Significant challenges face developing countries as a result of the maldistribution of access to healthcare throughout the world, specifically access to paediatric cardiac care. Sustainable paediatric cardiac programmes must be established in developing countries to provide care to all children with congenital heart disease. Education and research are essential components to sustainable paediatric cardiac programmes in developing countries to define local problems and the incidence of disease, and to generate solutions thereto related. Research can contribute to developing local expertise, improving technology, providing opportunities for local talent, generating financial resources, enhancing the dignity of people, and the facilitating resolution of health problems throughout the world. Clinical trials conducted in developing countries should meet the same ethical standards as trials based in developed countries.

We report the case of a 2-year-old boy with severe Langerhans cell histiocytosis who had tricuspid endocarditis caused by Staphylococcus lugdunensis and required surgery despite appropriate antimicrobial therapy. Through this case and literature review of endocarditis caused by S. lugdunensis in children, we highlight pitfalls and mistakes to be avoided in the management of this rare but serious infection.

We report two patients in whom an initially completely closed arterial duct reopened 6–12 months after implantation of the Rashkind USCI® umbrella. We are not aware of any other reports of this occurrence. No obvious reason could be found for the recanalization.

A male infant, aged 2 month, with Kawasaki disease had a myocardial infarction despite intravenous infusions of gamma globulin and aspirin at high dosage. He developed progressively a thin walled, dilated aneurysm of the apex of the left ventricle which became lined with thrombus despite treatment with warfarin. Another boy, aged 6 years, was noted on the 10th day of the evolution of Kawasaki disease to have developed a giant aneurysm of the main stem of the left coronary artery. Despite infusion of gamma globulin, the aneurysm remained unaltered and developed a thrombus. The thrombus resolved following treatment with warfarin, though the giant aneurysm has persisted. These two cases illustrate the serious consequences that can follow Kawasaki disease despite management optimal by current standards.

Occasionally complex congenital cardiac disease presents for the first time in adulthood, encouraging us to reflect anew on our understanding of familiar conditions. A 59-year–old woman, who had had two normal pregnancies, attended with breathlessness and was found to have a double inlet left ventricle with concordant ventriculoarterial connections and a straddling tricuspid valve adherent to the malaligned ventricular septum.

We report a 12-year-old girl who had multiple congenital cardiac lesions, specifically an arterial duct, left pulmonary arterial stenosis, an atrial septal defect in the oval fossa, and mild Ebstein's malformation of the tricuspid valve. Therapeutic transcatheter intervention was performed to stent the left pulmonary artery, occlude the arterial duct with a coil, and place a device to close the atrial septal defect. Subsequent to the catheterization, she complained of hoarseness, which was shown to be due to entrapment of the left recurrent laryngeal nerve between the coil used to close the arterial duct and the stent placed in the left pulmonary artery. Laryngoscopy confirmed paralysis of the recurrent laryngeal nerve.

Idiopathic calcification of the coronary arteries is a rare hereditary condition of infancy. Complications include cardiac ischaemia, cardiac failure, and systemic hypertension. We present three patients with this condition, which in each case masqueraded as other cardiac diseases, with no indication of the specific diagnosis prior to autopsy.

A 5 month old female presented with respiratory failure and hypertension and died within 24 hours of admission. All the coronary arteries were thick-walled, with narrow lumens. The aorta, great vessels, and renal arteries also showed thickening of the wall. Histology confirmed calcium in the internal elastic lamina of all vessels. The second patient was a female baby of 2 months, diagnosed with a large ventricular septal defect. She died suddenly prior to surgery. At autopsy, the orifice of the right coronary artery was reduced to a pinhole. The coronary arteries showed white patches of calcification, with associated ventricular infarction. The third patient was an 11 year old female who presented with cardiac failure, and had been diagnosed with dilated cardiomyopathy. Two weeks later, she died suddenly. The coronary arteries were patent, but firm with calcification and narrowed, with associated ventricular infarction. Our experience shows that idiopathic arterial calcification of infancy should always be considered in infants and children presenting with hypertension, cardiac failure, or sudden death.

