Coronary artery size and origin imaging in children: a comparative study of MRI and trans-thoracic echocardiography

BACKGROUND: The purpose of this study was to see how coronary magnetic resonance angiography (CMRA) compared to echocardiography for the detection of coronary artery origins and to compare CMRA measurements for coronary dimensions in children with published echocardiographic reference values.METHODS: Enrolled patients underwent dual cardiac phase CMRA and echocardiography under the same anesthetic. Echocardiographic measurements of the right coronary artery (RCA), left anterior descending (LAD) and left main (LM) were made. CMRA dimensions were assessed manually at the same points as the echocardiographic measurements. The number of proximal LAD branches imaged was also recorded in order to give an estimate of distal coronary tree visualization.RESULTS: Fifty patients (24 boys, mean age 4.0 years (range 18 days to 18 years)) underwent dual-phase CMRA. Coronary origins were identified in 47/50 cases for CMRA (remaining 3 were infants aged 3, 9 and 11 months). In comparison, origins were identified in 41/50 cases for echo (remaining were all older children). CMRA performed better than echocardiography in terms of distal visualization of the coronary tree (median 1 LAD branch vs. median 0; p = 0.001). Bland-Altman plots show poor agreement between echocardiography and CMRA for coronary measurements. CMRA measurements did vary according to cardiac phase (systolic mean 1.90, s.d. 0.05 mm vs. diastolic mean 1.84, s.d. 0.05 mm; p = 0.002).CONCLUSIONS: Dual-phase CMRA has an excellent (94 %) success rate for the detection of coronary origins in children. Newborn infants remain challenging and echocardiography remains the accepted imaging modality for this age group. Echocardiographic reference ranges are not applicable to CMRA measurements as agreement was poor between modalities. Future coronary reference values, using any imaging modality, should quote the phase in which it was measured.</p

Improved coronary magnetic resonance angiography using gadobenate dimeglumine in pediatric congenital heart disease

BackgroundCMRA in pediatrics remains challenging due to the smaller vessel size, high heart rates (HR), potential image degradation caused by limited patient cooperation and long acquisition times. High-relaxivity contrast agents have been shown to improve coronary imaging in adults, but limited data is available in children. We sought to investigate whether gadobenate dimeglumine (Gd-BOPTA) together with self-navigated inversion-prepared coronary magnetic resonance angiography (CMRA) sequence design improves coronary image quality in pediatric patients.MethodsForty consecutive patients (mean age 6 ± 2.8 years; 73% males) were prospectively recruited for a 1.5-T MRI study under general anesthesia. Two electrocardiographic-triggered free breathing steady-state free precession (SSFP) angiography sequences (A and B) with isotropic spatial resolution (1.3 mm3) were acquired using a recently developed image-based self-navigation technique. Sequence A was acquired prior to contrast administration using T2 magnetization preparation (T2prep). Sequence B was acquired 5–8 min after a bolus of Gd-BOPTA with the T2prep replaced by an inversion recovery (IR) pulse to null the signal from the myocardium. Scan time, signal-to noise and contrast-to-noise ratios (SNR and CNR), vessel wall sharpness (VWS) and qualitative visual score for each sequence were compared.ResultsScan time was similar for both sequences (5.3 ± 1.8 vs 5.2 ± 1.5 min, p = .532) and average heart rate (78 ± 14.7 vs 78 ± 14.5 bpm, p = .443) remained constant throughout both acquisitions. Sequence B resulted in higher SNR (12.6 ± 4.4 vs 31.1 ± 7.4, p &lt; .001) and CNR (9.0 ± 1.8 vs 13.5 ± 3.7, p &lt; .001) and provided improved coronary visualization in all coronary territories (VWS A = 0.53 ± 0.07 vs B = 0.56 ± 0.07, p = .001; and visual scoring A = 3.8 ± 0.59 vs B = 4.1 ± 0.53, p &lt; .001). The number of non-diagnostic coronary segments was lower for sequence B [A = 42 (13.1%) segments vs B = 33 (10.3%) segments; p = .002], and contrary to the pre-contrast sequence, never involved a proximal segment. These results were independent of the patients' age, body surface area and HR.ConclusionsThe use of Gd-BOPTA with a 3D IR SSFP CMRA sequence results in improved coronary visualization in small infants and young children with high HR within a clinically acceptable scan time.AbbreviationsBP-CA, blood pool contrast agent; BSA, body surface area; SSFP, steady state free precession; CHD, congenital heart disease; CMR, cardiovascular magnetic resonance; CMRA, coronary magnetic resonance angiography; CNR, contrast-to-noise ratio; CoA, aortic coarctation; EC-GBCA, extra-cellular gadolinium-based contrast agent; ECG, electrocardiogram; Gd-BOPTA, gadobenate dimeglumine; HR, heart rate; iNAV, image-based navigator; IR, inversion recovery; LAD, left anterior descending artery; LCx, left circumflex artery; MRI, magnetic resonance imaging; RCA, right coronary artery; SNR, signal-to-noise ratio; VWS, vessel wall sharpness

