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Intracardiac masses in the young occur in some conditions that are prevalent in Africa. Although usually non-malignant, they may present with refractory heart failure and other complications that can be fatal. In the majority of cases, the aetiologic differentiation can be achieved by careful history, physical examination, basic laboratory tests, and transthoracic echocardiography. We report three cases in young Africans and discuss the aetiology, clinical presentation, diagnosis, management, and outcome of selected conditions in resource-limited settings.
To evaluate the efficacy of flecainide and digoxin combination in foetal supraventricular tachycardia.
This study was carried out in a tertiary referral centre.
We conducted a retrospective review of 29 patients diagnosed with supraventricular foetal tachycardia between 2001 and 2009. Mode of presentation, foetal cardiac function, maternal anti-arrhythmic serum levels, drug tolerance, and maternal electrocardiogram recordings were assessed. The postnatal outcome of each infant was also evaluated for tachycardia recurrence.
In all, 27 foetuses were treated with digoxin and flecainide combination, and two foetuses were delivered without any treatment. Of the 27 foetuses, seven had atrial flutter and the remaining 22 had atrioventricular re-entry tachycardia. There were eight foetuses with hydrops (27%), of whom three had atrial flutter and five had atrioventricular re-entry tachycardia; 26 foetuses (96%) responded to flecainide and digoxin combination, with restoration of sinus rhythm in 22 (81.4%) and rate control in the other four. In one severely hydropic foetus, there was no response to treatment. In all, 26 treated infants were delivered alive, but one pregnancy was terminated for non-cardiac causes when the foetus was in sinus rhythm. There was no intrauterine death due to tachycardia. Although there were minor side effects to anti-arrhythmic medications, none of the pregnant women developed proarrhythmia.
Flecainide and digoxin combination treatment offers a safe and effective treatment for foetal supraventricular tachycardia with fast restoration of sinus rhythm.
Systemic right ventricular systolic dysfunction is common late after atrial switch surgery for transposition of the great arteries. Total isovolumic time is the time that the ventricle is neither ejecting nor filling and is calculated without relying on geometric assumptions. We assessed resting total isovolumic time in this population and its relationship to exercise capacity.
A total of 40 adult patients with transposition of the great arteries after atrial switch – and 10 healthy controls – underwent transthoracic echocardiography and cardiopulmonary exercise testing from January, 2006 to January, 2009. Resting total isovolumic time was measured in seconds per minute: 60 minus total ejection time plus total filling time.
The mean age was 31.6 plus or minus 7.6 years, and 38.0% were men. There were 16 patients (40%) who had more than or equal to moderate systolic dysfunction of the right ventricle. Intra- and inter-observer agreement was good for total isovolumic time, which was significantly prolonged in patients compared with controls (12.0 plus or minus 3.9 seconds per minute versus 6.0 plus or minus 1.8 seconds per minute, p-value less than 0.001) and correlated significantly with peak oxygen consumption (r equals minus 0.63, p-value less than 0.001). The correlation strengthened (r equals minus 0.73, p-value less than 0.001) after excluding seven patients with exercise-induced cyanosis. No relationship was found between exercise capacity and right ventricular ejection fraction or long-axis amplitude.
Resting isovolumic time is prolonged after atrial switch for patients with transposition of the great arteries. It is highly reproducible and relates well to exercise capacity.
Kawasaki disease is the leading cause of acquired coronary artery disease in young children. There is a lack of data on Kawasaki disease and its effect on coronary arteries in Jordan and other developing countries. We report clinical and demographic data of Kawasaki disease in Jordan from a single institution, with emphasis on cardiac involvement and short to intermediate follow-up. Review of the medical records of 34 patients with Kawasaki disease from 1997 to 2010 was done for clinical and demographic variables. Echocardiographic and angiographic images were reviewed for patients at presentation and follow-up. The median age at presentation was 19 months, ranging from 2 months to 8 years, with a male to female ratio of 3.9:1. In all, 12 patients (35%) had incomplete Kawasaki disease. There was a high incidence of coronary artery involvement (41%), where 20.5% had aneurysms and 20.5% had ectasia without aneurysm. Most coronary aneurysms were present at the time of diagnosis. The only independent variable for prediction of coronary involvement was age, with an odds ratio of 0.63 per year (95% confidence interval 0.41–0.95).
We performed a retrospective analysis of patients with pulmonary arterial hypertension receiving inhaled iloprost in a single centre to evaluate long-term tolerability, safety, and efficacy of chronic inhaled iloprost therapy in children.
