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.

Over a six year period, we identified four children with dilated cardiomyopathy associated with ventricular preexcitation, three in the absence of any documented tachyarrhythmias, and one with but a solitary episode of tachyarrhythmia. None of them had another identifiable aetiology for the cardiomyopathy. Based on the 12 lead electrocardiogram, and/or invasive electrophysiologic studies, we localised the accessory pathways to the right atrioventricular groove in all patients. We commenced antifailure medications in two of the four patients, but did not produce any measurable improvement in ventricular function. Catheter ablation was performed in two patients, with resolution of the cardiomyopathy. The ventricular preexcitation disappeared spontaneously in one child during follow-up, again with resolution of cardiomyopathy. The youngest patient continues to receive antifailure medications, albeit without improvement. We suggest, therefore, that dilated cardiomyopathy of reversible nature is associated with manifest ventricular preexcitation, even in the absence of sustained arrhythmias.

Parental stress is a universal experience for parents who have children diagnosed with CHD and has been studied within the context of the child’s illness, but not through a broader health disparity lens. This paper provides a thorough synthesis of the current literature on parental stress addressing disparities in parents of children with CHD. Several theories and models from within this literature are described and a new comprehensive framework, the Parental Stress and Resilience in CHD Model, is presented. Future research and clinical implications are discussed.

In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. The Nomenclature Working Group created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].

In previous publications from the Nomenclature Working Group, unity has been produced by cross-mapping separate systems for coding, as for example in the treatment of the functionally univentricular heart, hypoplastic left heart syndrome, or congenitally corrected transposition. In this manuscript, we review the nomenclature, definition, and classification of heterotaxy, also known as the heterotaxy syndrome, placing special emphasis on the philosophical approach taken by both the Bostonian school of segmental notation developed from the teachings of Van Praagh, and the European school of sequential segmental analysis. The Nomenclature Working Group offers the following definition for the term “heterotaxy”: “Heterotaxy is synonymous with ‘visceral heterotaxy’ and ‘heterotaxy syndrome’. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as ‘situs solitus’, nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as ‘situs inversus’.” “Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person. Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralised and thus have neither the usual, or normal, nor the mirror-imaged arrangements.”

The heterotaxy syndrome as thus defined is typically associated with complex cardiovascular malformations. Proper description of the heart in patients with this syndrome requires complete description of both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. The position of the heart in the chest, and the orientation of the cardiac apex, must also be described separately. Particular attention is required for the venoatrial connections, since these are so often abnormal. The malformations within the heart are then analysed and described separately as for any patient with suspected congenital cardiac disease. The relationship and arrangement of the remaining thoraco-abdominal organs, including the spleen, the lungs, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. One of the clinically important implications of heterotaxy syndrome is that splenic abnormalities are common. Investigation of any patient with the cardiac findings associated with heterotaxy, therefore, should include analysis of splenic morphology. The less than perfect association between the state of the spleen and the form of heart disease implies that splenic morphology should be investigated in all forms of heterotaxy, regardless of the type of cardiac disease. The splenic morphology should not be used to stratify the form of disease within the heart, and the form of cardiac disease should not be used to stratify the state of the spleen. Intestinal malrotation is another frequently associated lesion that must be considered. Some advocate that all patients with heterotaxy, especially those with isomerism of the right atrial appendages or asplenia syndrome, should have a barium study to evaluate for intestinal malrotation, given the associated potential morbidity. The cardiac anatomy and associated cardiac malformations, as well as the relationship and arrangement of the remaining thoraco-abdominal organs, must be described separately. It is only by utilizing this stepwise and logical progression of analysis that it becomes possible to describe correctly, and to classify properly, patients with heterotaxy.

Purpose: The aim of this study was to investigate the frequency and gender distribution of variations in the aortic arch branching pattern using 64-slice computed tomograhic angiography. Materials and methods: A total of 1000 patients with a normal left-sided aortic arch who underwent computed tomographic angiography for various reasons were analysed retrospectively for the frequency of variation of aortic arch branching; the variations were categorised into seven types. Results: Of the 1000 patients, 79.2% had a normal aortic arch branching pattern – type 1 – and 20.8% had variations. The frequency of type 2 variation – brachiocephalic and left common carotid arteries arising from the aortic arch in a common trunk – was 14.1%, that of type 3 – left vertebral artery originating from the aortic arch – was 4.1%, that of type 4 – coexistence of type 2 and type 3 – was 1.2%, that of type 5 – aberrant right subclavian artery – was 0.6%, that of type 6 – coexistence of aberrant right subclavian artery and bicarotid trunk – was 0.7%, and that for type 7 – thyroidea ima artery arising from the aortic arch – was 0.1%. The incidences of the variations of aortic arch branching were similar among males and females (20% versus 22.1%). The incidence of an aberrant right subclavian artery – type 5 and type 6 – was higher among females compared with males (2.5% versus 0.5), whereas the frequencies of the other variations were either equal or similar in both genders. Conclusion: Recognition of variations of aortic arch branching is important because they may cause symptoms due to tracheoesophageal compression or complications during surgical or endovascular interventional procedures of the aorta and its branches.

