Coronary artery fistulas are uncommon in children. We conducted a retrospective review of the case notes of 17 children who presented to our institution with the diagnosis of a coronary fistula since 1970. Their median age was 2.3 years. In five patients, there were associated congenital cardiac anomalies, with four having these as part of the spectrum of tetralogy of Fallot. Surgical correction was performed in nine patients, while in five closure was achieved using percutaneous embolization with coils. No complications or deaths were encountered related to the treatments chosen. In two patients, further procedures were needed to close residual fistulas. The median follow-up was 16 years, and on echocardiography, all fistulas appeared to be successfully closed, albeit that we lost three patients to follow up. We also conducted a review of 426 cases of coronary fistulas reported in children in the English literature. We have compared these findings with our institutional experience.

We determined the clinical value of routine transthoracic echocardiography performed after catheter ablation of supraventricular tachyarrhythmias in children. Between April 1996 and December 2003, 253 children, of whom 135 male, with the overall group having a median age of 9, ranging from 0.1 to 19 years, underwent 280 uncomplicated radiofrequency catheter ablation procedures for supraventricular tachyarrhythmias at three institutions. In every child, transthoracic Doppler echocardiography was performed before and after the procedure. The pre-ablation transthoracic echocardiograms were normal in all, and this was one of the criterions for inclusion. The post-ablation echocardiogram showed a disorder in four asymptomatic patients. In one patient, with focal atrial tachycardia, ablated via a retrograde aortic approach, there was mild aortic valvar insufficiency. This had resolved 6 months later. Pericardial effusions developed in 3 other children. In 2 the effusions resolved spontaneously but 1 patient required pericardial drainage. This same patient also developed clinically asymptomatic mild aortic insufficiency, which resolved spontaneously within 6 months. Routine echocardiography after uncomplicated catheter ablation procedures is of clinical value, and is especially indicated when a retrograde aortic approach has been used.

Neonatal cardiac hypertrophy associated with diabetic pregnancy is transient and regresses naturally, but is associated with increased morbidity and mortality.

This study was undertaken to analyse the changes in expression of 5 cardiac genes, including atrial natriuretic peptide, α- and β-myosin heavy chain, and cardiac and skeletal α-actin genes, using a rat neonatal model, in which cardiac hypertrophy was induced via maternal diabetes.

In the hypertrophied left ventricle of neonates from diabetic mothers, the levels of mRNA from all the above genes except skeletal α-actin were increased by between 1.8- and 12-fold compared with the controls at birth (p < 0.05). In the first 28 days, the level of mRNA for α-myosin heavy chain increased slightly, while that for atrial natriuretic peptide and β-myosin heavy chain decreased continuously similar to the controls, but at a significantly faster rate. No significant difference between the two groups of neonates was observed in all 5 genes after 1 month, indicating complete regression.

Expression of 5 cardiac genes in the neonatal cardiac hypertrophy was characterised in both hypertrophic and regressive phases. Hypertrophic regression provides a unique model for the testing of new drugs or genetic modifying factors in cardiac hypertrophy.

Objectives: To estimate the incidence and timing of spontaneous closure of atrial septal defects within the oval fossa, to study the modalities for diagnosis, and predict the need for therapeutic intervention. Methods: We reviewed retrospectively the medical records of patients with isolated atrial septal defects within the oval fossa, so-called “secundum defects”, diagnosed between January 1990 and February 2003. Based on the initial echocardiographic evaluation, we divided defects into small ones measuring from 3 to 5 mm, medium ones from 5 to 8 mm, and large ones greater than 8 mm. Results: We identified 121 patients, 50 (41.3%) of whom had failed to thrive, and 14 (11.6%) had congestive heart failure. At a mean of 44.9 ± 22.1 months following diagnosis, with a range from 12 to 102 months, the defects had closed spontaneously in 31 patients (25.6%). Of 22 patients having small defects, spontaneous closure occurred in 18 (82%) at a mean age of 18.9 ± 10.2 months. Of 27 patients with defects of medium size, 12 (44%) either experienced spontaneous closure, or else the defect effectively became a patent oval foramen, at a mean age of 51.2 ± 32.2 months. Only 1 (1.4%) of the 72 patients with a defect larger than 8 mm in size underwent spontaneous closure. The defects increased in size in 8 patients (6.6%). Intervention was necessary in 76 patients (63%) at a mean age of 75.5 ± 15.2 months. Conclusion: The initial size of a defect within the oval fossa at diagnosis is the best predictor of its natural history. Some defects increase in size with growth, irrespective of their initial size.

