Aims: To assess the cognitive, and behavioural and emotional functioning of children aged 3 months to 7 years shortly before elective cardiac surgery or elective interventional catheterisation. Methods: We used the Bayley Scales of Infant Development, and the McCarthy Scales of Children's Abilities, to measure cognitive functioning. The Child Behavior Checklist was used to assess behavioural and emotional problems. Results: We found no significant differences in mean cognitive scores for children scheduled for cardiac surgery or interventional catheterisation when compared with reference groups. This was also the case for children awaiting cardiac surgery as opposed to those awaiting interventional catheterisation, and for those below as compared to those above the age of 2.5 years. Overall, our results regarding behavioural and emotional functioning were comparable to those of normative reference groups. The only difference found was that the children scheduled for cardiac surgery and aged from 2 to 3 years had significantly higher scores on the Child Behavior Checklist than did peers from normative groups. Conclusion: Cognitive, and behavioural and emotional functioning, both for young children awaiting elective cardiac surgery and interventional catheterisation, can be considered as quite favourable.

Background: The hemodynamic status after a Fontan type procedure for definitive palliation of functionally univentricular hearts is dominated by a high central venous pressure, which seems to be one of several factors responsible for venous congestion appearing as a frequent complication in the early and late postoperative course. The purpose of our study was to find other hemodynamic parameters correlating with the presence of venous congestion and effusions in these patients. Methods: We compared the hemodynamic data of 18 patients who had an uneventful long-term course after a Fontan type procedure with the respective data of 10 patients who developed symptoms of venous congestion in the immediate postoperative period. Based on a theoretical model, we developed an algorithm to calculate mean hydrostatic capillary pressure from mean arterial pressure, systemic vascular resistance index and central venous pressure. Results: Pulmonary vascular resistance index (2.1 ± 1.0 mmHg L-1 min m2), mean left atrial pressure (9.7 ± 4.0 mmHg) and cardiac index (3.6 ± 0.6 1/min/m2) are mainly normal in patients with venous congestion in the immediate postoperative period, but mean hydrostatic capillary pressure is significantly higher compared to patients without venous congestion (24.3 ± 3.1 vs 18.3 ± 4.0 mmHg). Lower mean hydrostatic capillary pressures in these patients are due to a highly significant increase of systemic vascular resistance index (18.6 ± 4.2 versus 33.6 ± 6.6 mmHg L-1 min m2) and a concomitant decrease of cardiac index to 2.4 ± 0.3 1/min/m2. Conclusions: The increase of mean hydrostatic capillary pressure, caused by high central venous pressures but also by relatively low systemic vascular resistance indexes, seems to be the hemodynamic key parameter responsible for venous congestion and effusions in patients after a Fontan type procedure in the immediate postoperative period.

Dilation of the ascending aorta is rare in childhood. When seen, it is usually associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We describe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, also in infancy. A third child has had repair of an atrioventricular septal defect with exclusively atrial shunting, whereas the fourth patient had a structurally normal heart. The aortic valve had three leaflets, and was functionally normal in all. The dilation of the ascending aorta was progressive in all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.

Balloon dilation of coarctation of the aorta has been found to be an effective modality for treatment. Recently, in the older child and adult, implantation of endovascular stents has been considered a clinical alternative to dilation alone. Little is known, however, of the effect of implantation of stents on aortic compliance. To investigate this impact of implantation, we studied 18 piglets, divided into experimental and control groups. At median weight of 14 kg, 2 pairs of ultrasonic crystals were implanted on the aortic wall. After 1 week, all animals underwent catheterization. In the experimental group, a 3 cm long balloon expandable stent was implanted in the descending thoracic aorta between the pairs of crystals. Measurements of arterial pressure and dimensions were performed before implantation and immediately thereafter, and at follow-up catheterization. The index of stiffness, β, and the the elastic modulus of aortic pressure-strain, were calculated as indexes of arterial compliance. The change in compliance during the period of study was not different between groups. At follow-up, no difference was observed between groups in the velocity of the aortic pulse wave, the augmentation index, or the maximum velocity of flow of blood. The stents remained patent and did not affect aortic growth or medial wall thickness. There was no difference between groups in levels of plasma renin activity and serum aldosterone. In this animal model studied over the short term, therefore, implantation of stents does not affect aortic compliance. Further studies are required to elucidate the long term effects of stents on the hemodynamics affecting the aortic wall and local flow dynamics.