Application of the Boston Technical Performance Score to intraoperative echocardiography

Background: The Technical Performance Score (TPS) developed by Boston Children's Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre. Methods: After a period of training, intraoperative TPS (epicardial and/or transesophageal echocardiography) was instituted. TPS was used to inform intraoperative discussions and recorded on a custom-made database using the previously published scoring system. After a year, we reviewed the feasibility, results and relationship between the TPS and mortality, extubation time and length of stay. Results: From 01 September 2015 to 04 July 2016, there were 272 TPS procedures in 251 operations with 208 TPS recorded. Seven patients had surgery with no documented TPS, three had operations with no current TPS score template available. Patients left the operating theatre with TPS optimal in 156 (75%), adequate 34 (16%) and inadequate 18 (9%). Of those with an optimal score on leaving theatre, ten had more than one period of cardiopulmonary bypass. All four deaths &lt;30 days after surgery (1.9%) had optimal TPS. There was a statistically significant difference in extubation times in the RACHS category 4 patients (3 days vs 5 days, P &lt; 0.05) and in PICU and total length of stay in the RACHS category three patients (2 and 8 days vs 12.5 and 21.5 days respectively) if leaving theatre with an inadequate result. Conclusions: Application of intraoperative TPS is feasible and provides a way of objectively recording intraoperative imaging assessment of surgery. An 'inadequate' TPS did not predict mortality but correlated with a longer ventilation time and longer length of stay compared to those with optimal or adequate scores.</p

Cardiac Function After Cardiotoxic Treatments for Childhood Cancer-Left Ventricular Longitudinal Strain in Screening

Background:The majority of childhood cancer survivors (CCSs) have been exposed to cardiotoxic treatments and often present with modifiable cardiovascular risk factors. Our aim was to evaluate the value of left ventricular (LV) longitudinal strain for increasing the sensitivity of cardiac dysfunction detection among CCSs.Methods:We combined two national cohorts: neuroblastoma and other childhood cancer survivors treated with anthracyclines. The final data consisted of 90 long-term CCSs exposed to anthracyclines and/or high-dose chemotherapy with autologous stem cell rescue and followed up for > 5 years and their controls (n = 86). LV longitudinal strain was assessed with speckle tracking (Qlab) and LV ejection fraction (EF) by three-dimensional echocardiography (3DE).Results:Of the CCSs, 11% (10/90) had abnormal LV longitudinal strain (i.e., Conclusion:LV longitudinal strain is a more sensitive method than LV EF for the detection of cardiac dysfunction among CCSs. Therefore, LV longitudinal strain should be added to the screening panel, especially for those with modifiable cardiovascular risk factors.</p

Three-dimensional visualisation of the fetal heart using prenatal MRI with motion-corrected slice-volume registration:a prospective, single-centre cohort study

BACKGROUND: Two-dimensional (2D) ultrasound echocardiography is the primary technique used to diagnose congenital heart disease before birth. There is, however, a longstanding need for a reliable form of secondary imaging, particularly in cases when more detailed three-dimensional (3D) vascular imaging is required, or when ultrasound windows are of poor diagnostic quality. Fetal MRI, which is well established for other organ systems, is highly susceptible to fetal movement, particularly for 3D imaging. The objective of this study was to investigate the combination of prenatal MRI with novel, motion-corrected 3D image registration software, as an adjunct to fetal echocardiography in the diagnosis of congenital heart disease.METHODS: Pregnant women carrying a fetus with known or suspected congenital heart disease were recruited via a tertiary fetal cardiology unit. After initial validation experiments to assess the general reliability of the approach, MRI data were acquired in 85 consecutive fetuses, as overlapping stacks of 2D images. These images were then processed with a bespoke open-source reconstruction algorithm to produce a super-resolution 3D volume of the fetal thorax. These datasets were assessed with measurement comparison with paired 2D ultrasound, structured anatomical assessment of the 2D and 3D data, and contemporaneous, archived clinical fetal MRI reports, which were compared with postnatal findings after delivery.FINDINGS: Between Oct 8, 2015, and June 30, 2017, 101 patients were referred for MRI, of whom 85 were eligible and had fetal MRI. The mean gestational age at the time of MRI was 32 weeks (range 24-36). High-resolution (0·50-0·75 mm isotropic) 3D datasets of the fetal thorax were generated in all 85 cases. Vascular measurements showed good overall agreement with 2D echocardiography in 51 cases with paired data (intra-class correlation coefficient 0·78, 95% CI 0·68-0·84), with fetal vascular structures more effectively visualised with 3D MRI than with uncorrected 2D MRI (657 [97%] of 680 anatomical areas identified vs 358 [53%] of 680 areas; p&lt;0·0001). When a structure of interest was visualised in both 2D and 3D data (n=358), observers gave a higher diagnostic quality score for 3D data in 321 (90%) of cases, with 37 (10%) scores tied with 2D data, and no lower scores than for 2D data (Wilcoxon signed rank test p&lt;0·0001). Additional anatomical features were described in ten cases, of which all were confirmed postnatally.INTERPRETATION: Standard fetal MRI with open-source image processing software is a reliable method of generating high-resolution 3D imaging of the fetal vasculature. The 3D volumes produced show good spatial agreement with ultrasound, and significantly improved visualisation and diagnostic quality compared with source 2D MRI data. This freely available combination requires minimal infrastructure, and provides safe, powerful, and highly complementary imaging of the fetal cardiovascular system.FUNDING: Wellcome Trust/EPSRC Centre for Medical Engineering, National Institute for Health Research.</p

