Pediatric Cardiology

This study aimed to assess the outcome of cardiovascular diseases for patients with chronic active Epstein–Barr virus infection (CAEBV). The study enrolled 15 patients (7 boys and 8 girls) who fulfilled the diagnostic criteria for CAEBV, including 10 patients with T-cell type and 3 patients with natural killer (NK)-cell type. The median age at the CAEBV onset was 6.3 years (range, 1.2–17.8 years). Regular cardiologic studies were performed during the median follow-up period of 8 years (range, 2–20 years). Nine patients (60%) had cardiac diseases including coronary artery lesion (CAL) (n = 4, 44%), decreased left ventricular ejection fraction and pericardial effusion in (n = 3, 33%), complete atrioventricular block (n = 1), and sudden arrest (n = 1). The frequency of fever (78%, p = 0.04) or cytopenias (100%, p = 0.01), as the major symptom among patients with cardiac complications, was higher than among those without complications. The median time from disease onset to detection of CAL was 3.4 years (range, 1.8–8.6 years). The mean z-score increased to 3.98. Seven patients (78%) with cardiac complications died of disease progression, hematopoietic stem cell transplantation–related events, or both. In two patients, CAL regressed after allogeneic cord blood transplantation. Among CAEBV patients, CAL was the most common cardiac complication and could not be controlled without the eradication of EBV-infected T- and NK-cells.

Children with advanced heart failure may require ventricular assist devices (VAD) while awaiting heart transplantation. Currently, no data exist regarding the safety of exercise rehabilitation (ER) in children on VAD support. The purpose of this study was to determine the safety and feasibility of ER in children on VAD support awaiting heart transplantation. Eligible patients underwent VAD placement between 1998 and 2019; both inpatient and outpatient participants were included. After VAD implantation and when ambulatory, patients were enrolled in ER. Exercise sessions were scheduled three times a week and consisted of aerobic and musculoskeletal conditioning. A total of 29 patients (59% male, mean age 14 ± 3.2 years) were included with a median VAD duration of 120 ± 109 days. Cardiac diagnoses included cardiomyopathy (81%) and congenital heart disease (19%). VAD type included pulsatile (59%) and continuous-flow devices (41%). Eight hundred and sixty-four (85%) ER sessions were successfully completed and began at a mean of 49 days (range 19–108) after VAD implant. No adverse events, including episodes of hypotension, significant complex arrhythmia, or VAD malfunction occurred during exercise testing or ER, and no sessions were discontinued prematurely. Pediatric patients on VAD support can safely participate in ER with relatively high compliance, and sessions can be implemented early after VAD implantation. Given the safety profile, ER in pediatric VAD recipients, which is a modifiable pre-transplant risk factor that may improve functional capacity, warrants further study as a potential modality to improve post-transplant outcomes.

To evaluate complications and mortality following cardiac catheterization (CC) in patients under one year of age, we prospectively examined 312 CC on 273 patients from the centers in the New England Regional Infant Cardiac Program during a one-year period. Among CC performed on patients less than four months old, the incidence of complications requiring treatment was 12%; for patients 4–12 months, it was 1.5% (p<0.02). By pre-CC risk assessment, 13% of CC were high risk, 21% medium risk, and 66% low risk. The incidence of major complications was much greater among the High Risk (30%) compared with Medium Risk (14%) (p<0.05) and with Low Risk (4%) (p<0.001). The overall mortality rates 24 h, 48 h, and one week after CC were 3.8%, 8.3%, and 13.5%, including patients with inoperable lesions and those having cardiac surgery, while deaths directly attributable to a CC complication were 0, 0.3%, and 0.3%, respectively. We can predict, prior to CC, the high-risk cases where major complications and death are likeliest to occur. The incidence of death from CC-related complications is extremely low in patients under one year of age, compared with death from the underlying lesion or following cardiac surgery.

We prospectively employed a risk-stratified approach to first-stage palliation of hypoplastic left heart syndrome. High-risk features included severe tricuspid insufficiency, severe right ventricular dysfunction, a severely restrictive or intact atrial septum, an ascending aortic diameter ≤2 mm, late presentation, weight <2 kg, or significant extracardiac issues, Infants without high-risk features underwent a Norwood procedure (with Sano modification), whereas infants with high-risk features underwent a hybrid procedure consisting of bilateral pulmonary artery banding, ductal stenting, and atrial septostomy or a Norwood/Sano. Operative survival for 10 infants without high-risk features undergoing a Norwood/Sano procedure was 90%. Operative survival for 5 infants with high-risk features undergoing hybrid palliation was 100%, compared to 29% in 7 infants with high-risk features undergoing the Norwood/Sano procedure. Although only short-term data are available, this hybrid palliative procedure may have a role for infants with hypoplastic left heart syndrome and high-risk features.

A monozygotic twin boy with supravalvar aortic stenosis had reduction in the degree of obstruction with body growth. His twin brother had normal cardiac anatomy. To our knowledge, this is the first report of improvement without intervention in a patient with supravalvar aortic stenosis and of discordance of supravalvar aortic stenosis in monozygotic twins.