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This December Issue of Cardiology in the Young represents the 13th annual publication in Cardiology in the Young generated from the two meetings that composed “HeartWeek in Florida”. “HeartWeek in Florida”, the joint collaborative project sponsored by the Cardiac Centre at the Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, together with Johns Hopkins All Children’s Heart Institute of Saint Petersburg, Florida, averages over 1000 attendees every year and is now recognised as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of cardiac disease in the foetus, neonate, infant, child, and adult. “HeartWeek in Florida” combines the International Symposium on Congenital Heart Disease, organised by All Children’s Hospital and Johns Hopkins Medicine, and entering its 16th year, with the Annual Postgraduate Course in Paediatric Cardiovascular Disease, organised by The Children’s Hospital of Philadelphia entering its 19th year.
This December 2015 Issue of Cardiology in the Young features highlights of the two meetings that compose HeartWeek. Johns Hopkins All Children’s Heart Institute’s 15th Annual International Symposium on Congenital Heart Disease was held at the Renaissance Vinoy Resort & Golf Club, Saint Petersburg, Florida, from Friday, 6 February, 2015, to Monday, 9 February, 2015. This Symposium was co-sponsored by The American Association for Thoracic Surgery and its special focus was “Congenital Abnormalities of the Coronary Arteries”. The Children’s Hospital of Philadelphia’s annual meeting – Cardiology 2015, the 18th Annual Update on Paediatric and Congenital Cardiovascular Disease: “Challenges and Dilemmas” – was held at the Hyatt Regency Scottsdale Resort and Spa at Gainey Ranch, Scottsdale, Arizona, from Wednesday, 11 February, 2015, to Sunday, 15 February, 2015.
We would like to acknowledge the tremendous contributions made to paediatric and congenital cardiac care by Juan Valentín Comas, MD, PhD (13 May, 1960 to 16 June, 2015) and Donald Nixon Ross, FRCS (4 October, 1922 to 7 July, 2014); and therefore, we dedicate this December 2015 HeartWeek Issue of Cardiology in the Young to them.
Heart failure in children is a complex disease process, which can occur secondary to a variety of aetiologies, including CHD, cardiomyopathy, or acquired conditions as well. Although the overall incidence of disease is low, the associated morbidity and mortality are high. Mortality may have decreased slightly over the last decade, and this is likely due to our ability to shepherd patients through longer periods of significant morbidity, with lasting effects. Costs of heart failure are significant – on the order of $1 billion annually as hospital charges for inpatient admissions alone. The value, or benefit to patient life and quality of life at this cost, is not well delineated. Further research is needed to optimise not only outcomes for these patients but also the high costs associated with them.
A biomarker is any measurable, surrogate characteristic, which reflects either the presence or the absence of a disease state. This can be a blood test, an imaging characteristic, an exercise parameter, and even a genetic profile. Serum biomarkers are particularly attractive in that their cost to the patient is relatively low in terms of money, time, risk, and ease of obtaining a sample. The potential benefits of a good biomarker are manifold. This manuscript will review serum biomarkers of proposed utility in paediatric heart failure, especially with respect to their ability to aid clinical decision making, diagnosis, and prognosis.
Right ventricular function is a crucial determinant of long-term outcomes of children with heart disease. Quantification of right ventricular systolic and diastolic performance by echocardiography is of paramount importance, given the prevalence of children with heart disease, particularly those with involvement of the right heart, such as single or systemic right ventricles, tetralogy of Fallot, and pulmonary arterial hypertension. Identification of poor right ventricular performance can provide an opportunity to intervene. In this review, we will go through the different systolic and diastolic indices, as well as their application in practice. Quantification of right ventricular function is possible and should be routinely performed using a combination of different measures, taking into account each disease state. Quantification is extremely useful for individual patient follow-up. Laboratories should continue to strive to optimise reproducibility through quality improvement and quality assurance efforts in addition to investing in technology and training for new, promising techniques, such as three-dimensional echocardiography.
Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone’s complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.
The Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.
At present, there is significant interest in the morphology of the coronary arteries, not least due to the increasingly well-recognised association between anomalous origin of the arteries and sudden cardiac death. Much has also been learnt over the last decade regarding the embryology of the arteries. In this review, therefore, we provide a brief introduction into the recent findings regarding their development. In particular, we emphasise that new evidence, derived using the developing murine heart, points to the arterial stems growing out from the adjacent sinuses of the aortic root, rather than the arteries growing in, as is currently assumed. As we show, the concept of outgrowth provides an excellent explanation for several of the abnormal arrangements encountered in the clinical setting. Before summarising these abnormal features, we draw attention to the need to describe the heart in an attitudinally appropriate manner, following the basic rule of human anatomy, rather than describing the cardiac components with the heart in the “Valentine” orientation. We then show how the major abnormalities involving the coronary arteries in humans can be summarised in terms of abnormal origin from the pulmonary circulation, abnormal aortic origin, or fistulous communications between the coronary arteries and the cardiac cavities. In the case of abnormal aortic origin, we highlight those malformations known to be associated with sudden cardiac death.
