This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Background: Cyanotic CHD comprises up to 25% of cases of all causes of CHD. Rationale: There is lack of data about the present spectrum of congenital cyanotic heart disease in the paediatric age group. Objective: The present study was undertaken to determine the spectrum of patients with congenital cyanotic heart disease in the paediatric age group in tertiary paediatric cardiac care clinic. Design: Prospective observational study. Setting: Paediatric cardiac clinic of a tertiary cardiac care centre. Methods: All children aged 0–18 years with suspected cyanotic CHD were provisionally included in this study. They underwent a thorough echocardiographic evaluation, and those patients who had definitive diagnosis of congenital cyanotic heart disease were included for final analysis. Results: A total of 119 children met the inclusion criteria. Tetralogy of Fallot and its variant were the most common congenital cyanotic heart disease with proportion of about 44%. Other common malformations were double outlet right ventricle (14%), pulmonary atresia with ventricular septal defect (8%), total anomalous pulmonary venous connection (7%), d-transposition of the great arteries (9%), tricuspid valve anomalies − tricuspid atresia and Ebstein’s anomaly − hypoplastic left-heart syndrome, truncus arteriosus, and complex CHD such as single ventricle. Conclusion: Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.

Objective: The use of covered stents to close fenestration in total cavopulmonary connection is presented. Methods: We retrospectively reviewed data of all patients undergoing the procedure of a covered stent to close fenestration of total cavopulmonary connection between 2005 and 2012. Results: A total of 50 patients met the inclusion criteria. Median age and weight were 7.7 years and 20 kg, respectively. Median interval between Fontan completion and fenestration closure was 13 months. The femoral vein was used in 42 patients and the jugular vein in eight patients. Of the patients, seven received two stents. Covered stents were CP stents in 42 patients and Atrium Advanta V12 in eight patients. BIB balloons were used in 24 patients and simple balloons in 18 patients. Simultaneous occlusion of venous collaterals was observed in five patients. Median procedural and fluoroscopy times were 49 and 8 minutes, respectively. Mean central venous pressure rose from 10 to 12 mmHg. Mean oxygen saturation increased from 88% to 96%. Full occlusion was confirmed in 47 patients. The remaining had residual shunts: two patients had intracardiac Fontan, and one patient had a stent that could not be fully opened. Following the procedure, five patients had local bleeding, and three delayed discharge 48 hours after the procedure. There was no thromboembolic event after a mean follow-up of 49 months. Conclusion: Covered stent is a good option to close fenestration in extracardiac total cavopulmonary connection. It is safe, easily achievable with low fluoroscopy time, with very low risk of complication or failure. Good results are sustainable when excluding patients with none circular pathway.

Background: Most children in the Third World do not have access to treatment for heart diseases, as the priorities of health care are different from the developed countries. Materials and methods: Since 2009, teams supported by the Chain of Hope and Spanish medical volunteers have travelled twice a year to help develop paediatric cardiac services in the Cardiac Center in Ethiopia, undertaking four missions each year. As of December 2012, 296 procedures were performed on 287 patients. The procedures included 128 duct occlusions, 55 pulmonary valve dilations, 25 atrial septal defect closures, 14 mitral valve dilations, and others. The local staff were trained to perform a majority of these cases. Results: Procedural success was achieved in 264 (89.2%). There were three deaths, five device embolisations, and three complications in mitral valve dilation. During the visits, the local staff were trained including one cardiologist, six nurses, and two technicians. The local team performed percutaneous interventions on its own after a couple of years. The goal is also to enable the local team to perform interventions independently. Conclusion: Training of an interventional cardiology team in a sub-Saharan setting is challenging but achievable. It may be difficult for a single centre to commit to sending frequent missions to a developing country to make a meaningful contribution to the training of local teams. In our case, coordination between the teams from the two countries helped to achieve our goals.

