Review Article
The usefulness of brain natriuretic peptide in simple congenital heart disease – a systematic review
- Jannet A. Eindhoven, Annemien E. van den Bosch, Eric Boersma, Jolien W. Roos-Hesselink
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- Published online by Cambridge University Press:
- 20 September 2012, pp. 315-324
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Brain natriuretic peptide and N-terminal pro-brain natriuretic peptide are two well-established markers for cardiac failure in acquired heart disease. Nevertheless, the clinical utility of these markers in patients with congenital heart disease remains unclear. Therefore, the aim of this study was to evaluate the diagnostic and prognostic value of these markers in patients with congenital heart disease. A PubMed and EMBASE literature search was executed with focus on the most common simple congenital heart defects, atrial septal defect and ventricular septal defect. Data on brain natriuretic peptide measurement, cardiac function parameters, and follow-up were collected. In patients with atrial or ventricular septal defect, brain natriuretic peptide levels were mildly increased when compared with healthy age-matched controls. Shunt severity and pulmonary artery pressure correlated strongly with natriuretic peptide levels. A clear association between brain natriuretic peptide and functional class was demonstrated. After closure of the defect, a rise in brain natriuretic peptide levels in the first hours to days was observed. After longer follow-up, natriuretic peptide levels decreased and became comparable to pre-procedural values. In conclusion, this systematic review shows that brain natriuretic peptide levels are mildly increased in patients with unrepaired and repaired atrial or ventricular septal defect. Brain natriuretic peptide measurement might be a useful additional tool in the diagnostic work-up of patients with atrial or ventricular septal defect. Further investigation in a larger, prospective study with long-term follow-up is warranted to elucidate the true prognostic value of natriuretic peptides in patients with simple congenital heart disease.
Original Articles
Iron-deficiency anaemia, gastric hyperplasia, and elevated gastrin levels due to potassium channel dysfunction in the Jervell and Lange-Nielsen Syndrome
- Annika Winbo, Olof Sandström, Richard Palmqvist, Annika Rydberg
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- Published online by Cambridge University Press:
- 18 July 2012, pp. 325-334
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Aim
We investigated extra-cardiac clinical symptoms and signs in the rare Jervell and Lange-Nielsen Syndrome, characterised by impaired KCNQ1 function, a gene essential for gastric acid secretion.
MethodsAll Swedish Jervell and Lange-Nielsen cases with double KCNQ1 mutations (14 cases) were investigated by medical record review, an interview, and were offered laboratory testing for iron-deficiency anaemia and gastrointestinal markers.
ResultsA history of iron-deficiency anaemia in 12 of 14 patients and subjective gastrointestinal symptoms in 13 of 14 patients was revealed. Previous endoscopy in five cases had revealed no case of coeliac or inflammatory bowel disease but three cases of mucosal hyperplasia/dysplasia. Current signs of anaemia or iron substitution were present in 9 of 12 tested cases. Elevated levels of gastrin in seven of nine cases, pepsinogen in six of seven cases, and faecal calprotectin in nine of nine cases were present. A significant correlation between elevated gastrin levels and concurrent iron-deficiency and/or anaemia was revealed (p-value 0.039).
ConclusionsA high frequency of extra-cardiac clinical symptoms and previous medical investigations was found. We propose that the Jervell and Lange-Nielsen Syndrome phenotypically includes gastrointestinal symptoms/signs and secondary iron-deficiency anaemia owing to hypochlorhydria on the basis of KCNQ1 mutations. The resultant elevated gastrin level is a potential risk factor for later gastrointestinal cancer. Clinical monitoring with regard to developing anaemia and hypergastrinaemia should be considered in the Jervell and Lange-Nielsen Syndrome.
Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection
- Andrew M. Atz, Thomas G. Travison, Brian W. McCrindle, Lynn Mahony, Andrew C. Glatz, Aditya K. Kaza, Roger E. Breitbart, Steven D. Colan, Jonathan R. Kaltman, Renee Margossian, Sara K. Pasquali, Yanli Wang, Welton M. Gersony
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- Published online by Cambridge University Press:
- 24 July 2012, pp. 335-343
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Background
A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.
MethodsWe compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).
ResultsA prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.
