Triiodothyronine has been introduced as a promising new supplement for patients undergoing openheart surgery. This manuscript will review the existing literature regarding supplementation with triiodothyronine in children.

Infants and children undergoing cardiac surgery experience a profound decrease in levels of free and total triiodothyronine. These decreases are of sufficient magnitude to effect cardiac function. Supplementation with triiodothyronine has been shown to enhance myocardial performance, improve recovery of circulating levels of triiodothyronine, and to result in an increase in mixed venous oxygen saturation. As encouraging as these results are, there is no definitive study which demonstrates that triiodothyronine improves clinical outcome in children. Thus, while triiodothyronine appears to have promise as a supplement for children undergoing openheart surgery, more studies are needed to establish the efficacy of such therapy scientifically.

A left superior caval vein frequently occurs in the malformed, as well as in the structurally normal, heart. Its physiological impact varies, and is determined by its connections and whether there are associated cardiac lesions. In this review, we describe 3 patients with such a vein, 1 without other lesions and 2 with the anomalous venous channel as a component of a complex cardiac malformation. In all cases, transcatheter techniques were used to treat the physiological dysfunction caused by the presence of the vein. The connections of the vein, and the complexity of the associated cardiac lesions, determine the options for treatment. We define the role of surgical as opposed to transcatheter intervention, and discuss strategies for transcatheter treatment.

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.

Aim: To evaluate the failure of mechanically detachable spirals produced from tungsten (MDS, Balt, Montmorency, France) and the toxicity of elevated levels of tungsten in the serum subsequent to their implantation. Methods: We reviewed findings in 21 patients in whom tungsten coils had been used to occlude pathologic vessels, aneurysms and fistulas between 1996 and 1999. We achieved clinical follow-up, and measured renal and hepatic function, in 14 of the 21 patients. Results: Decreased radiopacity of the coils was observed in 9 of 13 patients who had follow-up fluoroscopy during repeat cardiac catheterization. Repeat angiography of the vessel occluded by the coil was performed in 7 patients, 5 of whom showed recanalization. Levels of tungsten in the serum were analyzed 6 to 35 months after implantation of coils in 8 patients. The mean concentration was 6.43 µg/l, with a range from 2 to 14.4 µg/l, normal values being less than 0.2 µg/l. Conclusion: Tungsten coils may dissolve over time and lead to markedly elevated levels of tungsten in the serum, with recanalization of previously occluded vessels. Despite lack of clinical and laboratory data in patients with elevated levels of tungsten in the serum, our study suggests that the clinical use of mechanically detachable coils produced from tungsten should no longer be recommended.

To clarify the contribution of afterload to left ventricular performance after repair of mitral regurgitation, we evaluated echocardiographically 8 children who had undergone surgical repair for isolated congenital mitral regurgitation. We examined the relationship between left ventricular systolic function and preload, afterload, and contractility. The left ventricular systolic function was strongly correlated with the afterload after the surgical repair. In the postoperative state, reducing afterload by vasodilators could be a useful means of treating cardiac failure, in addition to using catecholamines to increase the contractility.

