We describe the case of an 8-week-old infant with late presentation of severe refractory atypical Kawasaki disease. In addition to developing giant coronary arterial aneurysms and coronary thrombosis, she formed extensive bilateral arterial aneurysms throughout her systemic circulation.

Decision-making in congenital cardiac care, although sometimes appearing simple, may prove challenging due to lack of data, uncertainty about outcomes, underlying heuristics, and potential biases in how we reach decisions. We report on the decision-making complexities and uncertainty in management of five commonly encountered congenital cardiac problems: indications for and timing of treatment of subaortic stenosis, closure or observation of small ventricular septal defects, management of new-onset aortic regurgitation in ventricular septal defect, management of anomalous aortic origin of a coronary artery in an asymptomatic patient, and indications for operating on a single anomalously draining pulmonary vein. The strategy underpinning each lesion and the indications for and against intervention are outlined. Areas of uncertainty are clearly delineated. Even in the presence of “simple” congenital cardiac lesions, uncertainty exists in decision-making. Awareness and acceptance of uncertainty is first required to facilitate efforts at mitigation. Strategies to circumvent uncertainty in these scenarios include greater availability of evidence-based medicine, larger datasets, standardised clinical assessment and management protocols, and potentially the incorporation of artificial intelligence into the decision-making process.

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.

An 8-year-old girl, without any previous medical history, presented with a first short syncope. Physical examination was unremarkable. Transthoracic echocardiography revealed a thin-walled, echo-free cystic structure adjacent to the posterior wall of the left ventricle, and compressing it moderately. Other echocardiographic findings were normal. Both computed tomography and magnetic resonance imaging suggested a simple pericardial cyst, but during surgery we found an epicardial cyst with partial involvement of the circumflex branch of the left coronary artery. Cardiopulmonary bypass was necessary for successful resection of the cyst, leaving behind only the small area in continuity with the coronary artery.

Improving population-level cardiovascular health is a public health priority. Guided by the American Heart Association's metrics for ideal heart health, we examined the prevalence of behavioural heart health indicators using a representative sample of urban adolescents. Less than 1% (0.6%) of the sample had ideal heart health. The alarmingly low percentage of ideal behavioural heart health suggests that primary prevention approaches to address cardiovascular health in youth are warranted.

At some point during vertebrate evolution from species dwelling in water to living on land, the ancestral double or right aortic arches became single and left-sided in mammals, including humans, as the result of synchronous developments in cardiovascular and respiratory embryogenesis. Since left-sided aortic arches are unique to mammals, hemodynamics related to the placenta, specifically the requirement for a large arterial duct connecting to the descending aorta, may have led to switching from the right-sided to the left-sided arch. Additionally, development of a trilobar right lung and its bronchial tree, also unique to mammalian evolution, restricted the space above the high eparterial bronchus to a single large vessel. Consequently, mammals that mutated to the left-sided aortic arch avoided respiratory, digestive or circulatory problems that are often associated with an isolated right-sided aortic arch – something which could be considered a successful mistake. Due to natural selection, and survival of the fittest, the left-sided arch became the norm in mammals.

In congenital cardiac malformations where a large arterial duct is not mandatory in fetal life, as in Fallot's tetralogy or common arterial trunk, a right-sided aortic arch continues to occur, perhaps as an atavistic reversion to the anatomy seen in ancestral vertebrates.

Cardiac rhabdomyoma represents the commonest primary cardiac tumour. Its natural history is generally favourable, with resolution in the majority of cases. Surgical resection has been described for intractable arrhythmia, severe obstruction of the inflow or outflow tracts sufficient to compromise cardiac output, and systemic embolization. We describe an alternative palliative strategy, which was associated with regression of the tumour.

