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Endocardial fibroelastosis is not a disease but a reaction of the endocardium. I review the history of the term with emphasis on the gradual understanding of the many causes of this reaction. I include a comprehensive list of diseases or other cardiac stresses that authors have reported in association, and I try to explain the mechanism of the reaction. Although endocardial fibroelastosis is rare today, I issue a warning of a possible epidemic recrudescence of some of the associated diseases. My hope is for nosologic purity, therefore that outworn but surviving concepts will be firmly rejected.
Few paediatric cardiologists know of Maude Abbott. Yet before Helen Taussig, no one contributed more to founding the speciality than Maude Abbott. She achieved international fame as the early 20th century expert on cardiac malformations. We summarise here her life and contributions, indicating how she is more than justified in being inducted to the Hall of Fame.
The aim of this study was to investigate the relations between the P-wave dispersion and diastolic functions in type 1 diabetic children.
A total of 33 diabetic patients without any cardiovascular disease, with a mean age of 12.3 plus or minus 4.2 years, and 29 healthy controls, with a mean age of 10.4 plus or minus 3.9 years were enrolled for this study. Left and right ventricular functions were assessed by using standard pulsed-wave Doppler echocardiography. P-wave dispersion was calculated by measuring minimum and maximum P-wave duration values on the surface electrocardiogram.
For the diabetic patients, P-wave maximum duration and dispersion was found to be significantly increased compared with healthy controls. Likewise, mitral A velocity and A velocity time integral was significantly increased while the isovolumic contraction time was significantly higher in the diabetics. In tricuspid valve measurements, however, A velocity time integral was found to be significantly higher, whereas the deceleration time was significantly lower in the diabetics. No relation was found between the left ventricle diastolic functions and duration of diabetes, HbA1c levels and P-wave dispersion in the diabetic children. No correlation was found between the diastolic functions and P-wave minimum, maximum duration, and dispersion for all the participants.
In type-1 diabetic children, the diastolic functions of both the ventricles were observed to be affected negatively together. Diabetes might be causing the prolongation of P-wave dispersion, but there was no relationship between the diastolic functions and P-wave dispersion in the diabetic children.
Thromboembolic events are a serious complication occurring in critically ill children admitted to the cardiac intensive care unit. Although enoxaparin is one of the current anticoagulants of choice, dosages in children are extrapolated from adult guidelines. Recent data suggest that this population may need a higher dose than what is currently recommended to achieve target anti-factor Xa levels. The purpose of this study was to evaluate whether children less than 2 years old admitted to the cardiac intensive care unit require a higher enoxaparin dose than that currently recommended to achieve target anti-factor Xa levels.
Retrospective chart review including patients who received enoxaparin for the treatment or prophylaxis of venous thrombosis between January, 2005 and October, 2007. Patients were classified as younger and older as well as prophylactic and therapeutic on the basis of age and enoxaparin dose, respectively. Younger patients were those 2 month old or less and older patients were those older than 2 months of age.
A total of 31 patients were identified; 13 (42%) were 2 months or younger and 25 (81%) were postoperative patients. Ten (32%) received prophylactic and 21 (68%) received therapeutic enoxaparin doses. To achieve optimal anti-factor Xa levels, enoxaparin dose was increased in all groups and reached statistical significance in all patients except those older than 2 months who received prophylactic enoxaparin. An average of 2.8 dosage adjustments was needed. No bleeding complications were reported.
Young children, infants, and neonates admitted to the cardiac intensive care unit required a significantly higher enoxaparin dose than that currently recommended to achieve target anti-factor Xa levels.
Usually the literature results for device-closure of patent oval foramen concern a single type of device or different devices implanted without anatomical preferences. We propose a strategy of device type and selection based on intra-cardiac echocardiography measurements of inter-atrial septum characteristics.
We prospectively enrolled 100 consecutive patients with amean age of 43 plus or minus 15.5 years, 68 females, who had been referred to our centre for catheter-based closure of inter-atrial shunts over a 48-month period. On the basis of intra-cardiac echocardiography findings the operators selected the Amplatzer Occluder family (AGA Medical Corporation) or the Premere Closure System (St Jude Medical Inc.). Determinants of the selection process were presence and extension of atrial septal aneurysm, tunnel length, rims length, and thickness, presence of additional fenestrations.
According on intra-cardiac echocardiography study, 26 patients have a long channel patent oval foramen, 44 patients had a large atrial septal aneurysm (more than four RL), 24 patients had a moderate atrial septal aneurysm (more than two RL but less than four right-to-left), and six patients had hypertrophic rims. Thus, the Amplatzer PFO Occluder was selected in 24 patients, the Amplatzer ASD Cribriform Occluder in 44 patients, and the Premere device in 32 cases. No aortic erosions, device thrombosis, or recurrent ischaemic cerebral events were observed. Pre-discharge and follow-up occlusion rates were 91% and 96%, respectively.