Atrioventricular nodal reentrant tachycardia was proven during electrophysiologic study in 41 children, aged from 3.7 to 16 years, who were referred for catheter ablation of symptomatic supraventricular tachycardia. Using an abbreviated combined anatomical and electrogram-guided approach for selective ablation of the slow pathway, a steerable ablation catheter was placed at the inferior region of the vestibule of the tricuspid valve close to the orifice of the coronary sinus, with the intention of recording a multicomponent local atrial electrogramm during sinus rhythm. If application of radiofrequency current of 500 kHz at 70°C at this site did not result in a slowly accelerated junctional rhythm, at a rate of less than 120 beats per minute, the catheter was stepwise advanced up to a position midway towards the apex of the triangle of Koch for additional applications of energy. Ablation was achieved in 35 of the patients. In 6 patients, the slow pathway was modulated such that the tachycardia could no longer be induced. The number of applications of energy ranged from 1 to 19, with a median of 6 applications. The mean period of fluoroscopy was reduced to 15.6 (4.3 to 39.8) minutes, while the overall duration of the catheterization procedures ranged from 88 to 280 (mean 173.2) minutes. In none of the patients did we observe permanent high grade atrioventricular block. During follow-up over a mean of 4.1 years, two patients had recurrence of tachycardia, corresponding to a 95% rate of success in the midterm. We conclude that selective radiofrequency ablation of the slow pathway using the abbreviated anatomical and electrophysiological approach is a safe and curative therapeutic approach in children with atrioventricular nodal reentrant tachycardia. Periods required for fluoroscopy can be significantly reduced, and mid-term results are excellent.

Background: Non-invasive measurement of left ventricular output has been shown to be a repeatable technique. Little is known about the repeatability using echocardiography in determining pulmonary arterial diameters or right ventricular output. Aims: To find the most repeatable point at which to measure pulmonary arterial diameter, and to compare the repeatability of determining right ventricular output with left ventricular output. Methods: We assessed the Intra-observer and inter-observer repeatability for measuring the diameter of the pulmonary trunk in 24 term and 26 preterm infants, respectively. Interobserver repeatability was assessed for the diameters of the pulmonary trunk and aorta, for stroke distance, and for left and right ventricular output. Results: The coefficients of variation for intra-observer repeatability were 4%, 7.5% and 9% respectively for measurements of the pulmonary valve, the pulmonary trunk, and the right ventricular outflow tract. There were significant differences between observers for measurement of the pulmonary trunk (p< 0.001) and right ventricular outflow tract (p= 0.011) but not for the pulmonary valve measured in either its long (p= 0.22) or short axis (p= 0.22). Significant differences between observers were also found for the pulmonary stroke distance measured in the long axis (p= 0.004) and aortic diameter at end-diastole (p< 0.001). The other parameters did not differ significantly and were used to calculate right and left ventricular output, respectively. Mean left ventricular output was 241 mls/kg/min, with mean differences between observers of 0.6 mls/kg/min (95% confidence interval (CI): −39.2 to 40.3 mls/kg/min). Mean right ventricular output was 255 mls/kg/min, with mean differences of 0.3 mls/kg/min (95% CI: −24.1 to 23.4 mls/kg/min). Conclusion: Measuring the diameter of the pulmonary trunk at the base of the valvar hinge points was most repeatable. Repeatability of right ventricular output was similar to that of left, with absolute values similar to those published by other workers.

