Aortopathy in Turner syndrome is associated with aortic dilation, and the risk of dissection is increased when the aortic size index is ≥ 2–2.5 cm/m2. We evaluated the aortic biophysical properties in paediatric Turner syndrome using cardiac MRI to determine their relationship to aortic size index.

Turner syndrome patients underwent cardiac MRI to evaluate ventricular function, aortic dimensions, and biophysical properties (aortic stiffness index, compliance, distensibility, pulse wave velocity, and aortic and left ventricular elastance). Spearman correlation examined correlations between these properties and aortic size index. Data was compared to 10 controls.

Of 25 Turner syndrome patients, median age 14.7 years (interquartile range: 11.0–16.8), height z score −2.7 (interquartile range: −2.92 – −1.54), 24% had a bicuspid aortic valve. Turner syndrome had increased diastolic blood pressure (p < 0.001) and decreased left ventricular end-diastolic (p < 0.001) and end-systolic (p = 0.002) volumes compared to controls. Median aortic size index was 1.81 cm/m2 (interquartile range: 1.45–2.1) and 7 had an aortic size index > 2 cm/m2. Aortic and left ventricular elastance were greater in Turner syndrome compared to controls (both p < 0.001). Increased aortic size index correlated with increased aortic elastance (r = 0.5, p = 0.01) and left ventricular elastance (r = 0.59, p = 0.002) but not aortic compliance. Higher ascending aortic areas were associated with increased aortic compliance (r = 0.44, p = 0.03) and left ventricular elastance (r = 0.49, p = 0.01).

Paediatric Turner syndrome with similar aortic size index to controls showed MRI evidence of abnormal aortic biophysical properties. These findings point to an underlying aortopathy and provide additional parameters that may aid in determining risk factors for aortic dissection.

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 2020, reports revealed cases called multisystem inflammatory syndrome in children and adolescents temporally related to COVID-19 or multisystem inflammatory syndrome in children. A small proportion of patients suffer from persistent left ventricular dysfunction at discharge. The primary aim was to investigate if myocardial impairment persists during follow-up in these patients.

Children fulfilling the criteria for multisystem inflammatory syndrome in children with cardiac involvement hospitalized between December 2020 and February 2022 were included in this retrospective single centre study. Cardiac MRI was performed six months after the onset of symptoms to evaluate possible persistent myocardial damage.

Fifteen patients (80% male) with a median age of 8 years (interquartile range 4.5 - 13.5 years) were included. Upon admission, eight patients (53%) presented with reduced left ventricular function, with a median left ventricular ejection fraction of 54% (interquartile range 49.5%-61.5%) on transthoracic echocardiography. Elevated levels of cardiac-specific markers were found in 14 patients (93%). Cardiac MRI was performed in 12 patients at a median of 190.5 days after the onset of symptoms. Nine patients (75%) had normal left ventricular function, with a median left ventricular ejection fraction of 59.45%, while the remaining patients showed mildly to moderately reduced values. None of the patients showed signs of late gadolinium enhancement, indicating the absence of persistent myocardial scarring.

During a follow-up of 6.2 months, mild to moderate cardiac impairment was revealed in 25% of patients evaluated by cardiac MRI. Although a majority of patients do not show signs of cardiac impairment, close follow-ups should be performed in a proportion of patients.

Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal.

We conducted a retrospective cohort study of children with common atrioventricular canal who underwent MRI prior to repair. Associations between MRI and echocardiographic measures and surgical outcome were tested using logistic regression, and models were compared using area under the receiver operator characteristic curve.

We included 28 patients (median age at MRI: 5.2 months). The optimal MRI model included the novel end-diastolic volume index (using the ratio of left ventricular end-diastolic volume to total end-diastolic volume) and the left ventricle–right ventricle angle in diastole (area under the curve 0.83, p = 0.041). End-diastolic volume index ≤ 0.18 and left ventricle–right ventricle angle in diastole ≤ 72° yield a sensitivity of 83% and specificity of 81% for successful biventricular repair. The optimal multimodality model included the end-diastolic volume index and the echocardiographic atrioventricular valve index with an area under the curve of 0.87 (p = 0.026).

