Review Article
How should we diagnose and differentiate hearts with double-outlet right ventricle?
- Tara Bharucha, Anthony M. Hlavacek, Diane E. Spicer, Paraskevi Theocharis, Robert H. Anderson
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- Published online by Cambridge University Press:
- 19 September 2016, pp. 1-15
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Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called “200%” origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle.
This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.
Original Articles
Respiratory syncytial virus hospitalisation trends in children with haemodynamically significant heart disease, 1997–2012
- Patricia Y. Chu, Christoph P. Hornik, Jennifer S. Li, Michael J. Campbell, Kevin D. Hill
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- Published online by Cambridge University Press:
- 10 May 2016, pp. 16-25
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Objective
The aim of the study was to evaluate the trends in respiratory syncytial virus-related hospitalisations and associated outcomes in children with haemodynamically significant heart disease in the United States of America.
Study designThe Kids’ Inpatient Databases (1997–2012) were used to estimate the incidence of respiratory syncytial virus hospitalisation among children ⩽24 months with or without haemodynamically significant heart disease. Weighted multivariable logistic regression and chi-square tests were used to evaluate the trends over time and factors associated with hospitalisation, comparing eras before and after publication of the 2003 American Academy of Pediatrics palivizumab immunoprophylaxis guidelines. Secondary outcomes included in-hospital mortality, morbidity, length of stay, and cost.
ResultsOverall, 549,265 respiratory syncytial virus-related hospitalisations were evaluated, including 2518 (0.5%) in children with haemodynamically significant heart disease. The incidence of respiratory syncytial virus hospitalisation in children with haemodynamically significant heart disease decreased by 36% when comparing pre- and post-palivizumab guideline eras versus an 8% decline in children without haemodynamically significant heart disease (p<0.001). Children with haemodynamically significant heart disease had higher rates of respiratory syncytial virus-associated mortality (4.9 versus 0.1%, p<0.001) and morbidity (31.5 versus 3.5%, p<0.001) and longer hospital length of stay (17.9 versus 3.9 days, p<0.001) compared with children without haemodynamically significant heart disease. The mean cost of respiratory syncytial virus hospitalisation in 2009 was $58,166 (95% CI:$46,017, $70,315).
ConclusionsThese data provide stakeholders with a means to evaluate the cost–utility of various immunoprophylaxis strategies.
Intermediate- and long-term follow-up of device closure of patent arterial duct with severe pulmonary hypertension: factors predicting outcome
- Masood Sadiq, Asif U. Rehman, Najam Hyder, Ahmad U. Qureshi, Tehmina Kazmi, Shakeel A. Qureshi
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- 02 May 2016, pp. 26-36
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Background
In patients with large patent arterial ducts and severe pulmonary hypertension, the natural history of progression of pulmonary hypertension is very variable. Whether to close or not to close is often a difficult decision, as there are no established haemodynamic parameters predicting reversibility.
ObjectivesThe objectives of this study were to evaluate the results of device closure of large patent arterial ducts with severe pulmonary hypertension after 2 years of age and to determine haemodynamic variables associated with its regression during long-term follow-up.
MethodsA total of 45 patients, with median age of 10 (2–27) years, with large patent arterial ducts and severe pulmonary hypertension, were considered. Haemodynamic variables were assessed in air, oxygen, and after occlusion. The follow-up was performed to assess regression of pulmonary hypertension.
ResultsDevice closure was successful in 43 (96%) patients. Pulmonary artery systolic and mean pressures decreased from 79 to 67 mmHg and from 59 to 50 mmHg, respectively (p<0.001). At a median follow-up of 80 (41–151) months, severe pulmonary hypertension persisted in four (9.7%) patients. Multivariate analysis showed pulmonary vascular resistance index ⩽6 WU m2 and pulmonary artery systolic and mean pressures ⩽75 and ⩽55 mmHg (all in oxygen), having 97.8% predictive value for regression of pulmonary hypertension (p<0.001) in the long term. In 24 patients with catheterisation-based criteria, regression of pulmonary hypertension was associated with pulmonary vascular resistance index <8 WU m2 (p=0.001) and its fall of >25% (both in oxygen) (p=0.007).
ConclusionsDevice closure of large patent arterial ducts with severe pulmonary hypertension is safe and effective. Pulmonary vascular resistance index and systolic and mean pulmonary artery pressures in oxygen are the key prognostic variables predicting regression of pulmonary hypertension.
