Objectives: To review all cases of atrioventricular septal defects in Northern Ireland from January 1990 to February 1999, examining clinical and morphological features, management, and outcome. Methods: A retrospective case note analysis of 106 subjects with comparisons between subgroups. Results: An atrioventricular septal defect was part of a more complex abnormality in 50 of the patients (47%). Down's syndrome was present in 57 (54%). Cardiac surgery was performed in 81%. The defects were unrestrictive in 69 patients (65%), 45 of whom had Down's syndrome. Complex associated abnormalities existed in 36 patients, and 10 of these died without cardiac surgery. Operative mortality was 9.5% for those with co-existing Down's syndrome group, and 14.3% for the chromosomally normal patients. The ventricular components of the septal defect were restrictive in 23 patients (22%), with 9 having Down's syndrome. Spontaneous closure occurred in more than half of these patients. Mortality was zero. The septal defect was exclusively at atrial level in 14 patients (“primum” defects – 13%), and 3 of these had Down's syndrome. Operative mortality was again zero. Median duration of postoperative follow-up was 3 and a half years. Overall, moderate to severe left atrioventricular valvar regurgitation was observed postoperatively in 23% at follow-up. Conclusions: Mortality was highest in the atrioventricular septal defects with an unrestrictive ventricular component. Uncomplicated cases had good outcomes. Patients without Down's syndrome tended to have more associated cardiac abnormalities, and to have more postoperative arrhythmias. Approximately half of the defects with restrictive ventricular components closed spontaneously. Moderate postoperative left atrioventricular valvar regurgitation was commonest in patients with the defect exclusively at atrial level.

Background: The survival for patients with atrioventricular septal defect has improved markedly over the last decades and, during the same period, the survival of children with Down's syndrome has also increased. The aim of our study was to investigate long-term survival in patients having atrioventricular septal defect with common valvar orifice, but without associated significant congenital heart defects, in the setting of Down's syndrome, comparing the findings to those in chromosomally normal children with the same malformation. Methods and results: In a population-based retrospective study, we scrutinised the medical records from 801 liveborn children with atrioventricular septal defect born in Sweden during the period 1973 through 1997. Data on gender, presence or absence of Down's syndrome, associated congenital heart defects, date of birth, operation and death were recorded and followed up until 2001. An isolated atrioventricular septal defect with common atrioventricular valvar orifice was present in 502 children, of whom 86% had Down's syndrome. We found a significant reduction over time in age at operation, and in postoperative mortality at 30 days, from 28 to 1%. Using a multiple logistic regression model, we found no significant differences in mortality between genders, nor between those with or without Down's syndrome. Early corrective surgery could not be identified as a significant independent factor for survival. The 5-year postoperative survival in patients with Down's syndrome increased from 65% over the period from 1973 through 1977, to about 90% in the period 1993 through 1997, and the same trend was observed in chromosomally normal patients. Conclusions: Survival in uncomplicated atrioventricular septal defect with common atrioventricular valvar orifice has greatly increased, and surgical correction is now equally successful in patients with Down's syndrome and chromosomally normal patients, and for both genders. Death in connection with surgery is no longer the major threat, and focus must now be on long-term follow-up.

Objectives: Our study evaluates hospital survival following prenatal identification of candidates for the Norwood procedure, and the impact of prenatal diagnosis on survival, preoperative stability, and postoperative morbidity. Methods: We reviewed records of all patients who were identified prenatally as candidates for the Norwood procedure, and compared them to all postnatally diagnosed patients who underwent the Norwood procedure between August 1995 and May 2002. Results: Of the 98 patients studied, 45 (46%) were diagnosed prenatally. Of these, 35 underwent the Norwood procedure, 29 (83%) of who survived. Thus, 29 of 45 (64%) patients survived from prenatal diagnosis to discharge following the Norwood procedure. Of the 53 postnatally diagnosed patients who underwent the Norwood procedure, 42 (79%) survived. Prenatal diagnosis was not associated with improvement in survival, preoperative stability, or postoperative morbidity. By multivariate analysis, ascending aortic diameter equal to or greater than 2 mm (p = 0.01), and gestational age 36 weeks or greater (p = 0.01) independently predicted survival. Based on this, patients were stratified into groups at low risk, consisting of 69 patients, and at high risk, consisting of 19 patients. Prenatal diagnosis was unassociated with improved survival in either group. Results were unchanged when the analysis was restricted to patients with hypoplasia of the left heart. Conclusion: From the time of prenatal diagnosis, 64% of patients survived to discharge following the Norwood procedure. Prenatal diagnosis did not affect preoperative stability, survival or postoperative morbidity. This remained the case after stratifying patients by risk, or restricting analysis to patients with hypoplasia of the left heart. Ascending aortic diameter and gestational age independently predicted survival.

