tpeak-Tend intervals between the two groups. In the BB group, only two events occurred at postpartum, whereas 12 events occurred in the non-BB group during pregnancy (n=6) or postpartum period (n=6). The frequency of spontaneous abortion did not differ between the two groups. Fetal growth rate and proportion of infants with congenital malformation were similar between the two groups, but premature delivery and low birthweight infants were more common in those taking BB (OR 4.79, 95% CI 1.51 to 15.21 and OR 3.25, 95% CI 1.17 to 9.09, respectively). CONCLUSIONS: Early diagnosis and ß-blocker therapy for high-risk patients with LQTS are important for prevention of cardiac events during pregnancy and the postpartum period, and ß-blocker therapy may be tolerated for babies in LQT-P cases.

Three-dimensional echocardiographic pictures of isolated double-orifice mitral valve.

We present a case of a 12-year-old boy with a rare form of cardiac anomaly, isolated double-orifice mitral valve. He was referred to our hospital at 1.5 years old because of heart murmur. Two-dimensional echocardiography showed double-orifice mitral valve without any associated cardiac anomalies. He has been followed carefully without any medication for 11 years. He has had no symptoms and an excellent natural course thus far.