Background: Systemic right ventricular dysfunction is a major concern in the follow-up of patients who underwent an atrial redirection procedure for transposition (concordant atrio-ventricular and discordant ventriculo-arterial connections). No good non-invasive method is currently available for quantifying right ventricular function. Aims: We have used ultrasonically based imaging of strain rate and strain to quantify regional deformation in the right ventricle after the Senning operation, comparing properties of regional deformation of the right ventricle with right ventricular ejection fraction as measured using magnetic resonance imaging. Methods: In 20 asymptomatic patients who had undergone the Senning procedure, we measured peak systolic strain rate and systolic strain values in the right ventricular free wall, the septum and the left ventricular lateral wall using colour Doppler myocardial imaging, comparing the data with findings obtained in 30 healthy subjects. Global right ventricular ejection fraction was assessed using magnetic resonance imaging. Results: Properties of deformation of the right ventricular free wall were reduced and homogeneous after the Senning procedure compared to normals, with significantly lower values for peak systolic strain rate and systolic strain (−1.1 ± 0.4 vs. −2.5 ± 0.9 s−1; p < 0.05 and −16 ± 7% vs. −38 ± 13%; p < 0.05, respectively). There was a significant correlation between regional longitudinal right ventricular systolic strain values and right ventricular ejection fraction (r = −0.87, p < 0.001). In the septum, peak systolic strain rate was again reduced and homogeneous (−1.2 ± 0.4 vs. −1.8 ± 0.5 s−1; p < 0.05 vs. normals). Also in the left ventricle, the lateral wall peak systolic strain rate and systolic strain values were reduced (−1.5 ± 0.5 vs. −2.1 ± 0.9 s−1; p < 0.05 and −20 ± 6% vs.−25 ± 9%; p < 0.05, vs. normals, respectively). Conclusions: Properties of regional longitudinal deformation of the systemic right ventricle are reduced after the Senning procedure compared to normal controls, and correlate well with global right ventricular performance. These findings suggest that ultrasonic strain rate and strain imaging could be used in the non-invasive follow-up of ventricular function in these patients.

Congenitally corrected transposition is a rare congenital anomaly, with only a few cases diagnosed and reported prenatally even in the largest fetal series. To determine the morphologic features and outcome for the lesion as recognized during fetal life, we reviewed the fetal and postnatal echocardiograms and medical records of 11 consecutive cases of congenitally corrected transposition. These were identified among 230 (4.7%) consecutive cases of structural cardiac disease referred to our fetal cardiology unit over a period of 4 years. The mean gestational age at diagnosis was 24.7 weeks. Reasons for referral were suspected complete transposition, abnormal position of the heart, and bradyarrhythmias. Associated cardiac lesions included an abnormal cardiac position in 6 cases, ventricular septal defect in 8, obstruction of the subpulmonary outflow tract in 6, tricuspid valvar displacement in 5, and complete atrioventricular block in 2. Only 3 of the cases had mild tricuspid regurgitation prior to birth. Termination was chosen in 4 cases with severe obstruction to pulmonary flow. Of the remaining cases, 2 patients died at 3 and 12 months after birth, respectively. Both developed significant tricuspid regurgitation associated with unexpected major arrhythmias. The remaining 5 patients are alive and relatively well at a mean follow-up of 25.4 months. An epicardial pacemaker was inserted in 1 because of complete atrioventricular block. We conclude that prenatal counseling must be guarded following the diagnosis of congenitally corrected transposition, even in fetuses with an apparently favorable state at initial examination. Some of these cases may undergo major and unexpected changes, particularly with regard to cardiac rhythm and tricuspid valvar function, with concomitant significant changes in prognosis.

Patients: We studied retrospectively the echocardiographic features of newborns with persistent pulmonary hypertension who had been randomised to receive inhaled nitric oxide or conventional therapy. We sought to identify the predictors of extracorporeal membrane oxygenation therapy, death, and response to inhaled nitric oxide. Results: Among 85 neonates studied, an extrapulmonary right-to-left shunt through either an oval foramen and/or an arterial duct was present in 80 (94%) cases. In the 64 patients (75%) with adequate images for measurements, left ventricular ejection fraction was normal or mildly depressed in 87%, but there was a decreased index of left ventricular output, inferior to 2 l/min/m2, in 61% of the cases. Using multivariate analysis, an exclusive right-to-left ductal shunt was found to be an independent predictor of death, with odds ratio of 7.8, and 95% confidence intervals from 1.2 to 52.8, with a p value of 0.04. There was also a non-significant trend toward greater use of extracorporeal membrane oxygenation in patients with a predominant left-to-right ductal shunt, the odds ratio being 0.13, with 95% confidence intervals from 0.01 to 1.22, and a p value of 0.07. In the 40 patients randomised to receive inhaled nitric oxide, 28 had a positive response as defined by a 20% reduction in the index of oxygenation as measured from the post-ductal arterial blood gas sample. A left-to-right atrial shunt increased the risk of failing to respond to inhaled nitric oxide, with an odds ratio of 7.46, 95% confidence intervals from 1.23 to 45.1, and a p value of 0.028. Conclusion: Detailed Doppler echocardiographic screening of patients with suspected persistent pulmonary hypertension of the newborn may refine the selection of groups for specific treatment, and identify risk factors.

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.

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.

Objectives: We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998. Background: Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair. Methods: We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports. Results: The mean initial Z-score for the diameter of the tricuspid valve was −0.99 ± 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up. Conclusions: The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.

