Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05).

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.

The aim of this study was to clarify those morphological features and hemodynamic stress factors which influence the electrocardiographic findings in atrioventricular septal defect. In 64 patients with the incomplete form of atrioventricular septal defect (separate valvar orifices) with usual atrial arrangement, the length from the left ventricular apex to the aortic valve (outlet dimension), to the so-called cleft (scoop dimension), and to the lowest point of the left atrioventricular valve (inlet dimension) were measured by biplanar cineangiography. The size of the ostium primum defect, the ventricular pressure ratio, and the pulmonary/systemic flow ratio were also evaluated. The results showed that the lower the scoop/outlet ratio (r=0.60, p<0.001), or the higher the ventricular pressure ratio (r= −0.55, p<0.001), the more deviated to the left and superior was the mean frontal QRS axis. These factors also had significant correlation with the QRS patterns in the Frank vectorcardiogram. We conclude that the scoop/outlet ratio, supposedly reflecting the degree of scooping of the interventricular septum, is one of the most significant factors which influence the electrocardiographic findings in atrioventricular septal defect.

Aortico-left ventricular tunnel is a rare congenital heart defect. Reports on successful device closure of these defects are scarce. We are reporting the first case of device closure of aortico-left ventricular tunnel using an Amplatzer vascular plug III.

Valvar heart disease can be complicated by hemodynamic derangements, depending on the degree of the abnormality. Stenosis causes pressure overload of the chamber draining through the valve and regurgitation results in volume overload. Many lesions have a component of both, resulting in both pressure and volume overload. Increased wall stress causes myocardial stretching and fibrosis, resulting in scarring; a nidus for arrhythmia development. Arrhythmias can complicate the clinical picture and increase the morbidity and mortality in patients with both congenital and acquired valvar disease. In adults with congenital heart disease, arrhythmias are the most common cause of sudden death, followed by heart failure. Valvar stenosis and insufficiency certainly contribute to this. This article highlights the need for arrhythmia surveillance for high-risk valvar lesions.

We studied factors that predict in children catecholamine induced vasoconstriction of the persistent arterial duct. Epinephrine at 2.0–10.0 (4.9 ± 1.3, mean ± standard deviation) μg/kg was injected intravenously into 30 patients with persistent arterial duct whose age ranged from 1 to 91 (27 ± 26) months. In 11 patients aged 10–66 (34 ± 23) months (responders), a continuous murmur had almost completely disappeared on auscultation, and both the diameter and the shunt flow area of the arterial duct had become smaller as shown by Doppler echocardiography. On the other hand, there was no such change in 19 patients aged 1–91 (23 ± 27) months (non-responders). Although there was no significant difference in the age, the body weight, the minimal diameter of the arterial duct, or the doses of epinephrine between responders and non-responders, the gestational age at birth was slightly less (p=0.09) and the birth weight was significantly smaller in responders than in non-responders (p<0.05). The persistent arterial duct of those who had a history of low birth weight always responded to epinephrine. In infants younger than 6 months, the persistent arterial duct was never constricted by epinephrine regardless of the birth weight and gestational age. In low birth weight infants, the vascular smooth muscle of the persistent arterial duct can usually constrict beyond infancy. There may be some age dependent difference in responsiveness to epinephrine until 6 months of age.

We advocate primary repair of atrioventricular septal defect with a common atrioventricular orifice in symptomatic children presenting with intractable congestive heart failure. The single patch technique has been utilized as our procedure of choice. A low operative mortality and a low incidence of residual ventricular septal defect and regurgitation of the left atrioventricular valve requiring reoperation has been achieved. This report emphasizes the technical details of the repair. Careful delineation of anatomic detail is considered to be of the utmost importance. Precise suture placement, tailoring the patch, and reconstruction of the left atrioventricular valve are essential to achieve complete closure of the atrial and ventricular septal defects and functional integrity of the left atrioventricular valve.

We report our experience with an 8-year-old boy with complete atrioventricular block and syncopal bradycardia who required urgent pacing. Each attempt to cross the tricuspid valve with a femoral lead triggered ventricular standstill, followed by fibrillation, and pacing through the coronary sinus failed. Successful ventricular pacing was finally achieved through the oesophagus, allowing subsequent implantation of a transvenous pacemaker.

With the development of three-dimensional techniques for imaging, such as computed tomography and magnetic resonance imaging, it is now possible to demonstrate the precise sinusal origin and epicardial course of the coronary arteries with just as much accuracy as can be achieved by the morphologist holding the heart in his or her hands. At present, however, there is no universally accepted convention for categorising the various patterns found when the heart is congenitally malformed. In this review, we show how, to provide such a convention, it is necessary to take note not only of the sinusal origin of the three major coronary arteries, but also the relationship of the aortic root relative to the cardiac base. We summarise the evidence showing how the proximal portions of the developing coronary arteries grow into the aortic valvar sinuses subsequent to the separation of the aortic root from the subpulmonary infundibulum. We also discuss the evidence showing that the subpulmonary myocardium is impervious to the passage of epicardial coronary arteries, and suggest that the process of septation itself plays an integral role in guiding the arteries into the two aortic sinuses that are adjacent to the pulmonary root. We then show how marriage of convenience between the epicardial coronary arteries and the aortic valvar sinuses provides a good explanation for the known variations found in the setting of transposition. We point out that it is the absence of septation that likely governs the patterns seen in the setting of a common arterial trunk.

