We evaluated 6 patients with an anomalous subaortic left brachiocephalic vein. All cases were associated with some form of congenital heart disease: 4 patients had coexistent tetralogy of Fallot, one had congenitally corrected transposition, and the other an atrial septal defect with azygos connection of the inferior caval vein. Our incidence of an anomalous subaortic left brachiocephalic vein in patients with congenital heart diseases was 1.1% (6/527). It was observed in 4 out of 117 patients with tetralogy of Fallot (3.4%). Doppler echocardiography was useful in establishing the diagnosis. Furthermore, both digital subtraction angiography and magnetic resonance imaging demonstrated well the anomalous course of the vein, and were useful in making an accurate diagnosis.

We report our initial experience in three children with percutaneous transvenous closure of significant atrial septal defects. A newly developed one-piece nitinol device is delivered through a long venous sheath of 7 French calibre under fluoroscopic and transoesophageal echocardiographic monitoring. Complete closure of all defects was verified by echocardiography on the morning after the procedure. No complication occurred. In the first two patients, the device slipped sideways into the defect. This was easily discovered and corrected. Our experience shows that the Amplatzer® device is reliable, easy to implant, and presents very clearly on transoesophageal echocardiography and fluoroscopy. This makes implantation a controlled procedure. Until release, the device can easily be retracted into the sheath at any time and removed. The results suggest that closure of an atrial septal defect with this device is feasible, rapid, and safe.

We studied echocardiographically 17 children with depletion of carnitine due to treatment over 1–3 years with antibiotics containing pivalic acid. When the children were depleted of carnitine, the left ventriclar posterior wall was significantly thickened in diastole when compared to a reference group. Six months later, after normalisation of the concentrations of carnitine, the left ventricular posterior wall was significantly thinner and did not differ from the reference group. We conclude that drugs containing pivalic acid, when given for several months, decrease the stores of carnitine to levels where cardiac involvement occurs.

A bidirectional cavopulmonary shunt is performed in patients with single ventricle physiology to improve or maintain systemic oxygenation while decreasing the workload of the heart. During a period of 10 years, bidirectional cavopulmonary shunts were performed in 50 patients at our institution. The procedure was performed with an additional source of pulmonary flow through the pulmonary valve or a systemic to pulmonary shunt in 27 patients and without an additional source of pulmonary flow in 23 patients. Preoperative and postoperative chest radiographs, pulmonary angiograms, oxygenation, hemodynamic measurements, morbidity, and mortality were reviewed to determine whether a bidirectional cavopulmonary shunt with an additional source of pulmonary flow is an acceptable interim or final stage of palliation. Systemic oxygenation was improved only in patients with an additional source of pulmonary flow. The outcome of the bidirectional cavopulmonary shunt was not adversely affected by the presence of additional pulmonary flow. Twenty-five patients subsequently underwent an atrial to pulmonary or total cavopulmonary anastomosis. Oxygenation was improved by more definitive palliation; however, late complications of stroke, protein losing enteropathy, and arrhythmias requiring pacemaker therapy were more prevalent. In conclusion, systemic oxygenation can be improved by performing the bidirectional cavopulmonary shunt with an additional source of pulmonary flow through the pulmonary valve or a systemic to pulmonary shunt without an adverse effect on hemodynamic measurements, pulmonary arterial growth, morbidity, or survival. The bidirectional avopulmonary shunt may be an acceptable final stage of palliation in some patients with a functionally single ventricle.

Only small groups of children, and especially infants, with hypertrophic cardiomyopathy have been analysed thus far. The clinical features, and the evolution, of this potentially heterogeneous disease, therefore, remain to be clarified. We describe the clinical profile of 35 patients with hypertrophic cardiomyopathy, 18 being younger than 1 year when first seen. Electrocardiography, chest radiography and echocardiography (m-mode, cross-sectional and color-Doppler) were performed in all patients. Ambulatory electrocardiographic monitoring was performed in 20 patients, and cardiac catheterization in 5 patients. The duration of follow-up was 6.6 years, and ranged from 1 month to 15 years. First-degree relatives of 9 infants and 13 children underwent complete echocardiographic evaluation.

Inheritance, along with presence of congestive heart failure, cyanosis, cardiac enlargement and right ventricular hypertrophy proved to be different in infants who presented with hypertrophic cardiomyiopa-thy as compared with those presenting in childhood. In contrast, echocardiography showed that the distribution of ventricular hypertrophy was similar in the two groups. Intractable congestive heart failure, which was the commonest cause of death, was an ominous prognostic sign in infants. Older children, however, tended to die suddenly. Spontaneous resolution of severe ventricular hypertrophy occurred in four infants of non-diabetic mothers within their first 6 months of age. We conclude that hypertrophic cardiomyopathy may represent a constellation of disease entities. The observed spontaneous resolution in 4 infants may represent a new form of the syndrome which carries a good prognosis.