Our study suggested that such strategy driven from identification and measurements of the right atrium and inter-atrial septum components resulted in low complications and high-success rates, mandatory conditions when facing with otherwise healthy subjects, such as the patients with patent oval foramen.
The arterial switch operation is the corrective operation for transposition of the great arteries, defined as the combination of concordant atrioventricular and discordant ventriculo–arterial connections, but there have been concerns about silent subendocardial ischaemia on exercise and coronary artery growth. The arterial switch divides the majority of the sympathetic nerves entering the heart; we have studied the effects of coronary flow and sensitivity to catecholamine stimulation in an animal model.
A total of 10 piglets were operated on cardiopulmonary bypass with section and resuturing of aortic trunk, pulmonary artery and both coronary arteries, with 13 sham-operated controls. After 5–7 weeks of recovery, seven simulated switch survivors and 13 controls were studied.
Basal heart rate was significantly higher in switch piglets: in vivo mean (standard deviation) 112 (12) versus sham 100 (10) beats per minute, (p = 0.042); in vitro (Langendorff preparation): 89 (9) versus sham 73 (8) beats per minute (p = 0.0056). In vivo maximal heart rate in response to epinephrine was increased in switch piglets, 209 (13) versus 190 (17) beats per minute (p = 0.044). In vitro dose–response curves to norepinephrine were shifted leftward and upwards (p = 0.0014), with an 80% increase in heart rate induced by 0.095 (0.053) norepinephrine micromole per litre perfusate in switch hearts versus 0.180 (0.035) norepinephrine micromole per litre (p = 0.023). Increase in coronary flow on norepinephrine stimulation and maximal coronary flow were significantly reduced in switch hearts: 0.3 (0.2) versus 0.8 (0.4) millilitre per gram heart weight (p = 0.045) and 2.5 (0.4) versus 3.1 (0.4) millilitre per gram heart (p = 0.030), respectively.
A combination of increased intrinsic heart rate, increased sensitivity to chronotropic actions of norepinephrine, and a decreased maximal coronary flow creates potential for a mismatch between perfusion and energy demands.
This study was undertaken to review the spectrum and surgical outcome of adolescents and adults with congenitally malformed hearts from January, 1993 to December, 2008. The lack of data on this emerging problem from the West African sub-region prompted this report.
Patients and Method
This retrospective study is based on 135 adolescents and adults with congenitally malformed hearts. A review of their case notes and operative records was carried out and results analysed.
Selected patients made up 23% of all congenital cardiac surgeries performed at our institution in the same period. A total of 23 patients (17%) were non-Ghanaian West Africans. There was a female preponderance of 53.3%. The ages ranged from 16 to 70 years (mean 28.6 plus or minus 10.3 years). The mean follow-up was 7.5 plus or minus 4.4 years. Patients were functionally classified (New York Heart Association) as class I (23%), II (58%), and III (19%). In 14 (10.4%) patients, the defects were discovered incidentally. Ventricular septal defects, oval fossa type atrial septal defects, Fallot’s tetralogy, and patent arterial duct together accounted for 77.8% of the cases. Surgical correction was undertaken in 117 (86.7%) patients; the remainder had palliative procedures. There were six (4.3%) reoperations. The functional class improved to class I or II in 95% of patients within the first postoperative year. The overall hospital mortality was 3% with two late deaths (1.5%).
The study demonstrates the feasibility of surgery for adolescents and adults with congenitally malformed hearts in the sub-region with a good outcome. Majority (77.8%) of patients present with less complex lesions.
Neurocardiogenic syncope is a common disorder, which is considered as a benign condition. However, sudden loss of conscience and muscle tone causes anxiety among the family members due to its similarity to sudden death. Autonomic nervous system dysregulation is thought to be responsible in the aetiology. Heart rate variability is used for assessment of autonomic nervous system.
We evaluated 24 children between 6 and 18 years (mean plus or minus standard deviation is equal to 12.5 plus or minus 3.28, with neurocardiogenic syncope and 10 healthy controls, mean plus or minus standard deviation is equal to 12.48 plus or minus 3.27) by using 24 hour Holter monitorisation and head-up tilt test. Heart rate variability analysis was performed using the Holter recordings obtained both during head-up tilt test and throughout the day.
Our results revealed that, there is no significant difference between the study and the control groups in terms of the mean heart rate and all indices of the heart rate variability (p > 0.05). However, during the first 5 minutes of the head-up tilt test, standard deviation of all RR intervals and root mean square of successive differences were significantly lower in the syncope group compared with the control group, 42.17 plus or minus 12.56 versus 60.10 plus or minus 33.10 and 21.26 plus or minus 8.87 versus 36.80 plus or minus 31.03; p-values 0.02 and 0.03, respectively.
