Hall of Fame
The Paediatric Cardiology Hall of Fame – Donald Nixon Ross
- Jane Somerville
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- Published online by Cambridge University Press:
- 16 July 2015, pp. 1233-1237
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Donald Nixon Ross, FRCS (4 October 1922 to 7 July 2014) was a South African-born British cardiothoracic surgeon, who developed the pulmonary autograft, known as the Ross procedure, for the treatment of aortic valve disease, and also performed the first heart transplant in the United Kingdom in 1968. This paper, written by Jane Somerville, Professor of Cardiology [Retired], Imperial College London, London, United Kingdom, provides the personal recollections about Donald Ross from Jane Somerville, and thus provides a unique snapshot of cardiac surgical history.
Review Article
Executive function deficits in congenital heart disease: why is intervention important?
- Johanna Calderon, David C. Bellinger
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- 17 June 2015, pp. 1238-1246
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It is widely recognised that children with congenital heart disease (CHD) are at high risk for neurodevelopmental impairments including attention deficit hyperactivity disorder and autism spectrum disorder symptoms. Executive function impairments are one of the most prominent neurodevelopmental features associated with CHD. These deficits can have widespread debilitating repercussions in children’s neurocognitive, behavioural, and psycho-social development. There is a crucial gap in research regarding the efficacy of preventive or treatment strategies for these important cognitive morbidities. Executive functions are complex neurocognitive skills highly amenable to improvement. Evidence-based interventions have shown promising results in other paediatric populations, strongly suggesting that they might also benefit the growing population of children with CHD. In this review, we summarise the available data on executive function impairments in children and adolescents with CHD. We underline the important co-morbidity of executive dysfunction with other cognitive and psychiatric issues in CHD, which raises awareness of the crucial need to prevent or at least mitigate these deficits. Finally, we summarise future avenues for research in terms of interventions that may help reduce executive function impairments in youth with CHD.
Original Articles
Maternal hypothyroidism may be associated with CHD in offspring
- Michael J Grattan, Daina S Thomas, Lisa K. Hornberger, Robert M Hamilton, William K Midodzi, Sunita Vohra
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- Published online by Cambridge University Press:
- 02 October 2014, pp. 1247-1253
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Objectives: This study tested whether mothers with maternal hypothyroidism have increased odds of CHD in their offspring, and examined the relationship between CHD, maternal thyroid function, and nausea and vomiting in pregnancy. Background: Maternal hypothyroidism increases the risk for foetal demise and prematurity and can have a negative impact on neurodevelopment. Prior studies have postulated a relationship between maternal thyroid function, CHD, and maternal nausea and vomiting in pregnancy. Methods: A cross-sectional case–control study was conducted over a 17-month period to obtain a history of maternal thyroid status and nausea and vomiting in pregnancy. Paediatric echocardiograms were evaluated for CHD by a blinded paediatric cardiologist. Logistic regression analysis was performed to examine the association between CHD and maternal hypothyroidism. Results: Of the 998 maternal–child pairs, 10% (98/998) of the mothers reported a history of prenatal hypothyroidism. The overall prevalence of CHD in the study sample was 63% (630/998). Mothers with a history of hypothyroidism were significantly more likely to have offspring with CHD compared with mothers without a history of hypothyroidism (72 versus 62%; p=0.04). The adjusted odds ratio (95% confidence interval) of CHD in offspring associated with reported maternal hypothyroidism was 1.68 (1.02–2.78). Conclusion: This study suggests that maternal hypothyroidism is a risk factor for the development of CHD. Further prospective investigations are necessary to confirm this association and delineate pathogenic mechanisms.