In order to examine right ventricular diastolic function after complete correction of pulmonary atresia with intact ventricular septum and critical pulmonary valvar stenosis, we undertook a prospective analysis of seven patients who had previously undergone successful repair. Comparison was with age and sex-matched controls. The relationship between antegrade pulmonary arterial diastolic flow and other indices of right ventricular dysfunction were compared. The ratio of the velocity of early rapid filling to that of atrial systolic flow was no different from normal, but the deceleration of early rapid filling showed significant shortening with inspiration in patients, but not in control subjects. All patients, but none of the controls, demonstrated significant antegrade diastolic pulmonary arterial flow during late diastole. Despite “normal” ratios ofvelocity of early rapid filling to systolic atrial flow, patients after complete correction of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis have abnormal right ventricular diastolic function typical of a restrictive physiology. This presumably reflects incomplete adaptation due to the presence of endomyocardial fibrosis.

Improvement in operative survival of patients with hypoplastic left heart syndrome has led to increasing emphasis on prevention of interstage mortality. Many centres have improved interstage results through programmes of home monitoring following discharge after the Norwood (Stage 1) operation. Experience with heightened interstage surveillance has identified failure to thrive during infancy as a modifiable risk factor for this population, one that has been linked to concerning outcomes at subsequent palliative surgeries. Ensuring normal growth as an infant has thus become a priority of management of patients with functionally univentricular hearts. Herein, we review the existing evidence for best practices in interstage surveillance and optimal nutrition in infants with functionally univentricular hearts. In addition, we highlight data presented at HeartWeek 2011, from Cardiology 2011, the 15th Annual Update on Pediatric and Congenital Cardiovascular Disease, and the 11th Annual International Symposium on Congenital Heart Disease.

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up. During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].

This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both these two organizations to analyze outcomes of over 100,000 patients undergoing surgical treatment for congenital cardiac disease. Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between paediatric and congenital cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.

In finalising our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.

Optimising short- and long-term outcomes for children and patients with CHD depends on continued scientific discovery and translation to clinical improvements in a coordinated effort by multiple stakeholders. Several challenges remain for clinicians, researchers, administrators, patients, and families seeking continuous scientific and clinical advancements in the field. We describe a new integrated research and improvement network – Cardiac Networks United – that seeks to build upon the experience and success achieved to-date to create a new infrastructure for research and quality improvement that will serve the needs of the paediatric and congenital heart community in the future. Existing gaps in data integration and barriers to improvement are described, along with the mission and vision, organisational structure, and early objectives of Cardiac Networks United. Finally, representatives of key stakeholder groups – heart centre executives, research leaders, learning health system experts, and parent advocates – offer their perspectives on the need for this new collaborative effort.

Information about acute coronary syndrome caused by Kawasaki disease-related coronary artery lesions in adults is sketchy. We reviewed the clinical features of 50 adult patients who had an acute coronary syndrome caused by coronary artery lesions due to Kawasaki disease or probable Kawasaki disease from 1980 to 2008. Of the 50 patients, 43 (90%) were male and seven were female (10%). Their ages at the onset of acute coronary syndrome ranged from 18 to 69 years, with a median of 28 years. The culprit lesion in 43 patients was thrombotic occlusion of an aneurysm, and 40 patients had giant aneurysms. In the three patients in whom no aneurysms were seen in coronary angiograms performed at the time of acute myocardial infarction, either giant aneurysms or aneurysms had been visualised in childhood. The initial treatment of acute coronary syndrome was as follows: intracoronary thrombolysis, 11; primary percutaneous coronary intervention, 9; emergency coronary artery bypass grafting, 3; and medication, 26. Elective coronary artery bypass grafting was performed in 15 patients. Three patients (6%) died. Of the 27 patients with additional coronary risk factors, 20 were smokers. Giant aneurysms due to Kawasaki disease continued to cause acute coronary syndrome in adult life with onset at a younger age than typifies that due to atherosclerosis in the general population, especially in male population rather than female population. Even when giant aneurysms regressed after the acute phase, a few patients still developed acute coronary syndrome in adult life. Smoking appears to be the most prominent additional risk factor.

Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet (Fig. 1). The second feature is anterior and apical rotational displacement of the functional annulus (Fig. 2). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebstein's malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.

The function of the heart was studied in 20 asphyxiated term neonates by measuring the longitudinal peak systolic strain and peak systolic strain rate by tissue Doppler in 18 segments of the heart on days 1, 2, and 3 of life. The fractional shortening was assessed at each examination as well. Measurements were compared against measurements in 48 healthy term neonates examined by the same protocol. The function of the heart was lower in the asphyxiated neonates – peak systolic strain (mean (95% confidence interval) −19.4% (−20.4, −18.5), peak systolic strain rate −1.65 (−1.74, −1.56) per second) than in the healthy term neonates (peak systolic strain −21.7% (−22.3, −21.0), peak systolic strain rate −1.78 (−1.84, −1.74) per second; p < 0.001). Fractional shortening was similar in the asphyxiated (29.2% (26.8, 31.5)) and healthy term neonates (29.0% (27.9, 30.1); p = 0.874). The peak systolic strain differed significantly between the asphyxiated and healthy term neonates for the left basal and right basal groups of segments (p < 0.05) but not for the left apical, right apical, septum apical, or septum basal groups of segments. The peak systolic strain rate differed significantly only for the septum apical group of segments. The differences were largest on the second day of life. Measurements were similar in asphyxiated neonates with elevated and normal cardiac troponin T levels. The peak systolic strain and strain rate were in this study more sensitive indices than fractional shortening for assessing the reduced myocardial function in asphyxiated term neonates.