In conclusion; autonomic functions in children with neurocardiogenic syncope are similar to healthy children. However, sympathetic hyperactivation occurs during the early phase of orthostatic stress in children with neurocardiogenic syncope comparing to healthy controls. Parasympathetic innervation is not sufficient in compensation of this sympathetic hyperactivation. Management strategy in neurocardiogenic syncope should be based on these pathophysiologic mechanisms.
Mitral valvar prolapse is the most common anomaly of the mitral valve apparatus throughout childhood. Fibrillin is one of the structural components of the elastin-associated microfibrils found in the mitral valve. A case-controlled study has performed to investigate the relationship between fibrillin 1 gene intron 56 polymorphism and risk of mitral valvar prolapse in Turkish children.
Patients and methods
A total of 77 patients with mitral valvar prolapse diagnosed by clinical evaluation and echocardiography and 89 normal children of same age and sex were studied. The fibrillin-1 gene intron 56 polymorphism was identified by the polymerase chain reaction-based restriction analysis.
There was a significant difference in the distribution of fibrillin-1 gene intron 56 genotypes (p = 0.0001) and allelic frequency (p = 0.0001) between the cases and the controls.
Patients with mitral valvar prolapse have higher frequencies of fibrillin-1 gene intron 56 GC genotypes. Healthy children have higher frequencies of fibrillin-1 gene intron 56 CC genotypes. We speculate that the higher frequency of fibrillin-1 gene intron 56 G-allele increases the risk of mitral valvar prolapse.
Parents of children with congenital cardiac disease suffer from psychological stress and financial burdens. These costs have not yet been quantified.
Materials and methods
In cooperation with paediatricians, social workers, and parents, a questionnaire was devised to calculate direct non-medical and indirect costs. Direct non-medical costs include all costs not directly related to medical services such as transportation. Indirect costs include lost productivity measured in lost income from wages. Parents were retrospectively queried on costs and refunds incurred during the child’s first and sixth year of life. The questionnaire was sent out to 198 families with children born between 1980 and 2000. Costs were adjusted for inflation to the year 2006. Children were stratified into five groups according to the severity of their current health status.
Fifty-four families responded and could be included into the analysis (27.7%). Depending on severity, total direct non-medical and indirect costs in the first year of life ranged between an average of €1654 in children with no or mild (remaining) cardiac defects and an average €2881 in children with clinically significant (residual/remaining) findings. Mean expenses in the sixth year of life were as low as €562 (no or mild (remaining) cardiac defects) and as high as €5213 (potentially life-threatening findings). At both points in time, the highest costs were lost income and transportation; and day care/ babysitting for siblings was third.
Families of children with congenital cardiac disease and major sequelae face direct non-medical and indirect costs adding up to €3000 per year on average. We should consider compensating families from low socioeconomic backgrounds to minimise under-use of non-medical services of assistance for their children.
Many studies unequivocally indicate that air pollution is directly linked to the adverse cardiovascular outcomes in the general population. No data are currently available on cardiovascular effects of exposure to trafficked roads in healthy children. Distance of the residence to a major road has been shown to be a useful proxy for long-term traffic exposure and seem to be more consistently associated with atherosclerosis than particulate matter2.5. The aim of this study was to investigate a possible association between the distance to a major road and carotid arterial subclinical markers of atherosclerosis in a group of children in Italy.
The participants consisted of 52 healthy children living in a small town of the Amalphitan Coast with only one highly trafficked road. All children underwent an ultrasound carotid arterial examination.
A statistically significant difference was found in carotid arterial stiffness between children living closer to the main street and other children, both those living between 330 and 730 metres from the main street and those living more than 750 metres from the main street. No significant differences were detectable in carotid arterial thickness and arterial blood pressure among the three groups of children.
This study provides evidence in support of an association of exposure to air pollution with early atherosclerotic markers in healthy children. Impaired vascular health in childhood and adolescence gives further substance to the hypothesis that traffic exhausts are relevant to cardiovascular diseases even early in life.
Ectatic aortopathy and arterial abnormalities cause excess morbidity and mortality in Turner syndrome, where a state of vasculopathy seemingly extends into the major head and neck branch arteries.
We investigated the prevalence of abnormalities of the major intrathoracic arteries, their interaction with arterial dimensions, and their association with karyotype.
Magnetic resonance imaging scans determined the arterial abnormalities as well as head and neck branch artery and aortic dimensions in 99 adult women with Turner syndrome compared with 33 healthy female controls. Echocardiography determined aortic valve morphology.