A total of 20 patients with either idiopathic or associated pulmonary arterial hypertension were treated with iloprost between April, 2003 and January, 2010. The median age and weight of the patients were 3.8 years – ranging from 4 months to 19 years – and 12.3 kilograms – ranging from 4 to 73 kilograms – respectively. Pulmonary arterial hypertension was idiopathic or hereditary in eight patients (40%) and associated with congenital cardiac disease in 12 patients (60%).
Of the 20 patients, 15 had combined therapy – 12 patients with two and three patients with three different classes of drugs. In all, six patients died during follow-up. The median follow-up time was 18 months, ranging from 6 to 74 months. The 6-minute walking test was performed in 7 out of 20 patients at baseline and on follow-up. The median 6-minute walking test increased from 420 to 490 metres after iloprost therapy (p = 0.028). After initiation of iloprost therapy, one patient complained of headache and another had a rash around his mouth, none necessitating discontinuation of therapy. Overall compliance with inhaled iloprost was good.
Pulmonary hypertension is associated with significant morbidity and mortality. Careful assessment of each patient and timely combination of specific vasodilator therapy is needed to improve clinical outcomes. This study suggests that inhaled iloprost, with or without concomitant endotelin receptor antagonist and/or phosphodiesterase inhibitor, is safe and efficacious for treatment of pulmonary arterial hypertension in children.
Familial Mediterranean fever is a hereditary disease characterised by recurrent and self-terminated attacks of fever and polyserositis. An earlier study found that adult patients of Familial Mediterranean fever had an abnormally longer QT dispersion and corrected QT dispersion, markers for ventricular arrhythmogenicity. QT dispersion is a simple non-invasive arrhythmogenic marker that can be used to assess homogeneity of cardiac repolarisation; however, it has not been studied in children with Familial Mediterranean fever before. The aim of this study was to assess QT dispersion and corrected QT dispersion, and their relationship with systolic and diastolic function of the left ventricle in a group of children with Familial Mediterranean fever. We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum QT, minimum QT, QT dispersion, corrected QT, maximum corrected QT, minimum corrected QT, and corrected QT dispersion intervals were measured from standard 12-lead electrocardiography. No statistically significant differences were found between the groups in QT dispersion, corrected QT dispersion, and systolic–diastolic function of the left ventricle parameters. During the 12 months of follow-up, no ventricular arrhythmias were documented in either group.
The objective of this study was to detect structural and functional changes in the left and right ventricles in obese Egyptian adolescents.
Methods and results
Anthropometric and echocardiographic parameters, including tissue Doppler imaging, were obtained from 70 obese adolescents with average body mass index of 34 plus or minus 3.8 and compared with 50 age- and sex-matched controls, with a body mass index of 21.6 plus or minus 1.9. Cardiac dimensions, stroke volume, left ventricular and right ventricular systolic and diastolic functions were evaluated. The obese group had a higher end-diastolic septal and posterior wall thickness and left ventricular mass index than the non-obese group. Body mass index, mid-arm and hip circumference values showed significant correlations with these echocardiographic variables. Systolic and diastolic functions of the left ventricle were normal in both groups, although stroke volume was high in the obese group. The right ventricle tissue Doppler parameters were similar in both groups. However, the S wave of the septal/lateral tricuspid valve annulus was reduced in the obese group, but not to the level reflecting systolic dysfunction. This was inversely correlated with hip, waist, and mid-arm circumference. Stepwise multiple regression analysis showed that the mid-arm and hip circumference followed by the body mass index are significant predictors of these early cardiac abnormalities.
Left ventricular hypertrophy is present in obese children, although both systolic and diastolic functions are normal. Tissue Doppler imaging revealed a minor, but still significant, reduction in the right ventricular systolic function. Mid-arm and hip circumference are predictors of left ventricular hypertrophy.
Little is known about the quality of life, health, family, education, and employment status among adult men with repaired tetralogy of Fallot.
Material and methods
A total of 68 men who underwent repair of tetralogy of Fallot between 1971 and 1991 were studied. Fifty-three patients answered the SF-36 health survey and additional questions regarding offspring, education, and employment status. The men with repaired tetralogy of Fallot were compared with 32 healthy men and 40 women who also underwent repair of tetralogy of Fallot in the same period.
The patients scored lower than healthy men in the SF-36 categories physical functioning, general health, and physical component summary. There were no statistically significant differences in the scores from male and female patients except a lower score in bodily pain among women. Educational level for men operated for tetralogy of Fallot was similar to the general male population, whereas fewer were employed and more were retired, undergoing rehabilitation or receiving social benefits. The reproduction rate was lower compared with the general population (0.65 versus 1.02 children per man) but relatively higher than the rate among women with tetralogy of Fallot (0.88 versus 1.84 children per woman). The risk of having a child with congenital heart disease was 8.3%.