It is universally agreed that efforts to improve quality benefit from the analysis of outcomes. Yet, it is challenging to compare results across institutions because factors other than performance also impact outcomes. Two factors that complicate the analysis of outcomes after congenital cardiac surgery are case-mix and random statistical variation. Case-mix refers to differences in the mix of patients and their risk-factors at different institutions that may cause some centres to have more frequent complications and lower survival regardless of their true performance. Random statistical variation refers to fluctuations in outcomes that occur at random and follow the laws of probability. A variety of statistical methods exist to address these issues and make provider comparisons more fair. We explain a few common approaches including stratification, regression analysis, and confidence intervals. Concepts are illustrated using artificial data from two hypothetical hospitals, as well as real data from a multi-institution registry.

A 15-month-old child underwent percutaneous expansion of a Melody transcatheter pulmonary valve in the mitral position to accommodate growth after initial surgical implantation during infancy, but transiently decompensated after valvuloplasty owing to stent malformation. The Melody valve in the mitral position of small patients can be further expanded by percutaneous dilation, but there are a number of potential complications and technical improvements to consider.

To evaluate the influence of the size of the defect and the age of surgical repair on left ventricular mechanics, including geometry, shape, diastolic and systolic function as well as myocardial contractility, we used cross-sectional echo-Doppler to study 20 patients (12 males, 8 females) who had undergone successful surgical closure of a ventricular septal defect. The patients were divided in two groups, corrected early and late, on the basis of the degree of left-to-right shunting (ratio of pulmonary to systemic output of greater or less than 2.5/1) and the age at the surgical repair (older or younger than 2 years of age). The group undergoing early correction included 11 patients, mean age 7.1 ± 1.8 years (range 4.2–11.8 years) having surgery at mean age of 1.3±0.6 years for a large ventricular septal defect (mean ratio of pulmonary to systemic output of 3.1/1; range 3.4–2.7/1) with a mean postoperative follow-up 4.6±1.9 years. The group of nine patients undergoing late correction had a mean age of 11.3±4.9 years (range 6.7–17.2 years), with a later surgical repair (mean age 4.7±2.7 years) for a moderate-sized ventricular septal defect (mean pulmonary/systemic output ratio 2.1/1; range 2.3–1.7) and a mean postoperative follow-up of 7±4.2 years. Each group of surgically repaired patients was compared with a control group matched for age, body surface area and gender. No significant differences were found between the normal controls and those undergoing early correction for any assessed functional index regarding left ventricular geometry (normalized volumes and mass for body surface area, mass/volume and thickness/radius ratios), shape (long axis–short axis ratio), diastolic (mitral and pulmonary venous flow patterns) and systolic (fractional shortening and rate-corrected mean velocity of circumferential fibre shortening) function. In addition, the data points for each patient for the rate-corrected mean velocity of circumferential fibre shortening to end-systolic stress relationship were within the 95% confidence limits of normal, suggesting normal left ventricular contractility. On the other hand, the patients undergoing surgery at a later age showed a persistent increase of the normalized left ventricular end-diastolic volume and mass, with an higher mass/volume ratio and reduced end-systolic stress compared with normal controls. Furthermore, left ventricular shape (long axis–short axis ratio) was abnormal at end-diastole but with its normal values at end-systole. Our data suggest that, in the presence of a large ventricular septal defect, early successful surgical repair <2 years of age results in complete recovery of left ventricular mechanics in the postoperative follow-up. In ntrast, surgical closure at >2 years of age, even for a moderately sized ventricular septal defect, deleteriously affects postoperative left ventricular geometry and shape. Since prolonged volume overload may be detrimental to myocardial function, earlier surgical repair should be recommended.

We present a young infant with rapidly progressive idiopathic dilation of the right atrium, complicated by symptomatic and dynamic obstruction of the airways secondary to proximal obstruction which was reversed subsequent to surgical reduction of the right atrium. We discuss the surgical implications of the distorted cardiac anatomy, along with post-operative complications, which included refractory arrhythmias.

An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.

We describe a complex case of obstruction of the left ventricular outflow tract in one of Dr. Noonan's original patients. Intraoperative findings revealed pathology at the valvar, subvalvar and supravalvar positions. Patients with Noonan syndrome are traditionally described as having right-sided cardiac pathology. Review of the literature revealed left-sided lesions to occur in a substantial number of these patients. We therefore suggest the routine employment of cardiac ultrasonography in all patients with Noonan syndrome with attention directed toward left-sided pathology, as well as the frequent pulmonary valvar pathology.

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.