ConclusionsAfter controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.
Cardiac findings and long-term thromboembolic outcomes following pulmonary embolism in children: a combined retrospective-prospective inception cohort study
- Hayley S. Hancock, Michael Wang, Katja M. Gist, Elizabeth Gibson, Shelley D. Miyamoto, Peter M. Mourani, Marilyn J. Manco-Johnson, Neil A. Goldenberg
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- Published online by Cambridge University Press:
- 22 October 2012, pp. 344-352
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In paediatric pulmonary embolism, cardiac findings and thromboembolic outcomes are poorly defined. We conducted a mixed retrospective-prospective cohort study of paediatric pulmonary embolism at the Children's Hospital Colorado between March, 2006 and January, 2011. A total of 58 consecutive children – age less than or equal to 21 years – with acute pulmonary embolism were enrolled. Data collection included clinical and laboratory characteristics, treatments, serial echocardiographic and electrocardiographic findings, and outcomes of pulmonary embolism non-resolution and recurrence. The median age was 16.5 years ranging from 0 to 21 years. The most prevalent clinical risk factors were oral contraceptive pill use (52% of female patients), presence of a non-infectious inflammatory condition (21%), and trauma (21%). Thrombophilias included heterozygous factor V Leiden in 21%; antiphospholipid antibody syndrome was established in 31% overall. Proximal pulmonary artery involvement was present in 34%. At presentation, nearly half of the patients had hypoxaemia and 37% had tachycardia. The classic electrocardiographic finding of S1Q3T3 was present in 12% acutely; tricuspid regurgitation greater than 3 metres per second, septal flattening, and right ventricular dilation were each present on acute echocardiogram in 25%. Nearly all patients received therapeutic anticoagulation, with initial systemic tissue plasminogen activator administered in 16% for occlusive iliofemoral deep venous thrombosis and/or massive pulmonary embolism. Pulmonary embolism resolution was observed in 82% by 6 months. Recurrent pulmonary embolism occurred in 9%. There were no pulmonary embolism-related deaths. Right ventricular dysfunction was rare in follow-up. These data indicate that acute heart strain is common, but chronic cardiac dysfunction is rare, following aggressive management of acute pulmonary embolism in children.
Chest pain with elevated troponin assay in adolescents
- Matthew C. Schwartz, Shari Wellen, Jonathan J. Rome, Chitra Ravishankar, Shobha Natarajan
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- 20 September 2012, pp. 353-360
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Objective
We sought to describe the evaluation, treatment, and follow-up of adolescents who presented to a single institution with chest pain and an elevated troponin I value in the absence of typical symptoms of pericarditis or myocarditis.
Materials and methodsWe performed a retrospective review of patients in the age group of 10–18 years of age with no history of significant heart disease admitted to our institution from 2000 to 2010 after presenting with chest pain and an elevated troponin I value.
ResultsA total of 16 patients were identified with a median age of 16.5 years (range 11.2–17.8 years). Of these 13 (81%) were male and 10 (63%) showed evidence of localised ST elevations on electrocardiogram. The median peak troponin I level was 17.8 nanograms per millilitre (range 0.89–227, normal less than 0.4). There were eight patients (50%) with a diagnosis of coronary vasospasm, three patients (20%) with atypical myopericarditis, one patient with coronary anomaly, one patient with hypercoagulable disorder, and one patient with prolonged supraventricular tachycardia. In two patients, no definitive diagnosis was made. There was one patient who needed catheter-based intervention, which involved stenting of a coronary artery after a procedure-related complication.
ConclusionsIn our cohort of adolescents without history of significant cardiac disease, chest pain and elevated troponin I levels were attributed to a variety of causes. Although coronary vasospasm and atypical myopericarditis were seen most commonly, coronary anomaly was identified in one case. Magnetic resonance imaging proved a useful diagnostic tool to assess coronary artery anatomy and myocardial changes suggestive of myocarditis. On the basis of these results and a review of the literature, a general evaluation algorithm is presented.