Aim: We aimed to investigate the role of adiponectin in acute rheumatic fever by evaluating correlations with cytokines and acute-phase reactants. Methods: Patients were divided into three groups by clinical findings. Group 1 included 8 patients with only chorea, Group 2 included 13 patients with arthritis and carditis, and Group 3 included 12 patients with only carditis. A total of 54 age- and gender-matched children were enrolled in the control group. Blood samples were drawn for analysing acute-phase reactants, adiponectin, tumour necrosis factor-α, interleukin-6, and interleukin-8 levels at baseline on Days 2, 5, 10, and 15, and at 8 weeks. Results: There was no statistically significant difference between baseline age, gender, body mass index, serum triglyceride, total cholesterol, and low-density lipoprotein levels of the study and control groups (p>0.05). No correlation was found between baseline plasma adiponectin levels, age, body mass index, follicle-stimulating hormone, luteinising hormone, oestradiol, total testosterone, and blood lipid levels of the study and control groups (p>0.05). We found that adiponectin and interleukin-6 levels increased, tumour necrosis factor-α levels decreased, and interleukin-8 levels remained unchanged in acute rheumatic fever, which is an inflammatory disease. Moreover, adiponectin level was higher and tumour necrosis factor-α level was lower in the improvement period in comparison with the acute period, particularly in the carditis group. Conclusion: It was considered that, increasing throughout the treatment period, adiponectin may have anti-inflammatory effects in acute rheumatic fever. In addition, adiponectin levels are associated with a decline in inflammatory mediators in rheumatic fever.

Objectives: To evaluate long-term outcome of initial aortic valve intervention in a paediatric population with congenital aortic stenosis, and to determine risk factors associated with reintervention. Patients and methods: From 1985 to 2009, 77 patients with congenital aortic stenosis and a mean age of 5.8±5.6 years at diagnosis were followed up in our institution for 14.8±9.1 years. Results: First intervention was successful with 86% of patients having a residual peak aortic gradient <50 mmHg, and the proportion of patients with grade >1 regurgitation increased by 7%. Long-term survival after the first procedure was excellent, with 91% survival at 25 years. At a mean interval of 7.6±5.3 years, 30 patients required a reintervention (39%), mainly because of a recurrent aortic stenosis. Freedom from reintervention was 97, 89, 75, 53, and 42% at 1, 10, 15, 20, and 25 years, respectively. Predictors of reintervention were residual peak aortic gradient (p=0.0001), aortic regurgitation post-intervention >1 (p=0.02), prior balloon aortic valvuloplasty (p=0.04), and increased left ventricular posterior wall thickness (p=0.1). Conclusions: Aortic valve intervention is a safe and effective procedure for congenital aortic stenosis with excellent survival results. However, rate of reintervention is high and influenced by increased left ventricular posterior wall thickness pre-intervention, prior balloon valvuloplasty, higher residual peak systolic valve gradient, and more than mild regurgitation post-intervention. The study highlights that long-term follow-up is recommended for these patients.

Background: Children undergoing open heart surgery are at risk of neurological injury. A cohort of 35 patients, who had undergone cardiac surgery during infancy, had a significant reduction in Bayley Scale of Infant Development scores at a 12-month assessment. This cohort has now reached an appropriate age to reassess developmental progress. Methods: Detailed psychometric testing was conducted on 20 children from the original cohort using the Weschler Preschool and Primary Scale of Intelligence, the Wide Range Assessment of Memory and Learning, and the Wechsler Individual Achievement Test. Parents completed the Connor’s Rating Scale, the Behaviour Rating Scale of Executive Functioning, and the Child Behaviour Checklist. Results: The mean age of the cohort at assessment was 6.6 (standard deviation 0.4) years. Mean scores on all tests of intelligence, memory, academic achievement, and executive function fell within the average range. Of the children, 20–35% were found to have significant difficulties across these areas. Mean scores in the areas of social, emotional, behavioural, and psychological functioning also fell within the average range. Of the children studied, 35% had clinically significant problems in these areas. There was only a weak association between the 12-month scores and the Full-Scale Intelligence Quotient at 6 years. Conclusion: Detailed psychometric testing of these children suggests that they generally function in the average range; however, a significant proportion falls below age expectations in all the areas assessed. This highlights the importance of long-term follow-up with routine developmental screening to allow identification of a subgroup that may benefit from early educational and behavioural intervention.