Background: Pleural effusions after bidirectional cavopulmonary anastomosis remain a significant cause of morbidity. Prolonged effusions in such patients have been associated with persistent elevations in plasma renin and angiotensin II. Methods: We conducted a controlled study in 36 patients (median age 8 months) undergoing bidirectional cavopulmonary anastomosis. Enalapril (5 mcg/kg) was administered intravenously within 1 hour of surgery and every 12 hours thereafter in 18 patients; when these patients were tolerating feeds, enalapril was switched to enteral captopril (3 mg/kg/day) every 8 hours. The other 18 patients did not receive perioperative angiotensin converting enzyme inhibitors. Using standardized criteria for discontinuation of chest tubes (< 2 mL/kg/day), volume and duration of pleural drainage were compared between groups. Results. There were no differences between groups in demographic, diagnostic, or hemodynamic factors. There was no difference in cardiopulmonary bypass time between groups and no difference in postoperative pulmonary arterial pressures. The duration of pleural drainage was shorter (2.2 ± 1.4 vs 5.9 ± 1.4 days, p< 0.001) and the volume less during the first 24 hours (4.7 ± 1.2 vs 7.7 ± 2.1 mL/kg, p< 0.001) and overall (10.6 ± 2.4 vs 19.6 ± 4.5 mL/kg, p< 0.001) in patients who received angiotensin converting enzyme inhibitors than those who did not. Readmission for persistent effusions was required in 3 patients who did not receive angiotensin converting enzyme inhibitors and none who did (p= 0.11). Conclusions. Perioperative administration of angiotensin converting enzyme inhibitors is associated with decreased severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis.

Background: From January, 1997, as part of an international multicentric trial, we have been closing small-to-moderate atrial septal defects within the oval fossa using the Amplatzer Septal Occluder (ASO, AGA Medical). Methods: All patients with defects within the oval fossa deemed potentially suitable for transcatheter closure were investigated by transesophageal echocardiography with the aim of gaining extra information that might alter the decision to use the device to close the defect. Views were obtained in transverse and longitudinal planes, permitting measurements of the diameter of the defect, and its distance from the atrioventricular valves, coronary sinus, and pulmonary veins. Additionally, we sought to identify multiple defects, and to exclude sinus venosus defects. Results: Of 56 patients with left-to-right shunts, 41 (73.2%) were deemed suitable for closure with the Amplatzer Septal Occluder. All underwent the procedure successfully, with no complications. This includes 5 patients with multiple small defects that were sufficiently close to the main defect to be closed with a single device. Only two of these had been detected on the transthoracic study. In the remaining 15 of 56 patients, transcatheter closure was deemed unsuitable. In 9 patients, this was due to the limitation of the size of the device available during the period of study, this representing a relative contraindication. In the remaining 6 (10.7%), transcatheter closure was not performed because multiple defects were too far apart to be closed with a single device in 3 patients, two patients were noted to have a sinus venosus defect, and another was noted to have anomalous connection of the right upper pulmonary vein to the right atrium. Excluding patients contraindicated due to the size of the defect alone, transesophageal echocardiography provided extra information in one-tenth of our patients, which altered the decision regarding management. Conclusion: Transesophageal echocardiography is indispensable in the evaluation of patients undergoing transcatheter closure of atrial septal defect.

We describe 10 children with endomyocardial fibrosis who underwent surgical treatment between 1978 and 1999. Seven were male and 3 female, with an age range from 4 to 15 years, having a mean age of 11 years. All were in the final stage of heart failure. Three had biventricular disease, 6 had involvement of the right ventricle alone, and one had endomyocardial fibrosis confined to the left ventricle. There were 3 deaths (30%) in the postoperative period due to low cardiac output. The 7 survivors were followed up for a period ranging from 12 to 168 months, with a mean of 72 months. Two late deaths have occurred resulting from heart failure and infectious endocarditis. Five (50%) children are still alive. Two required 3 reoperations for dysfunction of the inserted valvar prosthesis. One patient is in functional Class IV, and 4 are in Class II to III, despite intensive medical treatment. It is concluded that surgery for endomyocardial fibrosis is an essentially palliative procedure and, especially in children, the results of surgical treatment leave much to be desired.

