Coronary sinus atrial septal defects are the rarest defects of the atrial septum comprising <1% of the five different types of atrial septal defects. Despite the widespread adoption of percutaneous device closure of secundum atrial septal defects, the published experience with percutaneous device closure of coronary sinus atrial septal defects is limited to only a few isolated case reports because of uncertainty regarding safety and efficacy. Open-heart surgical repair remains the treatment of choice for coronary sinus atrial septal defects, although this may not be the only treatment option in selected cases. Herein we describe our own experience with two patients with different clinical presentations and our method of successful percutaneous coronary sinus atrial septal defect closure in each. We then present a review of the anatomic spectrum of coronary sinus atrial septal defects along with a review of contemporary surgical and percutaneous device treatment.

Although the brain is the organ most sensitive to total bodily ischemia, and probably also most sensitive to the deleterious effects of cardiopulmonary bypass, it is also the most difficult organ in which to quantitate function accurately. Clinical studies allow sensitive neuropsychometric testing, as discussed elsewhere in this issue (see Wernovsky et al, p. 308). This is not possible in studies using animals. Even testing of gross neurological integrity in animals is complicated by the associated dysfunction of other organs which accompanies the insult of cardiopulmonary bypass with or without circulatory arrest on the whole body. For example, how should one evaluate an animal undergoing neurological testing which is unable to walk or eat, perhaps because of severe cardiorespiratory or gastrointestinal dysfunction following cardiopulmonary bypass and circulatory arrest. What is the contribution of neurological dysfunction to this state? Most importantly, how is the animal scored which does not survive, even though this may be a result of multiorgan dysfunction?

Clinical and Doppler echocardiography were performed in 65 patients who had undergone ligation of the arterial duct between January 1978 and December 1991. Of the patients, 12 (18%) patients had residual patency as demonstrated by color Doppler, while three (4.6%) of these had continuous murmurs. There was no association between residual patency and the age at surgery, the weight at surgery or the follow-up interval.

We sought to analyse the long-term follow-up after primary repair of tetralogy of Fallot in infancy in the first year of life, paying particular attention to growth of the pulmonary arteries and the need for reintervention. We performed a combined retro- and prospective echocardiographic study, including measurements of the pulmonary valve and right and left pulmonary arteries, indexed to the square root of body surface area, in 62 patients prior to primary repair, 18 to 24 months after this event, and at the most recent follow-up, with a mean of 80.4 ± 24 months. Of these, 38 patients, with an age at operation of 5.0 ± 3.4 months, had presented preoperatively with hypoxic spells or increasing cyanosis. The remaining 24 patients had been asymptomatic, with adequate flow of blood to the lungs. Their age at elective operation was 7.4 ± 3.0 months. A transannular patch was needed in 37 patients (63%). There were 3 early postoperative deaths (4.8%).

Cross-sectional echocardiography revealed a significantly smaller diameter for the pulmonary valve in patients who had been symptomatic preoperatively compared to the asymptomatic patients (1.09 versus 1.3 cm/BSA0.5; p = 0.019). The diameters of the right and left pulmonary arteries did not differ significantly between the groups. Examination of echocardiographic data obtained 18 to 24 months postoperatively in 43 patients revealed a significant increase in the diameter of the pulmonary arteries; 0.83 ± 0.17 cm/BSA0.5 versus 1.1 ± 0.26 cm/BSA0.5 for the diameter of the right pulmonary artery, 0.85 ± 0.2 cm/BSA0.5 versus 1.0 ± 0.25 cm/BSA0.5 for the left pulmonary artery. On comparison between individuals, 18.6% and 25.6% of the patients, respectively, did not show any change in the diameters of their right and left pulmonary arteries, whereas the increase in diameter reached or exceeded the measurements in normal controls in 55.8% and 46.5% of the patients, respectively. On recent follow-up, with a range from 56 to 147 months, no further increase in the indexed diameters of the pulmonaries could be documented in 21 of 33 patients. Of these, 9.1% and 18.2%, respectively, presented with small right and left pulmonary arteries compared with measurements obtained in normal controls. Moderate pulmonary incompetence was found on colour flow mapping in one-third. Of 56 longterm survivors, 8 (14%) had required reinterventions, which were surgical in 6 and achieved by transcatheter techniques in the other 2 patients. Thus, primary correction of tetralogy of Fallot in infancy, with restoration of normal pressures and flows, resulted in sustained increase in the diameters of the right and left pulmonary arteries. It allowed for early normal development of the proximal pulmonary arterial system in most patients regardless of their age and symptomatic status at operation. Patients with persistent subnormal diameter of the pulmonary arteries did not present with significantly elevated right ventricular pressure. Early one-stage repair of tetralogy of Fallot in infancy was associated with a low rate of reinterventions.

