Management of Left Ventricular Intramural Hematoma- an Unusual Complication of Complete Atrioventricular Septal Defect Repair in a Child.

Postoperative survival for complete atrioventricular septal defect (cAVSD) is excellent. Common complications of surgery include valvular stenosis/regurgitation, left ventricular outflow tract obstruction, arrythmias, and residual defects. We report a challenging case of a 7-month old girl with Trisomy 21, preoperative obstructive sleep apnea with severe pulmonary hypertension, who underwent AVSD repair and required veno-arterial extracorporeal membrane oxygenation (V-A ECMO) while unable to come off cardiopulmonary bypass and developed left ventricular intramural hematoma during the course. This case highlights the challenges in management of an unusual complication.

Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome.

BACKGROUND: Cardiovascular magnetic resonance (CMR) has established itself as the gold standard for serial assessment of systemic right ventricular (RV) performance but due to the lack of standardized RV reference values for hypoplastic left heart syndrome (HLHS) patients, the interpretation of RV volumetric data in HLHS remains difficult. Therefore, this study aimed to close this gap by providing CMR reference values for the systemic RV in HLHS patients. METHODS: CMR scans of 160 children, adolescents, and young adults (age range 2.2-25.2 years, 106 males) with HLHS were retrospectively evaluated. All patients were studied following total cavopulmonary connection. Short-axis stacks were used to measure RV end-diastolic and end-systolic volumes (RVEDV, RVESV), RV stroke volume (RVSV), RV ejection fraction (RVEF), and RV end-diastolic myocardial mass (RVEDMM). Univariable and multiple linear regression analyses were performed to assess associations between RV parameters and demographic and anthropometric characteristics. Following the results of the regression analysis, reference graphs and tables were created with the Lambda-Mu-Sigma method. RESULTS: Multiple linear regression analysis showed strong associations between body height and RVEDV, RVESV as well as RVSV. Age was highly associated with RVEDMM. Therefore, percentile curves and tables were created with respect to body height (RVEDV, RVESV, RVSV) and age (RVEDMM). The influence of demographic and anthropometric parameters on RVEF was mild, thus no percentile curves and tables for RVEF are provided. CONCLUSION: We were able to define CMR reference values for RV volumetric variables for HLHS patients. These data might be useful for the assessment and interpretation of CMR scans in these patients and for research in this field.

Detection of hypoplastic left heart syndrome anatomy from cardiovascular magnetic resonance images using machine learning.

OBJECTIVE: The prospect of being able to gain relevant information from cardiovascular magnetic resonance (CMR) image analysis automatically opens up new potential to assist the evaluating physician. For machine-learning-based classification of complex congenital heart disease, only few studies have used CMR. MATERIALS AND METHODS: This study presents a tailor-made neural network architecture for detection of 7 distinctive anatomic landmarks in CMR images of patients with hypoplastic left heart syndrome (HLHS) in Fontan circulation or healthy controls and demonstrates the potential of the spatial arrangement of the landmarks to identify HLHS. The method was applied to the axial SSFP CMR scans of 46 patients with HLHS and 33 healthy controls. RESULTS: The displacement between predicted and annotated landmark had a standard deviation of 8-17 mm and was larger than the interobserver variability by a factor of 1.1-2.0. A high overall classification accuracy of 98.7% was achieved. DISCUSSION: Decoupling the identification of clinically meaningful anatomic landmarks from the actual classification improved transparency of classification results. Information from such automated analysis could be used to quickly jump to anatomic positions and guide the physician more efficiently through the analysis depending on the detected condition, which may ultimately improve work flow and save analysis time.

Impact of compressed sensing (CS) acceleration of two-dimensional (2D) flow sequences in clinical paediatric cardiovascular magnetic resonance (CMR).

