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  • 1
    Online Resource
    Online Resource
    Is biventricular vascular coupling a better indicator of ventriculo-ventricular interaction in congenital heart disease?
    Cambridge University Press (CUP) ; 2021
    In:  Cardiology in the Young Vol. 31, No. 12 ( 2021-12), p. 2009-2014
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 31, No. 12 ( 2021-12), p. 2009-2014
    Abstract: Ventriculo-ventricular interactions are known to exist, though not well quantified. We hypothesised that the ventricular–vascular coupling ratio assessed by cardiovascular MRI would provide insight into this relationship. We also sought to compare MRI-derived ventricular–vascular coupling ratio to echocardiography and patient outcomes. Methods: Children with cardiac disease and biventricular physiology were included. Sanz’s and Bullet methods were used to calculate ventricular–vascular coupling ratio by MRI and echocardiography, respectively. Subgroup analysis was performed for right and left heart diseases. Univariate and multivariate regressions were performed to determine associations with outcomes. Results: A total of 55 patients (age 14.3 ± 2.5 years) were included. Biventricular ventricular–vascular coupling ratio by MRI correlated with each other (r = 0.41; p = 0.003), with respect to ventricle’s ejection fraction (r = −0.76 to −0.88; p 〈 0.001) and other ventricle’s ejection fraction (r = −0.42 to −0.47; p 〈 0.01). However, biventricular ejection fraction had only weak correlation with each other (r = 0.31; p = 0.02). Echo underestimated ventricular–vascular coupling ratio for the left ventricle (p 〈 0.001) with modest correlation to MRI-derived ventricular–vascular coupling ratio (r = 0.43; p = 0.002). There seems to be a weak correlation between uncoupled right ventricular–vascular coupling ratio with the need for intervention and performance on exercise testing (r = 0.33; p = 0.02). Conclusion: MRI-derived biventricular ventricular–vascular coupling ratio provides a better estimate of ventriculo-ventricular interaction in children and adolescents with CHD. These associations are stronger than traditional parameters and applicable to right and left heart conditions.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951121001426
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2021
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  • 2
    Online Resource
    Online Resource
    Extending fellowship for specialised training in paediatric cardiology: deciding when “enough is enough” and when “the sky’s the limit”
    Cambridge University Press (CUP) ; 2020
    In:  Cardiology in the Young Vol. 30, No. 11 ( 2020-11), p. 1557-1558
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 30, No. 11 ( 2020-11), p. 1557-1558
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951120003492
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2020
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  • 3
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    Online Resource
    Clinical application of three-dimensional printing to the management of complex univentricular hearts with abnormal systemic or pulmonary venous drainage
    Cambridge University Press (CUP) ; 2017
    In:  Cardiology in the Young Vol. 27, No. 7 ( 2017-09), p. 1248-1256
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 27, No. 7 ( 2017-09), p. 1248-1256
    Abstract: In recent years, three-dimensional printing has demonstrated reliable reproducibility of several organs including hearts with complex congenital cardiac anomalies. This represents the next step in advanced image processing and can be used to plan surgical repair. In this study, we describe three children with complex univentricular hearts and abnormal systemic or pulmonary venous drainage, in whom three-dimensional printed models based on CT data assisted with preoperative planning. For two children, after group discussion and examination of the models, a decision was made not to proceed with surgery. We extend the current clinical experience with three-dimensional printed modelling and discuss the benefits of such models in the setting of managing complex surgical problems in children with univentricular circulation and abnormal systemic or pulmonary venous drainage.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S104795111600281X
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2017
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  • 4
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    Online Resource
    Persistence of right ventricular dysfunction and altered morphometry in asymptomatic preterm Infants through one year of age: Cardiac phenotype of prematurity
    Cambridge University Press (CUP) ; 2019
    In:  Cardiology in the Young Vol. 29, No. 7 ( 2019-07), p. 945-953
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 29, No. 7 ( 2019-07), p. 945-953
    Abstract: Prematurity impacts myocardial development and may determine long-term outcomes. The objective of this study was to test the hypothesis that preterm neonates develop right ventricle dysfunction and adaptive remodelling by 32 weeks post-menstrual age that persists through 1 year corrected age. Materials and Methods: A subset of 80 preterm infants (born 〈 29 weeks) was selected retrospectively from a prospectively enrolled cohort and measures of right ventricle systolic function and morphology by two-dimensional echocardiography were assessed at 32 weeks post-menstrual age and at 1 year of corrected age. Comparisons were made to 50 term infants at 1 month and 1 year of age. Sub-analyses were performed in preterm-born infants with bronchopulmonary dysplasia and/or pulmonary hypertension. Result: In both term and preterm infants, right ventricle function and morphology increased over the first year (p 〈 0.01). The magnitudes of right ventricle function measures were lower in preterm-born infants at each time period (p 〈 0.01 for all) and right ventricle morphology indices were wider in all preterm infants by 1 year corrected age, irrespective of lung disease. Measures of a) right ventricle function were further decreased and b) morphology increased through 1 year in preterm infants with bronchopulmonary dysplasia and/or pulmonary hypertension (p 〈 0.01). Conclusion: Preterm infants exhibit abnormal right ventricle performance with remodelling at 32 weeks post-menstrual age that persists through 1 year corrected age, suggesting a less developed intrinsic myocardial function response following preterm birth. The development of bronchopulmonary dysplasia and pulmonary hypertension leave a further negative impact on right ventricle mechanics over the first year of age.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951119001161
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2019
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  • 5
    Online Resource
    Online Resource
    Left ventricular non-compaction in patients with single ventricle heart disease
    Cambridge University Press (CUP) ; 2020
    In:  Cardiology in the Young Vol. 30, No. 1 ( 2020-01), p. 12-18
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 30, No. 1 ( 2020-01), p. 12-18
    Abstract: Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function. Methods: Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio 〉 2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data. Results: The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m 2 , p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m 2 , p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71). Conclusions: Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951119001872
