Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

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.

Motor dysfunction and behavioural problems frequently coexist with congenital heart disease in school-age children.

AIM: Patients with congenital heart disease are at risk of neurodevelopmental deficits. Impairments in motor and behavioural function occur frequently, but no information is available concerning the coexistence of deficits in these two developmental domains. This study explored the occurrence of motor and behavioural deficits and their coexistence in children with surgically corrected congenital heart disease. METHODS: Outcome was assessed in 95 children with congenital heart disease who had undergone cardiopulmonary bypass surgery. Their mean age was 9.6 years (SD 2.5). Motor function was assessed with the Zurich Neuromotor Assessment and behaviour with the Strength and Difficulties Questionnaire. RESULTS: Children with congenital heart disease performed poorer in all motor domains compared with the reference population (all p ≤ 0.001). Behaviour was affected in the domains 'emotional symptoms' and 'hyperactivity/inattention' (both p < 0.01), and 54% of the children with motor abnormalities showed behavioural deficits. Children with coexistent abnormalities in behaviour and motor function had higher rates of remedial school services and therapeutic support. CONCLUSION: Children with congenital heart disease are at risk of long-term motor and behavioural problems, and there is a high rate of coexistence of problems in both domains. Early and longitudinal assessment of all developmental domains is necessary to provide adequate early support.

Pericarditis constrictiva in a 10-year-old boy after influenza A virus infection.

Pericarditis constrictiva is caused by fibrotic degeneration of the pericardium and leads to impaired diastolic ventricular filling. The diagnosis of constrictive pericarditis in children remains challenging and often requires a multimodal approach. We present a case of a pericarditis constrictiva in a 10-year old boy after influenza A virus infection. Clinicians should be aware of this complication, especially in patients with symptoms of exertional dyspnea and congestive heart failure.

Multicentre reference values for cardiac magnetic resonance imaging derived ventricular size and function for children aged 0-18 years.

AIMS: Cardiovascular magnetic resonance (CMR) imaging is an important tool in the assessment of paediatric cardiac disease. Reported reference values of ventricular volumes and masses in the paediatric population are based on small cohorts and several methodologic differences between studies exist. We sought to create steady-state free precession (SSFP) CMR reference values for biventricular volumes and mass by combining data of previously published studies and re-analysing these data in a standardized manner. METHODS AND RESULTS: A total of 141 healthy children (68 boys) from three European centres underwent cine-SSFP CMR imaging. Cardiac structures were manually contoured for end-diastolic and end-systolic phases in the short-axis orientation according to current standardized CMR post-processing guidelines. Volumes and masses were derived from these contours. Age-related reference curves were constructed using the lambda mu sigma method. Median age was 12.7 years (range 0.6-18.5). We report biventricular volumes and masses, unindexed and indexed for body surface area, stratified by age groups. In general, boys had approximately 15% higher biventricular volumes and masses compared with girls. Only in children aged <6 years old no gender differences could be observed. Left ventricle ejection fraction was slightly higher in boys in this study population (median 67% vs. 65%, P = 0.016). Age-related reference curves showed non-linear relations between age and cardiac parameters. CONCLUSION: We report volumetric SSFP CMR imaging reference values for children aged 0-18 years old in a relatively large multi-centre cohort. These references can be used in the follow-up of paediatric cardiac disease and for research purposes.

Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

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.

Cardiac disease in children and young adults with various lysosomal storage diseases: Comparison of echocardiographic and ECG changes among clinical groups.

BACKGROUND: Lysosomal storage disease (LSD) is a rare inherited disease group. Consecutively there are few data on cardiac changes in mucopolysaccharidosis (MPS), Anderson Fabry disease (AFD), and other LSD (oLSD) including Pompe disease (PD) and Danon disease (DD), I-cell disease ICD and mucolipidosis III (ML III). METHODS: Between 1994 and 2011, we identified 39 patients with LSD: 25 with MPS, 8 with AFD, and 6 with oLSD including PD (1), ML III (2), DD (1), and ICD (2) at our institution fulfilling the inclusion criteria of at least one echocardiogram and ECG. RESULTS: Median age was 11.4 years (range: 2-27), 22 were females (56%). Normal echocardiograms were present in 12 patients (31%): 4 with MPS (16%), 7 AFD (88%), and 1 oLSD (17%). Valvular heart disease was present in 23 patients (59%) occurring more often in MPS (76%) and oLSD (67%) than in AFD (0%) (p < 0.001). The most common ECG abnormality was a short PR interval in 10 of 35 patients (29%) occurring in all LSD groups. Median follow-up was 5.8 (0.2-22.2) years showing diminished 5-year survival compared to an age-matched group. However, no patient died due to a cardiac cause and no cardiovascular intervention was necessary. CONCLUSION: Echocardiographically detectable cardiovascular involvement in children with LSD is mostly confined to MPS and oLSD. Valve thickening in echo and a short PR interval in the ECG are the most frequent abnormalities. Routine repeat assessment is recommended in LSD. However, significant cardiac disease necessitating cardiac intervention is rare during a short follow-up.

Pectus excavatum: echocardiography and cardiac MRI reveal frequent pericardial effusion and right-sided heart anomalies.

AIMS: In patients with pectus excavatum (PEX), echocardiographic assessment can be difficult. There are little data on the impact of the chest deformity on echocardiographic findings and comparison of data obtained by echocardiography (echo) with cardiac magnetic resonance imaging (CMR) in PEX. METHODS AND RESULTS: In a prospective study, cardiac anomalies in PEX were analysed by echo and compared with CMR in consecutive patients with PEX referred for echo. If they agreed to participate, the patients were referred for CMR and included if the pectus index was ≥3.0 by CMR. Also, clinical data and electrocardiogram tracings were analysed. There were 18 patients (13 females; 72%), with a mean age of 53±16 years; mean pectus index was 4.7 (range: 3-7.3). Echo showed haemodynamically insignificant pericardial effusion in six patients (33%), tricuspid valve prolapse in five (28%), right ventricular (RV) localized wall motion anomalies (WMA) in five (28%) and diminished RV systolic function in two (11%); no patient had RV dilatation. CMR demonstrated cardiac displacement to the left in 9 patients (50%); minimal pericardial effusion was seen in 10 patients (56%; P value=0.13 compared with echo), RV localized WMA in 6 (44%; P value=1.0), diminished RV systolic function in 8 (44%; P=0.07), and RV dilatation in 5 (28%; P=0.06). A completely normal cardiac examination was found in six patients by echo (33%) and in 2 (11%) using CMR. Although some signs of arrhythmogenic RV cardiomyopathy (ARVC) were present, no patient fulfilled the ARVC criteria. CONCLUSION: In severe PEX, haemodynamically insignificant pericardial effusion, tricuspid valve prolapse and other RV anomalies possibly due to RV displacement are frequent as demonstrated by both CMR and echo. The cardiac assessment by echo and CMR did show discrepancies; however, they were not significant.