Assessment of inter-atrial, inter-ventricular, and atrio-ventricular interactions in tetralogy of Fallot patients after surgical correction. Insights from two-dimensional speckle tracking and three-dimensional echocardiography
- Mohamed Abd El Rahman, Tanja Raedle-Hurst, Axel Rentzsch, Hans-Joachim Schäfers, Hashim Abdul-Khaliq
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- 23 October 2014, pp. 1254-1262
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Background: We aimed to assess biatrial size and function, interactions on atrial and ventricular levels, and atrio-ventricular coupling in patients after tetralogy of Fallot repair. Methods: A total of 34 patients with a mean age of 20.9±9 years, and 35 healthy controls, underwent two-dimensional speckle tracking echocardiography for ventricular and atrial strain measurements and real-time three-dimensional echocardiography to assess ventricular and atrial volumes. Results: When compared with controls, tetralogy of Fallot patients had significantly reduced right atrial peak atrial longitudinal strain (p<0.01), right atrial peak atrial contraction strain (p<0.01), right atrial ejection fraction (p<0.01), left atrial peak atrial longitudinal strain (p<0.01), left atrial peak atrial contraction strain (p<0.05), and left atrial ejection fraction (p<0.01). In the tetralogy of Fallot group, left ventricular ejection fraction was negatively related to the right ventricular end-systolic volume normalised to body surface area (r=−0.62, p<0.01). An association was found in patients between the right atrial peak longitudinal strain and mean right ventricular strain (r=0.64, p<0.01). In patients, the left atrial peak longitudinal strain correlated negatively with right atrial end-diastolic volume normalised to body surface area (r=−0.67, p<0.01), whereas the left atrial ejection fraction correlated weakly with left ventricular ejection fraction (r=0.41, p<0.05). Conclusions: In asymptomatic tetralogy of Fallot patients, biatrial dysfunction exists and can be quantified via two-dimensional speckle tracking echocardiography as well as real-time three-dimensional echocardiography. Different forms of interactions on atrial and ventricular levels are evident among such cohorts.
A novel variation of GDF3 in Chinese Han children with a broad phenotypic spectrum of non-syndromic CHDs
- Jianmin Xiao, Guanyang Kang, Jing Wang, Tengyan Li, Jiuhao Chen, Jieyin Wang, Wei Li, Binbin Wang
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- 05 November 2014, pp. 1263-1267
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Background
The GDF3 gene plays a fundamental role in embryonic morphogenesis. Recent studies have indicated that GDF3 plays a previously unrecognised role in cardiovascular system development. Non-syndromic CHDs might be a clinically isolated manifestation of GDF3 mutations. The purpose of the present study was to identify potential pathological mutations in the GDF3 gene in Chinese children with non-syndromic CHDs, and to gain insight into the aetiology of non-syndromic CHDs.
MethodsA total of 200 non-syndromic CHDs patients and 202 normal control patients were sampled. There were two exons of the human GDF3 gene amplified using polymerase chain reaction. The polymerase chain reaction products were purified and directly sequenced.
ResultsOne missense mutation (c.C635T, p.Ser212 Leu, phenotype: isolated muscular ventricular septal defect) was found that has not been reported previously.
ConclusionsTo the best of our knowledge, this is the first study to investigate the role of the GDF3 gene in non-syndromic CHDs. Our results expand the spectrum of mutations associated with CHDs and first suggest the potentially disease-related GDF3 gene variant in the pathogenesis of CHDs.
Delayed enhancement imaging in a contemporary patient cohort following correction of tetralogy of Fallot
- Uta Preim, Philipp Sommer, Janine Hoffmann, Jana Kehrmann, Lukas Lehmkuhl, Ingo Daehnert, Matthias Gutberlet, Matthias Grothoff
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- 10 November 2014, pp. 1268-1275
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Objective
To test the hypothesis that myocardial scars after repair of tetralogy of Fallot are related to impaired cardiac function and adverse clinical outcome.
MethodsA total of 53 patients were retrospectively analysed after repair of tetralogy of Fallot. The median patient age was 20 years (range 2–48).
Cardiac MRI with a 1.5 T magnet included cine sequences to obtain volumes and function, phase-sensitive inversion recovery delayed enhancement imaging to detect myocardial scars, and flow measurements to determine pulmonary regurgitation fraction. In addition, clinical parameters were obtained.
ResultsAn overall 83% of patients were in NYHA class I. All patients with the exception of 2 (96%) had pulmonary insufficiency. Mean ejection fraction and end-diastolic volume index were 46% and 128 ml/m2 for the right ventricle and 54% and 82 ml/m2 for the left ventricle, respectively. Excluding enhancement of the septal insertion and prosthetic patches, delayed enhancement was seen in 11/53 cases (21%). Delayed enhancement of the right ventricle was detected in 6/53 patients (11%) and of the left ventricle in 5/53 patients (9%). The patient group with delayed enhancement was significantly older (p=0.003), had later repair (p=0.007), and higher left ventricular myocardial mass index (p=0.009) compared with the group without delayed enhancement.