Cardiac MRI can successfully predict successful biventricular repair in patients with unbalanced common atrioventricular canal utilising the end-diastolic volume index alone or in combination with the MRI left ventricle–right ventricle angle in diastole or the echocardiographic atrioventricular valve index. A prospective cardiac MRI study is warranted to better define the multimodality characteristic predictive of successful biventricular surgery.

Assessing the hepatic status of children with CHD is very important in the post-operative period. This study aimed to assess the usefulness of paediatric liver T1/T2 values and to evaluate the impact of respiration on liver T1/T2 values.

Liver T1/T2 values were evaluated in 69 individuals who underwent cardiac MRI. The mean age of the participants was 16.2 ± 9.8 years. Two types of imaging with different breathing methods were possible in 34 participants for liver T1 values and 10 participants for liver T2 values.

The normal range was set at 620–830 msec for liver T1 and 25–40 ms for liver T2 based on the data obtained from 17 healthy individuals. The liver T1/T2 values were not significantly different between breath-hold and free-breath imaging (T1: 769.4 ± 102.8 ms versus 763.2 ± 93.9 ms; p = 0.148, T2: 34.9 ± 4.0 ms versus 33.6 ± 2.4 ms; p = 0.169). Higher liver T1 values were observed in patients who had undergone Fontan operation, tetralogy of Fallot operation, or those with chronic viral hepatitis. There was a trend toward correlation between liver T1 values and liver stiffness (R = 0.65, p = 0.0004); and the liver T1 values showed a positive correlation with the shear wave velocity (R = 0.62, p = 0.0006).

Liver T1/T2 values were not affected by breathing patterns. Because liver T1 values tend to increase with right heart overload, evaluation of liver T1 values during routine cardiac MRI may enable early detection of future complications.

Young-onset hypertension is defined as hypertension diagnosed before the age of 40 years. Aortic pulse wave velocity is an indication of aortic stiffness. MRI assessment has been well verified compared to invasive pressure recordings for evaluating aortic pulse wave velocity. In this study, we aimed to determine whether aortic stiffness played a role in the aetiology of young-onset hypertension by calculating pulse wave velocity using MRI.

We enrolled 20 patients diagnosed with young-onset hypertension and 20 volunteers without hypertension. Aortic pulse wave velocity was measured by cardiac MRI and protocol for the pulse wave velocity measurement involved the use of a 1.5 T scanner to acquire velocity-encoded, phase-contrast transverse aortic cine images. Sagittal oblique images used to measure the distance (ΔX) between the ascending aorta and descending aorta for the calculation of pulse wave velocity. The aortic flow versus time curves of ascending aorta and descending aorta were automatically obtained from the phase-contrast MRI images. Using these curves, the temporal shift (ΔT) was measured by Segment Medviso.

The mean pulse wave velocity was 8.72 (SD 2.34) m/second (range: 7–12.8 m/second) for the patient group and 5.96 (standard deviation 1.86) m/second (range: 4.8–7.1 m/second) for the control group. The pulse wave velocity values were significantly higher in the patient group compared to the control group (p < 0.001).

Aortic stiffness may play a role in the aetiology of young-onset hypertension and serve as a non-invasive and reliable screening tool when measured by MRI.

T1 mapping is a recently developed imaging analysis method that allows quantitative assessment of myocardial T1 values obtained using MRI. In children, MRI is performed under free-breathing. Thus, it is important to know the changes in T1 values between free-breathing and breath-holding. This study aimed to compare the myocardial T1 mapping during breath-holding and free-breathing.

Thirteen patients and eight healthy volunteers underwent cardiac MRI, and T1 values obtained during breath-holding and free-breathing were examined and compared. Statistical differences were determined using the paired t-test.

The mean T1 values during breath-holding were 1211.1 ± 39.0 ms, 1209.7 ± 37.4 ms, and 1228.9 ± 52.5 ms in the basal, mid, and apical regions, respectively, while the mean T1 values during free-breathing were 1165.1 ± 69.0 ms, 1103.7 ± 55.8 ms, and 1112.0 ± 81.5 ms in the basal, mid, and apical regions, respectively. The T1 values were lower during free-breathing than during breath-holding in almost all segments (basal: p = 0.008, mid: p < 0.001, apical: p < 0.001). The mean T1 values in each cross section were 3.1, 7.8, and 7.7% lower during free-breathing than during breath-holding in the basal, mid, and apical regions, respectively.

