Aerosolized iloprost is now used as a therapeutic option in the treatment of pulmonary hypertension. We report on the administration of this derivative of prostacycline in treating severe pulmonary hypertension of the newborn. The combination of iloprost instilled endotracheally and inhaled was chosen as a last attempt at treatment in a critically ill patient who did not respond to advanced conventional treatments, including high frequency oscillation and inhalation of nitric oxide. The use of iloprost converted permanently the right-to-left shunting, leading to a substantial improvement in oxygenation.

Objectives: A retrospective study was undertaken to determine the degree of pulmonary regurgitation following percutaneous balloon valvoplasty for isolated pulmonary valvar stenosis. Background: Percutaneous balloon valvoplasty is the recognised treatment of choice in pulmonary valvar stenosis with effective relief of gradient. Few studies have reviewed the degree of pulmonary regurgitation after the balloon valvoplasty. Methods: We reviewed all patients with isolated pulmonary valvar stenosis undergoing percutaneous balloon valvoplasty at a tertiary centre between December 1984 and August 1996. Those with an associated haemodynamically insignificant atrial septal defect or patent oval foramen were also included. Their medical records, echocardiograms and angiograms were studied. Colour flow Doppler was used as a semi-quantitative way of assessing the pulmonary incompetence. Results: Over the period of review, 57 procedures had been performed in 49 patients. The median age at the time of the procedure was 1.08 years, with a range from 0.01 to 13.3 years. The median period of follow-up was 5.64 years, with a range from 3.00 to 14.26 years. Immediately following the dilation of the pulmonary valve, the peak-to-peak instantaneous systolic pressure gradient was significantly reduced. Seven patients required a second dilation of the valve. Their median age at the first valvoplasty, at 0.49 year, was significantly lower than those who required only one procedure, at a median of 1.50 years. Following the valvoplasty, mild pulmonary incompetence was noted in 26 out of 42 patients the day after the procedure, but only 7% had moderate incompetence. On follow-up, there was an increase in the number of patients with moderate to severe pulmonary incompetence, from 7% to 29%. Those patients in whom the procedure was performed at a younger age had more significant pulmonary incompetence. Neither the initial gradient across the pulmonary valve, nor the size of the balloon used, were related with statistical significance to the subsequent development of pulmonary incompetence. Conclusions: The majority of the patients with congenital isolated pulmonary valvar stenosis had only a mild increase in the degree of pulmonary incompetence following a single pulmonary valvoplasty. Patients who required the procedure early in life were more likely to develop significant pulmonary incompetence. The ratio of the size of the balloon to the diameter of the valve did not significantly affect the outcome. We suggest that those patients who had more severe stenosis because of a more abnormal pulmonary valve, and hence required early intervention, were more likely to develop greater pulmonary incompetence after the valvoplasty.

In the Eisenmenger syndrome, indirect estimation of blood volumes may provide quite inaccurate information when seeking to define therapeutic strategies. With this in mind, we analyzed directly the red cell mass, plasma volume, and total blood volume in patients with pulmonary hypertension associated with congenital cardiac defects and erythrocytosis, comparing the results with the respective estimated volumes, and examining the changes induced by therapeutic hemodilution.

Thus, we studied 17 patients with the Eisenmenger syndrome, aged from 15 to 53 years, in the basal condition, studying 12 of them both before and after hemodilution. We also investigated five individuals with minimal cardiac lesions, aged from 14 to 42 years, as controls. Red cell mass and plasma volumes were measured using [51 chromium]-sodium chromate and [131iodine]-albumin respectively. Hemodilution was planned so as to exchange 10% of the total blood volume, using 40,000 molecular weight dextran simultaneously to replace the removed volume. The mean values of the red cell mass, plasma volume and total blood volume as assessed by radionuclide techniques were 32%, 31% and 32% higher than the respective volumes as estimated using empirical mathematical formulas (p < 0.002). The measured total blood volume was also 19% higher in the patients compared with controls. Following a period of 5 days after hemodilution, we noted a 13% reduction in red cell mass (p = 0.046), and 10% reduction in total blood volume (p = 0.02), albeit with no changes in the plasma volume.

We conclude that direct measurement of blood volumes is useful for proper management of these patients, and provides results that are considerably different from those obtained by empirical estimations.

In recent years, three-dimensional imaging has provided new opportunities for visualizing congenital cardiac malformations. We present the initial clinical experience using a recently implemented system, which employs some of new interactive, real-time, techniques. We show how three-dimensional rendering based on magnetic resonance imaging can provide detailed spatial information on both intrinsic and extrinsic cardiac relations, and hence how a virtual examination can potentially provide new means to a better understanding of complex congenital cardiac malformations.

