An aortopulmonary window is a communication between the ascending aorta and pulmonary trunk in the presence of two separate arterial valves, and is often complicated by other associated defects. We sought to determine management and related outcomes in patients with this malformation.

We identified those patients presenting between 1969 and 1999 from the databases held in our Departments of Cardiology, Pathology and Cardiovascular Surgery. We obtained data relating to issues concerning demography, clinical findings, imaging, management and outcome.

The median age at presentation for the 42 patients identified, of whom 23 were female, was 62 days, with a range from birth to 6 years. Associated cardiac defects were present in 34 patients, including interruption of the aortic arch in 6 patients. The correct diagnosis was initially missed in 13 patients. Of the patients, six died without surgical repair, and 1 patient was lost-to-follow-up. Repair was performed in 35 patients, subsequent to repair of other defects in 4, in association with repair of other defects in 17, of whom 3 died, and as an isolated procedure in 14 patients, one of the latter being treated by transcatheter closure. Overall, there were 9 deaths, all in patients with complex associated defects, except 1 patient with a missed aortopulmonary window after repair of aortic coarctation. Kaplan-Meier estimates of survival were 81% at 3 months until 11.5 years, and 69% up to 21 years. Only the presence of interrupted aortic arch was independently associated with increased time-related mortality, the hazard ratio being 5.87 (p = 0.009).

The outcome for an isolated lesion is excellent. Mortality occurs mainly before repair, mostly with complex associated lesions, particularly interruption of the aortic arch.

Atrial arrhythmias are associated with enlarged atrial chambers and an increased duration of the P wave. Repair of atrial defects within the oval fossa is expected to normalize atrial size. Few studies, however, have evaluated electrical and morphological atrial features after repair. Our study was performed to determine if atrial abnormalities exist after surgical closure of such atrial septal defects, and whether early closure improves outcome. We recruited patients who had undergone surgical closure of a defect within the oval fossa, so-called “secundum” atrial septal defects. Electrocardiograms, signal averaged electrocardiograms, and echocardiograms were performed. Two-tailed test and Pearson correlation was utilized for statistical analysis. The population consisted of 20 patients and 27 controls, with the mean age of the patient being 11.25 ± 5.10 years, their age at surgery 6.55 ± 5.10 years, and the time since surgery 4.70 ± 2.61 years. The size of the right (23.88 ± 6.35 ml/m2 versus 18.84 ± 4.43 ml/m2) and left (21.91 ± 12.47 ml/m2 versus 17.72 ± 4.83 ml/m2) atrium were significantly larger in the patients. The duration of the P wave (108 ± 16 ms versus 96 ± 8 ms) and the duration of the PR interval (155 ± 18 ms versus 138 ± 23 ms) were longer. No correlation existed between age or interval since surgery with atrial sizes or measurements of the signal averaged electrocardiogram. We conclude that, despite surgical repair, abnormalities exist in patients with an atrial septal defect. Early surgery does not appear to prevent the atrial abnormalities.

The optimum treatment for myocarditis in children is unknown. We present outcomes for this disease as seen in a large series of children. Thus, we identified all children seen with myocarditis at Children’s Hospital of Pittsburgh since 1985, including only those with biopsy-proven myocarditis, or cardiac dysfunction and proof of concomitant cardiotropic viral infection. Outcomes were defined as complete recovery, incomplete recovery, and death or transplantation.

We identified 41 patients, 37 proven by histology, and 4 patients who were too unstable for biopsy but had proof of viral infection. Of the group, 27 (66%) made a complete recovery, 4 (10%) had incomplete recovery, and 10 (24%) either died (5) or underwent transplantation (5). The median time to death or transplantation was 8.4 months, with a range from 1 day to 49 months. Steroids had been administered to 16 patients, of whom 10 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. Intravenous immune globulin was given in isolation to one patient, who made a complete recovery, and to 18 in combination with steroids, of whom 12 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. The remaining 6 patients received neither steroids nor intravenous immune globulin, and of these, 4 made a complete recovery, 1 was transplanted, and 1 died. Freedom from death or transplantation was 81% at 1 year, and 74% at 5 years, with no difference between the modes of treatments. The median time to recovery of function was also comparable between the groups. Thus, in our patients, treatment with intravenous immune globulin appeared to confer no advantage to steroid therapy alone. These data emphasise the need for randomised trials to assess the efficacy of current treatments, as well as that of new therapies.

