Identifying thrombus formation in Fontan circulation has been highly variable, with reports between 17 and 33%. Initially, thrombus detection was mainly done through echocardiograms. Delayed-enhancement cardiac MRI is emerging as a more effective imaging technique for thrombus identification. This study aims to determine the prevalence of occult cardiac thrombosis in patients undergoing clinically indicated cardiac MRI.

A retrospective chart review of children and adults in the Duke University Hospital Fontan registry who underwent delayed-enhancement cardiac MRI. Individuals were excluded if they never received a delayed-enhancement cardiac MRI or had insufficient data. Demographic characteristics, native heart anatomy, cardiac MRI measurements, and thromboembolic events were collected for all patients.

In total, 119 unique individuals met inclusion criteria with a total of 171 scans. The median age at Fontan procedure was 3 (interquartile range 1, 4) years. The majority of patients had dominant systemic right ventricle. Cardiac function was relatively unchanged from the first cardiac MRI to the third cardiac MRI. While 36.4% had a thrombotic event by history, only 0.5% (1 patient) had an intracardiac thrombus detected by delayed-enhancement cardiac MRI.

Despite previous echocardiographic reports of high prevalence of occult thrombosis in patients with Fontan circulation, we found very low prevalence using delayed-enhancement cardiac MRI. As more individuals are reaching adulthood after requiring early Fontan procedures in childhood, further work is needed to develop thrombus-screening protocols as a part of anticoagulation management.

In neonatal vein of Galen aneurysmal malformation, vein of Galen aneurysmal malformation echocardiography remains the mainstay for early detection and explains various haemodynamic changes occurring due to a large systemic arterio-venous shunt. However, there is limited evidence of echocardiography in risk stratifying neonatal vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation. The objective of this study was to identify echocardiographic parameters that could be associated with major outcomes and guide timing of neuro-intervention.

In this retrospective chart review, infants < 28 days of age with the diagnosis of vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation were included. Demographic, clinical, and echocardiographic parameters were compared in neonates who survived or died with neonatal presentation. A risk algorithm model based on key echocardiographic parameters was developed to determine those who are at risk of early death.

Of the 19 neonates included, with median birth weight 3.1 kg (IQR 2.58–3.36), nine (47%) neonates died at median age of 5 days (IQR 4–17). All neonates showed retrograde diastolic flow at the level of descending aorta by colour Doppler on the first post-natal echocardiogram at median age of 2 days (IQR 1–5.5). An aortic antegrade-to-retrograde velocity time integral ratio of < 1.5 and supra-systemic pulmonary artery pressure had 100% positive predictive value of death (p = 0.029), whereas aortic antegrade-to-retrograde velocity time integral ratio of > 1.5 and sub-systemic pulmonary artery pressure had 100% positive predictive value of survival (p = 0.029).

Combination of aorta antegrade-to-retrograde velocity time integral ratio and degree of pulmonary hypertension on the first post-natal echocardiogram may help stratify the severity of disease and guide optimal timing for neuro-intervention for neonatal vein of Galen aneurysmal malformation.

During the COVID-19 pandemic, Kentucky prohibited elective medical procedures from 3/18/2020-4/27/2020. We sought to determine if cessation of elective procedures in Kentucky during the COVID-19 pandemic resulted in a decrease in the proportion of rarely appropriate outpatient transthoracic echocardiograms interpreted at the open echocardiography lab at Norton Children’s Hospital.

A retrospective chart review was conducted comparing proportions of rarely appropriate outpatient paediatric transthoracic echocardiograms performed pre-COVID (3/21/2019-4/28/2019) and during COVID (3/19/2020-4/27/2020). Transthoracic echocardiogram indication was determined by chart review and echocardiogram reports. Indication appropriateness was evaluated using paediatric appropriate use criteria for initial outpatient transthoracic echocardiogram or CHD follow-up as applicable.

Of transthoracic echocardiograms pre-COVID, 100 (37.7%) were rarely appropriate versus 18 (20.2%) during COVID. Pre-COVID, paediatric cardiologists tended to order fewer rarely appropriate transthoracic echocardiograms than paediatricians (35.9% versus 46.4%), although this difference was not statistically significant. Cardiologists ordered the majority of outpatient transthoracic echocardiograms during COVID (77/89, 86.5%), limiting the ability to compare transthoracic echocardiogram indications by provider type. There was no significant difference in diagnostic yield of initial outpatient transthoracic echocardiograms with (13.0%) abnormal studies pre-COVID versus 7 (15.5%) during COVID.

While elective procedures were prohibited in Kentucky during the COVID-19 pandemic, a decrease in the proportion of rarely appropriate outpatient paediatric transthoracic echocardiograms was observed. There was no significant difference in diagnostic yield of initial outpatient transthoracic echocardiograms between time periods, suggesting that clinically significant echocardiogram findings were still detected despite more prudent utilisation of echocardiography during this time.

To evaluate the association of systolic blood pressure percentile, race, and body mass index with left ventricular hypertrophy on electrocardiogram and echocardiogram to define populations at risk.

This is a retrospective cross-sectional study design utilising a data analytics tool (Tableau) combining electrocardiogram and echocardiogram databases from 2003 to 2020. Customized queries identified patients aged 2–18 years who had an outpatient electrocardiogram and echocardiogram on the same date with available systolic blood pressure and body measurements. Cases with CHD, cardiomyopathy, or arrhythmia diagnoses were excluded. Echocardiograms with left ventricle mass (indexed to height2.7) were included. The main outcome was left ventricular hypertrophy on echocardiogram defined as Left ventricle mass index greater than the 95th percentile for age.