Evolution of staged surgical management

Among the earliest reported attempts at palliation of hypoplastic left heart syndrome were those described by Cayler et al. in 1970,1 and Freedom et al. in 1977.2 Each case involved separate banding of the right and left pulmonary arteries, together with the construction of a direct aortopulmonary shunt. These two babies, one with critical aortic stenosis, and one with aortic atresia and ventricular septal defect, survived initial palliation but died without more definitive surgery. The separate banding of the pulmonary arteries had resulted in distortion of the distal pulmonary arterial branches and abnormal distribution of flow. Litwin et al.,3 in 1972, performed an operation for interrupted aortic arch in which a non-valved conduit was placed from the pulmonary trunk proximally to the descending thoracic aorta, and the pulmonary trunk was banded distal to the conduit. The one survivor again manifested severe distortion of the pulmonary arteries, and developed pulmonary vascular obstructive disease. An operation fundamentally similar to this, nonetheless, was subsequently used by several surgeons in an attempt to treat neonates with hypoplastic left heart syndrome. These included Albert et al. in 1978, and Mohri in 1979.

clinical experience in the treatment of patients with functionally univentricular hearts has shown the fontan operation to be much less than a perfect therapy. even in ideal patients, acquired elevation of the venous pressures in the systemic circulation, especially in the regions drained by the inferior caval vein, is only a matter of time. for this reason the treatment of this complex form of congenital heart disease is still the object of active scientific debate, as well as considerable ongoing medical and basic research.

Most adults with regular transposition (the combinations of concordant atrioventricular and discordant ventriculo-arterial connections) have undergone either the Mustard or Senning procedure in childhood. It is unclear whether adverse events differ according to the surgery performed. With this in mind, we conducted a systematic review and meta-analysis to compare long-term outcomes. We searched systematically entries to MEDLINE and EMBASE databases from January 1966 through August 2003, supplementing the search by secondary sources. Comparative studies were required to include at least 10 patients in each cohort of Mustard or Senning procedure, and to report overall survival. Data were extracted by two independent reviewers. We used a component approach to assess quality. On the basis of assessment of heterogeneity, we then used a random-effects model for pooled analyses. In all, we included seven studies, incorporating 885 patients. We found a trend towards lower mortality for the 369 patients undergoing a Mustard procedure when compared to 474 submitted to the Senning operation, with a hazard ratio of 0.63 and 95% confidence intervals between 0.35 and 1.14 (p = 0.13). This trend increased with the size of the sample (p = 0.004). Obstruction in the systemic venous pathway was more common in those having the Mustard procedure, with a risk ratio of 3.5 and 95% confidence intervals from 1.8 to 7.0 (p < 0.001), with a trend towards greater obstruction of the pulmonary venous pathway in those undergoing the Senning procedure, 7.6% vs. 3.8% (p = 0.27). A trend towards fewer residual shunts was observed for those with Mustard baffles, 7.0% vs. 14.1% (p = 0.10). Sinus nodal dysfunction, however, was more common after the Mustard procedure. Data regarding atrial tachydysrhythmias was inconclusive. Systemic cardiac failure and functional capacity, was similar. We conclude that outcomes are not uniform among patients submitted to the Mustard and Senning procedures. Knowledge of such differences may facilitate stratification of risk and 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.