Association of cardiac autonomic modulation with physical and clinical features of young people with type 1 diabetes
- Anne K. F. Silva, Diego G. D. Christofaro, Franciele M. Vanderlei, Marianne P. C. R. Barbosa, David M. Garner, Luiz C. M. Vanderlei
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- 16 March 2016, pp. 37-45
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Objective
The objective of this study was to verify possible associations between heart rate variability indices and physical activity, body composition, and metabolic and cardiovascular parameters in individuals with type 1 diabetes.
MethodA total of 39 young patients with type 1 diabetes were included. Body composition, physical activity, cardiovascular parameters, and metabolic parameters were assessed. For the heart rate variability analysis, heart rate was recorded beat-by-beat using a Polar S810i heart rate monitor for 30 minutes, with the volunteers in the supine position; subsequently, the following indices were considered: standard deviation of all normal RR intervals; root-mean square of differences between adjacent normal RR intervals in a time interval; percentage of adjacent RR intervals with a difference of duration >50 ms; high frequency component in milliseconds squared; high frequency component in normalised units; standard deviation of the instantaneous variability beat-to-beat; and standard deviation of the long-term variability. The association between the heart rate variability indices and independent variables was verified through linear regression in unadjusted and adjusted models (considering gender and age). The statistical significance was set at 5% and the confidence interval at 95%.
ResultsHigh values of at-rest heart rate were associated with reduced parasympathetic activity and global heart rate variability, and higher values of waist-to-hip ratio were related to lower parasympathetic activity, independent of age or gender.
ConclusionFor young patients with type 1 diabetes, increases in at-rest heart rate values are associated with reduced parasympathetic activity and global heart rate variability, whereas higher waist-to-hip ratio values are related to lower parasympathetic activity, both independent of age and gender.
Types of social supports predicting health-related quality of life among adult patients with CHD in the Institut Jantung Negara (National Heart Institute), Malaysia
- Sue K. Tye, Geetha Kandavello, Kah L. Gan
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- 25 February 2016, pp. 46-54
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Objectives
The objectives of this study were to examine which types of social supports – emotional/informational support, tangible support, affectionate support, and positive interactions – are the predictors of health-related quality of life (HRQoL) in adult patients with CHD and to assess the influence of demographic variables and clinical factors on these variables.
MethodsIn total, 205 adult patients with CHD from the National Heart Institute, Malaysia, were recruited. Patients were first screened by cardiology consultants to ensure they fit the inclusion criteria before filling in questionnaires, which were medical outcome studies – social support survey and AQoL-8D.
Results/conclusionsAll social supports and their subscales were found to have mild-to-moderate significant relationships with physical dimension, psychological dimension, and overall HRQoL; however, only positive interaction, marital status, and types of diagnosis were reported as predictors of HRQoL. Surprisingly, with regard to the physical dimension of quality of life, social supports were not significant predictors, but educational level, marital status, and types of diagnosis were significant predictors. Positive interaction, affectionate support, marital status, and types of diagnosis were again found to be predictors in the aspects of the psychological dimension of quality of life. In conclusion, positive interaction and affectionate support, which include elements of fun, relaxation, love, and care, should be included in the care of adult patients with CHD.
Is spontaneous closure of a patent arterial duct common?
- Julien I. E. Hoffman
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- 10 May 2016, pp. 55-58
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As closing a patent arterial duct is relatively simple, safe, and successful, most children with a patent arterial duct have it closed soon after diagnosis. The larger ducts are closed to prevent congestive heart failure, pulmonary vascular disease, or aneurysmal dilatation of the ductus, and smaller ducts are closed to prevent infective endocarditis. Consequently, there is no opportunity to determine whether spontaneous closure or diminution in size of the patent arterial duct is common.
If the duct does become smaller, flow through it may be so low that no murmur is produced – the silent ductus. The frequency and best management of the silent patent arterial duct are unknown, and we do not know whether these tiny ducts are the last stage before spontaneous closure.
Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population
- Bradley S. Marino, David J. Goldberg, Adam L. Dorfman, Eileen King, Heidi Kalkwarf, Babette S. Zemel, Margaret Smith, Jesse Pratt, Mark A. Fogel, Amanda J. Shillingford, Barbara J. Deal, Anitha S. John, Caren S. Goldberg, Timothy M. Hoffman, Marshall L. Jacobs, Asher Lisec, Susan Finan, Lazaros K. Kochilas, Thomas W. Pawlowski, Kathleen Campbell, Clinton Joiner, Stuart L. Goldstein, Paul Stephens, Jr, Alvin J. Chin
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- Published online by Cambridge University Press:
- 05 July 2016, pp. 59-68
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Background
Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients.
Methods and resultsThis study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman’s Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6–33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (−0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (−0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63–0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)].
ConclusionsHigher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.
Paediatric cardiology fellowship training: effect of work-hour regulations on scholarly activity
- Christina Ronai, Peter Lang
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- 09 March 2016, pp. 69-73
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Background
In 2003, work-hour regulations were implemented by the Accreditation Council for Graduate Medical Education. Much has been published regarding resident rest and quality of life as well as patient safety. There has been no examination on the effect of work-hour restrictions on academic productivity of fellows in training. Paediatric subspecialty fellows have a scholarly requirement mandated by the American Board of Pediatrics. We have examined the impact of work-hour restrictions on the scholarly productivity of paediatric cardiology fellows during their fellowship.
MethodsWe conducted a literature search for all paediatric cardiology fellows between 1998 and 2007 at a single academic institution as first or senior authors on papers published during their 3-year fellowship and 3 years after completion of their categorical fellowship (n=63, 30 fellows before 2003 and 33 fellows after 2003). The numbers of first- or senior-author fellow publications before and after 2003 were compared. We also collected data on final paediatric cardiology subspecialty career choice.
ResultsThere was no difference in the number of fellow first-author publications before and after 2003. Before work-hour restrictions, the mean number of publications per fellow was 2.1 (±2.2), and after work-hour restrictions it was 2.0 (±1.8), (p=0.89). By subspecialty career choice, fellows who select electrophysiology, preventative cardiology, and heart failure always published within the 6-year time period.
ConclusionsSince the implementation of work-hour regulations, total number of fellow first-authored publications has not changed. The role of subspecialty choice may play a role in academic productivity of fellows in training.
Screening high school students in Italy for sudden cardiac death prevention by using a telecardiology device: a retrospective observational study
- Claudio De Lazzari, Igino Genuini, Maria C. Gatto, Alessandra Cinque, Massimo Mancone, Alessandra D’Ambrosi, Elisa Silvetti, Antonio Fusto, Domenico M. Pisanelli, Francesco Fedele
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- Published online by Cambridge University Press:
- 04 March 2016, pp. 74-81
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Background
In 2010, an Italian project was launched aimed at using a telecardiology device in order to perform early diagnosis of young students at risk of sudden cardiac death.
MethodsOur retrospective observational study was conducted on a population of 13,016 students, aged between 16 and 19 years, in different Italian regions. It consisted of analysis of data recorded during a telecardiology pilot study. The recorded data were electrocardiograms and data concerning lifestyle habits and family history of cardiovascular diseases. In total, 14 alterations in the electrocardiogram signal have been considered in this study. Some of these alterations are as follows: ventricular ectopic beats, atrioventricular block, Brugada-like electrocardiogram pattern, left anterior/posterior fascicular block, left/right ventricular hypertrophy, long/short QT interval, left atrial enlargement, right atrial enlargement, short PQ interval, and ventricular pre-excitation Wolff–Parkinson–White syndrome. On the basis of the collected data, we implemented this retrospective observational study.
ResultsThe analysed data showed that 13.60% of students had a family history for cardiovascular diseases, 22.43% reported smoking habits, 26.23% reported alcohol consumption, and 7.24% reported abuse of drugs. A total of 24% of students had at least one of the 14 electrocardiogram pathological alterations considered in our study and 32% had electrocardiogram values within the normal range.
ConclusionsThis retrospective observational study analysed data registered during our telecardiology activity. This activity permitted to maximise data collection and minimise the costs for collecting such data. This activity of screening is being continued and in the next few years it will allow us to have a greater mass of data.