Men operated for tetralogy of Fallot have good quality of life and educational status. They start a family, although their reproduction rate is two-thirds that of the general population. The risk of having a child with congenital heart disease is higher compared with the background population. The overall quality of life is similar for men and women operated for tetralogy of Fallot.
Propranolol was recently discovered to be an effective treatment for infantile haemangiomas, and varying doses and monitoring regimens have been proposed. Adverse events, although uncommon, have been reported.
Materials and methods
This was a retrospective chart review of infants with haemangiomas who were started on propranolol at a dose of 3 milligrams per kilogram per day on an outpatient basis. After a baseline cardiac evaluation including an electrocardiogram and an echocardiogram, treatment was initiated during 6 hours of observation.
A total of 15 patients were identified; however, only 13 returned for at least one follow-up visit. This cohort was followed up for a median of 2.8 months with a range from 0.2 to 10.0. No hypotension, hypoglycaemia, bronchospasm, or clinically significant bradycardia occurred during treatment. All patients had clinical improvement of their haemangiomas.
This study suggests that initiating treatment during outpatient observation may be a reasonable alternative to inpatient admission. In addition, expensive testing may not be necessary during pre-treatment screening when the physical examination is normal.
The association between long QT interval and sudden infant death syndrome has been clearly established. Several studies have been conducted to determine the evolution of the QT interval in childhood from birth, but only in full-term newborns. However, data on the QT interval in pre-term infants are extremely scarce. The objective was to describe the development of the QT interval in premature infants.
Material and methods
In a prospective monocentric study in a neonatal intensive care unit, pre-term newborns born before 37 weeks of gestation without congenital heart disease, family history of long QT, unstable haemodynamic status, or administration of drugs inducing QT interval prolongation were included with parental consent. An electrocardiogram was recorded in similar conditions weekly until discharge in each child. The corrected QT was calculated with Bazett's formula.
In all, 309 echocardiograms were recorded in 87 children, with gestational age ranging from 24–36 weeks. QT first increased after birth in very premature infants – less than 30 weeks of gestation – and then started to decrease, whereas it only decreased in more mature infants. When plotted against postmenstrual age, QT first increased, and then decreased after 32 weeks.
Our data suggest that the QT interval varies with postmenstrual age in very premature infants, reaching a peak at 32 weeks. These developmental changes may induce specific vulnerability to QT-lengthening medications in premature infants. This study underlines the need for specific pharmacological studies in this population.
To evaluate the effect of a continuous infusion of basic fibroblast growth factor on the adaptive potential of the right ventricular myocardium after 30 days of mechanically induced overload in rats.
Materials and methods
We banded the pulmonary trunk, so as to increase the systolic workload of the right ventricle, in six Lewis/HanHsd rats at the age of 11 weeks, using six adult rats as controls. The six adult rats were also banded and received an additional continuous infusion of basic fibroblastic growth factor, using six rats with a continuous infusion of basic fibroblastic growth factor only as controls. We analysed the functional adaptation and structural changes of the right ventricular myocardium, blood vessels, and interstitial tissue 30 days after the increased afterload.
The pulmonary artery banding induced an increase in the right ventricular free wall thickness of banded rats when compared with controls, which was mainly justified by an increase in cardiomyocyte area and in the percentage of extracellular fibrosis. The infusion of basic fibroblastic growth factor promotes a more extensive capillary network in banded rats (p < 0.001), which modulates the compensatory response of the right ventricle, promoting the hypertrophy of contractile elements and limiting the areas in which fibrosis develops (p < 0.001).
The subcutaneous infusion with osmotic pumps was a valid and reproducible method of delivering basic fibroblast growth factor to heart tissue. This infusion contributed to better preserve the right ventricular capillary network, hampering the development of interstitial fibrosis.
To determine the gross motor skills of school-aged children after the Fontan procedure and compare the locomotor and object control skills with normative data.
This study followed a cross-sectional design.
This study was based on hospital outpatient visit, with accelerometry conducted at home.
This study included 55 patients, including 22 girls in the age group of 6–10 years, 5.1 years after Fontan.
Main outcome measures
Test of Gross Motor Development – Version 2, daily activity by accelerometer, medical history review, child and parent perceptions of activity.
