Congenitally corrected transposition is a rare cardiac anomaly characterized by the combination of discordant atrioventricular and ventriculoarterial connections. Young patients with this lesion can present with congestive cardiac failure, usually secondary to a large ventricular septal defect or pulmonary stenosis. We report here our experience with a lady aged 79, admitted to our unit because of deterioration of her congestive cardiac failure as a consequence of uncorrected congenitally corrected transposition associated with degenerative severe aortic stenosis.

Quadricuspid aortic valves are rare congenital anomalies, usually diagnosed in adult patients affected by severe aortic regurgitation. We have now encountered three such valves in children undergoing transthoracic echocardiography. All children were asymptomatic. The first child possessed a valve with two larger leaflets, and smaller leaflets of equal size. In the second child, 3 leaflets were of equal size, with 1 small accessory leaflet. The third child had a valve with four leaflets of approximately equal size. In one child, the aortic valve was functioning normally, and came to attention because of mitral valvar prolapse causing mild regurgitation. In the remaining two children, central mild aortic regurgitation was detected using colour flow analysis, and associated dilation of the aortic root was revealed by measurements of the cross-sectional images.

The familial variant of cardiac myxoma is known to be an autosomally dominant disease. Early diagnosis, and removal of the tumours, are of great importance. In this regard, echocardiography is considered the simplest and most reliable diagnostic method. We report our experience with echocardiographic diagnosis of a family with atrial myxomas.

We present the case of an isolated cystic lesion of the atrioventricular component of the membranous septum of unclear aetiology, but responsible for cardiomegaly and benign disturbances of cardiac rhythm. As far as we are aware, this type of lesion has not previously been documented.

We describe the results of myocardial perfusion scintigraphy performed 4 to 15 years after surgery in 12 patients with pulmonary atresia and intact ventricular septum. We used single photon emission computed tomography after injection of technetium Tc-99m tetrofosmin at submaximal exercise test. The patients, 7 girls and 5 boys, with a mean age of 11 years, and a range from 6 to 19 years, had either undergone biventricular repair, in 5 cases, or univentricular palliation in the remaining 7. This second group included 4 patients with ventriculo-coronary arterial communications. Of the children, 3 with biventricular repair and 6 with univentricular palliation had perfusion defects. Children with biventricular repair had perfusion defects in the ventricular septum, while those having univentricular palliation also had defects located to the left ventricular free wall. All children with ventriculo-coronary arterial communications had perfusion defects both in the ventricular septum and in the left ventricular free wall.

Meaningful evaluation of quality of care must account for variations in the population of patients receiving treatment, or “case-mix”. In adult cardiac surgery, empirical clinical data, initially from tens of thousands, and more recently hundreds of thousands of operations, have been used to develop risk-models, to increase the accuracy with which the outcome of a given procedure on a given patient can be predicted, and to compare outcomes on non-identical patient groups between centres, surgeons and eras.

In the adult cardiac database of The Society of Thoracic Surgeons, algorithms for risk-adjustment are based on over 1.5 million patients undergoing isolated coronary artery bypass grafting and over 100,000 patients undergoing isolated replacement of the aortic valve or mitral valve. In the pediatric and congenital cardiac database of The Society of Thoracic Surgeons, 61,014 operations are spread out over greater than 100 types of primary procedures. The problem of evaluating quality of care in the management of pediatric patients with cardiac diseases is very different, and in some ways a great deal more challenging, because of the smaller number of patients and the higher number of types of operations.

In the field of pediatric cardiac surgery, the importance of the quantitation of the complexity of operations centers on the fact that outcomes analysis using raw measurements of mortality, without adjustment for complexity, is inadequate. Case-mix can vary greatly from program to program. Without stratification of complexity, the analysis of outcomes for congenital cardiac surgery will be flawed. Two major multi-institutional efforts have attempted to measure the complexity of pediatric cardiac operations: the Risk Adjustment in Congenital Heart Surgery-1 method and the Aristotle Complexity Score. Both systems were derived in large part from subjective probability, or expert opinion. Both systems are currently in wide use throughout the world and have been shown to correlate reasonably well with outcome.

Efforts are underway to develop the next generation of these systems. The next generation will be based more on objective data, but will continue to utilize subjective probability where objective data is lacking. A goal, going forward, is to re-evaluate and further refine these tools so that, they can be, to a greater extent, derived from empirical data. During this process, ideally, the mortality elements of both the Aristotle Complexity Score and the Risk Adjustment in Congenital Heart Surgery-1 methodology will eventually unify and become one and the same. This review article examines these two systems of stratification of complexity and reviews the rationale for the development of each system, the current use of each system, the plans for future enhancement of each system, and the potential for unification of these two tools.

Rhabdomyomas are the most common cardiac tumours in children. They sometimes cause significant obstruction of the ventricular out flow tracts. We report a series of 3 neonates diagnosed antenatally with multiple rhabdomyomas, who developed significant obstruction of the ventricular outflow tracts following birth. They underwent surgical resection in the neonatal period with good outcome. Antenatal diagnosis of obstructive cardiac tumours allows for planning for appropriate early intervention.

Haemoptysis is a common feature of adults with congenitally malformed hearts suffering from Eisenmenger’s syndrome. While this situation is often treated conservatively, it may not settle with such treatment. A further therapeutic option for these patients is embolisation of appropriate systemic-to-pulmonary collateral arteries. We discuss here our experience in treating two such patients with different underlying anatomical substrates.

When designed in 2000, the Aristotle Complexity Score was entirely based on subjective probability. This approach, based on the opinion of experts, was considered a good solution due to the limited amount of data available. In 2008, the next generation of the complexity score will be based on observed data available from over 100,000 congenital cardiac operations currently gathered in the congenital cardiac surgery databases of the Society of Thoracic Surgeons and the European Association for Cardio-Thoracic Surgery.

A mortality score is created based on 70,000 surgeries harvested in the congenital databases of The Society of Thoracic Surgeons and The European Association for Cardio-Thoracic Surgery. It is derived from 118 congenital cardiovascular operations, representing 91% of the operations and including 97% of the patients. This Mortality Index of the new Aristotle Complexity Score could further be stratified into 5 levels with minimal within-group variation and maximal between-group variation, and may contribute to the planned unification of the Aristotle Complexity Score with the Risk Adjustment for Congenital Heart Surgery system.

Similarly, a score quantifying morbidity risk is created. Due to the progress of congenital cardiac surgery, the mortality is today reduced to an average of 4%. No instrument currently exists to measure the quality of care delivered to the survivors representing 96% of the patients. An objective assessment of morbidity was needed. The Morbidity Index, based on 50,000 operations gathered in the congenital databases of The Society of Thoracic Surgeons and The European Association for Cardio-Thoracic Surgery, is derived from 117 congenital cardiovascular operations representing 90% of the operations and including 95% of the patients. This morbidity indicator is calculated on an algorithm based on length of stay in the hospital and time on the ventilator.

The mortality and morbidity indicators will be part of the next generation of the complexity score, which will be named the Aristotle Average Complexity Score. It will be based on the sum of mortality, morbidity, and subjective technical difficulty. The introduction of objective data in assessment of mortality and morbidity in congenital cardiac surgery is a significant step forward, which should allow a better evaluation of the complexity of the operations performed by a given centre or surgeon.