OBJECTIVES: Two-dimensional (2D) through-plane phase-contrast (PC) cine flow imaging assesses shunts and valve regurgitations in paediatric CMR and is considered the reference standard for Clinical quantification of blood Flow (COF). However, longer breath-holds (BH) can reduce compliance with possibly large respiratory manoeuvres altering flow. We hypothesize that reduced BH time by application of CS (Short BH quantification of Flow) (SBOF) retains accuracy while enabling faster, potentially more reliable flows. We investigate the variance between COF and SBOF cine flows. METHODS: Main pulmonary artery (MPA) and sinotubular junction (STJ) planes were acquired at 1.5 T in paediatric patients by COF and SBOF. RESULTS: 21 patients (mean age 13.9, 10-17y) were enrolled. The BH times were COF mean 11.7 s (range 8.4-20.9 s) vs SBOF mean 6.5 s (min 3.6-9.1 s). The differences and 95% CI between the COF and SBOF flows were LVSV -1.43 ± 13.6(ml/beat), LVCO 0.16 ± 1.35(l/min) and RVSV 2.95 ± 12.3(ml/beat), RVCO 0.27 ± 0.96(l/min), QP/QS were SV 0.04 ± 0.19, CO 0.02 ± 0.23. Variability between COF and SBOF did not exceed intrasession variation of COF. CONCLUSION: SBOF reduces breath-hold duration to 56% of COF. RV flow by SBOF was biased compared to COF. The variation (95% CI) between COF and SBOF was similar to the COF intrasession test-retest 95% CI.

Short-term outcome of late gadolinium changes detected on cardiovascular magnetic resonance imaging following coronavirus disease 2019 Pfizer/BioNTech vaccine-related myocarditis in adolescents.

BACKGROUND: Rare cases of cardiac inflammation following vaccination for severe acute respiratory coronavirus 2 (SARS-CoV-2) have been reported. OBJECTIVE: To study paediatric patients with clinical findings of acute inflammation post coronavirus disease 2019 (COVID-19) Pfizer/BioNTech vaccination using cardiovascular magnetic resonance imaging (MRI) in acute and subacute phases. MATERIALS AND METHODS: We enrolled adolescents younger than 18 years who presented at one of two institutions between July 2021 and August 2022 with clinical and laboratory findings of acute myocarditis shortly following COVID-19 Pfizer/BioNTech vaccination. They all underwent cardiovascular MRI using the institutional myocarditis protocol. RESULTS: Five adolescents (four boys) underwent eight scans between 3 days and 109 days (mean 49 days) after the onset of symptoms following COVID-19 vaccination. Myocardial oedema appeared on short tau inversion recovery (STIR) T2-weighted images in three adolescents at presentation (3-12 days after symptom onset). In these children, the myocardial oedema/acute inflammation had resolved at follow-up cardiovascular MRI (53-68 days after first MRI). However, in all three adolescents, a persistent area of late gadolinium enhancement was evident at follow-up, suggesting post-myocarditic fibrosis. One adolescent scanned only once, 66 days after being symptomatic, had no acute inflammation but persistent fibrotic changes. This last adolescent, who underwent the first scan 109 days after symptom onset, had findings compatible with an episode of previous myocarditis, with mild ongoing regional myocardial oedema/inflammation. CONCLUSION: This study on post-vaccine myocarditis demonstrates residual lesions with persistent areas of late gadolinium enhancement/myocardial fibrosis with ongoing myocardial oedema after resolution of the initial myocardial oedema a few weeks after Pfizer/BioNTech vaccination. There is an urgent need to recognise and fully investigate the outcome of post-vaccination myocarditis.

Reference Values for Pediatric Atrial Volumes Assessed by Steady-State Free-Precession Magnetic Resonance Imaging Using Monoplane and Biplane Area-Length Methods.

BACKGROUND: Measurement of atrial volumes by MRI is becoming increasingly important in pediatric cardiac disorders. However, MRI normal values for atrial volumes in children are lacking. PURPOSE: To establish pediatric reference values for atrial volumes. STUDY TYPE: Retrospective. SUBJECTS: A total of 155 healthy children from two large institutions (103 male, age 13.9 ± 2.8 years, range 4-18 years). FIELD STRENGTH/SEQUENCE: A 1.5 T; balanced steady-state free precession (bSSFP) sequence. ASSESSMENT: The monoplane and biplane area-length methods were used to measure minimal and maximal left and right atrial volumes (LAmin , LAmax , RAmin , and RAmax ) from four-chamber (4ch) and two-chamber (2ch) MR cine images. Centile charts and tables for atrial volumes were created. STATISTICAL TESTS: Descriptive statistics, lambda-mu-sigma (LMS)-method of Cole and Green, univariable and multivariable linear regression models. A P value < 0.05 was considered to be statistically significant. RESULTS: In the multivariable linear model, body surface area was significantly associated with all atrial volumes and sex was significantly associated with RA volumes, LA volumes measured in the 2ch-view as well as biplane LAmax. Average atrial volumes measured: monoplane 4ch: LAmin 13.1 ± 4.8 mL/m2 , LAmax 33.4 ± 8.8 mL/m2 , RAmin 18.5 ± 6.8 mL/m2 , RAmax 33.2 ± 9.6 mL/m2 ; monoplane 2ch: LAmin 12.7 ± 4.9 mL/m2 , LAmax 30.5 ± 9.5 mL/m2 ; biplane: LAmin 12.3 ± 4.5 mL/m2 , LAmax 30.9 ± 8.7 mL/m2 . DATA CONCLUSION: Pediatric MRI reference values for atrial volumes have been provided. TECHNICAL EFFICACY: 2 EVIDENCE LEVEL: 4.

Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement.

AIMS: Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. METHODS AND RESULTS: The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich's ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne's muscular dystrophy (BMD/DMD, n = 15), Duchenne's carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. CONCLUSIONS: 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

Pediatric Cardiac Magnetic Resonance Reference Values for Biventricular Volumes Derived From Different Contouring Techniques.

BACKGROUND: Measurement of ventricular volumes and function using MRI is an important tool in pediatric congenital heart disease. However, normal values for children are sparce and analysis methods are inconsistent. PURPOSE: To propose biventricular reference values in children for two MRI postprocessing (contouring) techniques. STUDY TYPE: Retrospective. SUBJECTS: A total of 154 children from two institutions (13.9 ± 2.8 years; 101 male) that were referred for a clinical MRI study. FIELD STRENGTH/SEQUENCE: 1.5 T; balanced steady-state free precession (bSSFP) sequence. ASSESSMENT: Left ventricular (LV) and right ventricular (RV) end-diastolic and end-systolic volumes (LVEDV, LVESV, RVEDV, RVESV) and end-diastolic and end-systolic myocardial mass (LVEDMM, LVESMM, RVEDMM, RVESMM) were measured from short-axis images using two contouring techniques: 1) papillary muscles, trabeculations and the moderator band were included in the ventricular blood volume and excluded from the myocardial mass, 2) papillary muscles, trabeculations and the moderator band were excluded from the ventricular volume and included in the ventricular mass. STATISTICAL TESTS: Univariable and multivariable linear regression models were used to evaluate relationships between sex, weight, height, body surface area (BSA) and age and volumetric results. Reference graphs and tables were created with the LMS-method. Contouring techniques were compared by intraclass correlation, regression analysis and Bland-Altman plots. A P value < 0.05 was considered statistically significant. RESULTS: Height and BSA were significantly associated with LVESV (method 1) and with LVEDV and RVEDV (method 2). LVESV (method 2), RVESV (both methods), RVEDV (method 1), and LVEDMM and RVEDMM (both methods), showed significant associations with height and weight. LVSV and RVSV (both methods) were significantly associated with BSA and weight. RVESV (method 1) was significantly associated with age. Gender showed significant associations for all parameters. DATA CONCLUSION: The proposed pediatric reference values can be used in the diagnosis and follow-up of congenital or acquired heart disease and for research purposes. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: Stage 2.

Segmental and global longitudinal strain differences between Kawasaki disease and multi-system inflammatory syndrome in children.

BACKGROUND: Multi-system inflammatory syndrome in children and Kawasaki disease have overlapping clinical features but comparative echocardiographic studies are lacking. METHODS: We reviewed echocardiography findings of all multi-system inflammatory syndrome cases between 1st April and 31st July, 2020 and typical Kawasaki disease patients with coronary arteries abnormalities consecutively followed between 1st October, 2016 and June 30th, 2019. RESULTS: We included 40 multi-system inflammatory syndrome children (25 males, 62.5%) and 45 Kawasaki disease patients (31 males, 68.9%) at a mean age of 6.4 years old and 8 years old, respectively. Four out of 40 multi-system inflammatory syndrome children had coronary arteries abnormalities. Left ventricle ejection fraction was normal in both groups. Global longitudinal strain was normal although Kawasaki disease group had significantly lower values (-20.0 versus -21.7%; p = 0.02). Basal segments were the most affected in Kawasaki disease patients with significant differences in the basal anterior, anterolateral, and anteroseptal strain: -18.2 versus -23.0% (p = 0.002), -16.7 versus -22.0% (p < 0.001), -16.7 versus -19.5% (p = 0.034), respectively. The basal anterolateral and anteroseptal segments in Kawasaki disease patients were the only ones with an absolute reduction of longitudinal strain (-16.7% both) consistent with the greater left main coronary involvement in this cohort. CONCLUSIONS: Our findings are consistent with the transient cardiac involvement in multi-system inflammatory syndrome, as opposed to the subtle and chronic myocardial involvement in Kawasaki disease children with coronary arteries abnormalities. We speculate that the mechanism of cardiac impairment in the few multi-system inflammatory syndrome children with reduced global longitudinal strain is not related to coronary arteries abnormalities.