Objective: To assess haemodynamic patterns in head-up tilt testing on 400 paediatric cases with unexplained syncope. Methods: Medical records of 520 children who underwent head-up tilt testing in the preceding year were retrospectively evaluated, and 400 children, 264 (66%) girls and 136 (34%) boys, aged 12.6±2.6 years (median 13; range 5–18), with unexplained syncope were enrolled in the study. Age, sex, baseline heart rate, baseline blood pressure, frequency of symptoms, and/or fainting attacks were recorded. The test protocol consisted of 25 minutes of supine resting followed by 20 minutes of 70° upright positioning. Subjects were divided into nine groups according to their differing haemodynamic patterns. Results: There were no statistically significant differences between the groups with regard to age, gender, baseline blood pressure, and frequency of syncope (p>0.05). The response was compatible with orthostatic intolerance in 28 cases (7.0%), postural orthostatic tachycardia syndrome in 24 cases (6.0%), asymptomatic postural orthostatic tachycardia syndrome in 26 cases (6.5%), orthostatic hypotension in seven cases (1.7%), vasovagal syncope in 38 cases (9.5%), and negative in 274 cases (69.2%). Vasovagal syncope response patterns were of type 3 in nine cases (2.2%), type 2A in 10 cases (2.5%), type 2B in two cases (0.5%), and type 1 (mixed) in 17 cases (4.25%). Conclusions: In the 400 paediatric cases with unexplained syncope, nine different haemodynamic response patterns to head-up tilt testing were discerned. Children with orthostatic intolerance syndromes are increasingly referred to hospitals because of difficulty in daily activities. Therefore, there is need for further clinical trials in these patient groups.

Background: To study the feasibility and complications associated with the use of ductal occluders for closure of perimembranous ventricular septal defects. Methods: A total of 126 patients, ranging from 1 to 41 years of age (median – 8 years), underwent closure of ventricular septal defects from August 2010 to April 2013. Small- and moderate-sized defects were closed using first-generation Patent ductus arteriosus occluders or Amplatzer Duct Occluder-II. Patients were followed up for the development of complications such as heart block, aortic regurgitation, and tricuspid regurgitation. Results: Patent ductus arteriosus occluders were used in 81 patients, and the Amplatzer Duct Occluder-II device in 45 patients. The devices were successfully deployed in 99.2% of the cases. One patient had embolisation of an Amplatzer Duct Occluder-II device soon after deployment. There was one case of transient complete heart block (0.8%) needing temporary pacing, and two cases of isoarrhythmic atrioventricular dissociation (1.6%). One patient developed late-onset complete heart block 15 months after the procedure and underwent permanent pacemaker implantation. There were no instances of new-onset aortic regurgitation. New-onset mild tricuspid regurgitation was seen in two patients. Of the patients, three had small residual shunts on follow-up, without haemolysis. Conclusions: Duct occluders can be used to effectively close small- and moderate-sized ventricular septal defects. The incidence of complete heart block and valvular regurgitations are much less than reported with other devices, and they are cost-effective.

Background: Coronary artery lesions in Kawasaki disease invasively assessed by coronary angiography. Evaluation of myocardial perfusion by single-photon emission computed tomography may identify the haemodynamic significance of coronary lesions. Objective: To evaluate diagnostic accuracy of dipyridamole stress technetium-99 m tetrofosmin, single-photon emission computed tomography as a possible alternative to invasive coronary angiography for detection and follow-up of myocardial ischaemia in patients with Kawasaki disease, and pre- and post-coronary bypass grafting. Patients and methods: Coronary angiography and single-photon emission computed tomography were performed on 21 patients who were classified into three groups – group I (stenosis), group II (giant aneurysms), and group III (small aneurysms). Of the 21 patients, 16 (groups I and II) patients with myocardial perfusion defects, who underwent coronary bypass grafting, were followed up with single-photon emission computed tomography. Result: In group I, all patients had significant coronary stenosis and 100% of them had perfusion defects in the anterior and septal walls. In group II, all patients had giant aneurysms and 83% of them had inferior and inferolateral perfusion defects. In group III, all patients had small aneurysms and 100% of them had normal perfusion. Pre-coronary bypass grafting myocardial ischaemic defects disappeared in all patients after surgery. Sensitivity, specificity, and accuracy of single-photon emission computed tomography were 94, 100, and 95%, respectively. Conclusion: Technetium-99 m tetrofosmin single-photon emission computed tomography can be applied as an accurate non-invasive diagnostic technique for detecting myocardial perfusion defects with coronary artery lesions, and to show improved or even normalised perfusion of the myocardium in patients after surgical revascularisation.

