Background: At present, a considerable number of patients survive who underwent an atrial switch operation for correction of complete transposition. Our study aimed to assess their long-term exercise performance and the serial evolution of cardiac function. Methods: We studied 22 patients 5 to 17 years after an atrial switch operation, and followed them serially for 3.5 ± 2 years after the first evaluation. Cardiorespiratory exercise function was assessed by analysis of gas exhange and by determination of the ventilatory anaerobic threshold. Echocardiography was performed on all evaluations. Results: All patients were in Class I of the classification of the New York Heart Association at all assessments. Ventilatory anaerobic threshold, however, was significantly lower than normal. It averaged 77.9% ± 13.7 of the normal mean value at the initial evaluation, and remained stable when re-evaluated later (76.2 ± 13.7%). At the initial study, the increase in oxygen uptake during graded exercise was below the 95% confidence limit in 6 of the patients, and was below this level in 10 patients at re-assessment. The subnormal values for oxygen uptake during submaximal exercise were associated with moderate to severe haemodynamic dysfunction. At echocardiography, 15 of 17 patients studied twice had mild to moderate right ventricular dilation and tricuspidregurgiation, which remained virtually the same at reasssesment. A stable sinus rhythm was initially present in 17 patients, and persisted in 15 patients during follow-up. Conclusion: At medium term follow-up, cardiorespiratory exercise performance remains stable in patients after atrial switch repair. Serial exercise testing appears useful, because in individual patients in the present study, a decreasing exercise tolerance correlated with development of haemodynamic sequels.

Uncertainty exists regarding the degree to which infants with congenitally malformed hearts are at risk of behavioural disorders in childhood. Data was collected as part of a randomized clinical trial involving 155 children with surgically corrected transposition (concordant atrioventricular and ventriculo-arterial connections or alignments). As infants, they underwent the arterial switch operation, involving deep hypothermia with predominantly total circulatory arrest or predominantly low-flow continuous cardiopulmonary bypass as the method of providing support to the vital organs. Parents completed the Child Behavior Checklist when the patients were aged 4 and 8 years, and the Connors’ Parent Rating Scale at the age of 8 years. When the children were aged 8, teachers completed the Teacher’s Report Form and the Connors’ Teacher Rating Scale. In the cohort as a whole, the frequencies of behavioural problems identified by both parents and teachers were elevated, particularly on the scales for competence of the Child Behavior Checklist, and the Adaptive scales of the Teacher’s Report Form. Approximately 1 in 5 patients had scores for Total Problem Behavior in the range of clinical concern on both the Child Behavior Checklist and the Teacher’s Report Form. Few differences were found, however, according to the method of operative treatment. Postoperative seizures were associated with social and attention problems. Children experiencing academic problems at the age of 8 showed a larger increase in behavioural problems between the ages of 4 and 8 than did children making adequate academic progress. Children with congenitally malformed hearts who underwent reparative surgery in infancy using a strategy of severe haemodilution and alpha stat are at increased risk of behavioural problems in middle childhood.

Chick embryos are frequently used as animal models when researching the developing heart. In the past, every attempt to induce complete transposition (the combination of concordant arrioventricular and discordant ventriculo-arterial connections) failed in chicks, suggesting that it might be impossible to develop a chicken modle for this malformation. We demonstrate, to the best of our knowledge, the first well-documented case of complete transpositon occourrine in the chick

Objectives: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. Methods: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction. Results: We found 82 patients who satisfied the criterions for inclusion, of whom 52 patients had transposition with an intact ventricular septum, and 30 had either an associated ventricular septal defect or double outlet right ventricle. The median follow-up time was 8.8 years (4.1 to 16.4 years). The neo-aortic valve had been replaced in 1 patient. Of the patients, 3 had moderate, 66 had trivial to mild, and 12 had no neo-aortic valvar regurgitation at their most recent follow-up. The regurgitation had progressed by at least 1 grade in 38 of the 82 patients (46.4%). Neo-aortic dilation was noted at the basal attachment of the leaflets, and at mid-sinusal level, which was out of proportion to somatic growth. Conclusions: At mid-term follow-up, significant neo-aortic valve regurgitation is present in 3.7%, and trivial to mild regurgitation in 81.4% of patients. The regurgitation progressed in almost half of the patients over time. We also noted progressive dilation of the neo-aortic root out of proportion to somatic growth.

