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Coronary artery disease of non-atherosclerotic aetiology, while rare in incidence, can have a wide aetiology, such as fibromuscular dysplasia, which is a non-inflammatory arteriopathy of numerous histopathological types of fibromuscular tissue accumulation. This brief report describes the case of a 22-year-old male with a recently developed dilated cardiomyopathy and a history of aborted cardiac arrest at the age of 14 years. Coronary angiogram revealed severe three vessels disease, while optical coherence tomography established fibromuscular dysplasia as aetiology. Balloon and stent angioplasty was performed guided by fractional flow reserve with acceptable angiographic result.
Middle aortic syndrome is a severe form of aortic coarctation and mostly involves the thoracic and abdominal part of the aorta. Traditional surgical approach has some potential risks and repetitive operations. Percutaneous stent implantation is a feasible and safe method for the palliation of middle aortic syndrome in childhood. Herein, a child with middle aortic syndrome was presented who has been treated by transcatheter stent implantation.
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.
Two-dimensional speckle tracking echocardiography-derived left ventricular longitudinal systolic strain is an important myocardial deformation parameter for assessing the systolic function of the left ventricle. Strain values differ according to the vendor machine and software. This study aimed to provide normal reference values for global and regional left ventricular longitudinal systolic strain in Egyptian children using automated functional imaging software integrated into the General Electric healthcare machine and to study the correlation between the global longitudinal left ventricular systolic strain and age, body size, vital data, and some echocardiographic parameters.
Healthy children (250) aged from 1 to 16 years were included. Conventional echocardiography was done to measure the left ventricular dimensions and function. Automated functional imaging was performed to measure the global and regional peak longitudinal systolic strain.
The global longitudinal strain was −21.224 ± 1.862%. The regional strain was −20.68 ± 2.11%, −21.06 ± 1.84%, and −21.86 ± 2.71% at the basal, mid, and apical segments, respectively. The mean values of the systolic longitudinal strain become significantly more negative from base to apex. Age differences were found as regard to global and regional longitudinal strain parameters but no gender differences. The global peak longitudinal systolic strain correlated positively with age. No correlations were found with either the anthropometric parameters or the vital data.
Age-specific normal values for two-dimensional speckle tracking-derived left ventricular longitudinal regional and global systolic strain are established using automated functional imaging.
Double drainage of the confluence of all four pulmonary veins is extremely rare. We present the image findings in a child with double drainage of the pulmonary venous confluence into the coronary sinus and left superior caval vein with co-existent right superior caval venous stenosis.
We report a case of a patient with an anomalous left coronary artery originating from the opposite coronary sinus with evidence of intramural course at the level of the septal commissure and no slit-like deformation of the coronary ostium, acute take-off, or proximal vessel narrowing. According to previous publications, patients with anomalous coronary artery and intramural segments identified at surgery had coronary CT findings of acute take-off angle or proximal vessel narrowing; slit-like orifice; and elliptical cross-sectional shape. Although further investigation is required, we suggest that the intramural course may not be ruled out based on the absence of slit-like ostium, acute take-off, or proximal vessel narrowing.
Constrictive pericarditis is uncommon in children. It results from scarring and consequent loss of the normal elasticity of the pericardial sac and is most commonly seen as a late sequelae of idiopathic or viral pericarditis. Here, we report a case of protein losing enteropathy as a complication of constrictive pericarditis in a 2-year-old child. Pericardial thickening was demonstrated by cardiac MRI and subsequent pericardiectomy led to remarkable improvement and resolution of protein losing enteropathy.
Cardiovascular magnetic resonance serves as a useful tool in diagnosing myocarditis. Current adult protocols are yet to be validated for children; thus, it remains unclear if the methods used can be applied with sufficient image quality in children. This study assesses the use of cardiovascular magnetic resonance in children with suspected myocarditis.
Image data from clinical cardiovascular magnetic resonance studies performed in children enrolled in Mykke between June 2014 and April 2019 were collected and analysed. The quality of the data sets was evaluated using a four-point quality scale (4: excellent, 3: good, 2: moderate, 1: non-diagnostic).
A total of 102 patients from 9 centres were included with a median age (interquartile range) of 15.4(10.7-16.6) years, 137 cardiovascular magnetic resonance studies were analysed. Diagnostic image quality was found in 95%. Examination protocols were consistent with the original Lake Louise criteria in 58% and with the revised criteria in 35%. Older patients presented with better image quality, with the best picture quality in the oldest age group (13-18 years). Sedation showed a negative impact on image quality in late gadolinium enhancement and oedema sequences. No such correlation was seen in cardiac function assessment sequences. In contrast to initial scans, in follow-up examinations, the use of parametric mapping increased while late gadolinium enhancement and oedema sequences decreased.
Cardiovascular magnetic resonance protocols for the assessment of adult myocarditis can be applied to children without significant constraints in image quality. Given the lack of specific recommendations for children, cardiovascular magnetic resonance protocols should follow recent recommendations for adult cardiovascular magnetic resonance.
Growing evidence has emphasised the importance of ventricular performance in functionally single-ventricle patients, particularly concerning diastolic function. Cardiac MRI has been proposed as non-invasive alternative to pre-Fontan cardiac catheterisation in selected patients.
Aim of the study:
To identify clinical and cardiac magnetic resonance predictors of high pre-Fontan end-diastolic ventricular pressure.
In a retrospective single-centre study, 38 patients with functionally univentricular heart candidate for Fontan intervention, who underwent pre-Fontan cardiac catheterisation, beside a comprehensive cardiac MRI, echocardiographic, and clinical assessment were included. Medical and surgical history, cardiac magnetic resonance, cardiac catheterisation, echocardiographic, and clinical data were recorded. We investigated the association between non-invasive parameters and cardiac catheterisation pre-Fontan risk factors, in particular with end-diastolic ventricular pressure. Moreover, the impact of conventional invasive pre-Fontan risk factor on post-operative outcome as also assessed.