Performance of artificial intelligence for biventricular cardiovascular magnetic resonance volumetric analysis in the clinical setting.

Cardiovascular magnetic resonance (CMR) derived ventricular volumes and function guide clinical decision-making for various cardiac pathologies. We aimed to evaluate the efficiency and clinical applicability of a commercially available artificial intelligence (AI) method for performing biventricular volumetric analysis. Three-hundred CMR studies (100 with normal CMR findings, 50 dilated cardiomyopathy, 50 hypertrophic cardiomyopathy, 50 ischaemic heart disease and 50 congenital or valvular heart disease) were randomly selected from database. Manual biventricular volumetric analysis (CMRtools) results were derived from clinical reports and automated volumetric analyses were performed using short axis volumetry AI function of CircleCVI42 v5.12 software. For 20 studies, a combined method of manually adjusted AI contours was tested and all three methods were timed. Clinicians` confidence in AI method was assessed using an online survey. Although agreement was better for left ventricle than right ventricle, AI analysis results were comparable to manual method. Manual adjustment of AI contours further improved agreement: within subject coefficient of variation decreased from 5.0% to 4.5% for left ventricular ejection fraction (EF) and from 9.9% to 7.1% for right ventricular EF. Twenty manual analyses were performed in 250 min 12 s whereas same task took 5 min 48 s using AI method. Clinicians were open to adopt AI but concerns about accuracy and validity were raised. The AI method provides clinically valid outcomes and saves significant time. To address concerns raised by survey participants and overcome shortcomings of the automated myocardial segmentation, visual assessment of contours and performing manual corrections where necessary appears to be a practical approach.

Performance of Cardiac MRI in Pediatric and Adult Patients with Fontan Circulation.

Cardiac MRI has become a widely accepted standard for anatomic and functional assessment of complex Fontan physiology, because it is noninvasive and suitable for comprehensive follow-up evaluation after Fontan completion. The use of cardiac MRI in pediatric and adult patients after completion of the Fontan procedure are described, and a practical and experience-based cardiac MRI protocol for evaluating these patients is provided. The current approach and study protocol in use at the authors' institution are presented, which address technical considerations concerning sequences, planning, and optimal image acquisition in patients with Fontan circulation. Additionally, for each sequence, the information that can be obtained and guidance on how to integrate it into clinical decision-making is discussed. Keywords: Pediatrics, MRI, MRI Functional Imaging, Heart, Congenital © RSNA, 2022.

Myocardial Deformation in the Pediatric Age Group: Normal Values for Strain and Strain Rate Using 2D Magnetic Resonance Feature Tracking.

BACKGROUND: Myocardial deformation can be assessed from routine cardiac magnetic resonance (MR) images using two-dimensional feature tracking (2D-FT). Although reference values are essential for implementation of strain imaging in clinical practice, data for the healthy pediatric age group are limited. PURPOSE: To provide pediatric MR reference values for strain and strain rate for all four heart chambers. STUDY TYPE: Retrospective. SUBJECTS: One hundred and fifty-seven healthy children from two institutions (102 male, age 4.7-18 years). FIELD STRENGTH/SEQUENCE: 1.5 T; balanced steady-state free precession sequence. ASSESSMENT: Left ventricular (LV) global and regional longitudinal, circumferential, and radial strain and strain rate as well as right ventricular (RV) and atrial global and regional longitudinal strain and strain rate were measured in two-, three-, and four-chamber views and the short axis stack. The relationships between strain parameters and age, height, weight, and gender were investigated. Age- and height-specific centile curves and tables were created for LV strain and strain rate. For all other global strain parameters, the mean was calculated as a reference. STATISTICAL TESTS: Lambda-mu-sigma (LMS)-method of Cole and Green, univariable, and multivariable linear regression models. A P value <0.05 was considered to be statistically significant. RESULTS: Age, height and weight had a significant influence on LV global strain values. These parameters also showed an influence on RV strain but only in boys (girls P = 0.12) and none of the variables had a significant influence on atrial strain (P = 0.19-0.49). Gender differences were only found for RV strain values. DATA CONCLUSION: Pediatric potential reference values for myocardial deformation parameters of both ventricles and atria are provided. The values may serve as a reference in future studies and clinical practice. LEVEL OF EVIDENCE: 3 TECHNICAL EFFICACY: Stage 5.