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2020
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  • 6
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    Online Resource
    Utility of expert focused cardiac ultrasound in paediatric cardiology outreach clinics
    Cambridge University Press (CUP) ; 2019
    In:  Cardiology in the Young Vol. 29, No. 12 ( 2019-12), p. 1468-1473
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 29, No. 12 ( 2019-12), p. 1468-1473
    Abstract: This prospective observational study evaluates the utility of expert focused cardiac ultrasound (eFCU) with spectral Doppler to inform decision making in established patients with specific, selected congenital cardiac defects in outreach clinics. Secondary objectives include determining if the addition of eFCU expands capacity in paediatric cardiology outreach clinics and if it improves the patient experience. Methods: Patients aged 2 months to 19 years old with a diagnosis of ventricular septal defect, atrial septal defect, atrioventricular septal defect, patent ductus arteriosus, aortic valve stenosis, or pulmonary valve stenosis with the need for follow-up echocardiography in an outreach clinic from August 2017 to June 2018 were studied. A novel assessment tool was used to determine the success of eFCU. Results: Forty-two patients from 11 clinics underwent eFCU with one unsuccessful exam making the failure rate 2.3% (95% CI 0.0006–0.1256). Addition of eFCU led to a significant increase in volume of patients able to be seen 19 versus 15.5 (p 〈 0.01). A majority of parents/patients reported a positive experience with eFCU. Conclusion: Expert focused cardiac ultrasound with spectral Doppler can be used successfully for follow-up in patients with select CHD and the addition of eFCU permits increased patient capacity in outreach clinics and has the potential to improve the patient experience.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951119002427
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2019
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  • 7
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    Online Resource
    Echocardiographic examination of mitral valve abnormalities in the paediatric population: current practices
    Cambridge University Press (CUP) ; 2020
    In:  Cardiology in the Young Vol. 30, No. 1 ( 2020-01), p. 1-11
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 30, No. 1 ( 2020-01), p. 1-11
    Abstract: We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords “mitral regurgitation and/or stenosis, children.” The search was refined by adding the keywords “echocardiographic definition, classification, and evaluation.” Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived “gradients” has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951119003196
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2020
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  • 8
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    Online Resource
    LV non-compaction in patients with coarctation of the aorta: prevalence and effects on cardiac function
    Cambridge University Press (CUP) ; 2021
    In:  Cardiology in the Young Vol. 31, No. 9 ( 2021-09), p. 1445-1450
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 31, No. 9 ( 2021-09), p. 1445-1450
    Abstract: Left ventricular non-compaction has been associated with heart failure, arrhythmia, thromboembolism and sudden death. The prevalence of non-compaction in patients with coarctation of the aorta and its clinical significance remains unknown, although obstructive left heart disease is common in patients with non-compaction. We sought to evaluate the prevalence of left ventricular non-compaction in patients with repaired aortic coarctation as well as its effect on left ventricular size and systolic function. Methods and results: In total, 268 patients (Mean age 26 (inter-quartile range 21–37) years, 63% male) undergoing cardiac magnetic resonance imaging for clinical follow-up were included from three tertiary centres for adult congenital heart disease. Clinical data was obtained from medical records and correlated with ventricular volumes and function. Left ventricular non-compaction was defined as a diastolic non-compacted:compacted dimension ratio 〉 2.3 in the worst affected segment on a long-axis view. Left ventricular non-compaction was present in 8.2% of patients with repaired coarctation. Left ventricular end-diastolic volumes and stroke volumes were significantly higher in patients with non-compaction compared to those without. There were no significant differences in ventricular mass or ejection fraction in these two groups. Conclusions: Left ventricular non-compaction is relatively common in patients with repaired coarctation of the aorta and correlates with increased left ventricular end-diastolic volumes.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S104795112100038X
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2021
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  • 9
    Online Resource
    Online Resource
    Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging reveals abnormal blood flow patterns in the aorta and pulmonary trunk of patients with transposition
    Cambridge University Press (CUP) ; 2014
    In:  Cardiology in the Young Vol. 24, No. 1 ( 2014-02), p. 47-53
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 24, No. 1 ( 2014-02), p. 47-53
    Abstract: Flow profiles are important determinants of fluid–vessel wall interactions. The aim of this study was to assess blood flow profiles in the aorta and pulmonary trunk in patients with transposition and different ventriculoarterial connection, and hence different mechanics of the coherent pump. Methods In all, 29 patients with operated transposition – concordant atrioventricular and discordant ventriculoarterial connection, and no other cardiac malformation – and eight healthy volunteers were assessed with cardiac magnetic resonance imaging: n = 17 patients after atrial redirection, with a morphologic right ventricle acting as systemic pump and a morphologic left ventricle connected to the pulmonary trunk, and n = 12 patients after the arterial switch procedure, with physiologic ventriculoarterial connections. Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging was used to analyse systolic flow patterns in the aorta and pulmonary trunk, relating to helical flow and vortex formation. Results In the aorta, overall helicity was present in healthy volunteers, but it was absent in all patients independent on the operation technique. Partial helices were observed in the ascending aorta of 58% of patients after arterial switch. In the pulmonary trunk, mostly parallel flow was seen in healthy volunteers and in patients after arterial switch, whereas vortex formation was present in 88% of patients after atrial redirection. Conclusion Blood flow patterns differ substantially between the groups. In addition to varying mechanics of the coherent pumping ventricles, the absent overall helicity in all patients might be explained by the missing looping of the aorta in transposition.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951112002193
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2014
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