MAGNETIC RESONANCE IMAGING OF THE heart started in the 1970's, over the subsequent 25 years, enormous advances have been made in both the hardware and software of magnetic resonance imaging machines so that we can now obtain fast, detailed and accurate images of the heart and great vessels. In chronological terms, therefore, we can say that cardiac magnetic resonance imaging has come of age. In this article we will highlight some of the clinical applications of different magnetic resonance imaging techniques, as well as some recent developments. We will demonstrate that, in clinical investigation of congenital heart disease, magnetic resonance imaging has truly come of age.

We describe an unusual heart in which double outlet from the left atrium resulted in atrioventricular connections to a dominant left ventricle and to a left-sided rudimentary right ventricle, while the right atrium also connected to the left ventricle. This produced, in effect, double inlet left ventricle in association with discordant atrioventricular connections. The diagnosis was made by echocardiography and confirmed at post mortem examination.

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.

We report analysis of surgical results in 110 consecutive infants and children who underwent atrial repair of simple complete transposition using the Senning operation between February 1978 and May 1990. Mean age at operation was 5.4 months ± 6.1 (range 1 week to 4 years); 75 were less than 6 months old. There were 72 males and 38 females. Operative mortality rate was 5.5%, with one late death. Average follow-up is 48.1 months with 44 followed greater than 3 years, and 27 greater than 5 years. Postoperative cardiac catheterization was performed in 48 patients. Right ventricular ejection fraction averaged 0.52 ± 0.08 and was normal in 28 patients. Response of right ventricular ejection fraction to afrerload stress was abnormal in 12 of 14 patients tested. Right ventricular ejection fraction increased normally during exercise in 6 patients, but was abnormal in 15. Mild tricuspid regurgitation was noted in 12 patients. Mild obstruction of the superior caval vein was noted in 4 patients. Baffle leak requiring reoperation occurred in one patient. Fifty-seven of 90 patients are in sinus rhythm by latest electrocardiogram. Postoperative electrophysiologic studies were performed in 34 patients, and Holter monitoring in 25. Significant arrhythmia occurred in 26 patients: 6 patients required pacemakers for slow junctional rhythm or complete heart block; 20 additional patients have a junctional rhythm. Six patients have delayed sinus nodal recovery time. At last follow-up, 88 children (98%) are in New York Heart Association functional Class I, and 2 (2%) are in Class II. The Senning operation for compete transposition can be accomplished with a low operative and late mortality. Serious baffle complications requiring reoperation are rare. Surviving patients are clinically well, but arrhythmias and depressed right ventricular function may limit their long-term functional status.

I surveyed our echocardiographic database of the years between 1998 and 2012 for congenital abnormalities of the mitral valve in patients over 14 years. A total of 249 patients with mitral valve abnormalities were identified. Abnormalities included clefts in the mitral valve in 58 patients, double orifice of the mitral valve in 19, mitral stenosis with two papillary muscles in 72, and mitral stenosis with one papillary muscle in 51 patients. Supravalvar rings were found in 35 patients with a single papillary muscle, and mitral stenoses with two papillary muscles were found in 22 patients. Mitral prolapse occurred in 44 patients and mitral valvar straddle in five patients. The patients were evaluated by all modalities of ultrasound available over the course of time. Although some lesions were isolated, there were many lesions in which more than one mitral deformity presented in the same patient. The patients are presented showing anatomical correlation with autopsy specimens, some of which came from the patients in this series, and others matched to show correlative anatomy. These lesions remain rare as a group and continue to have high morbidity and mortality.