Background: We have previously identified risk factors for readmission following congenital heart surgery – Hispanic ethnicity, failure to thrive, and original hospital stay more than 10 days. As part of a quality initiative, changes were made to the discharge process in hopes of reducing the impact. All discharges were carried out with an interpreter, medications were delivered to the hospital before discharge, and phone calls were made to families within 72 hours following discharge. We hypothesised that these changes would decrease readmissions. Methods: The current cohort of 635 patients underwent surgery in 2012. Demographic, preoperative, operative, and postoperative variables were evaluated. Univariate and multivariate risk factor analyses were performed. Comparisons were made between the initial (2009) and the current (2012) cohorts. Results: There were 86 readmissions of 77 patients during 2012. Multivariate risk factors for readmission were risk adjustment for congenital heart surgery score and initial hospital stay >10 days. In comparing 2009 with 2012, the overall readmission rate was similar (10 versus 12%, p=0.27). Although there were slight decreases in the 2012 readmissions for those patients with Hispanic ethnicity (18 versus 16%, p=0.79), failure to thrive (23 versus 17%, p=0.49), and initial hospital stay >10 days (22 versus 20%, p=0.63), they were not statistically significant. Conclusions: Potential risk factors for readmission following paediatric cardiothoracic surgery have been identified. Although targeted modifications in discharge processes can be made, they may not reduce readmissions. Efforts should continue to identify modifiable factors that can reduce the negative impact of hospital readmissions.

Background: The term “borderline left ventricle” describes a small left heart that may be inadequate to provide systemic cardiac output and implies the potential need for a single-ventricle palliation. The aim of this study was to identify foetal echocardiographic features that help discriminate which infants will undergo single-ventricle palliation versus biventricular repair to aid in prenatal counselling. Methods: The foetal database at our institution was searched to identify all foetuses with borderline left ventricle, as determined subjectively by a foetal cardiologist, from 2000 to 2011. The foetal images were retrospectively analysed for morphologic and physiologic features to determine which best predicted the postnatal surgical choice. Results: Of 39 foetuses identified with borderline left ventricle, 15 were planned for a univentricular approach, and 24 were planned for a biventricular approach. There were significant differences between the two outcome groups in the Z-scores of the mitral valve annulus, left ventricular end-diastolic dimension, aortic valve annulus, and ascending aorta diameter (p<0.05). With respect to discriminating univentricular outcomes, cut-offs of mitral valve Z-score ⩽−1.9 and tricuspid:mitral valve ratio ⩾1.5 were extremely sensitive (100%), whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 provided the highest specificity (95.8%). Conclusion: In foetuses with borderline left ventricle, a mitral valve Z-score ⩾−1.9 or a tricuspid:mitral valve ratio ⩽1.5 suggests a high probability of biventricular repair, whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 confers a likelihood of single-ventricle palliation.

Despite many advances in recent years for patients with critical paediatric and congenital cardiac disease, significant variation in outcomes remains across hospitals. Collaborative quality improvement has enhanced the quality and value of health care across specialties, partly by determining the reasons for variation and targeting strategies to reduce it. Developing an infrastructure for collaborative quality improvement in paediatric cardiac critical care holds promise for developing benchmarks of quality, to reduce preventable mortality and morbidity, optimise the long-term health of patients with critical congenital cardiovascular disease, and reduce unnecessary resource utilisation in the cardiac intensive care unit environment. The Pediatric Cardiac Critical Care Consortium (PC4) has been modelled after successful collaborative quality improvement initiatives, and is positioned to provide the data platform necessary to realise these objectives. We describe the development of PC4 including the philosophical, organisational, and infrastructural components that will facilitate collaborative quality improvement in paediatric cardiac critical care.

Introduction: The diagnosis of trisomy 21 in children has been associated with failed extubation after CHD surgery. Dexmedetomidine may be a useful agent to improve postoperative outcomes in these patients, such as ventilator time, ICU length of stay, or hospital length of stay. Materials and methods: The Pediatric Health Information System database was queried from January, 2008 to December, 2010 for patients with trisomy 21 who underwent CHD surgery. Patients who received dexmedetomidine were matched to patients who did not by propensity score. The primary outcome was ventilator days charged, and secondary outcomes included ICU and hospital length of stay. Results: A total of 1088 patients (544 matched pairs) met inclusion criteria. Patient characteristics were similar, with the exception of more patients in the dexmedetomidine group undergoing repair of complete atrioventricular canal and fewer undergoing mechanical valve replacement (p<0.01). More patients in the dexmedetomidine group were administered milrinone, epinephrine, vasopressin, benzodiazepines, opiates, and adjunct pain and sedative medications (p<0.01). The dexmedetomidine group had greater time on the ventilator [7 (4.5–11) versus 6 (4–10) days (median, interquartile range) p<0.01] and similar ICU length of stay, hospital length of stay, and mortality compared with controls. Mixed-effects modelling clustered on institution did not show beneficial effect of dexmedetomidine on ventilator time, ICU stay, or hospital length of stay. Conclusions: The use of dexmedetomidine was not associated with the decreased ventilatory time. Routine use of dexmedetomidine is not warranted in this patient population.