Assessment of right ventricular volume and function is important in most congenital heart diseases before and after corrective or palliative surgery. Since transthoracic echocardiography is often substituted by transesophageal echocardiography in the perioperative setting, it is useful to compare transesophageal echocardiography with transthoracic echocardiography as performed preoperatively. We compared right ventricular volumes as calculated using these two methods from a four-chamber view in 21 children and adults with atrial septal defect. For right ventricular end-diastolic volumes of less than 70 ml, and end-systolic volumes of less than 40 ml, a close correlation was found between the techniques (r=0.99 and r=0.91, respectively), with a small degree of underestimation by transesophageal echocardio­graphy. For values larger than 70 nil and 40 ml, respectively, correlation decreased (r=0.41 for end-diastolic volumes and r=0.48 for end-systolic volumes) and underestimation of volume by transesophageal echocardiography increased. Underestimation of right ventricular end-diastolic volumes increased with increasing body surface area (r=0.74), and with progressive right ventricular enlargement (r=0.63). In patients with a body surface area of more than 1m2, the largest end-diastolic right ventricular length determined by transthoracic echocardiography was significantly longer than that derived by transesophageal echocardiography (p<0.001), whereas in smaller patients there was no significant difference between the two methods (p>0.1). If right ventricular volumes determined by transthoracic echocardiography using a four-chamber view are substituted by those obtained with transesophageal echocardiography in serial haemodynamic evaluation of patients with atrial septal defect, different correlation equations and, consequently, a different degree of underestimation by transesophageal echocardiography must be considered for large and small volumes. This increasing underestimation of larger right ventricular volumes seems to be based on foreshortening of the long cross-sectional axis of the right ventricle as seen in the transesophageal four-chamber view.

We report our experience using an originally designed thin walled metal-tipped arterial cannula for neonatal open heart surgery. Its external diameter is 2.1 mm and its internal diameter is 1.7 mm, it has a high internal – external diameter ratio. It was used on 36 patients, who were either neonates or small infants. In 32 of the patients, it was inserted into the ascending aorta for systemic perfusion. In the other 4, it was inserted into the brachiocephalic artery for selective cerebral perfusion during repair of the aortic arch. The relationship between the pressure drop and the bypass flow was obtained for the 7 most recently treated patients. The pressure drop at a bypass flow of 500 ml/min was 150 mmHg. Due to the high internal – external diameter ratio of this cannula, and its excellent flow characteristics, we consider it to be particularly useful in neonatal open heart surgery, while it also allows for successful selective cerebral perfusion during repair of the aortic arch in neonates.

The clinical course, noninvasive findings and outcome of 25 infants and children with idiopathic dilated cardiomyopathy were reviewed retrospectively to identify factors predictive of outcome both at diagnosis and at short-term follow-up. Patients, stratified by clinical status at last visit, were assigned to groups encompassing those who were asymptomatic and receiving on medication, those patients who were symptomatic or needed medication to control symptoms, and those who were dead, awaiting heart transplantation, or had undergone transplantation. Older age at diagnosis was strongly associated with poor clinical outcome (p=0.005), as was the presence of arrhythmias at the time of diagnosis (p=0.008) and at short-term follow-up-(p=0.003). Echocardiographic studies at diagnosis failed to predict eventual clinical outcome, although patients who ultimately recovered and did not need medications tended to demonstrate improvement in or resolution of echocardiographic abnormalities within 6 months of diagnosis.

Some patients with so called “secundum” atrial septal defect within the oval fossa show a large defect on the echocardiogram, although they do not have all the classical clinical features. Until now, a large atrial septal defect was thought to be characterized by a large shunt (functional defect). Experience indicates, nonetheless, that such large defects in size (anatomical defect) are not always accompanied by large shunts. In order to assess how often the classical clinical features of an atrial septal defect exist, and to investigate whether the surgical indication for closure of the defect are anatomical or functional, we carried out a retrospective study. We evaluated the records of 161 patients, with birth dates from 1973 to 1994 (age between 0 and 21 years), so as to study the classical clinical features (history, physical examination, electrocardiogram, chest X-ray, echo-Doppler studies, cardiac catheterization, surgery). Only patients with an atrial septal defect found in isolation were included.

We discovered that the classical clinical features are often not present when there is a large defect, be it functional or anatomical. The anticipated features are present in roughly two-thirds of the patients. There is also a discrepancy between the anatomical and the functional sizes of the defect. This finding creates a fundamental problem for the future, namely the indications fot closure of atrial septal defects within the oval fossa. Further studies are needed to answer this important question

Cross-sectional echocardiography was performed on 108 healthy children (7 days – 17 years old) and 55 children (6 months - 16.5 years old) with interatrial communication. Right ventricular end-diastolic volume, end-systolic volume, stroke volume, ejection fraction, muscle volume, and the ratio of muscle to cavity were calculated on the basis of outlined cavity and myocardium of an apical fourchamber view.

In the normal subjects right ventricular end-diastolic volume, end-systolic volume, stroke volume and muscle volume correlated with body surface area (end-diastolic volume: y=12.5x+7.8x2, r=0.99; end-systolic volume: y=4.8x+3.6x2, r=0.98; stroke volume: y=7.7x+4.2x2, r=0.98; muscle volume: y=14.1x+2.9x2, r=0.97), muscle/cavity ratio (0.85±0.17) and ejection fraction (58.9 ± 6.2%) were unrelated to body surface area. In the subjects with interatrial communication, the right ventricular volumes were significantly larger (p<0.001) than the normal values with a linear relationship to the ratio of pulmonary to systemic flows.