In a cohort of 13,539 patients, 6.7% of studies had left ventricular hypertrophy on echocardiogram. Systolic blood pressure percentile >90% has a sensitivity of 35% and specificity of 82% for left ventricular hypertrophy on echocardiogram. Left ventricular hypertrophy on electrocardiogram was a poor predictor of left ventricular hypertrophy on echocardiogram (9% sensitivity and 92% specificity). African American race (OR 1.31, 95% CI = 1.10, 1.56, p = 0.002), systolic blood pressure percentile >95% (OR = 1.60, 95% CI = 1.34, 1.93, p < 0.001), and higher body mass index (OR = 7.22, 95% CI = 6.23, 8.36, p < 0.001) were independently associated with left ventricular hypertrophy on echocardiogram.

African American race, obesity, and hypertension on outpatient blood pressure measurements are independent risk factors for left ventricular hypertrophy in children. Electrocardiogram has little utility in the screening for left ventricular hypertrophy.

Paediatricians with Expertise in Cardiology assess children with a full history, examination, and often perform an echocardiogram. A minority are then referred to an outreach clinic run jointly with a visiting paediatric cardiologist. The accuracy of the echocardiography diagnosis made by the Paediatrician with Expertise in Cardiology is unknown.

We conducted a retrospective review of clinic letters for children seen in the outreach clinic for the first time between March, 2004 and March, 2011. Children with CHD diagnosed antenatally or elsewhere were excluded. We recorded the echocardiography diagnosis made by the paediatric cardiologist and previously by the Paediatrician with Expertise in Cardiology.

The Paediatrician with Expertise in Cardiology referred 317/3145 (10%) children seen in the local cardiac clinics to the outreach clinic over this period, and among them 296 were eligible for inclusion. Their median age was 1.5 years (range 1 month–15.1 years). For 244 (82%) children, there was complete diagnostic agreement between the Paediatrician with Expertise in Cardiology and the paediatric cardiologist. For 29 (10%) children, the main diagnosis was identical with additional findings made by the paediatric cardiologist. The abnormality had resolved in 17 (6%) cases by the time of clinic attendance. In six (2%) patients, the paediatric cardiologist made a different diagnosis. In total, 138 (47%) patients underwent a surgical or catheter intervention.

Paediatricians with Expertise in Cardiology can make accurate diagnoses of CHD in children referred to their clinics. This can allow effective triage of children attending the outreach clinic, making best use of limited specialist resources.

In CHD, three-dimensional strain analysis may overcome limitations of Doppler and two-dimensional strain of the left ventricle. The aims of this study were to evaluate feasibility and reproducibility of three-dimensional longitudinal, circumferential, and radial systolic strain by three-dimensional speckle-tracking echocardiography compared with two-dimensional echocardiography.

Patients with CHD, biventricular circulation with a systemic left ventricle, and who had two- and three-dimensional imaging performed on the same day from 2010 to 2014 were included. Quantitative two- and three-dimensional strain analyses were performed (two-dimensional cardiac performance analysis version 1.2 and four-dimensional left ventricular analysis version 3.1). Intra- and inter-observer variabilities were calculated on 25 studies.

A total of 30 patients, including 19 (61%) males, with a median age of 3.6 years (0.1–22 years) were included. The mean fractional shortening was 34.6±5.3%, and the mean ejection fraction was 62.0±6.4%. Measurement of two- and three-dimensional strain was feasible in >95% of segments. Good correlation was observed between longitudinal and circumferential strain (r=0.92, p⩽0.001 and r=0.87, p⩽0.001), but not radial strain (r=0.29, p=0.2). Intra- and inter-observer agreements were better for three-dimensional compared with two-dimensional strain, and better for both two- and three-dimensional longitudinal and circumferential strains compared with radial strain.

Left ventricular three-dimensional strain analysis is feasible in children with CHD. The reproducibility of longitudinal and circumferential strain by three-dimensional analyses is better. Further longitudinal studies are warranted for the potential clinical application of this new technology.

Chest pain is a common presenting complaint to paediatrics, paediatric cardiology, and paediatric emergency departments. In this study, we prospectively evaluated clinical characteristics and causes of chest pain in children referred to our paediatric cardiology unit.

A total of 380 children were included. Associated symptoms and past and family histories were evaluated. All patients underwent physical examination. The following studies were performed: complete blood count in all patients; fasting lipid profiles in overweight and obese children and children with a family history of premature cardiovascular disease; and electrocardiogram, chest X-ray, and echocardiogram in all patients. If necessary, 24-hour electrocardiogram monitoring or exercise stress tests were performed. Patients with a history of positive psychological findings were evaluated by a child psychiatrist.

The most common causes of chest pain were musculoskeletal disorders (37.1%), idiopathic chest pain (29.2%), and miscellaneous disorders, for example precordial catch syndrome (15%), respectively. Only 1 of 380 (0.3%) patients had chest pain due to a cardiac disorder. Electrocardiograms were abnormal in 4 of 380 (1.1%) patients. A total of 9 of 380 patients (2.3%) had dyslipidaemia.

Although a paediatric cardiology referral may provide reassurance to the primary care and emergency department physicians, our results show that cardiac aetiologies for paediatric chest pain are very rare. We think that many patients in our study were adequately evaluated only by careful history, and physical examination. Therefore, we suggest that it may not be necessary to use echocardiogram in the routine evaluation of children with chest pain.