Congenital bronchial atresia is a rare malformation that causes recurrent lung infections and pneumothorax. Pulmonary hypertension has not been reported as a complication in pediatric population, to the best of our knowledge. Our present patient is a 2-year-old girl with left pulmonary emphysema and pulmonary hypertension. She received pulmonary vasodilators and underwent left upper lobectomy. Pulmonary vasodilators were only partially effective; lobectomy was ineffective for pulmonary hypertension. Six years later after lobectomy, the patient was still receiving pulmonary vasodilators, and her pulmonary hypertension was gradually improving.

Background: Echocardiographic assessment of increased left ventricular wall thickness (LVWT) is still undetermined in children and adolescents.

Methods: LVWT was measured by interventricular septal thickness [IVST] and left ventricular posterior wall thickness [PWT] in 579 school-age healthy volunteers (293 males and 286 females) at 11 hospitals in Japan. LVWT was adjusted by body surface area (BSA): (adjusted LVWT value)=(measured LVWT)/(BSA)^α, where α is a parameter of the exponent. Three different BSA formulae (Haycock’s, DuBois’, and Fujimoto’s formulae) were used. To create cut-off points for increased LVWT to predict whether myocardial hypertrophy would progress in the future, the estimated prevalence of childhood hypertrophic cardiomyopathy was taken into consideration.

Results: The exponents α in the exponential models to adjust LVWT for BSA were 0.398 in boys and 0.445 in girls for IVST, and 0.470 in boys and 0.479 in girls for PWT when Haycock’s formula was used. The adjusted IVST and PWT values by BSA^α were normally distributed in both sexes. Tentative cut-offs for increased adjusted LVWT were 9.0 mm for all three grades in males and 8.4 and 8.8 mm for the 1st and the 7th/10th grade females, respectively. Tentative cut-offs for increased measured (unadjusted) LVWT were 8.5, 10.5, and 11.5 mm for the 1st, the 7th, and the 10th grade males and 8.0 and 10.5 mm for the 1st, and the 7th/10th grade females, respectively.

Conclusions: These tentative echocardiographic values help with the early assessment of increased LVWT in school-age children and adolescents and might help with early assessment of myocardial hypertrophy potentially present.

Pulmonary hypoplasia and atelectasis due to significant cardiomegaly during fetal life can lead to poor ventilation after the Starnes procedure in patients with tricuspid valve hypoplasia or Ebstein disease, making the postoperative management challenging. We report a neonate who had been diagnosed with tricuspid valve dysplasia with severe regurgitation at 21 weeks of gestation. The female baby underwent right ventricular exclusion on the day of her birth. Postoperatively, veno-venous extracorporeal membrane oxygenation support effectively treated severe hypoxia and pulmonary dysfunction.

Background: It has been reported that electromagnetic interference (EMI) occurs in abdominal cardiac implantable electrical devices (CIEDs) caused by tablets and laptops nearby. The actual number of patients with abdominal CIEDs remains unknown in Japan as well as frequency and causes of EMI.

Objective: This study aimed to determine the number of patients with abdominal CIEDs and incidence of EMI nationwide.

Methods: We conducted a questionnaire survey on the number of patients with abdominal CIEDs, incidence of magnet response, and the situation when EMI occurred at major institutions in Japan. Such events could threaten either pediatric or adult patients with congenital heart disease. The collected data were analyzed.

Results: We received survey responses from 119 institutions all over this country. The total number of abdominal CIEDs was 2411. Magnet response was reported by 11 institutions (9.2%), seen in 31 patients (1.3%). The CIED types affected by magnet response were pacemakers in 30 (98.7%) and implantable cardiac defibrillator (ICD) in one patient (1.3%). EMI occurred at school, home, and unknown in 11 (35.5%), 3 (9.7%), and 17 patients (54.8%), respectively. The probable causes were internal speakers in 7 (22.5%), tablet case magnets in 2 (6.5%), and otherwise unknown in 22 patients (71.0%). One patient experienced magnet response when a computer was turned on. Neither symptom related to magnet response, adverse events such as ventricular arrhythmia induction or ICD therapy inhibition were reported.

Conclusions: In Japan, 1% of 2411 patients with abdominal CIEDs had magnet response caused by tablet cases, tablets, and laptops. The event might have caused adverse events, and the issue should be brought to the attention of healthcare professionals and patients. Additionally, we highly suspect that the actual incidence of magnet response should be high than the figure indicated in this survey related to various study limitations.