Constrictive pericarditis is a uncommom disease in children. We have now encountered pericardial thickening as the cause of severe constrictive physiology in two patients, one also having haemodynamic features of restrictive cardiomyopathy. Both patients, who had refractory ascites and evidence of increased systemic venous pressure, underwent Doppler echocardiography, cardiac catheterisation, and magnetic resonance imaging. Resonance imaging failed to show any thickning of the pericardium, but cardiac catheterisation revealed diastolic equalisation of pressures in all four chambers, with only mild elevation of pulmonary pressure in the first patient, but nearly equalisation of diastolic pressure, and a very high pulmonary arterial pressure with a difference of 7 mm Hg between the end diastolic pressures in the two ventricles in the second patient. Doppler revealed a restrictive pattern of mitral inflow, with high E and small A velocities and a short deceleration time. The clinical background did not suggest pericardial disease in either of the patients. We conclude that a careful search is needed to uncover constrictive pericarditis when there is no previous disease which may suggest late pericardial constriction. The haemodynamic features of restrictive cardiomyopathy can co-exist with pericardial restriction, and differentiation between the two entities is critical in view of the diverse management and prognosis of the two conditions.

Data on long-term follow-up for closure of so-called secundum type“ atrial septal defects within the oval fossa using recently developed devices are limited, and results focused on presence of residual shunting. The purpose of our study was to report the experience from a single center establishing the effectiveness of transcatheter closure in patients with various types of defect other than those located centrally within the oval fossa.

A total of 72 patients was included in this study. On transesophageal echocardiography, the size of the defects varied from 6 to 18 mm, with estimation of the stretched diameter from 11 to 21 mm. The ratio of stretched diameter to the extent of the residual septum ranged from 0.28 to 0.54. Mean follow-up was 30.5+ 7.4 months, with a range from 13 to 42 months. The rate of closure using devices with diameters from 28 to 40 mm increased from 80% immediately after implantation to 93% in the 57 patients examined 24 months after implantation. For further analysis, we compared the 44 patients with a solitary, centrally located, defect to 28 having morphological variations, including superiorly located defects with deficient superior and aortic rims, multifenestrated and aneurysmal defects, or isolated additional defects. There was no incidence of formation of thrombus, sustained atrial arrhythmia, or infective endocarditis. Residual shunting was not influenced by location or morphology of the defects, but increased with size, stretched diameter, and the ratio of pulmonary to systemic flows. Serial transthoracic echocardiographic findings revealed malposition of one right-sided superior arm of the device in 8 patients, while protrusion of one leftsided arm onto the right atrial aspect was observed in 3 patients. Fluoroscopy showed fatigue fracture of a single arm in 7 patients (9.7%) within the first 6 months after implantation.

These results demonstrate that transcatheter closure with the non self-centering double umbrella device was effective and safe on medium-term follow-up, and could be extended to defects within the oval fossa having various morphologies. Residual shunting resolved with time, and was not related to either morphology or the position of the device.

We describe the development and exacerbation of protein-losing enteropathy after relocating to an environment at an altitude of 3695 feet in El Paso, Texas, in a patient who had undergone a Fontan operation. This report should heighten awareness to the possibility of such patients developing protein-losing enteropathy at high-altitude, with hypoxemia-induced pulmonary vasoconstriction, and subsequent elevation of central venous pressure, the most likely underlying mechanism.

We report an infant with ectopic atrial tachycardia, due to an aneurysm of the right atrial appendage, who developed congestive heart failure. Although catheter ablation was transiently successful, tachycardia recurred 2 days later. The aneurysm of the right atrial appendage was resected successfully by surgery, and thereafter she did well, reverting to normal sinus rhythm.

Cardiac tamponade occurring late after interventional closure of defects within the oval fossa is a very rare but life-threatening complication. We describe such an occurrence after use of a Cardioseal device to close an interatrial communication. Two arms of the device had perforated left atrial wall. The device was removed at surgery, and the defect closed uneventfully. All available means should be used to identify this complication.

We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.

Noncompaction of the ventricular myocardium, sometimes referred to as “spongy myocardium”, appears as excessive and prominent trabeculations and deep intratrabecular recesses within the ventricular wall, usually involving the left ventricle, although the right ventricle and interventricular septum can also be affected. It may occur with or without additional heart malformations. Roifman syndrome is a constellation of antibody deficiency, spondyloepiphyseal dysplasia, facial dysmorphism, growth retardation, and retinal dystrophy. We report a patient with Roifman syndrome who presented with noncompaction of the left ventricular myocardium. Our findings expand the spectrum of diseases associated with noncompaction. The recognition of noncompaction among patients with Roifman syndrome is important, as the immune deficiencies may be subtle and undiagnosed until adulthood. Thus, some patients may first present with cardiac failure.