Aortic coarctation can now be repaired surgically with excellent results. Even though rare, injury to the spinal cord resulting in paraplegia remains a major concern. Preoperative evaluation showing the absence of collateral circulation is valuable in order to introduce protective actions. This report describes our experience using a temporary bypass from the ascending to the descending aorta bypass in children undergoing surgical correction of aortic coarctation in the setting of poorly developed collateral circulation.

Between 1990 and 2002, we undertook direct surgical repair in 56 patients with isolated aortic coarctation, 20 as neonates, 11 as infants, and 25 during childhood. From 1998 onwards, we introduced preoperative evaluation of the collateral circulation with magnetic resonance imaging. From that time, we placed a temporary bypass from the ascending to the descending aorta, using a polytetrafluoroethylene tube of 4 to 8 mm diameter, whenever distal pressures were shown to be 25 mmHg or less after test clamping, or when magnetic resonance imaging revealed absence of collateral circulation.

We found excellent correlations between the direct intra-operative measurements of distal pressure and the findings at magnetic resonance imaging. Following introduction of the temporary bypass, we observed no neurological complications, nor were there any complications related to bypass. Freedom from restenosis was 96%.

Preoperative magnetic resonance imaging, therefore, can accurately visualize poor collateral circulation in children with aortic coarctation. The use of a temporary bypass can possibly eliminate the risk of neurological sequels following direct repair of coarctation in children with poorly developed collateral circulation. The temporary bypass is both easy to apply and safe.

Positional cyanosis is an uncommon finding in young patients. We report three infants who presented with positional cyanosis due to a pedunculated tumour in the right heart. Arterial desaturation was the result of right-to-left shunting at the level of the oval foramen caused by obstruction and/or insufficiency of the tricuspid valve. The obstruction at the level of the tricuspid valve was variable because of the pedunculated nature of the tumours, which gave them considerable mobility. Hence, the degree of right-to-left shunting was dependent on the position of the patient. In all the patients, surgical resection of the tumours resolved the cyanosis.

We inserted covered Cheatham-Platinum stents in 4 patients, ranging in age from 12 to 19 years, who weighed between 45 and 94 kg. All the patients had aortic coarctation, with surgical repair having been attempted previously in one, and with balloon dilation having been performed as the primary treatment in two, resulting in formation of aneurysms. The fourth patient had not received any treatment. The gradients were reduced from 10 to 40 mmHg before insertion of the stent to 0 to 5 mmHg after stenting. No complications were encountered. All the patients are well at an interval of 3 to 14 months after stenting.

Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The mean period of follow-up for the 41 living patients was 16.5 years. An arrhythmia of variable severity appeared during the evolution of 27 patients (66%). At present, only 7 cases are in the functional class III or IV of the grading system of the New York Heart Association, but 25 patients (61%) needed some medical treatment, meanly for arrhythmic events. Thus, fetal and neonatal presentation of Ebstein's malformation is associated with a poor outcome. Moreover, the echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality. Arrhythmia and need of medical treatment are common in older children and adults. Survival after surgical treatment is associated with a good outcome.

Purpose: When there is partially anomalous pulmonary venous connection to the superior caval vein, intracardiac repair alone can result in obstruction. Although the Warden procedure involving translocation of the superior caval vein is commonly performed as an alternative to atriocavoplasty, follow-up of a larger number of patients in the modern era is lacking. We report and discuss the experience of a single institution with the Warden procedure for correction of partially anomalous pulmonary venous connection to the superior caval vein. Methods: Since 1995, all 16 patients presenting with partially anomalous pulmonary venous connection to the superior caval vein underwent the Warden procedure at a mean age of 7.1 ± 4.2 years, with a range from 0.2 to 14.3 years, and a mean weight of 24.7 ± 14.0 kg, with a range from 4.1 to 52.9 kg. There were 9 males and 7 females. In 8 patients, we performed 10 concomitant procedures, including closure of an atrial or ventricular septal defect in 7, and advancement of the aortic arch in the other. Results: There were no deaths, and only one episode of postoperative sinus bradycardia with intermittent junctional rhythm, which resolved spontaneously during temporary atrial pacing. All patients were discharged home in normal sinus rhythm at an average of 4.1 ± 2.2 days after the procedure, with a range from 2 to 10 days. All are currently in the first grade of the New York Heart Association up to 5.6 years postoperatively. There is currently no evidence of sinus nodal dysfunction, nor obstruction of the superior caval vein, in any patient. Conclusion: The Warden procedure for partially anomalous pulmonary venous connection to the superior caval vein produces excellent results, preserves the function of the sinus node, and should be routinely considered for the repair of this lesion.

