We describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.

An extremely low birthweight infant (940 grams) with a rare variant of obstructed infracardiac total anomalous pulmonary venous return underwent stenting of the venous duct as bridge to later surgical intervention. While technically challenging, this procedure represents a bridge to surgery for infants who might otherwise not be surgical candidates.

Cardiac strangulation is a rare and potentially deadly complication of epicardial pacemaker implantation. A young boy presenting with chest pain and tiredness almost 7 years after pacemaker implantation was found to have cardiac strangulation. Literature review revealed 22 cases reported to date with a worrying rise in the number of reports over the past 3 years. Strangulation is associated with implantation of leads at a young age and appears to be related to somatic growth. Serial assessment with chest X-ray and echocardiogram is recommended, at least until full adult growth is attained with further coronary artery imaging reserved for symptoms or suspicious echocardiographic findings. If cardiac strangulation is diagnosed prompt replacement of the offending system is needed.

To date, there are no reported cases of the Amplatzer Duct Occluder II: Additional Sizes’ use in percutaneous closure of an aortopulmonary window. We report a case of percutaneous closure of an aortopulmonary window in a 4.5-month-old, 6 kg child. Owing to the patient’s low weight, high risk of damage to the pulmonary valve, as well as the possibility of aortic and pulmonary artery obstruction, classic implants were deemed unsuitable and a decision was made to use the Amplatzer Duct Occluder II: Additional Sizes. The implant performed very well – the soft waist filled the aortopulmonary connection and both retention discs were properly shaped and pressed against vessel walls. Echocardiogram performed 12 hours after the procedure confirmed a correct occluder position. Low-profile retention discs had no impact on pulmonary valve function, despite the defect’s proximity to the valve. No obstruction of the aortic or pulmonary artery lumen was noted. The Amplatzer Duct Occluder II: Additional Size implant is a safe and useful device for percutaneous closure of an aortopulmonary window in a carefully selected group of patients.

We report two instances of transient isolated right-sided myocardial hypertrophy in patients with an intact ventricular septum, normal thickness of the posterior wall of the left ventricle, and normal ventricular function, diagnosed by echocardiography on the third day of life. The two neonates, born at 36 and 38 weeks gestation respectively, had perinatal distress. Both were diagnosed as having isolated right ventricular hypertrophy with mild pulmonary hypertension, which disappeared in both cases within 8 weeks without any specific therapy. Though the cause of the ventricular hypertrophy remains unclear, we believe that it is the consequence of remodeling of pulmonary vasculature secondary to acute perinatal distress, resulting in persistent pulmonary hypertension and producing pressure overload on the right ventricle, and hence right ventricular hypertrophy. The finding of early and transient right ventricular hypertrophy, with normal left-sided structures and normal ventricular function, has thus far failed to gain attention in the paediatric cardiologic literature.

Prolonged fever, 5 days or more, is the cardinal feature of Kawasaki disease. We described a 5-year-old boy with initial presentations of fever, conjunctivitis, and strawberry tongue. The fever only lasted for 3 days. However, giant coronary aneurysms developed later. This patient reminds us that coronary complications could happen even the fever is less than 5 days. Criteria of fever duration might need reconsideration.

Congenital cardiac disease is the most common birth defect, occurring in approximately 1 in 1000 live births. Congenital cardiac defects have associations, whether with gender, race, or specific chromosomal abnormalities, potentially allowing grouping of defects to be studied in an effort to develop an understanding of aetiological factors. The Baltimore-Washington Infant Study provides full ascertainment of a population of infants with congenital cardiac disease born in a defined geographic region. The fundamental hypotheses generated at the inception of the Baltimore-Washington Infant Study included the central idea that the outcome of birth, including the development of congenital cardiac malformations, was influenced by environmental factors and their route of introduction into a genetically susceptible host. Evidence exists that supports the concept that both genetic and environmental factors contribute to the development of diseases of the left heart.

We present a case of anomalous origin of the left pulmonary artery from a left-sided brachiocephalic artery via an arterial duct in a patient with a right aortic arch. Associated intracardiac anomalies were a large perimembranous ventricular septal defect and the persistence of a small vertical vein, connecting the pulmonary veins normally incorporated into the left atrium to the brachiocephalic vein. Spontaneous closure of the arterial duct occurred in the first days of life and was responsible for the circulatory “isolation” of the left pulmonary artery. The clinical course, surgical strategy and possible embryological explanation are described.

The postpericardiotomy syndrome occurs in up to one-third of children undergoing cardiac surgery. Its treatment includes anti-inflammatory agents, diuresis, and drainage of effusions. Administration of steroids can have a dramatic effect, but is limited by adverse effects. Usually the syndrome lasts weeks only, and persistence beyond six months is exceptional. We describe a rare case of chronic postpericardiotomy syndrome, with recurrent pericardial effusions and steroid dependency, that was treated successfully with a low weekly dose of methotrexate.

A left superior caval vein frequently occurs in the malformed, as well as in the structurally normal, heart. Its physiological impact varies, and is determined by its connections and whether there are associated cardiac lesions. In this review, we describe 3 patients with such a vein, 1 without other lesions and 2 with the anomalous venous channel as a component of a complex cardiac malformation. In all cases, transcatheter techniques were used to treat the physiological dysfunction caused by the presence of the vein. The connections of the vein, and the complexity of the associated cardiac lesions, determine the options for treatment. We define the role of surgical as opposed to transcatheter intervention, and discuss strategies for transcatheter treatment.

Hypertrophic cardiomyopathy is a genetic myocardial disorder. In such patients, myocardial bridging is the most frequent encountered coronary arterial anomaly. Patients may, however, on occasion, present with other much rarer malformations of the coronary arteries. Duplication of the right coronary artery is a very rare anomaly. We have now encountered a patient with hypertrophic cardiomyopathy in whom we found myocardial bridging of the anterior interventricular coronary artery and duplication of the right coronary artery. To the best of our knowledge, this association has not previously been described.

We report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.

Purpose: Previous studies have shown that the underlying pathophysiologic mechanism in children with breath holding may be generalised autonomic dysregulation. Thus, we performed cardiac rhythm and heart rate variability analyses using 24-hour Holter monitoring to evaluate the cardiac effects of autonomic dysregulation in children with breath-holding spells. Methods: We performed cardiac rhythm and heart rate analyses using 24-hour Holter monitors to evaluate the cardiac effects of autonomic dysregulation in children during a breath-holding spell. Our study group consisted of 68 children with breath-holding spells – 56 cyanotic type and 12 pallid type – and 39 healthy controls. Results: Clinical and heart rate variability results were compared between each spell type – cyanotic or pallid – and the control group; significant differences (p<0.05) in standard deviation of all NN intervals, mean of the standard deviations of all NN intervals for all 5-minute segments, percentage of differences between adjacent RR intervals >50 ms, and square root of the mean of the sum of squares of the differences between adjacent NN intervals values were found between the pallid and cyanotic groups. Conclusions: Holter monitoring for 24 hours and heart rate variability parameters, particularly in children with pallid spells, are crucial for evaluation of cardiac rhythm changes.

Device occlusion of perimembranous ventricular septal defect is gaining popularity with the emergence of newer, softer occluders and improved technical know-how. We report a 26-year-old lady with a moderate size perimembranous ventricular septal defect who had a new onset of bundle branch block shortly after device closure. The patient subsequently developed a complete atrio-ventricular heart block.

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.

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.

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.