Clinical characteristics and causes of chest pain in 380 children referred to a paediatric cardiology unit
- Ahmet Sert, Ebru Aypar, Dursun Odabas, Cem Gokcen
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- 05 July 2012, pp. 361-367
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Background
Chest pain is a common presenting complaint to paediatrics, paediatric cardiology, and paediatric emergency departments. In this study, we prospectively evaluated clinical characteristics and causes of chest pain in children referred to our paediatric cardiology unit.
MethodsA total of 380 children were included. Associated symptoms and past and family histories were evaluated. All patients underwent physical examination. The following studies were performed: complete blood count in all patients; fasting lipid profiles in overweight and obese children and children with a family history of premature cardiovascular disease; and electrocardiogram, chest X-ray, and echocardiogram in all patients. If necessary, 24-hour electrocardiogram monitoring or exercise stress tests were performed. Patients with a history of positive psychological findings were evaluated by a child psychiatrist.
ResultsThe most common causes of chest pain were musculoskeletal disorders (37.1%), idiopathic chest pain (29.2%), and miscellaneous disorders, for example precordial catch syndrome (15%), respectively. Only 1 of 380 (0.3%) patients had chest pain due to a cardiac disorder. Electrocardiograms were abnormal in 4 of 380 (1.1%) patients. A total of 9 of 380 patients (2.3%) had dyslipidaemia.
ConclusionsAlthough a paediatric cardiology referral may provide reassurance to the primary care and emergency department physicians, our results show that cardiac aetiologies for paediatric chest pain are very rare. We think that many patients in our study were adequately evaluated only by careful history, and physical examination. Therefore, we suggest that it may not be necessary to use echocardiogram in the routine evaluation of children with chest pain.
Behaviour problems in adolescents with cardiac disease: an exploratory study in a paediatric cardiology outpatient clinic
- Maria M. da Silva, Teresa H. Schoen-Ferreira, Maria S. B. Diógenes, Antonio C. Carvalho
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- 20 September 2012, pp. 368-376
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Aims
To assess behavioural problems in adolescents with congenital and acquired heart disease in comparison with healthy controls. The perception of behavioural problems by the patients’ parents was also assessed and compared.
MethodsA cross-sectional study was carried out in 130 adolescents with congenital and acquired heart disease and 246 healthy controls. The second part of the Youth Self-Report was applied to the patients and controls, and the Child Behavior Checklist to the patients’ parents.
ResultsMale patients showed significantly fewer behavioural problems compared with male controls. No significant difference was found in the female gender. Healthy male adolescents scored significantly higher in the Internalising, Externalising, and in the Total Problems scales. Patients scored significantly higher only on the Social Problems subscale. Female patients in middle and late adolescence and male patients in early adolescence displayed more problems. No significant difference was found between the diagnostic groups. Operated patients did not differ from the non-operated ones. Patients scored significantly lower than did their parents.
ConclusionsMale adolescents with cardiac disease reported fewer behavioural problems when compared with healthy controls, but no difference was observed in the female gender. Patients also reported fewer behavioural problems than did their parents. Adolescents with cardiac disease scored higher than did controls only on the Social Problems subscale. Analysing the patients’ behavioural profile, female patients in middle and late adolescence and male patients in early adolescence were the most problematic ones. No difference was observed between the diagnostic groups, nor between operated and non-operated patients.
Tips and pearls for “true” dextroposition of the aorta in tetralogy of Fallot
- Sahin Bozok, Mert Kestelli, Gökhan Ilhan, Orhan Gokalp, Berkan Ozpak, Muhammet Akyuz, Barcin Ozcem, Pınar Kestelli
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- 14 September 2012, pp. 377-380
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Infundibular stenosis may develop secondary to ventricular septal defect, and transannular patch plasty can affect mortality and morbidity rates. Therefore, dextroposition of the aorta has been investigated in order to eliminate discrepancies in the literature. Figures and illustrations from the selected references have been investigated and “actual dextroposition of the aorta” has been evaluated as far as the aorta is visible. A careful examination of these figures revealed the following tips and pearls for accurate diagnosis of dextroposition of the aorta: Aorta and ventricular septal defect should be adjacent for a “true” dextroposition of the aorta; the plane where the aorta exits from the ventricle should penetrate the plane of the ventricular septal defect towards the right ventricle; if the aorta and ventricular septal defect intersect at one edge, the aorta may seem to be dextroposed; new diagnostic modalities are necessary to evaluate the actual dextroposition status of the aorta for the proper planning of treatment.
Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults
- Giulia Tuo, Maurizio Marasini, Claudio Brunelli, Lucio Zannini, Manrico Balbi
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- 20 July 2012, pp. 381-386
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Objectives
To describe our experience in the management of coronary artery anomalies both in an adult and in a paediatric population and to compare the two groups for finding out differences in terms of angiographic incidence and treatment.
Patients and methodsDatabases at the Department of Cardiology of San Martino Adult's Hospital and of Gaslini Children's Hospital were searched for all patients with a diagnosis of coronary artery anomaly who underwent coronary angiography between 1994 and 2006.
ResultsCoronary anomalies were diagnosed in 76 (1%) adult patients. Anomalous left circumflex artery was the commonest coronary anomaly (25%). Anomalous left coronary artery from pulmonary artery and myocardial bridges were the only anomalies responsible for angina-like symptoms. No patients except the one with anomalous left coronary artery from pulmonary artery needed surgical intervention. In the paediatric population, we found 28 (0.9%) patients with coronary anomalies. Anomalous left coronary artery from pulmonary artery was the most common anomaly (48%) and always required emergency surgical treatment; in addition there were two patients with stenosis of the left main coronary artery.
ConclusionCoronary artery anomalies may be associated with very acute, even life-threatening symptoms in children, whereas they are usually clinically silent and detected by accident on coronary angiography in adults. Recognition of coronary artery anomalies enables early treatment or close follow-up in children, whereas it could be useful in case of cardiac surgery in adults.
Clinical response to arginine vasopressin therapy after paediatric cardiac surgery
- Christopher W. Mastropietro, Maria C. Davalos, Shivaprakash Seshadri, Henry L. Walters III, Ralph E. Delius
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- 18 July 2012, pp. 387-393
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Objective
To describe the haemodynamic response of children who receive arginine vasopressin for haemodynamic instability after cardiac surgery and to identify clinical variables associated with a favourable response.
Materials and MethodsWe reviewed patients less than or equal to 6 years undergoing open heart surgery in our institution between January, 2009 and July, 2010 who received arginine vasopressin during the first 7 days post operation. Favourable responders were defined as those in whom blood pressure was increased or maintained and catecholamine score was decreased, or blood pressure was increased by greater than or equal to 10% of baseline and catecholamine score was unchanged at 6 hours following arginine vasopressin initiation.
ResultsOf the 34 patients identified, 17 (50%) patients responded favourably to arginine vasopressin. At 6 hours, the mean blood pressure was increased by 32.2% in responders as compared with 4.6% in non-responders, with a p-value less than 0.001. The mean catecholamine score decreased by 30.1% in responders and increased by 7.6% in non-responders, with a p-value less than 0.001. Anthropometric, demographic, and intra-operative variables were similar in both groups, as was maximum dose of arginine vasopressin. The median time after arrival to the intensive care unit at which arginine vasopressin was initiated, however, was later in those who responded, 20 hours as compared with those who did not, 6 hours, with a p-value equal to 0.032.
ConclusionsArginine vasopressin therapy led to haemodynamic improvement in only half of the children in this study, and improvement was more likely to occur if arginine vasopressin was initiated after the post-operative night.
Familial congenital heart disease: data collection and preliminary analysis
- Xike Wang, Jing Wang, Pengjun Zhao, Ying Guo, Lanping Wu, Jing Sun, Kun Sun
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- 01 October 2012, pp. 394-399
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The aim of this study was to explore genetic mechanisms of congenital heart disease by analysing family data. Families with two or more affected members were studied, and information on family history and risk factors was collected. A total of 25 families with congenital heart disease were identified, and among them the condition was confirmed in 57. The prevalence of congenital heart disease in first-degree relatives was 43.0%, that is 46 out of 107, significantly higher than that in second-degree relatives, that is, 4.4%, 11 out of 252) (χ2 = 83.897, P < 0.01). The prevalence difference between twins (90%) and siblings (62.2%) (χ2 = 4.983, P < 0.05) was also significant among first-degree relatives. Eleven families were found to have the same phenotype (44%), including ventricular septal defect in six families, atrial septal defect in two families, conotruncal defects in two families, and hypoplastic left heart syndrome in one family. Both twins were diagnosed with congenital heart disease in 8 out of 10 twin families – all eight twins were monozygotic. The cardiac phenotype of the twins was consistent in three families (37.5%). The cardiac phenotype of first- and second-degree relatives was not fully consistent with their probands. There was an increased incidence of threatened abortion in early pregnancy in patients with familial congenital heart disease when compared with sporadic congenital heart disease (χ2 = 8.704, P < 0.05). Morbidity in relatives was related to blood relationship, with a closer relationship increasing the risk of congenital heart disease. Genetic factors appear to play an important role in congenital heart disease.