ConclusionsThis study reveals that scarring is common in patients after surgical repair of tetralogy of Fallot and is associated with older age and late repair. However, there was no difference in right ventricular function, NYHA class, or occurrence of clinically relevant arrhythmias between patients with and those without myocardial scars.
Plasma gelsolin as a biomarker of acute rheumatic carditis
- Mustafa Argun, Ali Baykan, Figen Narin, Abdullah Özyurt, Özge Pamukçu, Ferhan Elmalı, Kazım Üzüm, Nazmi Narin
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- 18 November 2014, pp. 1276-1280
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Background
Acute rheumatic fever is an autoimmune, inflammatory, and multi-systemic disease secondary to pharyngitis and is caused by group A streptococcus. In developing countries, acute rheumatic fever is the most common cause of acquired heart disease. Gelsolin is a calcium-dependent, multi-functional actin-regulatory protein circulating in the plasma of healthy human beings. The correlation between blood gelsolin levels and inflammatory conditions suggests the potential benefit of gelsolin as a prognostic marker. The aim of the present study was to appraise the association of gelsolin and acute rheumatic carditis in childhood.
Materials and MethodsPlasma gelsolin levels were measured and echocardiographic examinations were performed in patients (n=37) with acute rheumatic carditis and compared with those of age- and gender-matched healthy controls (n=24).
ResultsThe plasma gelsolin levels in children with acute rheumatic carditis were significantly lower compared with controls (197±218 versus 322±255 mg/L, p=0.039). There was a significant correlation among gelsolin levels and the grade of mitral regurgitation (p=0.030), left ventricular end-diastolic diameter (p=0.017), and left ventricular end-systolic diameter (p=0.028) at diagnosis.
ConclusionsLevels of the gelsolin plasma isoform were decreased in patients with acute rheumatic carditis compared with healthy controls. Gelsolin may be used as a biochemical marker for acute rheumatic carditis.
Arrhythmias in the paediatric intensive care unit: a prospective study of the rates and predictors of arrhythmias in children without underlying cardiac disease
- Gina N. Cassel-Choudhury, Scott I. Aydin, Iris Toedt-Pingel, H. Michael Ushay, James S. Killinger, Hillel W. Cohen, Scott R. Ceresnak
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- Published online by Cambridge University Press:
- 01 December 2014, pp. 1281-1289
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Objective
Arrhythmias are common in patients admitted to the paediatric intensive care unit. We sought to identify the rates of occurrence and types of arrhythmias, and determine whether an arrhythmia was associated with illness severity and paediatric intensive care unit length of stay.
DesignThis is a prospective, observational study of all patients admitted to the paediatric intensive care unit at the Children’s Hospital at Montefiore from March to June 2012. Patients with cardiac disease or admitted for the treatment of primary arrhythmias were excluded. Clinical and laboratory data were collected and telemetry was reviewed daily. Tachyarrhythmias were identified as supraventricular tachycardia, ventricular tachycardia, and arrhythmias causing haemodynamic compromise or for which an intervention was performed.
ResultsA total of 278 patients met the inclusion criteria and were analysed. There were 97 incidences of arrhythmia in 53 patients (19%) and six tachyarrhythmias (2%). The most common types of arrhythmias were junctional rhythm (38%), premature atrial contractions (24%), and premature ventricular contractions (22%). Tachyarrhythmias included three supraventricular tachycardia (50%) and three ventricular tachycardia (50%). Of the six tachyarrhythmias, four were related to placement or migration of central venous lines and two occurred during aminophylline infusion. Patients with an arrhythmia had longer duration of mechanical ventilation and paediatric intensive care unit stay (p<0.001). In multivariate analysis, central venous lines (odds ratio 3.1; 95% confidence interval 1.3–7.2, p=0.009) and aminophylline use (odds ratio 5.1; 95% confidence interval 1.7–14.9, p=0.003) were independent predictors for arrhythmias.
ConclusionsArrhythmias were common in paediatric intensive care unit patients (19%), although tachyarrhythmias occurred rarely (2%). Central venous lines and use of aminophylline were identified as two clinical factors that may be associated with development of an arrhythmia.