Background: Perceived correlation between the coronary arterial anatomy in patients with complete transposition, and the outcome of the arterial switch procedure, has made preoperative identification of their patterns standard practice. Purpose: Our purpose was to assess the accuracy of preoperative echocardiographic identification of coronary arterial patterns, to evaluate the necessity of preoperative imaging by angiography, and to determine the impact of the coronary arterial anatomy on outcome. Methods: We reviewed the medical records of all patients referred for an arterial switch operation between August 1995 and January 2000. The anatomy as described at the time of the operation using the Leiden convention was compared to the preoperative echocardiographic and angiographic findings. Results: The procedure had been performed in 67 patients, at a mean age of 9 days, with a range from 3 days to 15 months. In 42 patients, the ventricular septum was intact, while 21 patients had a ventricular septal defect, and the other four had double outlet right ventricle with the aorta anterior and rightward. In 52 patients, the left coronary artery arose from sinus #1, and the right from sinus #2. In 8 patients, the interventricular branch of the left coronary artery arose from sinus #1, with the circumflex coronary artery arising together with the right coronary artery from sinus #2. In three patients, all three coronary arteries arose from sinus #1, while in the remaining individual patients, a large conal branch arose with the left coronary artery from sinus #1, the right coronary and left anterior descending arteries arose from sinus #1, all three coronary arteries took origin from sinus #2, and the left anterior descending and right coronary artery arose from sinus #1 with no circumflex coronary artery identifiable, respectively. In two patients (4%), we identified an intramural coronary arterial course. Echocardiography and angiography were comparable (81% versus 86%) in delineating the coronary arterial anatomy. Patients with a single arterial orifice, or an atypical coronary arterial anatomy, had a slightly longer stay on the intensive care unit, and in the hospital, but showed no difference in mortality. In fact, there was no early mortality (70% confidence limits; 0–2.9%), while two patients died late (2.9%). Conclusion: We conclude that complex coronary arterial anatomy does not preclude a successful arterial switch procedure, although patients with a single coronary artery, or other arterial patterns, had a slightly longer hospital course. Preoperative echocardiographic evaluation is comparable to non-selective coronary angiography. Irrespective of complexity, nonetheless, the coronary arteries can successfully be translocated, obviating the need for preoperative coronary angiography.

In adults with Wolff-Parkinson-White syndrome, the likelihood of sudden death can be estimated based upon the presence or absence of symptoms. Comparable data in children do not exist. To date, therefore, invasive risk stratification has been used to guide management regarding radiofrequency ablation in symptomatic children. As the safety of electrophysiology study and radiofrequency ablation in children has improved, asymptomatic patients have been referred more commonly for invasive risk stratification. We sought to compare the findings from electrophysiologic studies in symptomatic children with Wolff-Parkinson-White syndrome to the findings in asymptomatic children with Wolff-Parkinson-White pattern on their electrocardiogram. Thus, we compared the findings from electrophysiologic studies carried out in patients seen at Stanford University and University of California, San Francisco, from April 1999 to February 2001 with a preexcitation pattern on their electrocardiogram. The patients were divided into three groups: 23 asymptomatic patients studied for risk stratification, 19 patients who presented with syncope, and 77 patients presenting with documented supraventricular tachycardia. Data were collected for commonly accepted invasive criterions for stratification of risk: an effective refractory period of the accessory pathway less than 270 ms, multiple pathways, septal location of pathway, and inducibility of supraventricular tachycardia. Groups were compared by chisquare. No differences were found between the groups for any of the variables. These data suggest that risk factors for sudden death, developed in studies of adult patients, are not clearly applicable to children. Further studies are needed better to define the indications for study and ablation in children.

We conducted a retrospective study at the Department of Paediatric Cardiology of the University Hospital Centre Rebro, Zagreb, over the period from 1988 to 1998, so as to assess the epidemiology of childhood cardiomyopathies. The patients were categorized according to the guidelines of the Task Force on Cardiomyopathies of the World Health Organization and the International Society and Federation of Cardiology. We identified 121 infants, children and adolescents as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 patients examined in our outpatient clinics for paediatric cardiology. Of the patients, 50 were female (41.3%) and 71 were male (58.7%). The cardiomyopathy was of the dilated variant in 52 patients (42.9%), with 43 patients (35.5%) having hypertrophic cardiomyopathy, and 6 patients (4.8%) identified with restrictive cardiomyopathy. We encountered no patients with arrhythmogenic right ventricular cardiomyopathy. In nine patients (7.4%), it proved impossible to classify the cardiomyopathy. We placed 11 patients (9.0%) in the group of specific cardiomyopathies. Most of those with dilated cardiomyopathy had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4–2.47). There were no statistically significant differences in the incidences of dilated as compared to hypertrophic cardiomyopathy (Z 0.923, p = 0.1779), but we encountered a significantly lower occurrence of restrictive cardiomyopathy (Z 6.044, p < 0.001). Of those with hypertrophic cardiomyopathy, 15 patients (34.8%) had the asymmetric variant, while 28 patients (65.2%) exhibited the concentric form. During the period of follow-up, 10 patients died, 4 with dilated cardiomyopathy, 4 with hypertrophic cardiomyopathy, 1 with restrictive cardiomyopathy, and 1 with a specific cardiomyopathy. We encountered 12 (9.9%) patients who, besides cardiomyopathies, also suffered from neuromuscular disorders. Most of these had dilated cardiomyopathy. Mitochondrial disorders, in contrast, were more frequently found in patients with hypertrophic cardiomyopathy.