Modified Calgary score in differential diagnosis between cardiac syncope and postural orthostatic tachycardia syndrome-associated syncope in children
- Jinyan Yang, Lulu Zhu, Stella Chen, Xueying Li, Qingyou Zhang, Fengwen Zhang, Li Chen, Chaoshu Tang, Junbao Du, Hongfang Jin
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- Published online by Cambridge University Press:
- 09 October 2012, pp. 400-404
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The present study was designed to analyse the usefulness of a modified Calgary score system during differential diagnosis between cardiac syncope and postural orthostatic tachycardia syndrome-associated syncope through a large sample sized clinical investigation. The study included 213 children, including 101 boys and 112 girls, with cardiac syncope or postural orthostatic tachycardia syndrome-associated syncope in the age group of 2–19 years (mean 11.8 ± 2.9 years). A modified Calgary score was created, which was analysed to predict differential diagnoses between cardiac syncope and postural orthostatic tachycardia syndrome-associated syncope using a receiver operating characteristic curve. The median of modified Calgary scores for cardiac syncope was −5.0, which significantly differed from that of postural orthostatic tachycardia syndrome (0.0; p < 0.01). The sensitivity and specificity of a differentiation score of less than −2.5 was 96.3% and 72.7%, respectively. Owing to the fact that the modified Calgary score was an integer, when less than −3.0 the diagnosis could be considered as cardiac syncope. The modified Calgary score could be used to make an initial differential diagnosis between cardiac syncope and postural orthostatic tachycardia syndrome-associated syncope in the clinic.
Successful introduction of interventional catheterisation and other paediatric cardiology services in a developing country
- Hideshi Tomita, Noriyuki Haneda, Takashi Higaki, Koichi Kataoka
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- 23 August 2012, pp. 405-408
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Background
Providing care for children with cardiac disease remains a challenge in developing countries.
Materials and methodsSince 2001, a team of paediatric cardiologists has been visiting Mongolia to perform children's cardiac catheterisation, as well as screening children for cardiac disease. Considering the limited medical resources, as well as cost, risk, and benefit, we focused our activities on diagnostic consultation by echocardiography, transcatheter closure of persistent arterial duct, balloon dilation of valvar pulmonary stenosis and aortic coarctation, and diagnostic catheterisation.
ResultsUp to 2011, we have completed echocardiography in 1200 patients; diagnostic catheterisations in 59 patients; and catheter interventions in 255 patients, including 224 for persistent arterial duct, 23 for valvar pulmonary stenosis, 6 for aortic coarctation, and a few others. We have visited 14 rural areas to screen for children's cardiac diseases. A total of 131 persistent ducts were closed with coils and a further 93 with a duct occluder. Migration of the coil or occluder to the pulmonary artery occurred once for each device. Pulmonary valvuloplasty was successful in 23 patients and a coarctation was effectively dilated in six patients.
ConclusionsThe limited healthcare resources in developing countries such as Mongolia make catheter interventional procedures an attractive alternative to surgery in treating children with simple but critical congenital heart diseases. Introduction of the duct occluder extended the application of transcatheter occlusion of persistent arterial duct in Mongolia to larger vessels and avoided the expense of coil occlusion using multiple coils.
