From January 1996 to April 2001, we treated eight patients with subdivided left atrium. Their ages at diagnosis ranged from newborn to 6.4 years. Prominent clinical symptoms were failure to thrive, respiratory symptoms, demand for oxygen, and congestive cardiac failure. Diagnosis was made by transthoracic echocardiography in all cases. Cardiac catheterization was necessary only in those patients who had associated cardiac anomalies, or suspected signs of pulmonary hypertension. In 7 patients, surgery was performed immediately after diagnosis, but one preterm infant died before operation due to neonatal sepsis and respiratory distress syndrome. In the postoperative period, one patient developed a severe capillary leak syndrome, and died due to irreversible congestive cardiac failure. The other 6 patients have all been followed up, with good results in the short- and intermediate-term at a mean of 34.3 ± 20.2 months. The infants were thriving, had a reduction in the frequency of infections of the respiratory tract, no significant arrhythmias, and showed early recovery from pulmonary hypertension and right ventricular hypertrophy.

Objectives of the study: to analyse the features and outcomes of different types of atrioventricular septal defects, detected during fetal life, as compared to postnatal data. Material and methods: We analysed retrospectively the data concerning 82 fetuses with atrioventricular septal defect, diagnosed from 19 through 37 weeks gestation with a median of 26 weeks. In 46 cases (56.1%), the diagnosis has been made before the age of 24 weeks. Results: Characteristics of the series – in 44 fetuses the atrioventricular septal defect was not associated with other cardiac anomalies, while 38 fetuses had a more complex form. Chromosomal anomalies were present in 33 of the fetuses (40.2%), more frequently in cases without associated intracardiac defects (56.8%). Trisomy 21 occurred in just over one quarter the series, and in 43.2% of cases without associated defects. In addition, 11% of fetuses had trisomy 18, and one had trisomy 13. Extracardiac anomalies were present in 12 of the fetuses (14.6%), more frequently in cases without associated abnormalities. Of fetuses with more complex defects, 46.4% had hypoplasia of the left ventricle and aorta. Complete atrioventricular block was present in 10 of the fetuses (12.2%), mainly in fetuses with other malformations, and particularly with left isomerism. Recurrence of congenital heart disease was observed in 5 of the fetuses (6.1%). Outcome: In 25 instances (30.5%) the parents opted for termination of pregnancy. Of 57 cases that continued through pregnancy, 9 fetuses died prior to term (15.8%), 32 died postnatally (56.13%) and only 16 fetuses (28.1%) survived. Overall, the mortality was higher in cases with associated malformations, in those with heart failure or those with atrioventricular block. Cardiac surgery was performed in 19 infants, with 5 dying postoperatively, and one late. Conclusions: Our data show a high prevalence of atrioventricular septal defect associated with other malformations when diagnosed during fetal life. This combination is less frequently associated with chromosomal and extracardiac anomalies, but more often with obstructive lesions of the left heart and with atrioventricular block. The association results in a less favourable outcome.

Differentiation between a cleft of the mitral valve and the cleft of the left side of an atrioventricular septal defect – a lesion commonly found in patients with Down's syndrome – is surgically important since the distribution of the conduction tissue varies between the 2 lesions. We sought to determine if cleft of the mitral valve occurs also in patients with Down's syndrome. We studied 5 patients with Down's syndrome and cleft of the mitral valve followed in our institution. Echocardiography showed in all 5 patients a cleft dividing the anterior (aortic) leaflet of mitral valve with normal papillary muscle position, mural leaflet size, and ratio of the inlet/outlet dimension of the left ventricle. Associated cardiac lesions were present in all 5 patients: perimembranous ventricular septal defect in 3, ostium secundum atrial septal defect in 2 and patent ductus arteriosus in 2 patients. During the 5.6 years (0.2–11) of the follow-up period, surgical repair of the cleft was never indicated since the mitral regurgitation through the cleft remained mild or absent in all the patients. Two patients underwent closure of a ventricular septal defect, with atrial septal defect closure in one and ductal ligation in 2. One patient died suddenly at home, without evidence of a cardiac cause. In conclusion, a cleft of the mitral valve has important developmental and morphologic differences with atrioventricular septal defect and may occur in patients with Down's syndrome. If surgical repair of the cleft or of associated cardiac lesion is indicated, it is necessary to distinguish it from atrioventricular septal defect where the conduction axis is displaced posteriorly and may be exposed during surgery.