Objective: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. Methods: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. Results: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent–child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. Conclusions: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair.

among the operations which lead to a partial rerouting of the systemic venous return into the pulmonary arteries, the bidirectional cavopulmonary anastomosis is frequently utilised in the staged surgical management of patients with functionally univentricular hearts. whereas some surgeons insist on closing any other source of pulmonary blood flow while performing a bidirectional cavopulmonary anastomosis, others maintain patency of either a stenosed native pulmonary outflow tract, or of a systemic-to-pulmonary arterial shunt. it remains controversial as to whether an additional source of pulmonary arterial blood flow can safely and usefully be left at the time of the bidirectional cavopulmonary anastomosis to increase systemic saturation, or whether it should be eliminated to reduce the volume load on the ventricle.

Recently, the well-known American political commentator, Robert Kagan, reapplied an existing populist statement in describing Americans as being from Mars, and Europeans from Venus.1 He said “they agree on little and understand one another less and less”. In general, it would be churlish to say that this applied to the medical fraternity, but it has great resonance with intercontinental differences in the management of hypoplastic left heart syndrome. In this review, I offer a personal view of those differences, potential explanations for them, and consider how the situation may change in coming years.

At 1 year we assessed the neurodevelopmental outcomes in infants undergoing cardiac surgery, seeking to explore the predictive value of perioperative markers of cerebral injury. We prospectively enroled 47 neurodevelopmentally normal infants prior to planned cardiac surgery. Postoperative monitoring consisted of 10-channel video synchronised, continuous electroencephalography from 6 to 30 h, Doppler assessment of cerebral blood flow in the anterior cerebral artery at 1, 2, 3 and 5 h, and measurement of serum S-100B at 0 and 24 h. Neurodevelopmental assessments were performed using the second edition of the Bayley Scale of Infant Development. Follow-up at 1 year was available on 35 infants. The mean age of these patients at surgery had been 57 ± 15 days. We observed clinical seizures in 1 patient, with 3 other patients having electroencephalographic abnormalities. At follow-up of 1 year, neurodevelopmental scores were lower than preoperative scores, with mean mental scores changing from 103 ± 5 to 94 ± 13 (p = 0.001), and mean motor scores changing from 99 ± 8 to 89 ± 20 (p = 0.004). No association was found between electroencephalographic abnormalities, reduced cerebral blood flow, or elevation of serum S-100B levels and impaired neurodevelopmental outcome at 1 year. Infants with electroencephalographic abnormalities had elevation of the levels of S-100B in the serum (p = 0.02). At 1 year of follow-up, infants undergoing cardiac surgery demonstrated a reduction in the scores achieved using the second edition of the Bayley Scale of Infant Development. They require ongoing assessment of their progress. Electroencephalographic abnormalities, cerebral blood flow, or levels of S-100B in the serum were not useful perioperative markers for predicting a poor neurodevelopmental outcome in the clinical setting.

Objective: To investigate the relationship between dialysate volume, intra-abdominal pressure, and cardio-respiratory function in infants following cardiac surgery. Design: Prospective pilot study. Setting: Paediatric intensive care unit. Patients: Six infants undergoing peritoneal dialysis within 24 h of cardiopulmonary bypass. Interventions: Manipulation of the volume of dialysate at levels of 0, 10, 20, and 30 ml/kg in variable order. Measurements and main results: Intra-abdominal pressure was measured at each volume of dialysate via a pressure transducer connected to the dialysis catheter. Haemodynamic data was collected, including cardiac output, which was measured by thermodilution via a 3.5-French gauge catheter placed in the pulmonary arterial pathway. Respiratory data included PaO2, PaCO2, and dynamic compliance. Intra-abdominal pressure increased with increasing volume of dialysate (p < 0.001), though there was considerable variation between patients in the magnitude of increase. Intra-abdominal pressure remained low even with 30 ml/kg in the abdomen. In three infants, intra-abdominal pressure was re-measured in the absence of muscle relaxants, and was found to be higher in each case. No negative effects on cardiac output, markers of delivery of oxygen, or respiratory function were seen even at volumes of 30 ml/kg. Cardiac index was significantly higher with 10 ml/kg than when the abdomen was empty or contained a larger volume (p < 0.05). Conclusions: In this small group of infants, intra-abdominal pressure increased with increasing volumes of dialysate but remained low, even with 30 ml/kg in the abdomen, and was not associated with any deleterious effects on cardio-respiratory performance.

The aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate arterial valves. This uncommon congenital anomaly is reported rarely in the literature. We present here our experience with 16 patients, emphasizing the importance of early closure of the defect by a transaortic approach.

We performed surgery on 16 patients over a period of 13 years using a transaortic approach under cardiopulmonary bypass. The median age of the patients at the time of operation was 6.5 months, with a range from 1 month to 11 years. Preoperative pulmonary arterial systolic pressure ranged from 30 to 100 mmHg. Associated cardiac anomalies were present in 7 of the patients, and were repaired at the same stage. The defect was between the ascending aorta and the proximal pulmonary trunk in 13 patients, and between the ascending aorta and the distal pulmonary trunk, with overriding of the orifice of the right pulmonary artery, in 3 patients. For closure, we used a patch of 0.4 mm Gore-Tex in 11, and gluteraldehyde-treated autologous pericardium in 5 of the patients.

One patient died during surgery. The mean follow-up period for the surviving 15 patients was 52.2 months, with a range from 12 to 130 months. All the patients were in good condition during the follow-up, and no residual defects have been detected.

Aortopulmonary window is a rare congenital cardiac anomaly, which can be repaired with very good operative results if surgery is performed before the development of irreversible pulmonary hypertension. We advise early correction of the defect with a transaortic patch, repairing all associated cardiac anomalies at the time of diagnosis.

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.

We investigated a group of 376 children, seen over a period of 7 years with different types of congenital cardiovascular defects, to assess the presence of chromosomal aberrations. The diagnostic approach, achieved in 3 consecutive steps, revealed conventional chromosomal aberrations in 30 of the patients (8%) excluding trisomies 13, 18, 21. Fluorescence in situ hybridisation for microdeletions showed 51 microdeletions (15%), with 43 patients having deletions of 22q11.2, 7 patients with deletion of 7q11.23, and 1 patient with deletion of 4p16.3. In 23 patients with additional clinical abnormalities, we carried out a subtelomeric screening. This revealed, in two cases (9%), different subtelomeric aberrations, namely deletions of 1p and of 1q. Thus, subtelomeric screening proved to be a very valuable as a new diagnostic approach. Our approach to genetic investigation in three phases makes it possible to detect a high rate of pathologic karyotypes in patients with congenital cardiovascular malformations, thus guaranteeing more effective genetic counselling of the families, and a more precise prognosis for the patient.

Aim: To assess the myocardial velocities of the mitral annulus, left ventricular lateral wall, and midseptum in healthy children, and to compare these parameters with transmitral and pulmonary venous velocities. Methods and results: We examined 72 children, half being male, who had no systemic or cardiac pathologies. Their mean age was 6.73 ± 5.10 years, with a range from 0.1 to 17.75 years, and a median age of 6.71 years. Each parameter was measured twice, at end inspiration and end expiration. The tissue velocities are similar in males and females (p > 0.05). The longitudinal velocity of the heart in early diastole has a positive correlation with age (p < 0.05; midseptum velocity r = 0.57, left ventricular lateral wall velocity r = 0.56, mitral annulus velocity r = 0.56), and the tissue velocities are not influenced by respiration (p > 0.05). The myocardial velocities of different segments of the left ventricle are not correlated with the transmitral or pulmonary venous flows (p < 0.05). When age is controlled for heart rate, age mainly affects the systolic velocity of the mitral annulus and the early diastolic velocity of the midseptum in longitudinal axis, as well as the early diastolic velocity of the midseptum in transverse axis (p > 0.05 for all, r = 0.34, 0.29, 0.30 respectively). Conclusion: This study, which has determined reference values for tissue velocities in a large healthy group of children, will now set the scene for further studies in children with heart disease.