In Turner syndrome, the relative risk of any congenital abnormality was 7.7 (p = 0.003) and 6.7 of ascending aortic dilation (p = 0.02). A bovine aortic arch was seen in both Turner syndrome and controls. Other abnormalities were only encountered in Turner syndrome: elongated transverse aortic arch (47%), bicuspid aortic valve (27%), aortic coarctation (13%), aberrant right subclavian artery (8%), and aortic arch hypoplasia (2%). The innominate and left common carotid arteries were enlarged in Turner syndrome (p < 0.001). Significant associations were first, bicuspid aortic valve with aortic coarctation, elongated transverse aortic arch, and ascending aortic dilation; second, aortic coarctation with elongated aortic arch and descending aortic dilation; third, 45,X with aortic coarctation, elongated transverse aortic arch and ascending aortic dilation; and fourth, branch artery dilation with bicuspid aortic valve, aortic coarctation, elongated transverse aortic arch and 45,X.
An increased risk of arterial abnormalities, aortic dilation, and enlargement of the branch arteries was found in Turner syndrome without distinct patterns of co-segregation.
The aim of the study is, by comparing cardiac parameters between children native to 1890 metres with children living at sea level, to find out whether there is any impairment in cardiac function related to that altitude.
Electrocardiographic, conventional, and tissue Doppler echocardiographic parameters were compared in 42 healthy children native to 1890 metres, and in 21 healthy age and gender matched children living at sea level. Plasma haemoglobin level and oxygen saturation measured by pulse oxymeter were also obtained from all patients.
Haemoglobin levels were higher, and oxygen saturation levels were lower in children native to 1890 metres. Conventional echocardiographic parameters and mitral annular myocardial parameters were all similar between children native to 1890 metres and children living at sea level. Tricuspid lateral annular early diastolic velocity and the ratio of early-to-late diastolic velocity were significantly lower and tricuspid lateral annular izovolumetric relaxation time was significantly higher in children native to 1890 metres than children living at sea level.
Children living at 1890 metres of altitude predispose to asymptomatic right ventricular diastolic dysfunction or otherwise they remain as healthy children.
Loeys–Dietz syndrome is a newly described entity characterised by a constellation of arterial tortuosity, cranial malformations, and hypertelorism. We report a case of a 7-year old boy with confirmed Loeys–Dietz syndrome and discuss magnetic resonance imaging as a complete technique for assessment and follow-up of aggressive vascular pathology in the brain, thorax, and abdomen, which may dictate early surgical intervention.
A 2-month-old baby was resuscitated from ventricular fibrillation attributed to a concurrent chaotic atrial tachycardia with Wolff-Parkinson-White syndrome. He underwent successful radiofrequency catheter ablation of an accessory pathway. Throughout the 4-year follow-up after the procedure, the boy remained free of any drugs, was in sinus rhythm without ventricular pre-excitation and his growth and development were normal.
We describe our experience with the closure of a synthetic Blalock–Taussig shunt using an Amplatzer vascular occluding device placed with the aid an exteriorised guidewire loop in an infant with congenital cardiac disease. The technique used in a neonate and the physiological benefits of this approach are discussed. We highlight the extended use of the Amplatzer vascular plug in this clinical setting and the advantages of using and the exteriorised guidewire loop for its placement.
Cystinosis is a rare autosomal recessive metabolic disorder characterised by an intracellular accumulation of cystine leading to severe organ dysfunction. It affects renal function, has extra-renal complications but has rarely been associated with cardiac disease. Renal transplantation and cysteamine have dramatically improved the prognosis in the nephropathic form. We present the case of a young adult Caucasian female diagnosed with nephropathic cystinosis and receiving haemodialysis who subsequently developed dilated cardiomyopathy. She presented with acute cardiac failure occurring early after stillbirth following an unplanned pregnancy when her cysteamine had been stopped. Transthoracic echocardiography showed typical features of dilated cardiomyopathy which was absent on pre-pregnancy scans. Investigations failed to identify an underlying cause for her cardiomyopathy. She responded to conventional treatment and currently has had full recovery of her cardiac function confirmed on follow-up echocardiography. As cardiomyopathy rarely co-exists with cystinosis, we believe that this case represents pregnancy-associated cardiomyopathy rather than direct involvement by her cystinosis, particularly as a minority of pregnant patients with associated cardiomyopathy develop heart failure early before the conventional period for peripartum cardiomyopathy. Patient characteristics and maternal outcomes are similar, albeit with higher risk of premature delivery suggesting the same underlying pathological process.
We present a case report of a pregnant woman with increased risk for trisomy 21 at first-trimester screening, assessed by ultrasonography, that was sent to our hospital. Amniocentesis was performed at 14 weeks and 2 days to obtain foetal karyotype that was normal (46,XX). At 19 weeks and 1 day, foetal hydrops associated with cardiac malformation was detected by routine scan. Foetal echocardiogram revealed a complete auriculoventricular septal defect, non-compacted myocardium, and a bradycardia of 70–90 beats per minute, which lead to the suspicion of left isomerism. Foetal death occurred at 20 weeks and 3 days. Autopsy was consistent with the prenatal diagnosis.