Aims: Measurement of electrocardiographic intervals to assess dispersion in ventricular repolarization may be helpful in the assessment of the risk of ventricular arrhythmia. We measured QTc, QT dispersion, and T wave intervals in premature infants before and while on treatment with the IKr blocker cisapride as markers for dispersion in ventricular repolarization. Methods and Results: We enrolled 15 non-ventilated premature infants with a mean gestational age of 30.5 weeks, ranging from 26.5 to 33.5 weeks, and mean postnatal age of 24 days, with a range from 5 to 51 days. A digital 12 lead electrocardiogram was recorded prior to and 3 days after administering cisapride at a dose of 0.8mg/kg/day. Serum electrolytes were simultaneously measured. Electrocardiographic measurements before and after included: QT, QTc Bazett, QT dispersion, R–R, T wave interval peak to end, T wave interval peak to end/onset Q to T wave peak, T wave axis, T wave maximum voltage and QRS–T angle. A paired t test and analysis of variance was used to compare the variables before and during treatment. The QTc, T wave interval peak to end and the ratio T wave interval peak to end/onset Q to T peak increased significantly following treatment with cisapride. Results expressed as before and during treatment were for QTc: 429 (65) ms versus 454 (29) ms p < 0.02; for T wave interval peak to end: 65 (11) ms versus 103 (24) p < 0.01, for the ratio T wave interval peak to end/onset Q to T peak: 0.32 (0.06) versus 0.55 (0.16) p < 0.001. Treatment with the IKr blocker did not significantly alter the QT dispersion, T wave voltage, angle or QRS–T angle. Conclusion: The interval from the peak to the end of the T wave and the ratio of this value to the onset Q to T peak interval, represents regional dispersion of repolarization across the ventricular wall. This is a potentially useful clinical index in the assessment of arrhythmic risk in premature infants being treated by blockade of the IKr channels.

We assessed the thickness of the normal left ventricular wall according to gender by performing twodimensional echocardiography in children. While a few studies have evaluated left ventricular wall thicknesses in children using two-dimensional echocardiography, there is little information related to the influence of gender throughout childhood. We evaluated 251 patients (128 males and 123 females) with innocent murmurs but without congenital heart anomalies, or coronary artery lesions or arrhythmias, as determined by repeated echocardiographic, and electrocardiographic examinations. Intra-ventricular septal thicknesses at end-diastole and end-systole, and left ventricular posterior thicknesses at end-diastole and end-systole were determined in the parasternal short-axis view. Significant gender differences were observed in the thickness of all four measurements. There was a linear relationship between the thickness of the left ventricular wall and height. Height was the most appropriate and practical index for assessing the thickness of the wall of the left ventricle in children.

Purpose: We investigated the quality and usefulness of spiral computed tomography and threedimensional reconstruction in children with obstruction of the central airways as a complication of congenital heart disease. Materials and methods: Spiral computed tomography with three-dimensional reconstruction was performed in 49 children with obstruction of the central airways seen as a complication of congenital heart disease. Spiral scanning was performed during breathing in 40, and after sedation with chloral hydrate in 38. Contrast medium was administered through a pedal venous route in 42. We analyzed the motion artifact, additional information provided by, and clinical usefulness, of the three-dimensional images. We also investigated the factors influencing the quality of the images. Results: Stenoses were seen in the trachea in 21 patients, and in bronchuses in 28. Their causes were an anomaly of the aortic arch in 6, posterior displacement of the aortic arch in 7, posterior displacement of the ascending aorta in 5, compression of the brachiocephalic artery in 5, absent pulmonary valve syndrome in 6, displaced or dilated cardiovascular structure in 17, and pulmonary arterial sling in 2. Motion artifact caused mild or negligible degradation of images in all patients except 6. Breath-holding in non-sedated children produced more severe motion artifact than did cardiovascular pulsation. Threedimensional images provided additional information over two-dimensional images in 11, and provided clinically useful information in 10. Contrast injection via the pedal route was better for the quality of threedimensional images than brachiocephalic injection (p = 0.013). Conclusions: Three-dimensional computed tomography is useful in evaluation of obstruction of the central airways in children with congenital heart disease. Despite the fact that motion artifact is unavoidable, the quality of three-dimensional images is acceptable for making a proper and accurate diagnosis. A pedal route is recommended for injection of contrast medium.

Background: Arterial access is traditionally considered mandatory during coil occlusion of the patent arterial duct. Arterial access necessitates heparinization and carries the risk of femoral artery occlusion in small children. Methods and Results: Between July 1999 and May 2001, we attempted coil occlusion of patent arterial ducts in 104 patients without arterial access. The patients were aged from 3 months to 14 years. The median age was 2 years. They weighed 3–35 kg. The median weight was 9.8 kg. The duct diameter at pulmonary artery insertion was 1.8–3.5 mm. The patients were selected on basis of echocardiographic evaluation of duct diameter at pulmonary artery insertion and morphology of the ampulla. Doppler color flow imaging was used in the catheterization laboratory to confirm duct closure. Arterial access was required in 21 patients. The reasons included accidental puncture in 5 patients, failure to obtain venous access in 1 patient, aortic embolization in 3 patients, poor echo images in 2 patients, requirement for additional coils in 8 patients and, failure to cross the duct from pulmonary artery in 2 patients. The fluoroscopic time ranged from 2.2 to 20 min with a mean of 5.3 ± 3.8 min. Immediate closure was achieved in 98 patients and this included 79 of the 83 patients in whom arterial access was avoided. Color Doppler 3–24 h later showed residual flow in 2 patients. Four patients had new-onset left pulmonary artery turbulence with peak gradients below 5 mm of mercury. Coil embolization occurred in 6 patients and all coils were retrieved. Three-month follow up information was available for 78 patients. Small residual ductal leaks were seen in 4 patients, 2 of whom had leaks at 24 h. Two patients had recanalized their ducts. Conclusion: It is feasible to occlude small patent arterial ducts with coils using venous access alone in carefully selected patients with excellent immediate and short-term results.