The Amplatzer septal occluder is an alternative to operative closure of atrial septal defects within the oval fossa. An issue when deploying the device is its distance from the mitral valve. The purpose of this study is to determine how this distance changes with growth of the patient. We identified, through a review of charts, all patients undergoing closure of defects within the oval fossa by insertion of an Amplatzer septal occluder. Data were obtained from the echocardiogram 24 hours after closure, and at most recent follow-up, including left ventricular end-diastolic diameter, left atrial diameter, degree of mitral valvar regurgitation, body surface area, and distance from the device to the mitral valve. We divided the patients into 2 groups based upon change in body surface area. The first group had an increase in body surface area of at least 10%. All others were in the second group.

We inserted 55 Amplatzer septal occluders in 54 patients. Of these we excluded 17 patients, 1 because quality of images was inadequate, 1 who underwent placement of 2 devices, 1 in whom the device embolised to the left ventricle the day after deployment, and 14 who have not yet had a follow-up echocardiogram.

The group which exhibited an increase in body surface area of greater than 10% demonstrated an increase in distance from the device to the mitral valve, left ventricular end-diastolic, and left atrial diameters. Those who did not undergo significant growth had no increase in distance from the device to the mitral valve, but did have an increase in left atrial and left ventricular end-diastolic diameters. No patient developed mitral regurgitation. We conclude that, when deploying an Amplatzer septal occluder close to the mitral valve in children, the distance from the device to the mitral valve can be expected to increase with growth of the patient.

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.

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.

Due to underlying cardiovascular anatomy and size, epicardial pacing may be the preferred method of pacing in small children. To assess long-term safety, we reviewed all epicardial pacemakers implanted in children between 1971 and 2001. We found that 122 patients, with a median age of 5.4 years, had a total of 181 pacemakers and 260 electrodes implanted over a total follow-up of 789 patient-years. Of the total, 12 patients died after the first implantation, with one death attributable to dysfunction of the pacemaker. Reintervention was required in 75 patients after 5.0 ± 3.2 years, due to depletion of the battery in 45 patients (60%), fracture or dysfunction of electrodes in 27 patients (36%), and infection in 3 patients (4%). In univariate analyses, risk factors for reintervention were an approach via a median sternotomy, with a relative risk of 2.3 (p = 0.0087), and an indication for pacing other than atrioventricular block, with a relative risk of 1.7 (p = 0.0314). In multivariate analyses, the approach via the median sternotomy independently predicted the need for reintervention, with a relative risk of 2.1, and 95% confidence intervals from 1.1 to 4.1 (p = 0.0256). The longevity of the second pacemaker and/or its electrode, assessed in 26 patients, was 3.7 ± 2.6 years, not shorter than the first implantation (p = 0.4037). We conclude that epicardial pacing is a reliable means of achieving permanent pacing in children, with low morbidity and mortality. A substantial proportion, nonetheless, requires reintervention within five years, warranting meticulous follow-up.

We aimed to assess the right atrial performance in patients after surgical correction of tetralogy of Fallot, and to clarify the relationship between the pump function of the right atrium and right ventricular systolic function.

We included in the study 50 asymptomatic patients following corrective surgery of tetralogy of Fallot, comparing them to 30 normal subjects. Right atrial areas were measured by echocardiography, and the active fractional area of emptying was expressed, in percentages, as the area measured at the onset of atrial contraction, minus the minimal area, divided by the area at the onset of atrial contraction. We used this value to assess the atrial pump function. Right atrial peak strain rates were measured by tissue Doppler imaging. Compared to controls, patients with tetralogy of Fallot had a significantly reduced right atrial active fractional area of emptying (p = 0.005), along with a reduced peak late diastolic strain rate (p = 0.002). Among 20 patients who underwent magnetic resonance tomographic examination, a relatively higher right atrial peak late diastolic strain rate was shown in patients with a right ventricular ejection fraction of less than 50% (p = 0.021).

Right atrial performance is reduced in patients after surgical correction of tetralogy of Fallot. When facing right ventricular systolic dysfunction, nonetheless, the right atrial pump function may be relatively enhanced. Tissue Doppler derived strain rate can provide quantitative analysis of regional right atrial performance.

We carried out a detailed clinical, epidemiological, and echocardiographic study in 41 patients ≤14 years of age who were admitted in a public hospital in Salvador, Brazil, with severe rheumatic heart disease.