Adolescent coarctation of aorta treated with subclavian-descending aorta bypass grafting
- Skander Benomrane, Khedija Soumer, Adel Khayati
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- 27 November 2014, pp. 1290-1292
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Background
Aortic coarctation in older children most frequently represents cases of re-coarctation following previous transcatheter or surgical therapy or missed cases of native coarctation.
MethodsWe describe three cases of adolescents – two girls and one boy – with aortic coarctation, operated between January, 2012 and December, 2013. Computed tomography angiography was performed as an essential diagnostic procedure.
ResultsHypertension was detected, and weaker arterial pulses in the lower limbs were noted in all cases. All operations were performed via left posterolateral thoracotomy. Aortic coarctation was treated surgically, with left subclavian-lower descending thoracic aorta bypass grafting. Postoperative course was uneventful in all cases. No residual brachial-ankle pressure gradient was observed, and all patients have remained in good condition after the operation.
ConclusionsSurgical treatment of aortic coarctation in adolescent patients can be achieved by resection with end-to-end anastomosis, interposition of a graft or bypass graft across the area of coarctation when the distance to be bridged is too long for end-to-end repair.
The extra-anatomic subclavian-descending aortic bypass grafting provides good results in adolescent patients, particularly in those with complex coarctation.
Effect of anti-heart failure therapy on diastolic function in children with single-ventricle circulations
- Deane L.S. Yim, Bryn O. Jones, Peta M.A. Alexander, Yves d’Udekem, Michael M.H. Cheung
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- 05 March 2015, pp. 1293-1299
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Background
Children with functionally univentricular circulations have chronic volume loading of the systemic ventricle, potentially affecting ventricular function. Medications including angiotensin-converting enzyme inhibitors and β-blockers are used to treat ventricular dysfunction, despite limited evidence of their efficacy in this population.
ObjectiveTo determine the effects of angiotensin-converting enzyme inhibitors on elevated filling pressures in children with single ventricle physiology.
MethodsWe performed a single-centre, retrospective review of patients with single ventricle physiology who underwent multiple cardiac catheterisations between 1991 and 2013. Study population comprised of patients who commenced or had optimised dosing of angiotensin-converting enzyme inhibitors between assessments in response to high ventricular filling pressures. Patients undergoing interventions influencing loading conditions between assessments were excluded.
ResultsA total of 17 patients were identified, with dominant morphologic right ventricle in eight patients (47.1%). Among them, 11 (64.7%) were pre-Fontan and six (35.3%) were post-Fontan completion. Median inter-assessment interval was 9.4 months (range 7.3–19.1). There was a reduction in end-diastolic pressure from 13 to 10 mmHg (p=0.002), mean pulmonary artery pressure from 16 to 13 mmHg (p=0.049), and mean atrial pressure from 12 to 9 mmHg (p=0.001). There was one cardiac transplant, and there were no patient deaths at median follow-up after 31 months.
ConclusionsWe observed a reduction in ventricular end-diastolic pressure, pulmonary artery pressure, and mean atrial pressure following treatment with angiotensin-converting enzyme inhibitors in patients with single ventricle physiology. Our study provides insights into the potential impact of anti-heart failure therapy in single ventricle circulations and calls for larger, controlled studies to assess for a therapeutic response.
Upgraded heart failure therapy leads to an improved outcome of dilated cardiomyopathy in infants and toddlers
- Stefan Rupp, Christian Apitz, Leonie Tholen, Heiner Latus, Stefan H. Ostermayer, Dorle Schmidt, Jürgen Bauer, Dietmar Schranz
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- 12 December 2014, pp. 1300-1305
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Objective
Dilated cardiomyopathy is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after establishing the diagnosis. New strategies are needed to improve the outcome in this high-risk patient population.