Child and teacher acceptability of school-based echocardiographic screening for rheumatic heart disease in Uganda
- Michelle Ploutz, Twalib Aliku, Tyler Bradley-Hewitt, Andrea Dantin, Bethan Lemley, Catherine W. Gillespie, Peter Lwabi, Craig Sable, Andrea Beaton
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- 17 March 2016, pp. 82-89
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Introduction
Rheumatic heart disease causes substantial morbidity in children in low-income countries. School-based echocardiographic screening has been suggested as a means to identify children with latent disease; however, little is known about the experience of children and teachers participating in screenings. The aim of our study was to assess students’ and teachers’ experience of school-based echocardiographic screening and identify areas for improvement.
Materials and methodsA school-based echocardiographic screening programme was conducted in five schools in Northern Uganda in 2013. After 8 months, an age- and gender-stratified population that included 5% of the participating students and teachers completed a questionnaire via an in-person interview. Responses were reviewed by question and coded to identify key themes.
ResultsA total of 255 students (mean 10.7 years; 48% male) and 35 teachers participated in our study. In total, 95% of the students and 100% of the teachers were happy to have participated in the screening; however, students reported feeling scared (35%) and nervous (48%) during the screening process. Programmatic strengths included the following: knowing one’s health status, opportunity to receive treatment, and staff interactions. Although 43% of the patients did not suggest a change with open-ended questioning, concerns regarding privacy, fear of the screening process, and a desire to include others in the community were noted.
DiscussionSchool-based echocardiographic rheumatic heart disease screening was well received by students and teachers. Future programmes would likely benefit from improved pre-screening education regarding the screening process and diagnosis of rheumatic heart disease. Furthermore, education of teachers and students could improve screening perception and establish realistic expectations regarding the scope of screening.
Effects of chronic treprostinil treatment on experimental right heart hypertrophy and failure
- Sofie Axelgaard, Sarah Holmboe, Steffen Ringgaard, Thomas K. Hillgaard, Stine Andersen, Mona S. Hansen, Asger Andersen, Jens E. Nielsen-Kudsk
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- Published online by Cambridge University Press:
- 18 April 2016, pp. 90-100
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Background
Right heart function is an important predictor of morbidity and mortality in pulmonary arterial hypertension and many CHD. We investigated whether treatment with the prostacyclin analogue treprostinil could prevent pressure overload-induced right ventricular hypertrophy and failure.
MethodsMale Wistar rats were randomised to severe pulmonary trunk banding with a 0.5-mm banding clip (n=41), moderate pulmonary trunk banding with a 0.6-mm banding clip (n=36), or sham procedure (n=10). The banded rats were randomised to 6 weeks of treatment with a moderate dose of treprostinil (300 ng/kg/minute), a high dose of treprostinil (900 ng/kg/minute), or vehicle.
ResultsPulmonary trunk banding effectively induced hypertrophy, dilatation, and decreased right ventricular function. The severely banded animals presented with decompensated heart failure with extracardial manifestations. Treatment with treprostinil neither reduced right ventricular hypertrophy nor improved right ventricular function.
ConclusionsIn the pulmonary trunk banding model of pressure overload-induced right ventricular hypertrophy and failure, moderate- and high-dose treatment with treprostinil did not improve right ventricular function neither in compensated nor in decompensated right heart failure.
The diagnostic value of plasma N-terminal connective tissue growth factor levels in children with heart failure
- Gang Li, Xueqing Song, Jiyi Xia, Jing Li, Peng Jia, Pengyuan Chen, Jian Zhao, Bin Liu
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- 16 March 2016, pp. 101-108
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Objective
The aim of this study was to assess the diagnostic value of plasma N-terminal connective tissue growth factor in children with heart failure.
Methods and resultsPlasma N-terminal connective tissue growth factor was determined in 61 children, including 41 children with heart failure, 20 children without heart failure, and 30 healthy volunteers. The correlations between plasma N-terminal connective tissue growth factor levels and clinical parameters were investigated. Moreover, the diagnostic value of N-terminal connective tissue growth factor levels was evaluated. Compared with healthy volunteers and children without heart failure, plasma N-terminal connective tissue growth factor levels were significantly elevated in those with heart failure (p<0.01). N-terminal pro-brain natriuretic peptide and left ventricular end-diastolic dimension were positively correlated with plasma N-terminal connective tissue growth factor levels (r=0.364, p=0.006; r=0.308, p=0.016), whereas there was a negative correlation between left ventricular ejection fraction and plasma N-terminal connective tissue growth factor (r=−0.353, p=0.005). Connective tissue growth factor was significantly correlated with the severity of heart failure (p<0.001). Moreover, addition of connective tissue growth factor to N-terminal pro-brain natriuretic peptide did not significantly increase area under curve for diagnosing heart failure (area under curve difference 0.031, p>0.05), but it obviously improved the ability of diagnosing heart failure in children, as demonstrated by the integrated discrimination improvement (6.2%, p=0.013) and net re-classification improvement (13.2%, p=0.017) indices.