We found that myocardial T1 values during free-breathing were about 3–8% lower in all cross sections than those during breath-holding. In free-breathing, it may be difficult to assess myocardial T1 values, except in the basal region, because of underestimation; thus, the findings should be interpreted with caution, especially in children.

Growth differentiation factor-15 is a novel biomarker of increasing importance in cardiovascular diseases. This study aimed to evaluate the relationship between ventricular measurements assessed by cardiac magnetic resonance imaging (MRI) and serum growth differentiation factor-15 levels in children with surgically corrected tetralogy of Fallot.

Serum growth differentiation factor-15 levels were measured in 40 patients (mean age: 15.2 ± 2.9 years; 52.5% male; 87.5% NYHA I). End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles and pulmonary regurgitation fraction were measured on cardiac MRI. The correlation between growth differentiation factor-15 levels and cardiac MRI parameters of the patients was investigated. Also, growth differentiation factor-15 levels of the patients were compared with healthy controls since reference values have not been determined in children.

The mean growth differentiation factor-15 level was 254.9 ± 6.3 pg/ml in the patient group. There was no correlation between growth differentiation factor-15 levels and cardiac MRI parameters in patients. Also, there was no significant difference in growth differentiation factor-15 levels between the patients and control groups.

The serum levels of growth differentiation factor-15 were uncorrelated with ventricular size, function, and pulmonary regurgitation fraction assessed by cardiac MRI in children with operated tetralogy of Fallot. Moreover, growth differentiation factor-15 levels were not different in these patients from healthy children.

Echo assessment of right ventricular function is difficult due to its complex geometry and regional differences in wall motion. In patients with repaired tetralogy of Fallot, cardiac MRI is the gold standard for assessment of right ventricular function. There is scant data regarding the correlation of echo measures of right ventricular function with cardiac MRI right ventricular function in the paediatric population.

Echocardiographic measures of regional and global right ventricular function were reviewed in 56 patients with repaired tetralogy of Fallot and 27 patients with structurally normal hearts. These measures were compared to global right ventricular ejection fraction by cardiac MRI.

Despite having the same right ventricular ejection fraction by cardiac MRI as normal controls, patients with repaired tetralogy of Fallot had significantly worse global and regional echocardiographic measures of right ventricular function. Right ventricular fractional area change and fractional shortening of the right ventricular outflow tract correlated best with global right ventricular function by MRI. Incorporation of these measures into a regression model allowed accurate prediction of global right ventricular function by cardiac MRI.

Children with repaired tetralogy of Fallot have abnormal regional and global right ventricular function compared to patients with normal hearts. Despite the presence of patch material, the right ventricular outflow tract significantly contributes to global right ventricular function by cardiac MRI. When incorporated into a model that includes regional and global echocardiographic measures of right ventricular function, right ventricular outflow tract fractional shortening helps predict global right ventricular function by MRI.

The objective of this study was to determine the evolution of fibrosis over time and its association with clinical status.

Children with repaired tetralogy of Fallot who had undergone at least two cardiac magnetic resonance examinations including T1 mapping at least 1 year apart were included.

Thirty-seven patients (12.7 ± 2.6 years, 61% male) were included. Right ventricular free wall T1 increased (913 ± 208 versus 1023 ± 220 ms; p = 0.02). Baseline cardiac magnetic resonance parameters did not predict a change in imaging markers or exercise tolerance. The right ventricular free wall per cent change correlated with left ventricular T1% change (r = 0.51, p = 0.001) and right ventricular mass Z-score change (r = 0.51, p = 0.001). T1 in patients with late gadolinium enhancement did not differ from the rest.

Increasing right ventricular free wall T1 indicates possible progressive fibrotic remodelling in the right ventricular outflow tract in this pilot study in children and adolescents with repaired tetralogy of Fallot. The value of T1 mapping both at baseline and during serial assessments will need to be investigated in larger cohorts with longer follow-up.