Method and resultsWe present our experience in 38 patients below the age of three years, who were diagnosed with dilated cardiomyopathy and who were treated at our institution between 2006 and 2012. The treatment strategy involved institution of β-blockers and angiotensin-converting enzyme inhibitors as soon as feasible. In selected cases, pulmonary artery banding or intracoronary autologous bone marrow-derived cell therapy was performed. The median age at presentation was six months (range 1–26 months). The median follow-up age was 16 months (range 2–80 months). Kaplan–Meier analysis of survival after dilated cardiomyopathy diagnosis revealed a one-year survival of 97% and a five-year survival of 86%. The rate of freedom from death or heart transplantation was 82% at one year and 69% at five years. Surviving patients who were free of transplantation, at the follow-up at 25 months (3–80 months), showed a significant improvement in left ventricular ejection fraction (from 19±11 to 46±16%) and left ventricular end-diastolic diameter (z-score from 4.6±2.4 to 1.4±1.6). In addition, the levels of B-type natriuretic peptide improved significantly (from 3330±3840 to 171±825 pg/ml).
ConclusionOur data suggest that the clinical approach described here may result in a markedly improved medium-term outcome in young children with dilated cardiomyopathy. Further studies are required to evaluate whether these approaches reduce end-points such as transplantation or death.
Alpha blocker and angiotensin-converting enzyme inhibitor in the management of severe pulmonary valve stenosis: from bench to bedside
- Mohammed O. Galal, Muhammad A. Khan
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- 29 December 2014, pp. 1306-1310
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Introduction
Neonates with severe pulmonary valve stenosis tend to remain oxygen dependent, despite resolution of the transpulmonary gradient. Alpha 2 blockers – phentolamine – and angiotensin-converting enzyme inhibitors – captopril – were reported to improve oxygen saturation.
ObjectiveTo describe the role of phentolamine and captopril in the treatment of these patients.
MethodsIn a retrospective cohort study, 28 neonates with severe pulmonary valve stenosis underwent balloon valvuloplasty. Among them, 20 remained oxygen or prostaglandin dependent after intervention, and were treated with phentolamine or captopril. Oxygen saturation was monitored before and after intervention and following treatment with these medications. Mean duration of hospitalisation was recorded.
ResultsMean age and weight were 25.2 days and 3.1 kg, respectively. Before balloon dilation, 18/20 (90%) neonates were on prostaglandin, whereas after the procedure only 6/18 patients required it. All 20 patients required oxygen after the procedure, and nine patients (45%) were started on phentolamine. Among them, one patient with severe infundibular stenosis did not respond favourably, and 11 patients (55%) were started on captopril. After starting phentolamine or captopril treatment, prostaglandin could be discontinued after a mean time of 15.86 hours. Within <2 days, there was an increase in mean oxygen saturation from 76.6 to 93.0%.
ConclusionPhentolamine and captopril seem to have therapeutic roles in neonates with severe pulmonary valve stenosis who remain oxygen dependent after balloon dilation. Both drugs led to vasodilation of the pulmonary and systemic vascularisation and facilitated inflow to the right ventricle. Right-to-left shunt across a patent foramen ovale or atrial septal defect decreased and saturation improved, leading to a significant reduction in the length of hospitalisation.
N-terminal pro-brain natriuretic peptide in acute Kawasaki disease correlates with coronary artery involvement
- Philippe M. Adjagba, Laurent Desjardins, Anne Fournier, Linda Spigelblatt, Martine Montigny, Nagib Dahdah
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- 29 December 2014, pp. 1311-1318
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Background
We have lately documented the importance of N-terminal pro-brain natriuretic peptide in aiding the diagnosis of Kawasaki disease.
ObjectivesWe sought to investigate the potential value of N-terminal pro-brain natriuretic peptide pertaining to the prediction of coronary artery dilatation (Z-score>2.5) and/or of resistance to intravenous immunoglobulin therapy. We hypothesised that increased serum N-terminal pro-brain natriuretic peptide level correlates with increased coronary artery dilatation and/or resistance to intravenous immunoglobulin.
MethodsWe carried out a prospective study involving newly diagnosed patients treated with 2 g/kg intravenous immunoglobulin within 5–10 days of onset of fever. Echocardiography was performed in all patients at onset, then weekly for 3 weeks, then at month 2, and month 3. Coronary arteries were measured at each visit, and coronary artery Z-score was calculated. All the patients had N-terminal pro-brain natriuretic peptide serum level measured at onset, and the Z-score calculated.