The role of right ventricular function in paediatric idiopathic dilated cardiomyopathy
- Abraham Groner, Jen Yau, Irene D. Lytrivi, H. Helen Ko, James C. Nielsen, Ira A. Parness, Shubhika Srivastava
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- 23 August 2012, pp. 409-415
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Introduction
The prevalence of right ventricular dysfunction in idiopathic dilated cardiomyopathy is incompletely studied in children. Furthermore, right ventricular function may signal worse outcomes. We evaluated recently published right ventricular function echocardiographic indices in identifying dysfunction in children with idiopathic dilated cardiomyopathy and the impact of right ventricular dysfunction on long-term prognosis.
MethodsA retrospective database review of right ventricular function indices in 30 patients with idiopathic dilated cardiomyopathy was compared with 60 age- and sex-matched controls from January, 2001 until December, 2010. Right ventricular function was assessed by Doppler tissue peak systolic S′, early and late diastolic E′ and A′ waves and isovolumic acceleration at the tricuspid valve annulus; pulsed wave Doppler tricuspid valve inflow E and A waves; right ventricular myocardial performance index; tricuspid annular plane systolic excursion; right ventricular fractional area change.
ResultsRight ventricular systolic and diastolic function in idiopathic dilated cardiomyopathy was significantly impaired. All measured indices except for isovolumic acceleration and fractional area change were significantly reduced, with a p-value less than 0.05. There was no right ventricular index predictive of death or transplantation. Patients with poor outcome were significantly more likely to need inotropic support (p-value equal to 0.018), be placed on a ventricular assist device (p equal to 0.005), and have a worse left ventricular ejection fraction z-score (p-value equal to 0.002).
ConclusionRight ventricular dysfunction is under-recognised in children presenting with idiopathic dilated cardiomyopathy. The need for clinical circulatory support and left ventricular ejection fraction z-score less than minus 8 were primary determinants of outcome, independent of the degree of derangement in right ventricular function.
Predictors of exaggerated exercise-induced systolic blood pressures in young patients after coarctation repair
- Peace C. Madueme, Philip R. Khoury, Elaine M. Urbina, Thomas R. Kimball
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- 12 September 2012, pp. 416-422
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Background
In normotensive subjects, an exaggerated blood pressure response to exercise is associated with the development of resting hypertension. We sought to determine the prevalence of elevated blood pressures during exercise in post-operative coarctation patients with normal resting blood pressure, and investigate associations with exercise-induced hypertension in this population.
MethodsA total of 38 patients were enrolled after end-to-end anastomosis repair and resting normotension. All patients underwent anthropometric and blood pressure measurements, echocardiographic evaluation of function, arterial stiffness assessment by pulse wave velocity, and a graded exercise test. An abnormal response was defined as a maximum systolic blood pressure greater than the 95th percentile of published normal values. Correlation analyses and stepwise regression analyses were performed.
ResultsThe mean age was 12.7 years, including 79% male patients. The mean resting systolic blood pressure was 111.3 millimetres of mercury and the mean exercise systolic blood pressure was 178.1 millimetres of mercury. The prevalence of a systolic blood pressure greater than the 95th percentile was 16.7%. In multivariate analysis, the exercise systolic blood pressure index was associated with body mass index, age, aortic valve annulus, shortening fraction, and pulse wave velocity (R2 equal to 0.79, p equal to 0.0009). Estimates of ventricular filling and indexed left ventricular mass were elevated.
ConclusionsThere is a risk of elevated systolic blood pressure during exercise in normotensive patients after coarctation repair. Resting blood pressures are useful but not sufficient. Echocardiography demonstrated abnormalities suggestive of a chronic cardiac burden despite resting normotension. Regular imaging may be necessary to improve long-term outcomes. New paradigms for the continued follow-up of these patients are necessary.
Tetralogy of Fallot with unilateral absent pulmonary artery
- Murat Ugurlucan, Ahmet H. Arslan, Yahya Yildiz, Sibel Ay, Resmiye T. Besikci, Sertac Cicek
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- 18 July 2012, pp. 423-430
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Background
Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity.
Patients and methodsThere were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy.
ResultsUrgent modified Blalock–Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively.
ConclusionOur modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.