Mitral insufficiency was severe in 90%, and moderate in 10%, of the patients. A posteriorly directed jet was seen in 93% of the patients. We identified three mechanisms producing the regurgitation: prolapse of the aortic leaflet of the mitral valve in 13 (32%) patients, rupture of tendinous cords in 14 (34%), and a retracted, non-coapting mural leaflet in 14 (34%). The mean ages, with standard deviations, for these three groups were 7.0 (1.6) years, 7.9 (2.2) years, and 10.5 (2.4) years, respectively (p < 0.001). Rheumatic activity was diagnosed in 58.5% of them. Evidence of previous rheumatic fever was present in 54% of patients with prolapse, in all patients with rupture, and in 93% of those with non-coapting leaflets (p = 0.002).

Prolapse of the aortic leaflet, rupture of tendinous cords, and a retracted, non-coapting mural leaflet are the mechanisms responsible for mitral valvar insufficiency in children and adolescents with severe rheumatic heart disease. Prolapse seems to be an early phenomenon in the natural history of rheumatic heart disease, while rupture and non-coaption of the leaflets were associated with older age and signs of chronic rheumatic disease.

Repair of complex malformations that necessitate restoration of continuity between the right ventricle and the pulmonary arteries can now safely be performed with low morbidity and mortality. Major concerns still remain on the long-term outlook for these patients, and about the durability of the different prostheses used to restore that continuity, whether during initial correction or at the time of reintervention for failure of the conduit or pulmonary regurgitation. In this review, we discuss the salient morphologic features of the right ventricular outflow tract, and then focus on the indications for early and late intervention, current therapeutic options, and outcomes.

We describe two cases of an isolated cleft of the mitral valve in transposition with intact ventricular septum. The cleft is positioned leftward in the pulmonary leaflet, at about 2 o’clock, when viewed from below looking at the cardiac short axis with the right ventricle to one’s left hand. Such clefts, when seen in the Taussig-Bing malformation are also positioned leftward.

In keeping with our current knowledge of cardiac development, our cases provide further evidence that transposition with an intact ventricular septum is the end-point of the Taussig-Bing spectrum.

We describe an infant with hypoplasia of the left heart diagnosed prenatally who, at birth, had signs of severe pulmonary venous obstruction. Echocardiography indicated normally connecting pulmonary veins, and showed a paradoxical right-to-left shunt across a patent oval foramen. Postmortem examination revealed that the obstruction was due to a divided left atrium, or cor triatriatum sinister, with an imperforate muscular diaphragm separating completely the two components of the divided atrium.

The unguarded tricuspid valve is uncommon. We describe herein a fetus with a grossly dilated right ventricle and atrium, with severe tricuspid and pulmonary valvar regurgitation. The right ventricle was akinetic, and no tricuspid tissue or valvar apparatus was identified. Colour Doppler showed a highly unusual retrograde flow of blood through the right heart. The pregnancy was terminated, and necropsy examination confirmed the gross dilation of the right heart chambers, with severely dysplastic valvar tissue at the right atrioventricular junction effectively giving an unguarded orifice. There was no valvar displacement, and the left heart was normal. The fetus had a normal karyotype, albeit with absent kidneys.

We found spontaneous echoes in two teenagers with nephrotic syndrome and profound hypoalbuminemia, both having normal cardiac structure, function and output. The phenomenon disappeared after the level of albumin normalized. In one patient, all spontaneous echoes disappeared following convalescence, the level of albumin in the serum then being documented at normal levels. The second patient, who presented with profound hypoalbuminemia, was infused with human albumin because of oliguria, following which the spontaneous echoes disappeared.

It is rare to find multiple fistulas arising from all three coronary arteries and draining into the left ventricle. Coronary angiography revealed this anomaly in a one-year-old girl with tricuspid atresia after conversion to the Fontan circulation. To the best of our knowledge, this is the first report of such multiple fistulas in the setting of tricuspid atresia, and also the first report in childhood.

I discuss a newborn whose mother is addicted to alcohol. On the third day of life, the newborn was found to have ventricular tachycardia. After spontaneous termination of the abnormal rhythm, the duration of the corrected QT interval was 0.48 s. During the next days, the duration of the interval normalized, and has now remained stable for 5 years. I conclude that the so-called “alcohol withdrawal syndrome of the newborn” might cause postnatal prolongation of the QT interval.