ConclusionsPlasma N-terminal connective tissue growth factor is a promising diagnostic biomarker for heart failure in children.
Elevated birth prevalence of conotruncal heart defects in a population with high consanguinity rate
- Moshe Stavsky, Renana Robinson, Maayan Yitshak Sade, Hanah Krymko, Eli Zalstein, Viktorya Ioffe, Victor Novack, Aviva Levitas
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- 16 March 2016, pp. 109-116
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Background
The aetiology of conotruncal heart defects is poorly understood and the birth prevalence varies geographically. The known risk factors for developing conotruncal heart defects are as follows: CHD in siblings, genetic chromosomal abnormalities, paternal age >30 years, high parity, low birth weight, prematurity, and maternal diabetes.
ObjectiveThe aim of this study was to characterise conotruncal heart defects, birth prevalence, mortality, and morbidity in the population of southern Israel, of whom 75% are Jewish and the rest are mostly Bedouin Arabs.
MethodsThe data were obtained from Soroka University Medical Center database of births and newborns. Conotruncal heart defects cases were identified by ICD9 codes.
ResultsDuring 1991–2011, there were 247,290 singleton live births and 393 conotruncal heart defects in Soroka University Medical Center. The birth prevalence per 10,000 live births of tetralogy of Fallot, transposition of the great arteries, and truncus arteriosus was 9.5, 5, and 1.8, respectively. In the multivariate analysis, Bedouin descent (adjusted odds ratio 2.40, p<0.001), maternal age >35 years (1.66, p=0.004), and siblings with congenital heart defects (1.98, p=0.005) were associated with tetralogy of Fallot, and Bedouin descent (1.61, p=0.05), siblings with congenital heart defects (2.19, p=0.004), and diabetes mellitus (7.15, p<0.001) were associated with transposition of the great arteries. In a univariate analysis, Bedouin descent (p=0.004) and congenital heart defects in siblings (p<0.001) were associated with truncus arteriosus.
ConclusionWe observed higher birth prevalence of conotruncal heart defects compared with the birth prevalence reported worldwide, specifically among the Bedouins, a population characterised with high consanguinity rate. Therefore, genetic counselling and early fetal echocardiograms should be encouraged, especially in high consanguinity rate populations. Naturally, further educational efforts are needed in order to decrease consanguinity and its related consequences.
Reduced arterial stiffness in very fit boys and girls
- Heidi Weberruß, Raphael Pirzer, Thorsten Schulz, Birgit Böhm, Robert Dalla Pozza, Heinrich Netz, Renate Oberhoffer
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- 29 March 2016, pp. 117-124
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Low cardiorespiratory fitness is associated with higher cardiovascular risk, whereas high levels of cardiorespiratory fitness protect the cardiovascular system. Carotid intima-media thickness and arterial distensibility are well-established parameters to identify subclinical cardiovascular disease. Therefore, this study investigated the influence of cardiorespiratory fitness and muscular strength on carotid intima-media thickness and arterial distensibility in 697 children and adolescents (376 girls), aged 7–17 years. Cardiorespiratory fitness and strength were measured with the test battery FITNESSGRAM; carotid intima-media thickness, arterial compliance, elastic modulus, stiffness index β, and pulse wave velocity β were assessed by B- and M-mode ultrasound at the common carotid artery. In bivariate correlation, cardiorespiratory fitness was significantly associated with all cardiovascular parameters and was an independent predictor in multivariate regression analysis. No significant associations were obtained for muscular strength. In a one-way variance analysis, very fit boys and girls (58 boys and 74 girls>80th percentile for cardiorespiratory fitness) had significantly decreased stiffness parameters (expressed in standard deviation scores) compared with low fit subjects (71 boys and 77 girls<20th percentile for cardiorespiratory fitness): elastic modulus −0.16±1.02 versus 0.19±1.17, p=0.009; stiffness index β −0.15±1.08 versus 0.16±1.1, p=0.03; and pulse wave velocity β −0.19±1.02 versus 0.19±1.14, p=0.005. Cardiorespiratory fitness was associated with healthier arteries in children and adolescents. Comparison of very fit with unfit subjects revealed better distensibility parameters in very fit boys and girls.