Circumferential myocardial calcification within the left ventricular wall is rarely reported during the perioperative period of pediatric open heart surgery. This case report highlights a unique instance of whole circumferential myocardial calcification after surgery leading to fatal biventricular diastolic dysfunction. A 9-month-old female patient, diagnosed with type B interrupted aortic arch and 22q.11.2 microdeletion, underwent the Yasui operation following stage I hybrid palliation. Postoperatively, despite initial improvements, she developed lactic acidosis and peripheral edema, with catecholamines being unable to be discontinued. Serial imaging and cardiac catheter examinations revealed progressive whole circumferential myocardial calcification and worsening ventricular diastolic pressures. Surgical attempts to remediate these complications were unsuccessful, and the patient unfortunately succumbed shortly after the fourth intervention. Pathological examination revealed diffuse granular calcification deposits in the pericardium. Calcification was also observed in the submitted left ventricular myocardial tissue, with only a small amount of viable myocardial cells present. This case underscores the severity of myocardial calcification that can complicate the postoperative course in pediatric cardiac surgery, potentially leading to severe diastolic dysfunction and death. Early recognition and management would be critical, though effective treatment modalities need further exploration.

Numerous studies have substantiated the viability of aortic valve neocuspidization (AVNeo) in the pediatric demographics; however, the potential complications associated with this technique remain inadequately elucidated. A 12-year-old female patient presented with aortic regurgitation following the unroofing procedure 10 months earlier for the anomalous origin of the left coronary artery from the right coronary cusp exhibiting an intramural course. She underwent AVNeo this time. It appeared there had been a constriction at the sino-tubular junction, despite the absence of a notable pressure gradient. During the surgical procedure, all three leaets were replaced with patches of the glutaraldehyde-treated autologous pericardium that had degenerated and thickened due to prior surgery. During the weaning process from cardiopulmonary bypass, episodes of ventricular fibrillation recurrently occurred, coinciding with significant aortic stenosis (AS) indicated on intraoperative transesophageal echocardiography. Consequently, a decision was made to proceed with mechanical valve replacement in conjunction with annular enlargement. Following this revision, weaning from cardiopulmonary bypass was successfully achieved, culminating in patient’s discharge on the postoperative day 25. AVNeo was performed in an adolescent, necessitating intraoperative conversion to mechanical valve replacement. It is conceivable that both supra-aortic stenosis, clinically insignificant but morphologically significant, and the pericardium inadequately flexible limited opening of the reconstructed leaflets.

Background: Studies have indicated varying prevalence rates of Wolff–Parkinson–White (WPW) syndrome in young people, with various ages at initial episode of tachycardia. We aimed to investigate the incidence of subsequent tachycardia in asymptomatic schoolchildren who are first diagnosed as having WPW syndrome via secondary screening at our hospital.

Methods: From April 2011 to December 2023, 82 patients with suspected WPW syndrome based on school-based electrocardiograms (ECGs) underwent the second cardiac screening at our department. Of these, 62 patients (37 male patients) were diagnosed with WPW syndrome. Whether tachycardia occurred or not, age at onset if occurred, and ECG features were analyzed.

Results: Tachycardia occurred in 11 (18%) of the 62 patients, not significantly differing between sexes. Age at onset of tachycardia ranged from 10 to 16 years old, with the highest frequency observed at 12 years old (36%). Tachycardia was most frequently observed in patients with type A WPW syndrome (positive delta wave in lead V1); whereas the type of WPW syndrome did not differ between the tachycardia and the non-tachycardia groups. Additionally, no tachycardic episodes were observed in the 11 patients with intermittent WPW syndrome. The delta-wave amplitude seemed higher in the tachycardia group versus the non-tachycardia group, but this result was not statistically significant.

Conclusion: In the clinical follow-up of asymptomatic patients with WPW syndrome, when tachycardia attacks tend to occur should be taken into account to avoid delay in appropriate therapeutic intervention.

Vascular Ehlers-Danlos syndrome (vEDS) is a severe connective tissue disorder caused by pathogenic variants in the collagen type III alpha I chain gene (COL3A1), characterized by increased fragility of arteries and hollow organs. Typically, vEDS is diagnosed after complications related to arterial or intestinal tissue fragility. We herein report a case of an 11-year-old girl who was asymptomatically diagnosed with vEDS owing to a family history of sudden death. She was referred to our hospital at 5 years of age, after her father died suddenly at 35 years of age due to a ruptured thoracoabdominal aortic aneurysm. Despite showing no significant clinical features or vascular abnormalities indicative of connective tissue disorders during follow-up, genetic testing at 11 years of age identified a missense mutation in COL3A1, confirming the diagnosis of vEDS. Brain magnetic resonance imaging (MRI) and contrast-enhanced MRI of the thoraco-abdominal viscera revealed no abnormalities. Oral celiprolol therapy was initiated to prevent cardiovascular complications. Although diagnosing vEDS in childhood can be challenging due to its rarity and subtle clinical presentation, it is vital to consider this syndrome in pediatric patients with a family history of early-onset arterial aneurysms, dissections, or gastrointestinal vascular rupture.