Objectives: We evaluated the efficacy of percutaneous transluminal coronary angioplasty for anastomotic stenosis after coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease. Subjects and Methods: From July 1997 to April 2000, four boys and one girl underwent percutaneous transluminal coronary angioplasty for 6 anastomotic lesions following coronary arterial bypass grafting using the left or right internal thoracic artery. Progressive severe stenosis of the grafts in the follow-up angiograms after grafting, and evidence of ischemia, were regarded as indications for percutaneous transluminal coronary angioplasty. Age at coronary angioplasty ranged from 4.2 to 16.7 years, with a median of 6.9 years, while the interval from operation ranged from 0.3 to 3.0 years, with a median of 1.1 years. The diameter of the balloon catheter employed varied from 1.5 to 2.5 mm, and the pressure of inflation ranged from 8 to 16 atmospheres. Results: The degree of stenosis decreased from 63 to 99%, with a median of 88%, to 0 to 40%, with a median of 17% immediately after angioplasty. A follow-up angiogram either 3 months or 1 year later revealed no restenosis in any patient. Conclusion: Percutaneous transluminal coronary angioplasty is a feasible and useful procedure for treating anastomotic stenosis following coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease.

Atherosclerotic disease of the heart likely has its origins in childhood. The promotion of cardiovascular health in children, however, has been studied only for those practicing in general paediatrics. We hypothesised that paediatric cardiologists do not consistently discuss cardiovascular risk factors with patients and their families. We therefore, carried out a nationwide survey of paediatric cardiologists to determine how often they discussed atherosclerosis and 4 modifiable risk factors, specifically weight, smoking, diet and nutrition, and physical activity. Only two-fifths reported that they discussed atherosclerotic disease frequently to always. For patients with cardiovascular disease, weight was discussed frequently to always by 59%, smoking by 61%, diet and nutrition by 63%, and physical activity by 92%. In contrast, for patients without cardiovascular disease, weight was discussed frequently to always by 35%, smoking by 46%, diet and nutrition by 39%, and physical activity by 62%. These differences are statistically significant (p < 0.003 by χ2 analysis). Cardiovascular risk factors were discussed more consistently as children grew older (p < 0.0001). Respondents stated that, in their opinion, the promotion of cardiovascular health was a role more appropriate for providers of primary care than for paediatric cardiologists (p < 0.0001). Constraints of time, and the perceived role of the cardiologist, were the most common barriers to anticipatory guidance. We suggest that these findings indicate that paediatric cardiologists can assume a more prominent role in preventive cardiology and education, although their precise role or roles, and the optimal methods of anticipatory guidance, remain controversial.

A 17-year-old girl with Turner's syndrome underwent two cardiac operations due to severe mitral stenosis with pulmonary hypertension, caused by a parachute-like mitral valve. The anomaly was associated with persistence of the left superior caval vein, which drained to the coronary sinus, and non-compaction of the left ventricular myocardium. The association of these lesions is rare in patients with Turner's syndrome.

Aims: Our aims were to use transthoracic three-dimensional echocardiography to assess the morphology of atrial septal defects in children prior to closure, and to compare the three-dimensional echocardiographic data with transcatheter and surgical findings. Methods and results:We used transthoracic three-dimensional echocardiography in 62 consecutive patients, aged from 2 to 18 years, with atrial septal defects, measuring the maximal diameter and the extent of the rims. Subsequent to the study, we referred 42 patients for transcatheter closure, the rims being measured at greater than 4 mm. We found a good correlation between the maximal diameter of the defect as measured at transthoracic three-dimensional echocardiography and using a balloon (y = 3.45 − 0.73x; r = 0.78; p < 0.0001), the mean difference between the measurements being 2.4 ± 2.8 mm. Successful closure with the Amplatzer septal occluder, having a mean size of 22 ± 4 mm, was achieved in 95% of the patients. Of the original cohort, 20 patients were referred for surgical closure. In these patients, the inferior rim had been deemed insufficient in 5, the postero-superior rim in 6, and the postero-inferior rim in 9. Complete agreement was found when the deficiency of the rim as judged using transthoracic three-dimensional echocardiography was compared with intraoperative findings. The correlation between measurements of the deficiency of the rim achieved by transthoracic three-dimensional echocardiography and at surgery was excellent (y = 0.2 + 0.98x; r = 0.93; p < 0.0001), the mean difference between the measurements being no more than 0.6 ± 0.4 mm. Conclusions: Transthoracic three-dimensional echocardiography proved accurate in measuring the maximal diameter and rims of atrial septal defects within the oval fossa. This non-invasive method will be valuable in selecting children for transcatheter or surgical closure of such defects.