Role of anxiety and depression in adolescents with chest pain referred to a cardiology clinic
- Zahra Khairandish, Leila Jamali, Saeedeh Haghbin
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- Published online by Cambridge University Press:
- 16 March 2016, pp. 125-130
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Background
We carried out this study in order to evaluate the causes of chest pain in teenagers and the role of anxiety and depression in this age group compared with the normal population.
MethodsIn this prospective case–control study, all patients aged 11–18 years with chest pain and no history of trauma and referred to a paediatric cardiology clinic from March, 2009–April, 2010 were selected. A chest pain protocol including a detailed history, full physical examination, required blood tests, electrocardiography, and echocardiography was performed for all. The presence of depression and anxiety and their severity were assessed by Beck questionnaires. The patients were compared with age- and sex-matched, randomly selected healthy controls.
ResultsIn total, 194 patients with a mean age of 14±2 years were selected. The most frequent presentation was idiopathic chest pain (43.3%), followed by the psychological group (29.9%). These groups had no abnormal points in history, physical, and para-clinical tests. Moderate-to-severe depression was found in 45.9% in the patients group, compared with 17.6% of controls, which was statistically significant (p=0.016). Moreover, anxiety was detected in 67.5% of patients versus 15.4% in controls, which is a statistically significant difference (p=0.009). Cardiac chest pain with 9.27% was the most common type of organic causes.
ConclusionChest pain during teenage is more prevalent, but not risky. Undergoing a detailed history and full physical examination can help diagnose the causes in the majority of cases. Given the prevalence of a psychological group as well as role of anxiety and depression in most patients, referring to a psychiatrist is suggested.
Interstage somatic growth in children with hypoplastic left heart syndrome after initial palliation with the hybrid procedure
- Fiona T. S. Chan, Hannah R. Bellsham-Revell, Hannah Duggan, John M. Simpson, Tony Hulse, Aaron J. Bell
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- Published online by Cambridge University Press:
- 08 April 2016, pp. 131-138
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Introduction
The hybrid procedure is one mode of initial palliation for hypoplastic left heart syndrome. Subsequently, patients proceed with either the “three-stage” pathway – comprehensive second stage followed by Fontan completion – or the “four-stage” pathway – Norwood procedure, hemi-Fontan, or Fontan completion. In this study, we describe somatic growth patterns observed in the hybrid groups and a comparison primary Norwood group.
MethodsA retrospective analysis of patients who have undergone hybrid procedure and Fontan completion was performed. Weight-for-age and height-for-age z-scores were recorded at each operation.
ResultsWe identified 13 hybrid patients – eight in the three-stage pathway and five in the four-stage pathway – and 49 Norwood patients. Weight: three stage: weight decreased from hybrid procedure to comprehensive second stage (−0.4±1.3 versus −2.3±1.4, p<0.01) and then increased to Fontan completion (−0.4±1.5 versus −0.6±1.4, p<0.01); four stage: weight decreased from hybrid procedure to Norwood (−2.0±1.4 versus −3.3±0.9, p=0.06), then stabilised to hemi-Fontan. Weight increased from hemi-Fontan to Fontan completion (−2.7±0.6 versus −1.0±0.7, p=0.01); primary Norwood group: weight decreased from Norwood to hemi-Fontan (p<0.001) and then increased to Fontan completion (p<0.001). Height: height declined from hybrid procedure to Fontan completion in the three-stage group. In the four-stage group, height decreased from hybrid to hemi-Fontan, and then increased to Fontan completion. The Norwood group decreased in height from Norwood to hemi-Fontan, followed by an increase to Fontan completion.
ConclusionIn this study, we show that patients undergoing the hybrid procedure have poor weight gain before superior cavopulmonary connection, before returning to baseline by Fontan completion. This study identifies key periods to target poor somatic growth, a risk factor of morbidity and worse neurodevelopmental outcomes.