Right ventricular volumes can be determined in normal children with acceptable repeatability using a standard apical four-chamber view. The growth related normal values provide a basis for future quantitative studies.

Accurate bedside assessment of the ratio of pulmonary to systemic flow (Qp/Qs ratio, referred to as “the flow ratio” or “the ratio”) plays an important role in the management of many congenital heart defects, especially the complexes unified by univentricular atrioventricular connections. Arterial oxygen saturation can be a misleading measure of the ratio, and may not reflect derangements until they are quite large. Theoretical analysis suggests that systemic venous oxygenation may be a better indicator of the ratio. To examine this, we created a widely patent atrial septal defect in neonatal piglets (weight =4–6.5 kg, n=6). Snares aruond the aorta and pulmonary trunk were adjusted to alter the flow ratio from 0.1 to 6.5. Venous oxygen saturations, measured in the mid-inferior caval vein, were at a maximum at a ratio about 1, and declined rapidly with increases or decreases in the ratio beyond a limited range. The venous oxygen saturation was found to vary much more than arterial oxygen saturation, with arterial oxygen saturation only falling when the ratio dropped below 0.5. Oxygen delivery (Oxygen Content x Cardiacoutput) was found to parallel closely systemic venous oxygen saturation, and was at a maximum at the same ratio that produced a maximum value of systemic venous oxygen saturation. The study suggests that systemic venous oxygen saturation provides a better estimate than does systemic arterial oxygen saturationof the flow ratio and oxygen delivery. Interventions that maximize systemic venous oxygen saturation should maximize oxygen delivery, and determination of systemic venous oxygen saturation should be a helpful addition in managing children with a number of congenital heart defects.

The availability of echo Doppler cardiography enables monitoring of the natural course of congenital aortic valvar stenosis more adequately than before. Between 1986 and 1993, 129 children with such stenosis were examined echocardiographically, 83 of them over a prolonged period with repeated studies of at least one per year. The pressure gradient between the left ventricle and ascending aorta, as well as the left ventricular wall thickness, were measured. When the pressure gradient detected increased to more than 60 mmHg, the patients were treated by balloon valvoplasty or surgical valvotomy. The last examination prior to intervention was taken as the final measurement.

We found a significant increase (≥10 mmHg) in the measured pressure gradient in 40 of the 83 patients (48%). The onset of severe stenosis was not observed at a specific age, rather the stenosis was found suddenly to increase in severity at any age. The gradients measured in children younger than 2 years of age, however, increased twice as rapidly as those measured in the older children. A high gradient was not always accompanied by evidence of left ventricular hypertrophy.

Based on our findings, we suggest that, since a sudden increase in severity of aortic valvar stenosis might be expected, the pressure gradient should be measured once every 6 months in children younger than 2 years of age and once a year after that age

We report on a case of a newborn with echocardiographic diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. Intraoperative inspection demonstrated a proximal anomalous course of the right coronary artery but a normal origin from the right sinus of Vansalva of the Aorta. We suggest a complete investigation, including coronary angiography, when isolated and rare congenitaj malformations of the coronary arteries are suspected.

Patients undergoing the modified Fontan procedure may develop low cardiac output postoperatively. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate the influence of triiodothyronine supplementation. Ten consecutive patients under-going the Fontan procedure were administered intravenous triiodothyronine (0.4 mcg per kg) following surgery. Clinical outcome and thyroid hormone profiles were assessed and then compared to a previous series of patients undergoing the Fontan procedure who had not received triiodothyronine supplementation. Both groups initially demonstrated marked decreases in serum free triiodothyronine levels. The group which received triiodothyronine supplementation demonstrated a more rapid return of serum triiodothyronine levels to baseline [259±17 vs 121±15 pg/dl (p<0.05) on the fifth postoperative day and 336±18 vs 178±12 pg/dl (p<0.05) on the eighth day]. In addition, patients receiving supplemental triiodothyronine demonstrated more rapid recovery of total triiodothyronine, free thyroxine, total thyroxine and thyroglobulin levels. The group which received triiodothyronine supplementation had a shorter length of hospital stay [9±2 vs 14±3 (p<0.05)] as compared to patients who did not receive exogenous triiodothyronine. The results of this study demonstrate that triiodothyronine supplementation aids in the recovery of thyroid hormone levels following Fontan procedure. This endocrinologic finding correlated with improved clinical outcome.

Over a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.

Anomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly. Diagnosis is usually made at autopsy or incidentally in asymptomatic adults, but cardiopul-monary arrest and sudden death have been reported. The angiographic findings have been well described but the echocardiographic findings are less well documented.

This report describes the echo and colour Doppler findings in a 3-year-old child with an anomalous right coronary artery arising from the pulmonary trunk, as well as observations on myocardial perfusion demonstrated by nuclear angiography.