ResultsThere were 109 patients enrolled at 6.58±2.82 days of fever, age 3.79±2.92 years. High N-terminal pro-brain natriuretic peptide level was associated with coronary artery dilatation at onset in 22.2 versus 5.6% for normal N-terminal pro-brain natriuretic peptide levels (odds ratio 4.8 [95% confidence interval 1.05–22.4]; p=0.031). This was predictive of cumulative coronary artery dilatation for the first 3 months (p=0.04–0.02), but not during convalescence at 2–3 months (odds ratio 1.28 [95% confidence interval 0.23–7.3]; p=non-significant). Elevated N-terminal pro-brain natriuretic peptide levels did not predict intravenous immunoglobulin resistance, 15.3 versus 13.5% (p=1).
ConclusionElevated N-terminal pro-brain natriuretic peptide level correlates with acute coronary artery dilatation in treated Kawasaki disease, but not with intravenous immunoglobulin resistance.
Left ventricular function by echocardiogram in children with sickle cell anaemia in Mumbai, Western India
- Abhay Tidake, Pranil Gangurde, Anup Taksande, Ajay Mahajan, Pratap Nathani
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- 18 December 2014, pp. 1319-1325
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Introduction
Cardiovascular events and complications are the leading cause of mortality and morbidity in patients with sickle cell disease. Cardiac abnormalities occur frequently and at an early stage in sickle cell anaemia patients, despite being more evident in adulthood. Sickle cell anaemia patients are increasingly able to reach adulthood owing to improved healthcare, and may, therefore, suffer the consequences of chronic cardiac injury. Thus, the study of cardiac abnormalities is essential in children
ObjectiveThe aim of this study was to determine the echocardiographic changes in left ventricular function in children suffering from sickle cell disease in Mumbai, Western India.
MethodsThe study comprised of 48 cases of sickle cell anaemia and 30 non-anaemic controls with normal haemoglobin and electrophoresis pattern. M-mode, two-dimensional, and Doppler echocardiographic measurements of patients and controls were performed according to the criteria of the American Echocardiography Society.
ResultsOn Doppler study, the A wave height was increased and the E/A ratio was decreased, whereas the deceleration and isovolumetric relaxation times were prolonged, which is typically seen in slowed or impaired myocardial relaxation (p<0.001). Although chamber dilatations were present, echocardiographic parameters showed no statistically significant correlation with severity of anaemia and age among the sickle cell patients.
ConclusionsWe conclude that the increased left ventricular stiffness, compared with controls, might be due to fibrosis related to ischaemia caused by SS disease in addition to wall hypertrophy.
Association of MTHFR A1298C polymorphism with conotruncal heart disease
- Beyza D. Sayin Kocakap, Cihat Sanli, Feryal Cabuk, Murat Koc, Ali Kutsal
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- 30 December 2014, pp. 1326-1331
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Congenital heart diseases are common congenital anomalies with 1% prevalence worldwide and are associated with significant childhood morbidity and mortality. Among a wide range of aetiologically heterogeneous conditions, conotruncal anomalies account for approximately one-third of all congenital heart defects. The aetiology of conotruncal heart diseases is complex, with both environmental and genetic causes. Hyperhomocysteinaemia, which is often accompanied by the defects of folic acid metabolism, is known to cause conotruncal heart anomalies. In this study, we have evaluated three polymorphisms in the following two hyperhomocysteinaemia-related genes: methylenetetrahydrofolate reductase (MTHFR C677T and A1298C) and nicotinamide N-methyl transferase (NNMT rs694539) in 79 children with conotruncal heart disease and 99 children without conotruncal heart disease. Genotype distribution of the MTHFR A1298C polymorphism showed a statistically significant difference between the two groups. In the case group, AC and CC genotypes were higher than the control group (p<0.05). We have found that MTHFR A1298C polymorphism is associated with conotruncal heart disease; C allele (p=0.028), AC (OR[95% CI]=2.48[1.24–4.95], p=0.010), CC (OR[95% CI]=3.01[1.16–7.83], p=0.023), and AC+CC (OR[95% CI]=2.60[1.36–4.99], p=0.004) genotypes are more frequent in the patient group. Genotype distributions of the MTHFR C677T and NNMT rs694539 polymorphisms were similar in the two groups when evaluated separately and also according to the dominant genetic model (p>0.05). Our results suggest that MTHFR 1298C allele is a risk factor for conotruncal heart disease.