Natural history and outcomes in paediatric RASopathy-associated hypertrophic cardiomyopathy

Aims: This study aimed to describe the natural history and predictors of all-cause mortality and sudden cardiac death (SCD)/equivalent events in children with a RASopathy syndrome and hypertrophic cardiomyopathy (HCM). Methods and results: This is a retrospective cohort study from 14 paediatric cardiology centres in the United Kingdom and Ireland. We included children <18 years with HCM and a clinical and/or genetic diagnosis of a RASopathy syndrome [Noonan syndrome (NS), NS with multiple lentigines (NSML), Costello syndrome (CS), cardiofaciocutaneous syndrome (CFCS), and NS with loose anagen hair (NS-LAH)]. One hundred forty-nine patients were recruited [111 (74.5%) NS, 12 (8.05%) NSML, 6 (4.03%) CS, 6 (4.03%) CFCS, 11 (7.4%) Noonan-like syndrome, and 3 (2%) NS-LAH]. NSML patients had higher left ventricular outflow tract (LVOT) gradient values [60 (36–80) mmHg, P = 0.004]. Over a median follow-up of 197.5 [inter-quartile range (IQR) 93.58–370] months, 23 patients (15.43%) died at a median age of 24.1 (IQR 5.6–175.9) months. Survival was 96.45% [95% confidence interval (CI) 91.69–98.51], 90.42% (95% CI 84.04–94.33), and 84.12% (95% CI 75.42–89.94) at 1, 5, and 10 years, respectively, but this varied by RASopathy syndrome. RASopathy syndrome, symptoms at baseline, congestive cardiac failure (CCF), non-sustained ventricular tachycardia (NSVT), and maximal left ventricular wall thickness were identified as predictors of all-cause mortality on univariate analysis, and CCF, NSVT, and LVOT gradient were predictors for SCD or equivalent event. Conclusions: These findings highlight a distinct category of patients with Noonan-like syndrome with a milder HCM phenotype but significantly worse survival and identify potential predictors of adverse outcome in patients with RASopathy-related HCM

The relationship between maximal left ventricular wall thickness and sudden cardiac death in childhood onset hypertrophic cardiomyopathy

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1–16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3–9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM

Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy

BACKGROUND Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. OBJECTIVES The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. METHODS Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. RESULTS At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. CONCLUSIONS Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages. (C) 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.Peer reviewe

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [+/- 4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was = 10 to = 20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score >= 20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores = 10 to = 20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.Peer reviewe

Sex differences in children and adolescents with hypertrophic cardiomyopathy

BACKGROUND: Sex differences have been described in adults with hypertrophic cardiomyopathy (HCM), but it is unknown if similar differences exist in childhood-onset disease. OBJECTIVES: This study aimed to investigate the influence of biological sex on the clinical characteristics and outcomes of children with HCM. METHODS: An international retrospective cohort of patients diagnosed with nonsyndromic HCM ≤16 years was formed. Sex differences in baseline characteristics and clinical outcomes were investigated. Primary outcome was all-cause mortality or cardiac transplantation. Secondary outcomes include major arrhythmic cardiac event and heart failure event. RESULTS: Of 1,433 patients diagnosed at a median age of 11 years (IQR: 6-14), 471 (33.0%) were female. Although there were no sex differences in phenotype in preadolescent patients (<12 years), adolescent female patients were more likely to have heart failure symptoms (n = 53 [31.9%] vs n = 86 [22.5%]; P = 0.019). Adolescent female patients had larger left atrial size (1.4 z-score [±2.3] vs 2.1 z-score [±2.5]; P = 0.0056) but there was no difference in degree of hypertrophy or proportion with obstructive disease. Over a median follow-up of 5.3 years (IQR: 2.9, 8.0) annual incidence of all-cause mortality or cardiac transplantation, major arrhythmic cardiac event or heart failure events did not vary by sex. CONCLUSIONS: Young female patients with HCM are more likely to experience heart failure symptoms and have echocardiographic features of diastolic impairment. Despite differences in phenotype, outcomes during childhood and young adulthood are not different. Further studies are required to explore the underlying mechanisms for these observed differences