Background: Perceived correlation between the coronary arterial anatomy in patients with complete transposition, and the outcome of the arterial switch procedure, has made preoperative identification of their patterns standard practice. Purpose: Our purpose was to assess the accuracy of preoperative echocardiographic identification of coronary arterial patterns, to evaluate the necessity of preoperative imaging by angiography, and to determine the impact of the coronary arterial anatomy on outcome. Methods: We reviewed the medical records of all patients referred for an arterial switch operation between August 1995 and January 2000. The anatomy as described at the time of the operation using the Leiden convention was compared to the preoperative echocardiographic and angiographic findings. Results: The procedure had been performed in 67 patients, at a mean age of 9 days, with a range from 3 days to 15 months. In 42 patients, the ventricular septum was intact, while 21 patients had a ventricular septal defect, and the other four had double outlet right ventricle with the aorta anterior and rightward. In 52 patients, the left coronary artery arose from sinus #1, and the right from sinus #2. In 8 patients, the interventricular branch of the left coronary artery arose from sinus #1, with the circumflex coronary artery arising together with the right coronary artery from sinus #2. In three patients, all three coronary arteries arose from sinus #1, while in the remaining individual patients, a large conal branch arose with the left coronary artery from sinus #1, the right coronary and left anterior descending arteries arose from sinus #1, all three coronary arteries took origin from sinus #2, and the left anterior descending and right coronary artery arose from sinus #1 with no circumflex coronary artery identifiable, respectively. In two patients (4%), we identified an intramural coronary arterial course. Echocardiography and angiography were comparable (81% versus 86%) in delineating the coronary arterial anatomy. Patients with a single arterial orifice, or an atypical coronary arterial anatomy, had a slightly longer stay on the intensive care unit, and in the hospital, but showed no difference in mortality. In fact, there was no early mortality (70% confidence limits; 0–2.9%), while two patients died late (2.9%). Conclusion: We conclude that complex coronary arterial anatomy does not preclude a successful arterial switch procedure, although patients with a single coronary artery, or other arterial patterns, had a slightly longer hospital course. Preoperative echocardiographic evaluation is comparable to non-selective coronary angiography. Irrespective of complexity, nonetheless, the coronary arteries can successfully be translocated, obviating the need for preoperative coronary angiography.

We report obstruction of the reconstructed left ventricular outflow tract diagnosed 18 months after an arterial switch operation that included closure of a ventricular septal defect. We speculate that either turbulences created by the patch used to close the ventricular septal defect, or postoperative modifications of ventricular geometry, were responsible for producing this unusual complication.

Deteriorating ventricular function is a major concern after the Senning operation. A Doppler-derived non-geometric measurement, the so-called myocardial performance index, has been described for use in adults and children. We aimed to assess the utility of this index as a method for quantification of right ventricular function in patients in the late follow-up of the Senning procedure, and to correlate the right ventricular ejection fraction and the first derivative of right ventricular pressure as derived using echocardiography with the ejection fraction determined using magnetic resonance imaging.

We studied 44 patients within a mean postoperative period of 15.3 years. We calculated the right ventricular myocardial performance index by pulsed wave Doppler interrogation of tricuspid inflow and aortic outflow, the ejection fraction by Simpson's rule, and the first derivative of right ventricular pressure by continuous wave Doppler from tricuspid regurgitation.

Mean values of right ventricular myocardial performance index, ejection fraction, and the first derivative of right ventricular pressure were 0.50, 39 percent and 1,398 millimetres of mercury per second, respectively. A cut-off value of 0.47 for the right ventricle myocardial performance index was determined, with a sensitivity of 75 percent and a specificity of 62.5 percent. We found no correlation between ejection fraction and the first derivative of right ventricular pressure as estimated by echocardiography and the ejection fraction as shown by magnetic resonance imaging (r2 equal to 0.29 and 0.04 respectively).

We concluded, first, that patients with preserved right ventricular function had values for the right ventricular myocardial performance index lower than 0.47, and second, that ejection fraction and the first derivative of right ventricular pressure as determined echocardiographically did not correlate with values derived using magnetic resonance imaging.

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.

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.