Being involved in active team sports increased locomotor percentile score by 10.3 points (CI: 4.4, 16.1). Preference for weekend outdoor activities (6.9, CI: 2.0, 11.8), performing at least 30 minutes of moderate-to-vigorous physical activity daily (24.5, CI: 7.3, 41.8), and reporting that parents seldom criticise the child's physical activity (21.8, CI: 8.9, 34.8) were also associated with higher locomotor percentile scores (p < 0.01). Object control percentile scores were higher (p < 0.03) with involvement in formal instruction (5.9, CI: 1.1, 10.6) and being restricted to “activities within comfortable limits” (27.6, CI: 7.7, 47.5). Older chronological age (r = 0.28), a more complicated medical history (r = 0.36), and older age at Fontan (r = 0.28) were associated with greater skill delay (p < 0.04).
Children after Fontan attain basic motor skills at a later age than their peers, and deficits continue for more complex skills as age increases, suggesting a need for longitudinal monitoring of gross motor skill development through the elementary school years. Future research might investigate whether a gross motor skill rehabilitation programme can provide these children with the motor skills needed to successfully participate in a physically active lifestyle with peers.
Although hypertension has been shown to be one of the most important risk factors for atherosclerosis, data about the presence of subclinical atherosclerosis in normotensive offspring with parental history of hypertension are scarce. Accordingly, the current study was designated to evaluate flow-mediated dilatation and aortic stiffness, which are early signs of atherosclerosis in young subjects with parental history of hypertension.
A total of 102 healthy, non-obese subjects in the age group of 18–22 years were included in this study and divided into two groups. The first group included 70 offspring of hypertensive parents and the second group included 70 offspring of normotensive parents as controls. In all subjects, endothelium-dependent and endothelium-independent vasodilatation of the brachial artery and aortic elastic parameters were investigated using high-resolution Doppler echocardiography.
Offspring of hypertensive parents demonstrated higher values of aortic stiffness (7.1 plus or minus 1.88 and 6.42 plus or minus 1.56, respectively) but lower distensibility (9.47 plus or minus 1.33 and 11.8 plus or minus 3.36 square centimetres per dyne per 106) and flow-mediated dilatation (4.57 plus or minus 1.3 versus 6.34 plus or minus 0.83 percent, p equals 0.0001, respectively) than offspring of hypertensive parents.
We observed blunted endothelium-dependent dilatation and aortic stiffness in offspring of hypertensive parents compared with offspring of hypertensive parents. This is evident in the absence of overt hypertension and other diseases, suggesting that parental history of hypertension is a risk for subclinical atherosclerosis and it may contribute to the progression to hypertension and overt atherosclerosis in later life.
This case report describes a unique form of a left ventricular diverticulum in a 17-year-old patient. Echocardiography, angiography, and magnetic resonance imaging including virtual endoscopy complete a detailed picture of the size and texture of a diverticulum, as well as the localisation of the two connections into the left ventricle.
A 2-kilogram child had a pacemaker implanted by a subxyphoid approach with the generator located under the rectus sheath. Days later, the battery eroded the abdominal wall and the peritoneum. The whole system was removed and a new one was implanted inside the pericardium on an emergent basis.
Kawasaki disease constitutes an acute febrile vasculitis of unknown aetiology. It is considered the most common cause of acquired cardiac failure in children. Although standard treatment comprises intravenous immunoglobulin and aspirin, some children exhibit refractory disease, necessitating the use of alternative therapies such as corticosteroids and anti-tumour necrosis factor-alpha. For these cases, few controlled data are available. This report focuses on an extremely refractory classical Kawasaki disease with coronary artery aneurysms and ongoing inflammation. We discuss the therapeutic approaches and the potential pitfalls undertaken, which led to an unfavourable clinical outcome.
Tall stature is a common feature of both Marfan syndrome and XYY syndrome. Differential diagnosis between these entities has important prognostic implications. We report the case of a 21-year-old young man with a previously known diagnosis of XYY syndrome, in whom the identification of a fibrilin-1 mutation was determinant to establish an appropriate diagnosis, medical follow-up, and genetic counselling.
Anomalies of the innominate vein are uncommon in congenital cardiac disease. We report a case of duplicate innominate veins forming a vascular ring encircling the ascending aorta. We postulate that this vascular ring represents the failure of both a dorsal and ventral precardinal anastomosis to regress.
Congenital aortocaval fistula is a very rare anomaly. Clinically, it resembles conditions that cause left-to-right shunt of blood. We report a case of such anomaly in combination with a secundum atrial septal defect in a 13-month-old girl who presented with failure to thrive and exertional respiratory symptoms. The aortocaval fistula was occluded percutaneously using an Amplatzer® Duct Occluder.