A 6-year follow-up study of adult patients with congenitally corrected transposition
- Mirta Koželj, Marta Cvijić, Pavel Berden, Tomaž Podnar
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- 19 December 2014, pp. 1332-1339
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The aims of this study were to assess the development of heart failure in patients with congenitally corrected transposition of the great arteries in a medium-term follow-up, to identify the impact of tricuspid regurgitation on the development of heart failure, and to determine the most reliable marker for its identification. The prospective 6-year follow-up study included 19 adult patients. All patients were evaluated clinically by the determination of N-terminal pro-hormone brain natriuretic peptide levels, exercise stress testing, echocardiography magnetic resonance, or CT. Among them, two patients died of heart failure. There was a decline in exercise capacity and systolic systemic ventricular function (p=0.011). Systemic ventricular ejection fraction decreased (48.3±13.7 versus 42.7±12.7%, p=0.001). Tissue Doppler imaging showed a decline in peak tricuspid systolic annular velocity (10.3±2.0 versus 8.3±2.5 cm/second, p=0.032) and peak tricuspid early diastolic annular velocity (14.6±4.3 versus 12.0±4.5 cm/second, p=0.048). The tricuspid regurgitation did not increase significantly. N-terminal pro-hormone brain natriuretic peptide levels increased (127.0 ng/L(82.3–305.8) versus 226.0 ng/L(112.5–753.0), p=0.022). Progressive exercise intolerance in congenitally corrected transposition of the great arteries appears to be driven mainly by a progression in systemic right ventricular dysfunction. Tricuspid regurgitation is likely to play a role, especially in patients with structural abnormalities of the tricuspid valve – Ebstein anomaly. The N-terminal pro-hormone brain natriuretic peptide levels and tissue Doppler parameters appear sensitive in detecting changes over time and may guide management.
Site of interstage outpatient care and growth after the Norwood operation
- Mehul D. Patel, Karen Uzark, Sunkyung Yu, Janet Donohue, Sara K. Pasquali, David Schidlow, David W. Brown, Sarah Gelehrter
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- Published online by Cambridge University Press:
- 02 January 2015, pp. 1340-1347
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Background
Recent efforts have focused on optimising interstage outcomes, including growth, for infants following the Norwood operation. The impact of the site of interstage care remains unclear, and it has been hypothesised that care at the surgical site may be beneficial due to greater access to resources such as nutritional support. This study evaluated the relationship between site of interstage care and weight gain in a large multicentre cohort.
MethodsInfants enrolled in the National Paediatric Cardiology Quality Improvement Collaborative (2008–2013) surviving up to Stage 2 were included. Change in weight-for-age z-score between Norwood discharge and Stage 2 admission was compared in those receiving care at the surgical versus non-surgical site.
ResultsOf the 487 interstage survivors, 60% received all care at the surgical site, and 40% received care at a non-surgical site. There was no significant difference between groups in change in weight-for-age z-score: +0.36±0.96 for the surgical site group versus +0.46±1.02 for the non-surgical site group, p=0.3. Results were unchanged in multivariable analysis adjusting for differences in important baseline characteristics, duration of interstage, and home surveillance strategy. The proportion of all patients with weight-for-age z-score <−2 decreased from 40% at Norwood discharge to 29% at Stage 2, with no significant difference in change between the two groups (p=0.1).
ConclusionsThe site of interstage care was not associated with weight gain during the interstage period. Nearly one-third of patients overall had a weight-for-age z-score <−2 at Stage 2. Further study is required to identify methods to optimise weight gain in these patients.
Comparison of invasive and non-invasive pressure gradients in aortic arch obstruction
- Bethany L. Wisotzkey, Christoph P. Hornik, Amanda S. Green, Piers C. A. Barker
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- Published online by Cambridge University Press:
- 20 January 2015, pp. 1348-1357
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Background
Aortic arch obstruction can be evaluated by catheter peak-to-peak gradient or by Doppler peak instantaneous pressure gradient. Previous studies have shown moderate correlation in discrete coarctation, but few have assessed correlation in patients with more complex aortic reconstruction.
MethodsWe carried out retrospective comparison of cardiac catheterisations and pre- and post-catheterisation echocardiograms in 60 patients with native/recurrent coarctation or aortic reconstruction. Aortic arch obstruction was defined as peak-to-peak gradient ⩾25 mmHg in patients with native/recurrent coarctation and ⩾10 mmHg in aortic reconstruction.
