We report our experience in radiofrequency catheter ablation between April, 1992 and December, 1998, in which we treated 287 parients less than 18 years of age (mean 14.3±3.1 years) with supraventricular tachycardia. Accessory pathways were the arrhythmic substrate in 252 of the patients (87.8%), the patients having a total of 265 accessory pathways. Atrioventricular nodal re-entry was the cause of tachycardia in 26 patients (9.0%), while atrial flutter was detected in the remaining 9 patients (3.1%). We were able successfully to eliminate the accessory pathway in 236 patients (89%), but 25 patients had recurrent arrhythmias. Ablation proved successful in all cases of atrioventricular node re-entry tachycardia, the slow pathway being ablated in 25 patients, and the fast pathway in only one case. Recurrence of the arrhythmia occurred in three patients (11.5%). We performed a second ablation in these children, all then proving successful. The ablation was successful in all cases of atrial flutter, with one recurrence (11.1%). Overall, therefore, ablation was immediately successful in 271 patients (94.4%), with a recurrence of the arrhythmia in 29 cases (10.7%). The incidence of serious complications was 2.09% There was one late death due to infective endocarditis, 3 patients suffered complete heart block, 1 had mild mitral regurgitation, and 1 patient developed an haematoma in the groin. We conclude that radiofrequency catheter ablation can now be considered a standard option for the management of paroxysmal supraventricular tachycardias in children and young adults.

We successfully visualized the brachiocephalic arteries and aortic arch in a fetus seen at 19 weeks of gestation with a common arterial trunk and interrupted aortic arch by means of color power Doppler angiography, a new diagnostic development of color Doppler echocardiography. Power Doppler imaging is more sensitive to the state of low flow in fetal vessels, thus providing better visualization of fetal vascular structures from an early gestational stage.

The Amplatzer Septal Occluder is made from a Nitinol wire mesh shaped into 2 disks with a connecting waist, which serves to center the device in the defect while occluding it. The Amplatzer device is also available in a configuration with no central waist for use in patients with patent oval foramen, or multiperforated aneurysm of the interatrial septum. For the purposes of this review, we analysed our experience using the Amplatzer device in 150 patients with interatrial communications. Of these, 104 had a defect within the oval fossa, 33 a patent oval foramen, and 13 had undergone fenestration of a Fontan procedure. Of those with defects within the oval fossa, a device was implanted in 100 patients, and 2 of these patients subsequently required surgical intervention, 1 because of migration and the other because of malformation of the device. Of the remaining 98 patients, complete occlusion has been achieved in 90% at 1 year. Any residual leaks are either trivial or small. In those with a patent oval foramen, the septal occluder was used to close 20, whilst the device designed specifically for this purpose was used in 13. On follow-up contrast echocardiography, only 2 patients have a small residual right-to-left shunt. Complete occlusion was achieved for all the Fontan fenestrations, although 1 patient later underwent surgery for baffle dehiscence. Other significant complications occurred in 2 patients who developed deep vein thrombosis, and 3 patients who suffered transient supraventicular arrhythmias. Although the Amplatzer device has been in clinical use for only 3 years, its unique design, and ease of use, has resulted in its widespread adoption by many entres. The results to date are very encouraging, but it must be remembered that there is, as yet, no long-term followup data available for this life-long implant.

A young female athlete is described with anomalous origin of the main stem of the left coronary artery from the non-facing sinus of Valsalva who sustained myocardial infarction and died suddenly after physical exertion. Autopsy findings illustrated the mechanistic importance of acute angle take-off of the left main coronary artery and a slit-like orifice, which was likely compressed and obstructed by acute expansion of the aortic wall. This rare type of coronary anomaly has been regarded as having little clinical significance, but it can lead to sudden cardiac death under physical exertion.

Deletions of chromosome 22q11 are common in patients with tetralogy of Fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.

Abstract Severe Ebstein's malformation has a poor prognosis when diagnosed during the neonatal period. Surgical options that have proved successful in older patients have been associated with high mortality and morbidity in neonates. We report here our success with a policy of induction at term and immediate surgical intervention when Ebstein's malformation was diagnosed prenatally.

A woman, having already delivered one child, underwent fetal echocardiography during thre subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process.

An atrial septal defect is commonly thought of as a benign cardiac lesion especially in infancy. The haemodynamic consequences for the lungs, nonetheless, can be comparable to that produced by patency of the arterial duct. In a preterm boy, this lesion led to the development of broncho-pulmonary dysplasia, and dependence on a respiratory ventilator. He could be extubated shortly after surgical closure of the septal defect.

Tetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.

Abstract To investigate the pattern of flow in the superior caval vein in children after open heart surgery, we examined the flow velocity in 37 partients who had undergone surgical correction of either ventricular or atrial septal defects. The control group consisted of 64 age-matched children. We used pulsed Doppler examinations to record the velocity curves. We measured the hightest and lowest peak flow velocities during systole and diastole, and calculated ratios for the various measurements.

We found that the velocity of the wave in systole is lower than that of the wave in diastole in the postoperative patients, which is contrary to the normal pattern. The velocity in systole increases more than that in diastole during inspiration, which is also diffetent from the normal pattern. The change in pattern of flow velocity cure during respiration was smaller in patients after surgery than in normal subjects, which might indicate that there is less reserve ability in the right atrium in postoperative patients.

An hydropic fetus seen at 28-weeks gestation had a saccular aortic aneurysm in the descending thoracic aorta. Histology disclosed marked fibrointimal hyperplasia, thrombus, and attenuation of the tunica media. The remainder of the descending thoracic aorta showed fibrointimal hyperplasia. We speculate that the narrowed lumen and rigid aortic wall resulting from this vasculopathy provided an increased afterload leading to cardiac failure.

A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.

The perigraft reaction is an unusual complication found in patients in whom a modified Blalock Taussig shunt has been created using a polytetrafluoroethylene graft. We found that, in two infants, consistent laboratory findings during such a perigraft reaction were hypofibrinogenemia, increased levels of thrombin-antithrombin III complex, prothrombinfragment 1 and 2 and products of degradation of fibrin. Normalization of the levels of fibrinogen produced resolution of the perigraft reaction.