Among populations of patients with the congenital heart disease, there is considerable diversity in the anatomy of the coronary arteries. Understanding these anatomical differences is vitally important in directing interventions and surgical repair. In this report, the authors describe the echocardiographic evaluation of the variants of coronary artery anatomy in the following lesions: transposition of the great arteries, congenitally corrected transposition of the great arteries, double-inlet left ventricle, common arterial trunk, tetralogy of Fallot, and double-outlet right ventricle.
In the 1970s, diagnosing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was often uncertain using imaging alone; however, with the advances in high-frequency transducers, advanced image processing, and other ultrasound modalities such as Doppler colour flow imaging, tissue Doppler imaging, and speckle tracking to asses regional wall motion abnormalities, modern echocardiography now permits accurate diagnosis of ALCAPA with greater certainty. Although many consider ultrasound to be the only imaging test necessary if there is a question as to the diagnosis, other imaging modalities such as MRI, CT, and cardiac catheterisation with angiography remain valuable complementary tests, especially in older patients.
Stress echocardiography in paediatrics is used to evaluate pre- and post-operative coronary artery conditions, as well as to gain haemodynamic information for a variety of diagnoses, although evidence regarding sensitivity, specificity, and predictive value is lacking. This review will consider the available literature with a focus on anomalous aortic origin of the coronary arteries and discuss a practical approach to test selection and use.
An infant presented with features suggestive of an anomalous left coronary artery was found to have normal origins of both coronary arteries. Echocardiography during episodes of ischaemia showed marked aortic regurgitation with retrograde coronary flow. The left coronary leaflet was mildly hypoplastic. Surgical re-suspension of this leaflet prevented aortic regurgitation and the patient had no further symptoms and recovered cardiac function.
Congenital anomalies of the coronary arteries, although uncommon, have the potential to cause serious myocardial damage, ischaemic cardiomyopathy, and sudden cardiac death. This article summarises aspects of care for these patients in the outpatient setting, including clinical history and physical examination findings, ancillary testing, decision-making regarding the need for surgical intervention, and recommendations for physical activity. Although there are limited data regarding some of these recommendations, it is hoped that these can be used as an initial benchmark against which further data will lead to a more evidence-based approach.
Anomalous aortic origins of the coronary arteries comprise approximately one-third of all coronary artery anomalies and are characterised by coronary arteries with anomalies of aortic origin involving abnormal courses, stenoses, and compression that can lead to myocardial ischaemia and sudden death. Operative techniques to treat these anomalies have not been standardised yet. Moreover, the management of potential complications has not been addressed. Common and rare forms of anomalous aortic origins of the coronary arteries are reviewed and understood standard techniques for an uncomplicated unroofing procedure are illustrated. Also noted are techniques that can be applied to unexpected anatomical findings and unwanted complications that could prove to be life-threatening. Several technical recommendations are offered.
Congenital abnormalities of the coronary arteries in the absence of structural heart disease account for a small but interesting percentage of cardiac lesions in children. Their presentation may vary from incidental identification to aborted/sudden cardiac death. Patients with aborted sudden death episodes will require significant support if they develop extensive ischaemic myocardial injury. Ultimately, surgical repair should be carried out as soon as haemodynamic stability is attained and the neurological status is evaluated. The aims of this article were to provide a review of congenital abnormalities of the coronary arteries most commonly seen in children in the ICU as well as to review the current critical-care management thereof.
The anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with an interarterial, intramural, and/or intraconal course is a relatively rare congenital defect of the heart that may be associated with an increased risk of ischaemia of the myocardium and sudden death, notably in children and young adults. Data are limited regarding stratification of risk and long-term outcomes of these patients. In 2009, the Anomalous Coronary Artery Working Group formed the Congenital Heart Surgeons’ Society Registry of Anomalous Aortic Origin of a Coronary Artery to obtain information on large numbers of young patients with anomalous aortic origin of a coronary artery with the goal to better understand the natural and surgical history of this anomaly as well as to develop evidence-based treatment and management guidelines. In this report, we describe the data we have collected from the registry and the current state of the registry.
Previous analyses have suggested an association between centre volume and in-hospital mortality, post-operative complications, and mortality in those patients who suffer from a complication. We sought to determine the nature of this association using a multicentre cohort.
All the patients, aged 18 years or younger, undergoing heart surgery at centres participating in the European Congenital Heart Surgeons Database (2003–2013) were included. Programmes were grouped as follows: small <150; medium 150–250; large 251–349; very large >350. Multivariable logistic regression was used to identify the differences between groups with the adjusted in-hospital mortality, onset of any and/or major complication, and in-hospital mortality in those patients with any and/or major complication. The outcomes were adjusted for patient specific risk factors and surgical risk factors.
The data set consisted of 119,345 procedures performed in 99 centres. Overall, in-hospital mortality was 4.63%; complications occurred in 23.4% of the patients. In-hospital mortality in patients with complications was 13.82%. Multivariable logistic regression showed that the risk of in-hospital death was higher in low- and medium-volume centres (p<0.001). The rate of the occurrence of any post-operative complication in small, medium, and large programmes was lower compared with very large centres (p<0.001). Low- and medium-volume centres were associated with significantly higher mortality in patients with any complication (p<0.001).
Our analysis showed that the risk of in-hospital mortality was lower in higher-volume centres. Although the risk of complications is higher in high-volume centres, the mortality associated with complications that occurred in these centres was lower.
In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children’s Hospital.