ResultsDiastolic continuation of flow was not associated with aortic arch obstruction in either group. Doppler peak instantaneous pressure gradient, with and without the expanded Bernoulli equation, weakly correlated with peak-to-peak gradient even in patients with a normal cardiac index (r=0.36, p=0.016, and r=0.49, p=0.001, respectively). Receiver operating characteristic curve analysis identified an area under the curve of 0.61 for patients with all types of obstruction, with a cut-off point of 45 mmHg correctly classifying 64% of patients with arch obstruction (sensitivity 39%, specificity 89%). In patients with aortic arch reconstruction who had a cardiac index ⩾3 L/min/m2, a cut-off point of 23 mmHg correctly classified 69% of patients (71% sensitivity, 50% specificity) with an area under the curve of 0.82.
ConclusionThe non-invasive assessment of aortic obstruction remains challenging. The greatest correlation of Doppler indices was noted in patients with aortic reconstruction and a normal cardiac index.
Abnormal myocardial blood flow in children with mild/moderate aortic stenosis
- Erin Madriago, Ronald Wells, David J. Sahn, Brian S. Diggs, Stephen M. Langley, Daniel J. Woodward, Michael Jerosch-Herold, Michael Silberbach
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- Published online by Cambridge University Press:
- 10 February 2015, pp. 1358-1366
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Objective
To quantify myocardial blood flow in infants and children with mild or moderate aortic stenosis using adenosine-infusion cardiac magnetic resonance.
BackgroundIt is unclear whether asymptomatic children with mild/moderate aortic stenosis have myocardial abnormalities. In addition, cardiac magnetic resonance-determined normative myocardial blood flow data in children have not been reported.
MethodsWe studied 31 infants and children with either haemodynamically normal hearts (n=20, controls) or mild/moderate aortic stenosis (n=11). The left ventricular myocardium was divided into six segments, and the change in average segmental signal intensity during contrast transit was used to quantify absolute flow (ml/g/minute) at rest and during adenosine infusion by deconvolution of the tissue curves with the arterial input of contrast.
ResultsIn all the cases, adenosine was well tolerated without complications. The mean pressure gradient between the left ventricle and the ascending aorta was higher in the aortic stenosis group compared with controls (24 versus 3 mmHg, p<0.001). Left ventricular wall mass was slightly higher in the aortic stenosis group compared with controls (65 versus 50 g/m2, p<0.05). After adenosine treatment, both the absolute increase in myocardial blood flow (p<0.0001) and the hyperaemic flow significantly decreased (p<0.001) in children with mild/moderate aortic stenosis compared with controls.
ConclusionAbnormal myocardial blood flow in children with mild/moderate aortic stenosis may be an important therapeutic target.
Safety of therapeutic hypothermia in children on veno-arterial extracorporeal membrane oxygenation after cardiac surgery
- Song Lou, Graeme MacLaren, Eldho Paul, Derek Best, Carmel Delzoppo, Yves d’Udekem, Warwick Butt
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- Published online by Cambridge University Press:
- 27 February 2015, pp. 1367-1373
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Objective
The aim of this study was to evaluate whether the use of therapeutic hypothermia in patients receiving extracorporeal membrane oxygenation after paediatric cardiac surgery is associated with increased complication rates.
MethodsWe undertook a retrospective study to compare the complication rates and clinical course of children after cardiac surgery in two groups – extracorporeal membrane oxygenation without therapeutic hypothermia (group 1) and extracorporeal membrane oxygenation with therapeutic hypothermia (group 2). Therapeutic hypothermia was performed via the extracorporeal membrane oxygenation circuit heater–cooler device.
ResultsA total of 96 patients were included in this study (59 in group 1 and 37 in group 2). Complications were comparable between group 1 and group 2, except that more patients with therapeutic hypothermia had hypertension while on extracorporeal membrane oxygenation. Therapeutic hypothermia was not independently associated with in-hospital mortality (adjusted odds ratio 1.16, 95% CI: 0.33–4.03; p=0.82).
ConclusionTherapeutic hypothermia can be safely provided to children on extracorporeal membrane oxygenation after cardiac surgery without an increase in complication rates.