In my Mannheimer lecture, designed to meet the needs of a mainly clinical audience, I present aspects of cardiac development that link basic science to clinically relevant problems. During development of the cardiac tube, and its subsequent changes as a dextrally looped structure, which is still connected to the dorsal body wall by a venous and an arterial pole, there are basic requirements. These consist of the development of myocardium, endocardium and the interposed cardiac jelly from the cardiogenic plates. In this primitive heart tube, septation and valvar formation then take place to convert it into a four-chambered heart. I demonstrate that the refining of the above events cannot take place without the addition of extracardiac populations of cells. These are presented as the “quintessence of heart development”, and consist of cells derived from the neural crest, along with epicardially derived cells. Without these contributions, the embryos uniformly die of cardiac insufficiency. Important contributions are made by the cells derived from the neural crest to septation and the formation of the arterial valves, and possibly in differentiation of the central conduction system. The epicardially derived cells are essential for formation of the interstitial fibroblasts and the myocardium, as well as the coronary vascular system. I conclude by discussing specific malformations of the heart that might be linked to these extracardiac contributions.

We describe a new method of three-dimensional magnetic resonance imaging of the heart that has been used to produce high quality diagnostic images in 274 patients with congenital cardiac disease, ranging in age from 1 day to 66 years. Using a steady state free precession gradient echo technique and parallel imaging, rapid acquisition of the entire cardiac volume is possible during 8 to 15 sequential breath-holds, each lasting between 8 and 15 s. We obtained high-resolution images, with a resolution of 1 mm3, at between 3 and 10 phases of the cardiac cycle.

While images of diagnostic quality were obtained in all cases, in 52 patients there was some degradation due to various factors. Children under 8 years were ventilated, and ventilation was suspended for the breath-holds. For patients breathing spontaneously a novel respiratory navigator technique was developed, using a navigator echo placed over the right hemidiaphragm. This was used successfully in 20 patients, and reduced the misalignment of images obtained during different breath-holds.

Images were analysed using multi-planar reformatting and volume rendering. Image processing took approximately five minutes for each study. End-diastolic images were processed for all patients. Systolic images were also processed in selected cases.

Further improvements in parallel imaging should reduce imaging times further, so that it is possible to obtain the full volume image in a single breath-hold. This will enable imaging of complex anatomy to be obtained using a standard imaging protocol that does not require the operator to understand the cardiac malformation, making the magnetic resonance imaging of congenital cardiac disease faster and more effective.

During the last two decades, several advances have resulted in marked improvement in medium-term survival, with excellent quality of life, in children undergoing cardiac transplantation. Improved outcomes reflect better selection of donors and recipients, increased surgical experience in transplantation for complex congenital heart disease, development of effective surveillance for rejection, and wider choice of immunosuppressive medications. Despite all of these advances, recipients continue to suffer from the adverse effects of non-specific immunosupression, including infections, induction of lymphoproliferative disorders and other malignancies, renal dysfunction, and other important end-organ toxicities. Furthermore, newer immunosuppressive regimes, thus far, appear to have had relatively little impact on the incidence of chronic rejection. Progress in our understanding of the immunologic mechanisms of rejection and graft acceptance should lead to more targeted immunosuppressive therapy and avoidance of non-specific immunosupression. The ultimate goal is to induce a state of tolerance, wherein the recipient will accept the allograft indefinitely, without the need for long-term immunusupression, and yet remain immuno-competent to all non-donor antigens. This quest is currently being realized in many animal models of solid organ transplantation, and offers great hope for the future.

The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.

An 11-year-old female, in apparent good health, was referred to our Institute because of exercise-induced left bundle branch block detected during a Master's step test performed to obtain the clearance for competitive sport. The tests that we performed to investigate the possible causes were all negative. We suspect, however, that the left bundle branch block is the initial sign of cardiomyopathy. If our suspicions are correct, close follow-up is important to chart the possible progression of the disease.

We report a patient with Down's syndrome referred to our Department of Echocardiography for evaluation after an episode of tachyarrhythmia. We diagnosed Ebstein's malformation. As far as we know, this association has been reported previously on only three occasions. We review these previous cases, describe our echocardiographic techniques, and discuss our experience.

An 11-month-old infant had undergone a primary arterial switch operation, including the Lecompte maneuver, for correction of discordant ventriculo-arterial connections and closure of an accompanying ventricular septal defect.

At discharge, there were no signs of aortic valvar incompetence. Regurgitation across the aortic valve was detected first at the age of 2 years, and then increased progressively, as documented by serial echocardiographic studies. There had been no history of bacterial endocarditis. At the age of 10 years, echocardiography revealed severe aortic valvar incompetence. At operation, the aortic valve had three leaflets, all of which were short, with very restricted movement. Absence of sufficient leaflet tissue precluded a durable valvar reconstruction, so the aortic valve was replaced with a 21 mm mechanical prosthesis. Histological examination of the removed leaflets revealed nodular swelling due to mucous changes of the matrix, as well as fibrous alteration and formation of scar tissue, including areas of fibroblastic and capillary proliferation. There were no signs of calcification or acute inflammatory changes. Improvement of left ventricular function was observed both early postoperatively and later on. Our observation shows that aortic valvar incompetence after an arterial switch operation can be caused by degenerative changes of the neo-aortic leaflets, which prevent plastic reconstruction of the valve, necessitating valvar replacement.