Proposed competencies for the performance of cardiovascular computed tomography in pediatric and adult congenital heart disease.

Cardiovascular computed tomography (CCT) is rated appropriate by published guidelines for the initial evaluation and follow up of congenital heart disease (CHD) and is an essential modality in cardiac imaging programs for patients of all ages. However, no recommended core competencies exist to guide CCT in CHD imaging training pathways, curricula development, or establishment of a more formal educational platform. To fill this gap, a group of experienced congenital cardiac imagers, intentionally inclusive of adult and pediatric cardiologists and radiologists, was formed to propose core competencies fundamental to the expert-level performance of CCT in pediatric acquired and congenital heart disease and adult CHD. The 2020 SCCT Guideline for Training Cardiology and Radiology Trainees as Independent Practitioners (Level II) and Advanced Practitioners (Level III) in Cardiovascular Computed Tomography (1) for adult imaging were used as a framework to define pediatric and CHD-specific competencies. Established competencies will be immediately relevant for advanced cardiac imaging fellowships in both cardiology and radiology training pathways. Proposed future steps include radiology and cardiology society collaboration to establish provider certification levels, training case-volume recommendations, and continuing medical education (CME) requirements for expert-level performance of CCT in pediatric and adult CHD.

Ventricular arrhythmias following balloon-expandable transcatheter pulmonary valve replacement in the native right ventricular outflow tract.

BACKGROUND: Ventricular arrhythmia incidence in children and adolescents undergoing transcatheter pulmonary valve replacement (TPVR) within the native right ventricular outflow tract (nRVOT) is unknown. We sought to describe the incidence, severity, and duration of ventricular arrhythmias and identify associated risk factors in this population. METHODS: This was a retrospective cohort study of 78 patients <21 years of age who underwent TPVR within the nRVOT. Patients were excluded for pre-existing ventricular arrhythmia or antiarrhythmic use. Study variables included surgical history, valve replacement indication, valve type/size, and ventricular arrhythmia. Univariable logistic regression models were used to evaluate factors associated with ventricular arrhythmias, followed by subset analyses. RESULTS: Nonsustained ventricular arrhythmia occurred in 26/78 patients (33.3%). The median age at the procedure was 10.3 years (interquartle range [IQR]: 6.5, 12.8). Compared with other nRVOT types, surgical repair with transannular patch was protective against ventricular arrhythmia incidence: odds ratio (OR): 0.35 (95% confidence interval [CI], 0.13-0.95). Patient weight, valve type/size, number of prestents, and degree of stent extension into the RVOT were not associated with ventricular arrhythmia occurrence. Beta blocker was started in 16/26 (61.5%) patients with ventricular arrhythmia. One additional patient was lost to follow-up. The median beta blocker duration was 46 days (IQR 42, 102). Beta blocker was discontinued in 10 patients by 8-week follow-up and in the remaining four by 9 months. CONCLUSIONS: Though common after balloon-expandable TPVR within the nRVOT, ventricular arrhythmias were benign and transient. Antiarrhythmic medications were successfully discontinued in the majority at 6- to 8-week follow-up, and in all patients by 20 months.

Left ventricular thrombus in the multisystem inflammatory syndrome in children associated with COVID-19.

A 3-year-old girl presenting with fever, mucocutaneous inflammation, and acute gastrointestinal symptoms met criteria for the multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C). Echocardiography showed severely decreased left ventricular (LV) function with an apical mass. After treatment with intravenous (IV) immunoglobulin, IV steroids, anakinra, milrinone, and systemic anticoagulation, her LV function rapidly improved and the mass became increasingly mobile. Given the risk of systemic embolization, the mass was excised through left ventriculotomy and pathology confirmed a thrombus.

Short-Term Left Ventricular Reverse Remodeling after Transcatheter Aortic Valve Replacement in Children.