Purpose: Nitinol, a nickel-titanium alloy, is a valuable material in the construction of interventional endoluminal devices because of its biocompatibility, super elasticity, high resiliency and shape memory. The possibility of nickel toxicity has been raised with devices constructed of Nitinol. Our investigation examines the long-term corrosive behavior of this alloy in experimental and biological environments. Methods: We performed three levels of study. Microscopic examination was made of 64 devices of various sizes, randomly selected from 240 Amplatzer® Septal Occluders that had been exposed to saline solution at 37°C for fourteen months. All samples were studied by electron microscopy ranging from 50 to 5000 times magnification. We also studied microscopically 3 Amplatzer® devices explanted 18–36 months after implantation in dogs, and 2 Amplatzer Septal Occluders removed from patients 18 months (cardiac transplant) and 19 months (died of causes unrelated to device placement) after implantation, which were examined grossly and by electron microscopy up to 5000 times magnification. We then measured the levels of nickel in the blood using inductive plasma mass spectroscopy in 19 patients with implanted Amplatzer® devices, making measurements before and 6 months after implantation. Results: Electron microscopy showed an intact titanium oxide layer with no evidence of corrosion in vitro and in vivo. One explanted device in direct contact with the platinum leads of a pacemaker for eighteen months showed minor pitting of the titanium oxide layer believed to be galvanic in nature. No wire fractures were found in vitro after cycle testing with 400 million cycles, nor in devices taken from the animals and humans. Biochemical studies showed no significant elevation of levels of nickel levels after implantation. Conclusion: Nitinol wire of Amplatzer® septal occlusion devices is resistant to corrosion when exposed to physiologic saline solution, and in experimental animals as well as humans. A device in contact with a platinum pacemaker electrode developed minimal pitting of the titanium oxide layer, believed to be galvanic in nature and of no structural or clinical significance. There is no increase of concentrations of nickel in the blood of patients who have received Amplatzer® nitinol devices. These favorable testing results reveal that nickel-titanium is an inert, corrosion resistant alloy.

Objectives: To evaluate difficulties in daily life, and satisfaction with level of knowledge about their disease, in patients with congenital cardiac disease in order to improve counselling. Methods: A self-administered questionnaire was completed by 80 patients with mild, and 76 with complex, congenital cardiac disease. They were aged from 17 to 32 years. Results: Even patients with only mild malformations experienced difficulties related to their disease, but being found in only 11%, these were significantly less than those uncovered in 87% of those with complex disease (p < 0.001). Those patients with complex malformations frequently felt restricted in choices because of their disease in areas such as sport (59%), employment (51%), and education (34%). Other difficulties reported were: paying a higher premium for life insurance (29%), having to give up on a sport (28%), and being excluded from a job (18%). Depending on the item, between one-fifth and two-thirds of participants reported gaps in knowledge, most frequently for “causes of congenital cardiac disease”, “future consequences”, and “family planning”. For 53% of those with mild anomalies, and 93% of severely affected patients, the cardiologist is the most important source of information. Conclusions: A minority of adults with mild, and a majority of those with complex congenital cardiac disease report difficulties in daily life. A substantial number of these patients feel that they have an inadequate level of knowledge about their disease. Our results suggest the need for a specific programme of counselling.