Post-operative complications were associated with higher end-diastolic ventricular pressure and Mayo Clinic indexes (p < 0.01 and p = 0.05, respectively). At receiver operating characteristic curve analysis end-diastolic ventricular pressure ≥ 10.5 mmHg predicted post-operative complications with a sensitivity of 75% and specificity of 88% (AUC: 0.795, 95% CI 0.576;1.000, p < 0.05). At multivariate analysis, both systemic right ventricle (OR: 23.312, 95% CI: 2.704–200.979, p < 0.01) and superior caval vein indexed flow (OR: 0.996, 95% CI: 0.993–0.999, p < 0.05) influenced end-diastolic ventricular pressure ≥ 10.5 mmHg.
A reduced superior caval vein flow, evaluated at cardiac magnetic resonance, is associated with higher end-diastolic ventricular pressure a predictor of early adverse outcome in post-Fontan patients.
We report the case of a 14-year-old male presented with raised myocardial injury biomarkers, on the workout, Campylobacter coli was identified on stool culture, treated with antibiotics with total resolution. Cardiac magnetic resonance showed interventricular septum and lateral wall hypokinesia and subepicardial delayed enhancement, with preserved ventricular systolic function. To our knowledge, this is the first report linking Campylobacter coli to myopericarditis in children.
This article reports three children who presented with negative pressure pulmonary oedema before or after removal of the airway foreign bodies. Of them, two cases were type I negative pressure pulmonary oedema and one case was type II negative pressure pulmonary oedema following foreign body removal from the airways. Pathogenesis and location differences between type I and type II negative pressure pulmonary oedema caused by airway foreign body obstruction, as well as diagnosis and treatment modalities of the negative pressure pulmonary oedema were discussed.
Machine learning uses historical data to make predictions about new data. It has been frequently applied in healthcare to optimise diagnostic classification through discovery of hidden patterns in data that may not be obvious to clinicians. Congenital Heart Defect (CHD) machine learning research entails one of the most promising clinical applications, in which timely and accurate diagnosis is essential. The objective of this scoping review is to summarise the application and clinical utility of machine learning techniques used in paediatric cardiology research, specifically focusing on approaches aiming to optimise diagnosis and assessment of underlying CHD. Out of 50 full-text articles identified between 2015 and 2021, 40% focused on optimising the diagnosis and assessment of CHD. Deep learning and support vector machine were the most commonly used algorithms, accounting for an overall diagnostic accuracy > 0.80. Clinical applications primarily focused on the classification of auscultatory heart sounds, transthoracic echocardiograms, and cardiac MRIs. The range of these applications and directions of future research are discussed in this scoping review.
We report on a patient with bicuspid aortic valve and anomalous right coronary artery from the opposite sinus without evidence of intramural course. Different authors support the universal presence of intramural course in patients with origin of the right coronary artery from the opposite sinus of Valsalva in normal heart. The occurrence of both bicuspid aortic valve and the absence of intramural course may not be accidental. This might suggest a developmental interaction between bicuspid aortic valve and anomalous coronary artery. Large observational study including characterisation by intravascular ultrasonography in patients with bicuspid aortic valve and anomalous coronary is needed.
Patent ductus arteriosus stenting for ductal-dependent pulmonary blood flow is a technically challenging neonatal procedure to maintain a stable pulmonary circulation. Pre-procedural computed tomography imaging aids in outlining ductal origin, insertion, size, course and curvature. Computed tomography imaging may add value to procedural outcomes and reduce overall procedural morbidity in neonatal patent ductus arteriosus stenting. We conducted a single centre retrospective chart review of neonates with ductal-dependent pulmonary blood flow who underwent patent ductus arteriosus stenting between January 1, 2014 and June 31, 2020. We compared patients variables between patients who underwent pre-procedural computed tomography imaging to those who did not. A total of 64 patients were referred for patent ductus arteriosus stenting with 33 (52%) obtaining pre-procedural computed tomography imaging. Average age [19 days; range 1–242 days (p = 0.85)] and weight [3.3 kg (range 2.2–6.0 kg; p = 0.19)] was not significantly different between the groups. A diagnosis of pulmonary atresia was made in 42 out of 64 (66%) patients prior to patent ductus arteriosus stenting. The cohort with pre-intervention computed tomography imaging had a significant reduction in the total number of access sites (1.2 versus 1.5; p = 0.03), contrast needed (5.9 versus 8.2 ml/kg; p = 0.008), fluoroscopy (20.7 versus 38.8 minutes; p = 0.02) and procedural time (83.4–128.4 minutes; p = 0.002) for the intervention. There was no significant difference in radiation burden between the groups (p = 0.35). Pre-procedural computed tomography imaging adds value by aiding interventional planning for neonatal patent ductus arteriosus stenting. A statistically significant reduction in the number of access sites, contrast exposure, as well as fluoroscopic and procedural time was noted without significantly increasing the cumulative radiation burden.
This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years.
In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan–Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model.
The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948–271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047–15.376; p = 0.043).
Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.
Coronary sinus abnormalities are usually associated with arrhythmia disorders when symptomatic. We report a 5-year-old 14 kg patient with a giant diverticulum of coronary sinus and Wolff-Parkinson-White syndrome. Catheter ablation therapy was decided during follow-up due to inadequate response to multidrug therapy. Posteroseptal and left posterolateral accessory pathways were established and radiofrequency ablation was performed successfully through coronary sinus.
Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery–pulmonary artery fistula. We present two cases with coronary artery–pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.
Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging.
Retrospective review of all patients from February 2008 to January 2019.
Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5−15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6−15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter.
Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.