Background: Premature ventricular complexes (PVCs) are generally benign but can sometimes be life-threatening and may cause sudden cardiac death. Although a standard 12-lead resting electrocardiogram (resting ECG) is commonly used for screening, its capability for detecting PVCs is limited, and a convenient alternative method is needed.

Methods: We retrospectively evaluated 106 patients who underwent both the Master’s two-step exercise test (Master’s two-step test) and 24-hour Holter monitoring (Holter monitoring) as part of a single outpatient evaluation. The primary outcome was defined by the frequency and consecutive occurrence of PVCs observed during Holter monitoring. The predictive performance of the resting ECG and the Master’s two-step test was compared using sensitivity, specificity, positive and negative predictive values, likelihood ratio, and decision curve analysis (DCA).

Results: Twelve patients (11%) had highly significant PVCs, defined as more than 10% of total heartbeats or at least three consecutive PVCs. The Master’s two-step test showed higher sensitivity (91.7%) and negative predictive value (98.7%) and a lower negative likelihood ratio (0.11) compared to the resting ECG. DCA indicated that the Master’s two-step test provided the greatest net benefit when the prevalence of highly significant PVCs ranged from 1.3% to 25%.

Conclusion: Incorporating the Master’s two-step test as a screening tool was superior to relying solely on the resting ECG to identify patients with clinically significant PVCs. This method may more effectively identify patients who require further evaluation with Holter monitoring.

Background: Despite improved fetal diagnosis rates (FDRs) for congenital heart disease (CHD), regional disparities persist. This study evaluated FDRs for critical CHD in Okinawa and assessed efficacy of fetal echocardiography in this district.

Methods: We conducted a retrospective review of 271 CHD cases from 2013 to 2022, all of whom underwent cardiac surgery or catheter intervention at our center. The cohort was divided into two groups; the earlier term (ET: 2013–2017, 154 cases) and the latter term (LT: 2017–2022, 117 cases). Critical CHD was defined as CHD requiring surgical or catheter-based intervention.

Results: FDRs for critical CHD significantly increased from 32% in the ET group to 54% in the LT group (p<0.05), markedly improving in patients with CHD and biventricular physiology (from 17% in the ET to 43% in the LT). Pre-22-week gestation antenatal diagnosis increased from 2.0% in the ET to 16.1% in the LT (p<0.05); nonetheless, 79% were diagnosed after 25 weeks of gestation. For hypoplastic left heart syndrome (HLHS), FDRs increased from 38% in the ET to 60% in the LT, with ductus closure-related shock decreasing from 38% to 20%.

Conclusion: Over the past decade, significant improvement in FDRs for critical CHD has occurred in Okinawa; however, it still trails behind urbane areas by 5 years. Although diseases with biventricular physiology and HLHS showed increasing FDRs, similar figures for total anomalous pulmonary venous connection and pre-22-week gestation diagnoses remained rather low. Enhancing primary screening efforts may involve visits to local hospitals by a fetal cardiologist or use of parameters such as nuchal translucency. To improve rural FDRs, training obstetric sonographers further and applying remote diagnosis techniques will be necessary.

ClearSight^© finger plethysmography is a noninvasive hemodynamic monitoring system which consists of an inflatable cuff that compresses the digital artery to achieve a constant blood volume by varying the pressure, which is displayed as a beat-to-beat pulse waveform. The main limitations are its lower accuracy when hypotension and the lack of validation in pediatric patients. We present a case of a 5-year-old child, with a history of chronic dilated cardiomyopathy due to perinatal myocardial infarction, who underwent elective strabismus surgery. A ClearSight^© System device was used off-label for continuous noninvasive monitoring, and allowed the optimal management of different hemodynamic events, even before they had an impact on blood pressure, during the entire procedure. ClearSight^© System monitoring could represent a feasible alternative to arterial cannulation in procedures without significant blood loss or shock and an effective decision-making tool during goal-directed anesthetic management of children with cardiomyopathy.

Patients with the Fontan circulation complicated by protein-losing enteropathy (PLE) often require prolonged intravenous antibiotic therapy. Extended hospitalizations may further compromise their functional status and quality of life. We report a 27-year-old woman with Fontan physiology and PLE who developed cellulitis and Streptococcus agalactiae bacteremia. According to the 2023 Duke–ISCVID criteria, the patient met one major criterion (typical streptococcal bacteremia) and two minor criteria, fulfilling the definition of possible infective endocarditis despite the absence of echocardiographic vegetation. The patient was treated with intravenous ceftriaxone for 36 days during hospitalization, followed by an additional 6 days of outpatient parenteral antimicrobial therapy (OPAT) administered via a peripherally inserted central catheter. After structured teach-back education and daily home-nursing visits, she remained afebrile with negative follow-up blood cultures, successfully completed a full 42-day regimen, and experienced no catheter-related complications or relapse at five-month follow-up. Remarkably, she was able to attend a company award ceremony during the treatment. This case illustrates that, with careful selection of patients, standardized education, and consistent nursing supervision, OPAT can be a safe and complete therapeutic option for high-risk patients with a Fontan-PLE circumstance while preserving functional capacity and psychosocial well-being.