Bilateral arterial ducts represent an uncommon form of pulmonary or systemic blood supply associated with complex congenital cardiac disease. We sought to determine the role of high-resolution cross-sectional echocardiography and color flow Doppler ultrasonography in assessing this condition, as well as to describe the management and outcome in a group of patients. A retrospective review was conducted of 11 newborns identified over a 16-year period as having bilateral arterial ducts. Pulmonary atresia associated with non-confluent pulmonary arteries was the dominant lesion, with the heterotaxy syndrome also frequently being recognized. Echocardiography best identified the source of blood supply to either the pulmonary or systemic circulations, allowing differentiation from collateral vessels. Stenosis of the right or left pulmonary artery at the initial site of ductal insertion needs careful evaluation on follow-up. Management of patients with this condition remains a challenge, as indicated by the poor outcomes observed in our series.

Isolated non-compaction of the ventricular myocardium is characterized by numerous and prominent trabeculations and deep intertrabecular recesses. This rare disease is due to an arrest of myocardial morphogenesis. Most cases, when seen in children, are associated with obstructive malformations. Isolated non-compaction is even rarer in childhood, and affects predominantly the myocardium of the left ventricle. Morbidity and mortality resulting from cardiovascular complications is high. In most cases, transplantation is the final option. To our knowledge, this rare cardiac malformation has yet to be diagnosed in the fetus. We report here two sporadic cases, one male and one female, and 2 familial cases, both male, which were diagnosed prenatally and followed by fetal echocardiography. Our study indicates that isolated non-compaction is a primary disorder of early fetal development. Our cross-sectional echocardiographic examinations revealed a fetal cardiomyopathy, with prominent and numerous trabeculations and deep intertrabecular recesses of the myocardium at the apex of the ventricles. In contrast with postnatal experience, we found isolated non-compaction mostly in the right ventricle. Systolic dysfunction was found in all cases. The diagnosis was confirmed by histology in 3 fetuses dying with cardiac failure, and by postnatal cross-sectional echocardiography in the fetus who survived. Two male fetuses belonged to a family in which 3 individuals were subsequently found to be affected. We discuss the issues of prenatal diagnosis, natural history, and myocardial histology.

A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Followup angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. Angioplasty has only partially relieved these obstructions. The pathology and possible etiology are reviewed.

We performed an electrophysiological study, using non-contact mapping, in an 8-year-old girl weighing 39.9 kg who had suffered recurrent symptomatic episodes of exercise-induced non-sustained ventricular tachycardia. Color-coded isopotential maps of the ventricular tachycardia identified the area of earliest endocardial activation high and anterior in the right ventricular outflow tract. Although partial deflation of the balloon was required to position the ablation catheter at the earliest site of activation, this site was still identified accurately, as demonstrated by termination of the ventricular tachycardia and ectopy upon mechanical pressure, as well as application of radiofrequency current.

In this young patient, precise mapping of the earliest endocardial activation using the non-contact mapping system was safe and effective, allowing successful radiofrequency ablation of the tachycardia.

Haemoptysis was the presenting symptom in a 27-year-old male. He had undergone a Mustard operation for connection of complete transposition at the age of 2 years. For 6 months prior to admission, he had complained of dyspnoea without chestpain, and swelling of the fingers during hard physical work. Chest radiography and computer tomographic scans showed normal features of the pulmonary parenchyma, and no sign of cardiomegaly or vascular stasis. Fiberoptic bronchoscopy demonstrated a blood clot in the upper right bronchus, without any associated abnormalities of the bronchial tree. Doppler echocardiography showed obstruction of the superior caval vein, which was verified by cardiac catheterization. Balloon dilation at the site of obstruction increased the diameter of the vein from 0.5 to 1.7 cm, and the mean pressure in the superior caval vein was reduced significantly from 18 to 10 mmHg. The haemoptysis did not recur, and no complaints of dyspnoea or swelling of fingers during physical activity was reported 2 years later. Transthoracic echocardiography undertaken at this time revealed no obstruction of the superior caval vein. We conclude that hemoptysis is a rare complication of increased venous pressure in the upper body of patients with superior caval venous obstruction, which can be treated by balloon dilation or stenting.

We describe a patient with a coronary arteriovenous fistula, atresia of the orifice of the coronary sinus, and persistence of the left superior caval vein. Depression of her ST segments was revealed by exercise electrocardiography long after the initial surgical reconstruction of the coronary sinus.