Cardiac troponin I after cardiopulmonary bypass in infants in comparison with older children
- Monesha Gupta-Malhotra, Jeffrey H. Kern, Patrick A. Flynn, Myles S. Schiller, Jan M. Quaegebeur, Deborah M. Friedman
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- Published online by Cambridge University Press:
- 20 July 2012, pp. 431-435
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Objective
At the present time, there is a trend towards performing open heart surgery at a younger age. Myocardium of infants has been thought to be more vulnerable to cardiopulmonary bypass in comparison with adults. For this study, we evaluated the degree of myocardial injury by measurement of cardiac troponin levels in infants in comparison with older children for similar surgeries.
MethodsSerum was collected before bypass, after bypass, and daily after surgery and serum cardiac troponin I level (micrograms per litre). The demographic data, cardiac diagnoses, types of surgery performed, and peri-operative parameters were collected.
ResultsOf the 21 children enrolled consecutively, five were infants. Among the 21 patients, four patients had post-operative peak troponin values greater than 100 (three were infants) and all four patients survived and had normal left ventricular systolic function upon discharge echocardiogram. The five infants had peak troponin levels of 222.3, 202, 129, 26.7, and 82.3. The post-operative peak troponin levels were significantly higher in infants (mean 132.5 with a standard deviation of 81.6) than in the older children (mean 40.3 with a standard deviation of 33.4), although there was no significant difference in bypass time, bypass temperature, cross-clamp time, or the length of stay in the intensive care unit between the two age groups.
ConclusionsHigher troponin release is seen in infants in comparison with older children after bypass for similar surgeries. A troponin level greater than 100 after bypass does not necessarily predict death or a severe cardiovascular event in the very young.
Brief Reports
Managing a traumatic ventricular septal defect with atrial septal defect occluder device
- Mohammad Alidoosti, Seyed K. Hoseini, Akbar Shafiee
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- 10 September 2012, pp. 436-439
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Traumatic ventricular septal defects are rare complications of blunt and penetrating chest trauma. Patients are usually referred because of shock or cardiac tamponade. Focusing on the critical condition of the patient leads to missing the presence of traumatic ventricular septal defects. In this case report, we introduce a patient with a large traumatic ventricular septal defect, which was diagnosed 40 days after a penetrating cardiac trauma and was finally treated with transcatheter closure.
A large intracardiac thrombus in a child with steroid-resistant nephrotic syndrome
- Filiz Ekici, Nilgün Çakar
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- Published online by Cambridge University Press:
- 20 July 2012, pp. 440-442
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Intracardiac thrombus is a rare complication of nephrotic syndrome in children, and only a few cases have been reported. We report the case of a 3.5-year-old child diagnosed with Henoch–Schönlein Purpura and steroid-resistant nephrotic syndrome for 2 months. The patient had massive oedema but no cardiovascular instability. Echocardiography displayed a large thrombus within the right atrium and the patient was successfully managed with enoxoparine.
Recurrent pulmonary haemorrhage in an infant with tetralogy of Fallot and absent pulmonary valve: interventional treatment by coil occlusion of systemic-to-pulmonary collateral arteries
- Matthias Kumpf, Jürgen Schäfer, Michael Hofbeck
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- Published online by Cambridge University Press:
- 17 July 2012, pp. 443-446
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Although clinically silent in the majority of cases, enlarged bronchial arteries or systemic-to-pulmonary collateral arteries may complicate congenital heart disease in infants, causing significant left-to-right shunting with subsequent pulmonary congestion and respiratory compromise. So far, pulmonary haemorrhage, a well-known complication in older patients with cyanotic congenital heart disease, has not been described in infancy. We describe the case of a 6-month-old girl with tetralogy of Fallot and absent pulmonary valve who developed haemoptysis with severe respiratory distress following corrective surgery of the cardiac malformation. High-resolution computed tomography of the thorax followed by selective angiography revealed a systemic-to-pulmonary collateral artery originating from the left internal mammary artery. Pulmonary haemorrhage stopped immediately following coil occlusion of the collateral. A second episode of pulmonary haemorrhage occurred at the age of 9 months during mechanical ventilation for treatment of pneumonia. Repeat angiography revealed two more collateral vessels. Again coil occlusion resulted in prompt resolution of pulmonary haemorrhage. According to our experience, enlarged bronchial arteries or systemic-to-pulmonary collateral arteries should be considered in infants with cyanotic heart disease with unexplained pulmonary congestion or prolonged respiratory problems.