The aim of our study is to provide data on the prevalence of disturbances of rhythm in the general population of children. Accurate estimates of true prevalence of such disturbances of rhythm from large samples are mandatory if we are to interpret properly electrocardiographic abnormalities. We analysed prevalence of disturbances of rhythm in a population of 152,322, comprised of 71,855 elementary school students, 36,692 males and 35,163 females, aged from 5 to 6 years, and 80,467 students of junior high school, 41,842 males and 38,625 females, aged from 12 to 13 years. We analysed the prevalence of premature atrial and ventricular contractions, first, second and third degree atrioventricular block, incomplete and complete right bundle branch block, Wolff-Parkinson-White syndrome, and prolongation of the QT interval. The prevalence of disturbances of rhythm in total rose with age, being found in 1.25% of elementary school students and 2.32% of junior high school students, and was higher in males than females, at 2.00% as opposed to 1.38%, both values being statistically significant at a level of less than 0.0001. Prevalences of all types of rhythmic disturbances were higher in junior high school students than elementary school students (p < 0.0001). Premature atrial and ventricular contractions and prolongation of the QT interval were higher in female than male students, at percentages of 0.089, 0.497, and 0.02 for males, and 0.123, 0.534 and 0.027 in females (p < 0.0001). In contrast, incomplete and complete right bundle branch blocks were higher in males than females, at 0.983% and 0.083% in males versus 0.410% and 0.161% in females (p < 0.0001). Disturbances of rhythm increased with age, and conduction disturbances were higher in male students than female, although premature atrial and ventricular contractions and prolongation of the QT interval were more frequent in female. These data may be useful for future comparative studies of disturbance of rhythm in children.

We describe an asymptomatic 1-year-old boy who presented a continuous murmur, and was found to have a tunnel between the right atrium and the aorta. The definitive diagnosis was established by both echocardiography and aortography, and surgical interruption of the tunnel was successful. We emphasise the rarity of this condition, and discuss its major features.

A 12-year-old boy presented with tachycardia and cardiomegaly. An electrocardiogram showed atrial flutter with a ventricular rate of 170 beats/min. The echocardiogram showed left ventricular dilation, with decreased contractility. Electromyography confirmed the diagnosis of myotonic dystrophy. The atrial flutter was converted to sinus rhythm by electroversion, leading to restoration of normal cardiac function. Our case illustrates that atrial flutter can be an early sign of the cardiac complications of myotonic dystrophy in childhood.

An acute injury to a coronary artery was recognized during the surgical construction of the Fontan circulation. Surgical manipulation of the site of injury was not successful in restoring normal myocardial blood flow. A stent was therefore placed intraoperatively under direct vision, with restoration of normal coronary arterial flow acutely and at short-term follow-up.

We report a patient in whom the circumflex coronary artery arose anomalously from the right aortic sinus. The patient also had atrial and ventricular septal defects in the setting of Eisenmenger physiology. We recommended transplantation of the heart and lungs.

As far as we are aware, a bicuspid aortic valve has not previously been reported in the setting of tetralogy of Fallot and pulmonary atresia. We describe this association in a newborn who presented with a murmur and cyanosis. Echocardiography showed tetralogy of Fallot with pulmonary atresia, and also a moderately stenotic bicuspid aortic valve. The patient underwent open-heart surgery guided by transesophageal echocardiography. Postoperatively, there was only mild obstruction across both outflow tracts. We have also reviewed the pertinent data from our Jesse E. Edwards Registry of Cardiovascular Disease to establish the incidence of bicuspid aortic valve in the setting of obstruction of the right ventricular outflow tract, finding the association in no patients with pulmonary atresia and tetralogy of Fallot, in 0.7% of those with tetralogy of Fallot and pulmonary stenosis, but in 6.6% of those with pulmonary atresia and intact ventricular septum.