A 3-month-old infant developed signs of cardiac failure, which was initially attributed to cardiomyopathy. At 8 months, further investigations showed evidence of myocardial ischaemia with reversal of the flow of blood in the left coronary artery, which received no demonstrable inflow from the aorta. An anomalous connection of this artery with the pulmonary trunk was diagnosed but, at surgery, it was found that the arterial orifice was completely atretic, although the main stem was of normal size. A left internal thoracic arterial graft to the anterior descending coronary artery was performed, but he died on the third day after the operation. Postmortem examination showed a small dimple within the aorta at the site of the orificial atresia, extensive myocardial infarction, and two zones of myocardial bridging of the anterior descending coronary artery. We discuss the relationship of coronary orificial atresia with single coronary artery. Although they are related, they typically have different and contrasting clinical presentations. The possible role of the myocardial bridging is also considered.

A boy with familial eosinophilia had the hypereosinophilic syndrome, with involvement of mitral and tricuspid valves. Between the ages of 11 and 20 years, he underwent eight surgical procedures on his atrioventricular valves. The pathology included recurrent thrombotic vegetative masses related to hypereosinophilia. Initial repair of the mitral valve was shortlived, but recurrent repairs of the tricuspid valve were helpful. Mechanical prostheses inserted in the mitral position thrombosed despite anticoagulant therapy, and bioprosthetic valves deteriorated with thrombus, fibrosis, or tearing. The hypereosinophilic syndrome is unusual in children, and produces additional problems with valvar surgery.

Omphaloceles and left ventricular diverticulums are rare disorders. Although either is known to occur on its own, the combination is highly suggestive of the so-called pentalogy of Cantrell. This syndrome is a combination of deformities involving midline structures, with exteriorisation of the heart, or ‘ectopia cordis’, as the most severe malformation. A cause has yet to be identified, though genes located on the X-chromosome may be involved. We discuss a neonate who presented with an omphalocele and a palpable diverticulum of the left ventricle. An omphalocele, especially when above the umbilicus, is an indication for further investigation for deformities as seen in the spectrum of Cantrell's pentalogy, especially cardiac malformations and anterior diaphragmatic herniation. A left ventricular diverticulum is usually associated with Cantrell's syndrome. When found, it is usually accompanied by other intracardiac malformations, so that again further examination is indicated. In our patient, there was an atrial septal defect within the oval fossa, along with a ventricular septal defect and unobstructed albeit anomalous venous pulmonary return to the left atrium. Early surgical intervention seems to be indicated, as spontaneous rupture, arrhythmias, and thromobogenicity of the ventricular diverticulum have all been reported.

We describe an infant born prematurely at 30 weeks gestation with Down's syndrome who became dependent on oxygen at 3 weeks of age after an uneventful initial neonatal period. There had been no evidence of bronchopulmonary dysplasia. An isolated aortopulmonary collateral artery of moderate size was mistakenly diagnosed as persistent patency of the arterial duct on echocardiography, and subsequently successfully occluded using two coils with an excellent clinical result. To the best of our knowledge, this is the first description of a congenital aortopulmonary collateral artery in a symptomatic infant with Down's syndrome and no evidence of bronchopulmonary dysplasia. We discuss the possible etiologies of these collateral arteries. It is important to include aortopulmonary collateral arteries in the differential echocardiographic diagnosis of an arterial duct.

We report a 12-year-old child with anomalous origin of the left coronary artery from the right coronary aortic sinus, the artery taking a proximal intramural course. The anomalous artery was reimplanted into the left coronary aortic sinus. Postoperative stenosis was successfully treated with percutaneous transluminal angioplasty and implantation of a stent.

A 7-year-old boy developed increasing cyanosis after a total cavopulmonary connection with a 3mm fenestration in the baffle. Catheterisation performed 4 years and 7 months after the operation showed reopening of a left superior caval vein draining into the pulmonary venous atrium. Due to the large size of the left superior caval vein, and the absence of intrinsic stenosis, we chose to use an Amplatzer ductal device to occlude the reopened vein. The procedure was safe and successful.