Reliability of pediatric ventricular function analysis by short-axis "single-cycle-stack-advance" single-shot compressed-sensing cines in minimal breath-hold time.

OBJECTIVES: Cardiovascular magnetic resonance (CMR) cine imaging by compressed sensing (CS) is promising for patients unable to tolerate long breath-holding. However, the need for a steady-state free-precession (SSFP) preparation cardiac cycle for each slice extends the breath-hold duration (e.g. for 10 slices, 20 cardiac cycles) to an impractical length. We investigated a method reducing breath-hold duration by half and assessed its reliability for biventricular volume analysis in a pediatric population. METHODS: Fifty-five consecutive pediatric patients (median age 12 years, range 7-17) referred for assessment of congenital heart disease or cardiomyopathy were included. Conventional multiple breath-hold SSFP short-axis (SAX) stack cines served as the reference. Real-time CS SSFP cines were applied without the steady-state preparation cycle preceding each SAX cine slice, accepting the limitation of omitting late diastole. The total acquisition time was 1 RR interval/slice. Volumetric analysis was performed for conventional and "single-cycle-stack-advance" (SCSA) cine stacks. RESULTS: Bland-Altman analyses [bias (limits of agreement)] showed good agreement in left ventricular (LV) end-diastolic volume (EDV) [3.6 mL (- 5.8, 12.9)], LV end-systolic volume (ESV) [1.3 mL (- 6.0, 8.6)], LV ejection fraction (EF) [0.1% (- 4.9, 5.1)], right ventricular (RV) EDV [3.5 mL (- 3.34, 10.0)], RV ESV [- 0.23 mL (- 7.4, 6.9)], and RV EF [1.70%, (- 3.7, 7.1)] with a trend toward underestimating LV and RV EDVs with the SCSA method. Image quality was comparable for both methods (p = 0.37). CONCLUSIONS: LV and RV volumetric parameters agreed well between the SCSA and the conventional sequences. The SCSA method halves the breath-hold duration of the commercially available CS sequence and is a reliable alternative for volumetric analysis in a pediatric population. KEY POINTS: • Compressed sensing is a promising accelerated cardiovascular magnetic resonance imaging technique. • We omitted the steady-state preparation cardiac cycle preceding each cine slice in compressed sensing and achieved an acquisition speed of 1 RR interval/slice. • This modification called "single-cycle-stack-advance" enabled the acquisition of an entire short-axis cine stack in a single short breath hold. • When tested in a pediatric patient group, the left and right ventricular volumetric parameters agreed well between the "single-cycle-stack-advance" and the conventional sequences.

Myocardial deformation assessed by CMR in children after multisystem inflammatory syndrome (MIS-C).

BACKGROUND: Short-term sequelae of Multisystem Inflammatory Syndrome in Children (MIS-C), recently published by our institution, showed rapid improvement of the cardiac abnormalities within a few weeks after the onset of the disease. However, subtle residual abnormalities, affecting mainly the myocardial interstitium, were shown in some of the patients. The current study aimed to assess myocardial deformation with CMR shortly after MIS-C. METHODS: Sixty children were included into the study; 30 following MIS-C (onset-to-scan mean 27 days, SD 11) and 30 controls. Strain values were compared between patients and controls and additionally to published paediatric normal CMR values. U-Mann Whitney test was used for comparison of the myocardial deformation between patients and controls. RESULTS: Median age of the patients was 9.0 years (range 0.99-14.4) and controls 9.8 years (range 4.7-14.9). All conventional CMR parameters in patients were in normal range. Strain values were significantly lower in patients than in controls. When compared to published centile graphs, radial and circumferential global strain was within 2.5th and 97.5th centile in all patients. Eleven patients had global longitudinal strain between 2.5th centile and 50th centile, 1 patient was below 2.5th centile and all the others above 50th centile. Only 3 controls had global longitudinal strain between 2.5th centile and 50th centile, all other had higher strain. CONCLUSIONS: This study demonstrates that myocardial deformation indices measured by CMR are within normal range in the vast majority of the patients within a few weeks after the onset of MIS-C. However, when compared to healthy controls, all strain parameters were lower in patients.