The study was designed to investigate the value of assessing right ventricular volume by transoesophageal 3-dimensional echocardiographic techniques compared with the standard transoesophageal cross-sectional approach. Echocardiography was performed using a multiplane probe. The 3-dimensional data sets were reconstructed after electrocardiographic and respiratory gated scanning, calculating the 3-dimensional volumes by the method of multiple slices. Cross-sectional determination of volume was performed using a modified area-length method, and the biplane multiple slice method following Simpson's rule. We studied 15 patients, with ages ranging from 6 to19 years, and body surface areas from 1.1 to 1.67 square metres. It proved possible top determine volumes with both methods in all patients. As determined by 3-dimensional echo, volumes were greater, being 113.0 plus or minus 61.2 millilitres at end-systole, and 61.7 plus or minus 36 millilitres at end-diastole, than those calculated from cross-sectional images using Simpson's rule, which gave values of 92.5 plus or minus 52 millilitres, and 41.3 plus or minus 22 millilitres. Compared to the values obtained using the area-length method, at 116.9 plus or minus 61 millilitres, and 60.3 plus or minus 30 millilitres, there were only small differences at end-systole, with a bias of 1.4, and limits of agreement of 20.9 millilitres, as well as at end-diastole, when bias was minus 3.8, and limits of agreement 22.3 millilitres. Correlation was also good, with coefficients of 0.93, and 0.91, respectively. The mean difference between the volumes by 3-dimensional acquisition and the multiple slice method was larger, with higher limits of agreement, at end-diastole showing bias of 20.5, and limits of agreement of 30.1 millilitres, and for end-systole bias of 20.4, and limits of agreement of 32.2 millilitres. Our data confirm that cross-sectional echocardiographic assessment of right ventricular volumes in children with atrial septal defects is quick, and reasonably reliable in clinical practice when employing the area-length method.

Foetal echocardiography has become an established subspecialty in paediatric cardiology. The skills to perform detailed assessment of foetal cardiac anomalies in a tiny, moving foetus surrounded by sometimes challenging layers of maternal tissue are not easy to obtain, and require prolonged periods of specific training and supervised scanning in expert centres. The goal of this overview is to discuss the importance of the awareness of the fact that expert examination of the foetal heart and in-depth knowledge of newborn surgical interventions are only a part of the picture; many cardiac abnormal foetuses do not just have an isolated cardiac defect. The extracardiac malformations, when present, play a major role in determining the treatment and prognosis. The challenge of providing optimal prenatal care to the pregnant woman, who remains the primary patient at least until the umbilical cord is cut, carrying a foetus with a cardiac malformation is to organise rapid, effective, and complete evaluation of all possible foetal anomalies in a multidisciplinary team approach, followed by clear and honest non-directive counselling on all possible options. Only then, with the maximum involvement of the parents, decisions on perinatal management can be taken by the team. Controversies do exist in view of various obstetric, paediatric, and other specialists on how to best organise this multidisciplinary care programme. In this paper, we provide a practical, logical, and efficient path of care for the pregnant woman suspected to carry a foetus with a heart defect.

Recent studies have shown that elevated red blood cell distribution width is associated with poor outcome in cardiovascular diseases. In order to assess the predictive value of red blood cell distribution width, before treatment with intravenous immunoglobulins, for coronary artery lesions in patient with Kawasaki disease, we compared 83 patients with coronary artery lesions and 339 patients without coronary artery lesions before treatment with intravenous immunoglobulin. Clinical, echocardiographic, and biochemical values were evaluated along with red blood cell distribution width. A total of 422 consecutive patients with Kawasaki disease were enrolled into our study. According to receiver operating characteristic curve analysis, the optimal red blood cell distribution width cut-off value for predicting coronary artery lesions was 14.55% (area under the curve was 0.721; p=0.000); eighty-three patients (19.7%) had coronary artery lesions, and 70% of the patients with coronary artery lesions had red blood cell distribution width level >14.55%. Logistic regression analysis revealed that fever duration >14 days (odds ratio was 3.42, 95% confidence interval was 1.27–9.22; p=0.015), intravenous immunoglobulin resistance (odds ratio was 2.33, 95% confidence interval was 1.02–5.29; p=0.04), and red blood cell distribution width >14.55% (odds ratio was 3.49, 95% confidence interval was 2.01–6.05; p=0.000) were independent predictors of coronary artery lesions in patients with Kawasaki disease. In Conclusion, red blood cell distribution width may be helpful for predicting coronary artery lesions in patients with Kawasaki disease.