The palliation of the cyanotic child with a dominant morphologically left ventricle, discordant ventriculo-arterial connections, and obstruction to the pulmonary outflow tract has continued to evolve and mature. The evolution began in the early days of surgical palliation with the Blalock–Taussig shunt, extended to construction of cavopulmonary shunts, if required, and then to the Fontan procedure and its subsequent modifications. This journey took nearly 30 years to complete. There is increasing clinical data to document the beneficial effects of this approach, with ever-improving outcomes. Some aspects of the history of the cavopulmonary shunt have been previously reviewed in this journal and elsewhere,1–5 as have analysis of outcomes for some groups of patients considered for surgical completion of the Fontan circulation.6–12 While there has been some ongoing interest in ventricular septation since the early success of Sakakibara et al.,13 this approach has largely been abandoned. Considerably more challenges and debate resonate in the surgical algorithms defined for patients whose hearts are characterized by a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs. This latter group will be the focus of this review, as will the aetiology of the myocardial hypertrophy that is particularly frequent in this group of patients, its clinical recognition, indeed its anticipation, and the multiple surgical strategies designed to prevent or treat it. All these manoeuvres are considered to optimise suitability for, and outcome from, creation of the Fontan circulation.

the bidirectional cavo-pulmonary anastomosis is a well-established palliative procedure for patients with a functionally univentricular circulation. it is usually considered one step in preparation for fontan procedure, but it may be performed as a long-term palliation for patients deemed to be at high-risk. in this subset of patients, a valuable surgical option could be to add, or maintain, an additional source of flow of blood to the lungs, either derived from a patent but banded trunk or one protected by native pulmonary stenosis, or a systemic-to-pulmonary arterial shunt. the risk and benefits of providing an additional source of pulmonary flow after construction of a bidirectional cavopulmonary anastomosis are strongly debated. in terms of benefit, the arterial saturation of oxygen is increased due to the greater ratio of pulmonary-to-systemic flow, arteriovenous fistulas are prevented and, as a consequence of the arterial pulsatile flow, the pulmonary arteries are stimulated to grow. the most significant drawbacks are volume overload of the functionally single ventricle, and higher pressures compared to an isolated bidirectional cavopulmonary anastomosis.

Video assisted thoracoscopic surgery for closure of the persistently patent arterial duct is an effective answer to the modern effort of seeking repair of congenital cardiac malformations with minimally invasive techniques.

Between June, 1994, and December, 2002, 150 consecutive patients with an echocardiographic diagnosis of isolated patency of the arterial duct were referred to our Institution for video assisted thoracoscopic closure. The median age at the time of operation was 45 months, with a range from 3 to 161 months, and mean weight was 18 kg, with a range from 4.2 to 73 kg. Video assisted closure was possible in 139 patients (93%). The mean operative time was 37 min, with a range from 14 to 89 min. In the remaining 11 patients (7%), seen early in our experience, we converted to a mini-thoracotomy to achieve closure. In no instance did we encounter major haemorrhage requiring blood transfusion, and there were no hospital deaths. The mean period of stay in hospital was less than 24 h in 61 patients, less than 48 h in 88 patients seen earlier in our experience, and 21 days in 1 patient because of a persistent chylothorax. Other complications included palsy of the left recurrent laryngeal nerve in 1 patient, with partial regression at 6 months follow-up.

We conclude that closure of the arterial duct by video-assisted thoroscopy combines the advantages of safe and complete extraluminal occlusion with an excellent clinical and aesthetic result, minimal postoperative pain, and short stay in hospital at low cost.