Effects of milk flow on the physiological and behavioural responses to feeding in an infant with hypoplastic left heart syndrome
- Britt F. Pados, Suzanne M. Thoyre, Hayley H. Estrem, Jinhee Park, George J. Knafl, Brant Nix
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- Published online by Cambridge University Press:
- 16 March 2016, pp. 139-153
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Infants with hypoplastic left heart syndrome often experience difficulty with oral feeding, which contributes to growth failure, morbidity, and mortality. In response to feeding difficulty, clinicians often change the bottle nipple, and thus milk flow rate. Slow-flow nipples have been found to reduce the stress of feeding in other fragile infants, but no research has evaluated the responses of infants with hypoplastic left heart syndrome to alterations in milk flow. The purpose of this study was to evaluate the physiological and behavioural responses of an infant with hypoplastic left heart syndrome to bottle feeding with either a slow-flow (Dr. Brown’s Preemie) or a standard-flow (Dr. Brown’s Level 2) nipple. A single infant was studied for three feedings: two slow-flow and one standard-flow. Oral feeding, whether with a slow-flow or a standard-flow nipple, was distressing for this infant. During slow-flow feeding, she experienced more coughing events, whereas during standard-flow she experienced more gagging. Disengagement and compelling disorganisation were most common during feeding 3, that is slow-flow, which occurred 2 days after surgical placement of a gastrostomy tube. Clinically significant changes in heart rate, oxygen saturation, and respiratory rate were seen during all feedings. Heart rate was higher during standard-flow and respiratory rate was higher during slow-flow. Further research is needed to examine the responses of infants with hypoplastic left heart syndrome to oral feeding and to identify strategies that will support these fragile infants as they learn to feed. Future research should evaluate an even slower-flow nipple along with additional supportive feeding strategies.
Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation
- Lucia Manuri, Stefano Morelli, Salvatore Agati, Michele B. Saitta, Lilia Oreto, Giuseppe Mandraffino, Enrico Iannace, Fiore S. Iorio, Paolo Guccione
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- Published online by Cambridge University Press:
- 17 March 2016, pp. 154-160
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Background
The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure.
MethodsWe reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol.
ResultsThe median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1–70 days), and the median hospital length of stay was 16 days (6–70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events.
ConclusionsOur experience indicates that the combination of an “early hybrid approach”, systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis.
Correlating the morphological features of tetralogy of Fallot and the Eisenmenger malformation
- Angelo Restivo, Robert H. Anderson, Raffaella Carletti, Cira R. T. di Gioia
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- Published online by Cambridge University Press:
- 08 April 2016, pp. 161-172
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We studied a series of 43 autopsied cases of tetralogy of Fallot, assessing the mode of insertion of the outlet septum relative to the limbs of the septomarginal trabeculation, and compared the findings in retrospective fashion with our previous observations of a group of hearts with the so-called Eisenmenger malformation. In the majority of hearts with tetralogy of Fallot, the outlet septum inserted frontally relative to the septomarginal trabeculation, but in a minority of cases the outlet septum inserted in lateral fashion, as had been observed in all our hearts studied with the Eisenmenger malformation. The different modes of insertion were found to correlate, first, with the axis of anatomical aortic rightward rotation, coincident with the commissure between the right coronary and the left coronary leaflets of the aortic valve. The different modes of insertion of the outlet septum also correlated with the level of attachment of the arterial valvar leaflets on its subpulmonary and subaortic surfaces; concomitantly, correlation was found between the length of the subpulmonary infundibulum and the length of the muscular outlet septum itself. In the majority of hearts showing tetralogy of Fallot, the elongated infundibulum was also uniformly narrow, but in a minority the infundibulum was well expanded, obstructed only at its mouth, but widening at the valvar level. In all the hearts with the Eisenmenger malformation, in contrast, the unobstructed infundibulum was well expanded. The morphological findings of the present study show unequivocally that tetralogy of Fallot and Eisenmenger malformation are two phenotypically different congenital cardiac anomalies.
Images in Congenital Cardiac Disease
Cardiogenic unilateral pulmonary oedema in an infant with severe residual mitral regurgitation
- Anna Joong, Wyman W. Lai, Anne Ferris
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- Published online by Cambridge University Press:
- 17 October 2016, pp. 173-175
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An infant with residual severe mitral regurgitation following mitral commissurotomy developed cardiogenic unilateral pulmonary oedema and subsegmental atelectasis that resolved with mechanical mitral valve replacement.