Objective: To explore whether prenatal diagnosis of congenital heart disease is associated with lower levels of parental distress and greater satisfaction with decisions about cardiothoracic surgery when compared to postnatal diagnosis. Methodology: A combined quantitative–qualitative design was used. Participants included the parents of 31 neonates (30 mothers and 22 fathers) admitted to the cardiac intensive care unit between 1 November 2001 and 1 May 2002 for repair of congenital cardiac malformations. Participants completed self-report measures of anxiety, optimism, and life events pre-operatively, and semi-structured qualitative interviews assessing satisfaction with decision-making within 1 week of the operation. Results: At the time of surgery, mothers of neonates receiving the diagnosis prenatally did not differ from mothers of neonates receiving the diagnosis postnatally on measures of anxiety, optimism, and life events. Fathers of neonates receiving the diagnosis prenatally, however, reported more optimism, lower state and trait anxiety, and fewer negative life events than fathers of neonates receiving the diagnosis postnatally. When we analyzed the interviews, we found that, regardless of the timing of the diagnosis, parents felt as though they made a genuine choice for their baby to have surgery. Conclusions: In this pilot study, fathers who learned prenatally that their child had a congenital cardiac malformation were less distressed than those who discovered this fact only postnatally. From the parental perspective, nonetheless, distress and urgency do not impair their ability to make decisions about neonatal cardiac surgery.

Objectives: To determine the long-term outcomes and risk factors for, reintervention after balloon dilation of congenital aortic stenosis in children aged 6 months or older. Background: Although balloon dilation of congenital aortic stenosis has become a primary therapeutic strategy, few data are available regarding long-term outcomes. Methods: We carried out a retrospective review of 87 children who had undergone balloon dilation of the aortic valve at median age of 6.9 years. Results: The procedure was completed in 98% of the children, with an average reduction in the gradient across the valve of 64 ± 28%, and without mortality. Of the children, 76 had been followed for a mean of 6.3 ± 4.2 years. Reintervention on the aortic valve was required in 32 children, with 12 undergoing reintervention within 6 months, with 1 death. Another patient had died over the period of follow-up due to a non-cardiac event. Estimated freedom from reintervention was 86% at 1 year, 67% at 5 years, and 46% at 12 years. Parametric modeling of the hazard function showed a brief early phase of increased risk, superimposed on an ongoing constant risk. The only incremental risk factor for the early phase was a residual gradient immediately subsequent to the procedure greater than 30 mmHg. Incremental risk factors for the constant phase included the presence of symmetric valvar opening, and greater than moderate regurgitation immediately after dilation. Conclusion: Long-term survival was excellent, albeit that the need for further reintervention was high due to the palliative nature of the procedure.

The use of a conduit of polytetrafluoroethylene placed between the right ventricle and the pulmonary arteries as source of pulmonary arterial supply during the first stage of palliation for the hypoplastic left heart syndrome has facilitated post-operative management and resulted in decreased mortality. We describe here the use of a cryopreserved saphenous vein inserted in reversed direction to create the connection between the right ventricle and the pulmonary arteries in a neonate with low birth weight undergoing the modified Norwood procedure.

We occluded a large arterial duct in a patient with atrial fibrillation, using an Amplatzer duct occluder, with no residual shunt at the end of the procedure. Follow-up revealed recanalization, which was successfully treated with a 4 mm Amplatzer ASD occluder. It is speculated the reopening of the initially occluded duct was the consequence of anticoagulation needed because of the atrial fibrillation.

Based on experience in several centers, the double switch operation has reportedly become the standard surgical therapy for congenitally corrected transposition. We report and discuss here the use of a “ventricle and a half” double switch operation performed due to the concerns raised intraoperatively because of the size of the morphologically right ventricle. Although the long-term course of such a procedure in this setting remains unknown, we submit that the proposed benefits of the double switch operation, even when used in the “ventricle and a half” configuration, may be superior to the alternatives.

We report our experience with an 8-year-old boy with complete atrioventricular block and syncopal bradycardia who required urgent pacing. Each attempt to cross the tricuspid valve with a femoral lead triggered ventricular standstill, followed by fibrillation, and pacing through the coronary sinus failed. Successful ventricular pacing was finally achieved through the oesophagus, allowing subsequent implantation of a transvenous pacemaker.

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.

Contrary to what the name implies, the left atrial appendage is increasingly becoming an organ of clinical importance. Its role in the pathogenesis of thromboembolism is now well established. Enlargement of the appendage due to haemodynamic disturbances or anatomic weakness increases this risk, along with the potential for arrhythmia. We report a rare case of gross enlargement of the left atrial appendage within the pericardial cavity.

Training in all medical disciplines is currently undergoing a major overhaul. There is a move away from the old concept of training as an apprentice, towards more structured programmes of training. Proposals have recently been made to shorten higher specialist training to 3–4 years, thus producing “generalist” consultants to meet the growing need for service. Advanced subspeciality training can then be undertaken by those with the ability, and desire, to do so following the core training. In the near future, therefore, subspeciality training will need to meet the needs of those undertaking core training, and those wishing to develop a higher degree of expertise. It will have to be focused, to allow skills appropriate to the future practice to be undertaken as a consultant to be developed efficiently. We propose a new scheme for training in interventional paediatric cardiology as a template for subspeciality training. The scheme proposes training at three levels. The first level, basic training, is required of all trainees. Training for the intermediate level will be required for those proposing to carry out diagnostic cardiac catheterisation and basic intervention. Training at the advanced level will be needed by those who wish to carry out a wide range of interventional procedures. A curriculum is proposed for each level, detailing skills that must be attained. Attendance at procedures needs to be driven by the educational needs of the trainee, rather than the requirements of the service. Objective methods are suggested to allow assessment of competence. These should supersede log-books, which document only procedural numbers. Training may be needed for the trainers to ensure that teaching and assessment achieve agreed standards of excellence.