BACKGROUND: There are no published data on left ventricular (LV) reverse remodeling after transcatheter aortic valve replacement (TAVR) in children. The aim of this study was to assess changes in LV echocardiographic parameters 6 months after TAVR in children. METHODS: This single-center, retrospective study included all 22 patients (age < 21 years) who underwent TAVR. The median age was 14.7 years (interquartile range, 13.3-15.9 years), median weight was 57 kg (interquartile range, 46.0-66.3 kg), and 59% of patients were male. Demographics, type and duration of aortic valve dysfunction, symptom and treatment data, and preprocedural and 6-month follow-up echocardiographic data (LV volume, mass, end-diastolic dimension, end-systolic dimension, ejection fraction [EF], sphericity, and longitudinal strain) were collected. Failure to reverse remodel at 6 months was defined as meeting at least two of the following: Z score ≥ 2 that was unchanged or increased from baseline for LV volume, mass, end-diastolic dimension, or end-systolic dimension; abnormally high sphericity index that was unchanged or increased; and abnormally low EF or longitudinal strain. Median, interquartile range, and range are reported for continuous variables, and pre- and post-TAVR data were compared using the Wilcoxon signed rank test. RESULTS: Eight patients (36%) had isolated aortic stenosis, four (18%) had isolated regurgitation, and 10 had (46%) mixed disease. Twelve (55%) had symptoms and 20 (91%) had prior surgical or catheter valve interventions. The primary complication was left bundle branch block, occurring in four children (18%). At 6 months, LV volume, mass, end-diastolic dimension, end-systolic dimension, and sphericity index improved. EF and strain were normal at baseline and at follow-up. Of three patients who failed to reverse remodel, two had left bundle branch block. Of three patients with persistent symptoms, one had failure of reverse remodeling. CONCLUSIONS: Most pediatric patients had evidence of reverse LV remodeling 6 months after TAVR, suggesting a possible alternative to surgical aortic valve replacement in this population. Functional parameters (EF and strain) were normal at baseline and follow-up. Future studies are needed to determine optimal timing of TAVR and to explore the association of postprocedural left bundle branch block on failed reverse remodeling and outcomes in this population.

Outcomes of Myocarditis in Patients with Normal Left Ventricular Systolic Function on Admission.

The objective of this study was to describe a cohort of patients with clinical myocarditis and normal left ventricular (LV) systolic function on admission. A retrospective chart review at seven tertiary pediatric hospitals identified patients aged < 19 years admitted with an ICD-9 code of myocarditis between 2008 and 2012. Patients were excluded if admission LV systolic ejection fraction was < 50%, fractional shortening (FS) was < 28% or if the admitting or consulting cardiologist did not suspect myocarditis. A total of 75 patients met inclusion criteria. The median age was 15.5 years with an Interquartile Range (IQR) of 13.6-16.6. 33% were female. Patients presented most commonly with chest pain (75%) and dyspnea (24%). On admission, median B-type natriuretic peptide (BNP) was 132 pg/mL (IQR 57-689) and median troponin I (TnI) was 8.4 ng/mL (IQR 2.0-20.3). Electrocardiogram revealed ST elevation in the majority (55%). Magnetic resonance imaging was obtained on 40%, with 63% of those showing evidence of inflammation. Therapies included inotropic support (15%), mechanical ventilation (12%), antiarrhythmic medications (9%), and Extracorporeal Membrane Oxygenation (5%). Those with poor outcomes were noted to have significantly higher BNP, TnI, and creatine kinase levels on presentation. One patient was transplanted and 35% were discharged on heart failure medications. At one year follow-up one patient had died of unspecified causes, 15% required readmission for cardiac reasons, and 21% continued on heart failure medications. The risk associated with clinical myocarditis in the setting of normal ventricular function at presentation may be higher than previously suspected.

Cardiac allograft vasculopathy following fecal microbiota transplantation for recurrent C. difficile infection.

We report the case of a 3-year-old male who developed recurrent Clostridium difficile infection after receiving an orthotopic heart transplant. Despite multiple courses of antibiotics, C. difficile infection was persistent and he underwent a fecal microbiota transplant. The patient responded with resolution of his diarrhea. However, within 2 months he developed severe mixed rejection with high circulating donor-specific antibodies and significant coronary vasculopathy. Organ dysfunction led to the need for re-transplantation. The patient's postoperative course has since been complicated by pneumatosis intestinalis and recurrent C. difficile infection.