Background: “ALARA – As Low As Reasonably Achievable” protocols reduce patient radiation dose. Addition of electroanatomical mapping may further reduce dose. Methods: From 6/11 to 4/12, a novel ALARA protocol was utilised for all patients undergoing supraventricular tachycardia ablation, including low frame rates (2–3 frames/second), low fluoro dose/frame (6–18 nGy/frame), and other techniques to reduce fluoroscopy (ALARA). From 6/12 to 3/13, use of CARTO® 3 (C3) with “fast anatomical mapping” (ALARA+C3) was added to the ALARA protocol. Intravascular echo was not utilised. Demographics, procedural, and radiation data were analysed and compared between the two protocols. Results: A total of 75 patients were included: 42 ALARA patients, and 33 ALARA+C3 patients. Patient demographics were similar between the two groups. The acute success rate in ALARA was 95%, and 100% in ALARA+C3; no catheterisation-related complications were observed. Procedural time was 125.7 minutes in the ALARA group versus 131.4 in ALARA+C3 (p=0.36). Radiation doses were significantly lower in the ALARA+C3 group with a mean air Kerma in ALARA+C3 of 13.1±28.3 mGy (SD) compared with 93.8±112 mGy in ALARA (p<0.001). Mean dose area product was 92.2±179 uGym2 in ALARA+C3 compared with 584±687 uGym2 in ALARA (p<0.001). Of the 33 subjects (42%) in the ALARA+C3 group, 14 received ⩽1 mGy exposure. The ALARA+C3 dosages are the lowest reported for a combined electroanatomical–fluoroscopy technique. Conclusions: Addition of CARTO® 3 to ALARA protocols markedly reduced radiation exposure to young people undergoing supraventricular tachycardia ablation while allowing for equivalent procedural efficacy and safety.

The aim of this study was to determine the left ventricular myocardial deformation and segmental myocardial dysfunction by speckle tracking echocardiography and tissue Doppler imaging among the operated patients with anomalous origin of the left coronary artery from the pulmonary artery. The study was conducted on 12 patients diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, who had been operated upon between 2001 and 2013 at the medical centres of Shiraz University of Medical Sciences, Shiraz, Iran. The mean age of the patients at the time of surgical correction was 12.6 years ranging from 6 months to 43 years, and the duration of postoperative follow-up was between 1 and 12 years. Comparison of the strain rate between the patients with acceptable ejection fraction and the control group by tissue Doppler imaging showed significant differences between the two groups regarding the lateral wall (p<0.001), but not the septal wall of the left ventricle (p=0.65). Moreover, the strain values by the speckle tracking method revealed significant differences between the patient and the control group regarding the global strain (p=0.016) and anterior, lateral, and posterior segments of the left ventricle. Although postoperative conventional echocardiography revealed normal global left ventricular function with acceptable ejection fraction, abnormal myocardial deformation of the variable segments of the left ventricle with regional and global myocardial dysfunction were well defined by speckle tracking echocardiography.

Objectives: This study sought to evaluate left ventricular, right ventricular, and left atrial mechanics and their interactions in patients with congenital scoliosis without clinical heart failure. Methods: A total of 23 patients with a median age of 14 years and a median Cobb’s angle of 61° were studied. Ventricular and atrial myocardial deformation was measured using speckle tracking echocardiography. The results of the patients were compared with 22 controls. Results: Compared with controls, the patients had a significantly greater annular a velocity (p=0.04) and lower e/a ratio (p=0.03); the left ventricular deformation significantly decreased in radial global (p=0.04) and segmental systolic strain and early diastolic strain rate (p=0.03); the left atrial deformation showed a significantly lower positive strain (p=0.02), greater negative strain (p=0.01), and active contractile strain rate (p=0.01). For the patients, the Cobb’s angle was negatively correlated with the left ventricular global radial systolic strain (r=−0.65, p=0.001), left atrial positive strain (r=−0.68, p<0.001), and the left atrial negative strain was positively correlated with the left ventricular circumferential late diastolic strain rate (r=0.46, p=0.01). The left atrial conduit strain rate was positively correlated with the left ventricular circumferential early diastolic strain rate (r=0.42, p=0.03). The left atrial active contractile strain rate was positively correlated with the left ventricular longitudinal late diastolic strain rate (r=−0.4, p=0.03). Conclusions: Impaired left ventricular and altered left atrial mechanics occur relatively early in patients with congenital scoliosis, and are correlated with the severity of their scoliosis. Our findings provide evidence of preclinical heart dysfunction in patients with this disorder.