Propionic acidemia is a rare autosomal recessive inborn error of metabolism. It is relatively common in Middle East. Dilated cardiomyopathy is one of the leading causes of morbidity and mortality for patients with propionic acidemia. Liver transplantation has been used for patient with frequent metabolic decompensations and was shown to be beneficial in propionic acidemia-related dilated cardiomyopathy. Up to our knowledge, there has been one reported case of recurrent dilated cardiomyopathy 3 years after liver transplantation. We report the first case, from Middle East, of recurrent dilated cardiomyopathy, 6 years after liver transplantation.

Deteriorating ventricular function is a major concern after the Senning operation. A Doppler-derived non-geometric measurement, the so-called myocardial performance index, has been described for use in adults and children. We aimed to assess the utility of this index as a method for quantification of right ventricular function in patients in the late follow-up of the Senning procedure, and to correlate the right ventricular ejection fraction and the first derivative of right ventricular pressure as derived using echocardiography with the ejection fraction determined using magnetic resonance imaging.

We studied 44 patients within a mean postoperative period of 15.3 years. We calculated the right ventricular myocardial performance index by pulsed wave Doppler interrogation of tricuspid inflow and aortic outflow, the ejection fraction by Simpson's rule, and the first derivative of right ventricular pressure by continuous wave Doppler from tricuspid regurgitation.

Mean values of right ventricular myocardial performance index, ejection fraction, and the first derivative of right ventricular pressure were 0.50, 39 percent and 1,398 millimetres of mercury per second, respectively. A cut-off value of 0.47 for the right ventricle myocardial performance index was determined, with a sensitivity of 75 percent and a specificity of 62.5 percent. We found no correlation between ejection fraction and the first derivative of right ventricular pressure as estimated by echocardiography and the ejection fraction as shown by magnetic resonance imaging (r2 equal to 0.29 and 0.04 respectively).

We concluded, first, that patients with preserved right ventricular function had values for the right ventricular myocardial performance index lower than 0.47, and second, that ejection fraction and the first derivative of right ventricular pressure as determined echocardiographically did not correlate with values derived using magnetic resonance imaging.

A two-year-old boy with a background history of Down syndrome and partial atrioventricular septal defect presented with acute respiratory distress requiring intubation and mechanical ventilation. He continued to deteriorate, despite ventilation; direct laryngoscopy, bronchoscopy, and computed tomography demonstrated severe long segment tracheal stenosis. He was placed on extracorporeal membrane oxygenation to stabilise his condition. A slide tracheoplasty and complete repair of the partial atrioventricular septal defect was successfully undertaken. His post-operative recovery was complicated by myocardial infarction and stroke but he made a full recovery. This represents the first report of slide tracheoplasty and partial atrioventricular septal defect repair in a child following extracorporeal membrane oxygenation support.

A young child with exertional chest pain, and an electrocardiographic pattern suggesting reversible ischaemia of the anterior ventricular wall documented by Cardiolyte stress-testing, underwent cardiac catheterization and selective coronary angiography. Although the coronary arteries were entirely normal, the recirculation phase demonstrated marked dilation of the coronary sinus, with atresia of its mouth. At surgery, the patient was confirmed to have muscular atresia at the mouth of the coronary sinus, and underwent unroofing of the coronary sinus to the left atrium, with ligation of a persistent left superior caval vein. Post-operatively, the patient continued to have persistent chest pain, albeit without inducible ischaemia on stress-testing.

Pericardial effusion requires attention because of its underlying pathology and its potential for producing hemodynamic compromise. It was our hypothesis that the etiology of and danger from large pericardial effusions are different at different ages. We reviewed the clinical data on 44 consecutive patients seen between 1986 and 1993. All (age range 0-19 years) had a large pericardial effusion identified by echocardiography, and 38 had fluid available for analysis, pericardiocentesis in these cases being undertaken on the basis of the clinical decision of the attending physician. History, physical examination, chest x-ray, electrocardiogram, and echocardiographic Doppler findings were evaluated retrospectively. Of the patients, 17 presented with tamponade. Of the 14 patients aged less than one year, 12 had tamponade (85%), whereas in the 30 patients older than one year, only five (16%) had tamponade. Surgical drainage was needed in seven patients. Typical physical and electrocardiographic findings (distant heart sounds, pericardial friction rub, decreased QRS voltage, and ST-T wave abnormalities) were present in less than half the patients. Etiologies of the effusion included infection in 10 (four less than one year old, and six greater than one year of age). Among these, two unusual pathogens were detected: Mycobacterium avium-intracellulaire and Candida tropicalis. In eight patients, the effusion was due to trauma (six less than one year old, and two older than one year), and in 11 it was due to post-pericardiotomy syndrome (two less than one year of age and nine older than one year). Malignancy was the cause in six (all older than one year of age), radiation in two, and collagen vascular disease, drug induced, and uremia in one each, all older than one year. Only three patients had idiopathic effusions (6%). These data suggest that, unlike adults, an etiologycan be found in most children with large pericardial effusions. Those less than one year of age are much more likely to present in cardiac tamponade.