The Fontan procedure provides a means of separating the systemic and pulmonary circulations in patients with congenital heart defects characterized by a functionally single ventricle. The procedure has undergone numerous changes since its introduction 25 years ago. Technical modifications have now increased its applicability to virtually all forms of univentricular atrioventricular connection, as well as many hearts with biventricular connections. More recently, philosophical changes have been introduced to address increased rates of morbidity and mortality observed in certain subsets of patients. These changes appear to have improved short- and midterm results. The purpose of this review is to offer our strategy for management of such patients. We will discuss the interrelationship between the bidirectional Glenn and Fontan procedures. We will also focus on a number of observations concerning the possible role of accessory pulmonary blood flow, aortopulmonary collateral arteries, and endocrinologic changes.

We report amiodarone-induced pulmonary toxicity in an 18-year-old who had undergone corrective surgery for tetralogy of Fallot 4 years earlier, and was treated with amiodarone because of recurrent malignant postoperative ventricular tachyarrhythmias. Toxicity was recognized on the basis of clinical features, chest X-ray, high-resolution contrast enhanced computerized tomography, and resolution of the findings subsequent to withdrawal of amiodarone and treatment with steroids. pulmonary toxicity due to amiodarone, as far as we know, has not been reported in children and young adults, and its occurence even in young adults requires wider appreciation.

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

Scimitar syndrome is a rare congenital anomaly that affects classically the right lung and the heart. We present a rare variant that involves both lungs in association with totally anomalous pulmonary venous connection, horseshoe lung, and right pulmonary cyst.

The recent developments in imaging techniques have created new opportunities to give an accurate description of the three-dimensional morphology of vessels. Such three-dimensional reconstruction of anatomical structures from medical images has achieved importance in several applications, such as the reconstruction of human bones, spine portions, and vascular districts.

Over the past 20 years, the successes of neonatal and infant surgery have resulted in dramatically changed demographics in ambulatory cardiology. These school-aged children and young adults have complex and, in some cases, previously unexpected cardiac and non-cardiac consequences of their surgical and/or transcatheter procedures. There is a growing need for additional cardiac and non-cardiac subspecialists, and coordination of care may be quite challenging. In contrast to hospital-based care, where inpatient care protocols are common, and perioperative expectations are more or less predictable for most children, ambulatory cardiologists have evolved strategies of care more or less independently, based on their education, training, experience, and individual styles, resulting in highly variable follow-up strategies. We have proposed a combination proactive–reactive collaborative model with a patient’s primary cardiologist, primary-care provider, and subspecialists, along with the patient and their family. The goal is to help standardise data collection in the ambulatory setting, reduce patient and family anxiety, increase health literacy, measure and address the non-cardiac consequences of complex cardiac disease, and aid in the transition to self-care as an adult.

Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinico-pathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.

Cognitive, functional, educational achievement and behavioural measures were employed to assess neurobehavioral status in 57 of 60 participants who were initially enrolled in the Baltimore–Washington Infant Study, and who survived surgical correction of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). Charts were reviewed to investigate the relationship between birth variables, surgical strategy and developmental outcomes. Higher preoperative weight was associated with better outcomes on the Stanford–Biner Short-term Memory subtest, while lower preoperative oxygen tension was associated with better outcomes on the Abstract/Visual Reasoning subtest and a test of Visual–Motor Integration. Longer total bypass time was associated with poor outcomes on the Short-term Memory subtests. Higher average flow rates during cooling and rewarming were associated with higher scores in the test of short term memory but poorer outcomes on a test for visual motor integration. Longer cooling times were associated with higher scores on the test for Visual–Motor Integration. Patients suffering seizures scored lower on the Stanford–Biner Composite, as well as in their tests of achievement. The data indicate that non-verbal. skills may be particularly sensitive to variations in surgical strategies employed to correct complete transposition. Overt neurological events, such as seizures, were related to global deficits in intellectual functioning. Prospective studies evaluating systemic variations in surgical procedures and attempts to prevent and manage perioperative neurological events are important for further investigation of neurodevelopmental outcomes in children surviving surgical correction.

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.