We report a successful surgical case for progressive outflow graft obstruction in a patient with a left ventricular assist device for end-stage congenital heart disease. A 41-year-old female, who had physiological repair of congenitally corrected transposition of the great arteries at four years of age and left ventricular assist device implantation for systemic ventricular dysfunction at 38 years of age, was transported to our institution following a sudden episode of syncope with frequent low-flow alarms three years post-implantation. Despite inotropic support and volume optimization, device flow remained unimproved. Contrast-enhanced computed tomography revealed obstruction across the outflow graft near the aortic anastomosis, presumably due to external compression. Emergent surgical intervention was carried out, and device flow improved immediately after a full redo sternotomy. A hematoma and fibrous tissues were found near the outflow graft and they were completely removed. The patient had an uneventful postoperative recovery. Histopathological examination of the resected specimens showed features of a hematoma and a fibroadipose tissue with proliferation of vessels.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is an exceptionally rare congenital coronary anomaly. We report a rare pediatric case in which ARCAPA coexisted with partial anomalous pulmonary venous connection (PAPVC), an association not previously described. A 2-year-old girl was referred for evaluation of coronary dilatation noted on echocardiography. Enhanced computed tomography confirmed ARCAPA and revealed right upper pulmonary vein drainage into the superior vena cava. Myocardial perfusion and ventricular function remained preserved. Surgical intervention was undertaken due to the potential risk of life-threatening arrhythmia related to chronic coronary steal. The patient underwent right coronary artery reimplantation and rerouting of the PAPVC drainage to the left atrial cavity. Postoperative imaging demonstrated excellent patency of the reimplanted coronary artery and unobstructed pulmonary venous return. She remained asymptomatic during one year of follow-up.

Extramedullary lesions of pediatric acute lymphoblastic leukemia (ALL) can present as tumorous masses outside the bone marrow and, in rare cases, may occur within the cardiac chambers. We report a case of a 14-year-old girl previously diagnosed with ALL at the age of 12 years. At that time, chest CT and echocardiography performed as part of a systemic evaluation revealed multiple masses approximately 20 mm in size in the right atrium and right ventricle, raising suspicion of extramedullary involvement of ALL. Due to poor general condition, surgical intervention was deferred in favor of prioritizing chemotherapy for the underlying disease. Serial follow-up using echocardiography and electrocardiography demonstrated no significant change in tumor size, and the patient’s overall condition remained stable. After induction of remission, pulmonary perfusion scintigraphy was performed for the first time, revealing a segmental perfusion defect in the right lower lobe, leading to a diagnosis of asymptomatic pulmonary embolism (1 year and 6 months after leukemia onset). Given the presence of multiple residual masses within the cardiac chambers, surgical resection was performed, and several nodular lesions were removed. Pulmonary embolism associated with intracardiac extramedullary lesions of ALL may be life-threatening. To prevent tumor detachment and embolization, early surgical resection should be considered in conjunction with systemic treatment of the underlying malignancy.

In some cases of Kawasaki disease (KD), low-grade fever and mildly elevated inflammatory markers persist following initial intravenous immunoglobulin (IVIg) therapy. These cases are associated with a higher incidence of coronary arterial lesions (CALs) compared to those with a favorable response to the initial treatment, emphasizing the importance of prompt inflammation resolution. However, it remains challenging to determine the optimal additional treatment in each patient. We report a case series documenting the efficacy of cyclosporine A in such instances of KD. Of the 90 patients diagnosed with KD and admitted to our institution between 2020 and 2024, 10 received cyclosporine A therapy. In these patients, a persistent fever (≥37.5°C), mild primary symptoms, and mildly elevated inflammatory markers persisted after initial IVIg administration. Cyclosporine A monotherapy was initiated, and its blood concentration was monitored. The patients’ age ranged from 3 months to 4 years and 5 months. All patients initially received administration of IVIg and moderate-dose aspirin; prednisolone was added if the IVIg resistance prediction score was ≥5. Cyclosporine A was effective in 8 of the 10 cases, and no CALs developed after cyclosporine A administration. In the remaining 2 cases where cyclosporine A was ineffective, additional treatment with IVIg and infliximab were administered. Compared with other additional treatments, cyclosporine A is less invasive, more easily achieved, and does not obscure inflammatory activity. Furthermore, cyclosporine A was safely given even in infants, without adverse events. Our findings suggest that cyclosporine A may be an effective additional treatment for KD patients with mild and persistent inflammation.