We present the case of a five-year-old child with an inlet ventricular septal defect, subpulmonic stenosis, hypoplastic right ventricle, and straddling tricuspid valve who received a successful one-stage biventricular repair with right ventricular rehabilitation, right ventricular outflow tract augmentation, papillary muscle transposition, ventricular septal defect closure, and fenestrated atrial septation. This report outlines the surgical decision making and operative technique.

Congenital rupture of tricuspid chordae tendinea leading to severe tricuspid insufficiency is an extremely rare pathology associated with signs and symptoms of congestive heart failure presenting at birth. If the diagnosis and treatment of this pathology are not made early in life fetal demise may become inevitable.

We herein present a neonate with central cyanosis and congestive heart failure due to rupture of an anterior leaflet chordae resulting in severe insufficiency of the tricuspid valve who was treated with appropriate surgery.

Tricuspid valvectomy is a rare surgical intervention, and knowledge regarding long-term outcome in children is lacking. We report a favourable outcome 11 years after tricuspid valvectomy in early infancy without subsequent surgery or other cardiac interventions. Specific criteria for timing of re-intervention are lacking. Application of adult tricuspid and pulmonary regurgitation recommendations is helpful but has limitations.

Ebstein’s anomaly is a rare CHD that is characterised by caudal displacement of the functional tricuspid annulus and a dysfunctional tricuspid valve owing to a failure of proper leaflet coaptation. We present a balanced variant of Ebstein’s anomaly, in which the overgrowth of the septal leaflet had allowed proper coaptation of the tricuspid leaflets, thus preserving the valve function.

Libman–Sacks endocarditis may be the first manifestation of systemic lupus erythematosus. The risk of its occurrence increases with the co-existence of the anti-phospholipid syndrome. Changes usually involve the mitral valve and the aortic valve. In this report, we present a case of Libman–Sacks endocarditis of the tricuspid valve in a teenage girl.

This December Issue of Cardiology in the Young represents the 12th annual publication generated from the two meetings that compose “HeartWeek in Florida”. “HeartWeek in Florida”, the joint collaborative project sponsored by the Cardiac Center at the Children’s Hospital of 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 15th year, with the Annual Postgraduate Course in Pediatric Cardiovascular Disease, organised by The Children’s Hospital of Philadelphia and entering its 18th year. This December, 2014 Issue of Cardiology in the Young features highlights of Johns Hopkins All Children’s Heart Institute’s 14th Annual International Symposium on Congenital Heart Disease, which was held at the Renaissance Vinoy Resort & Golf Club, Saint Petersburg, Florida, from 15–18 February, 2014. This Symposium was co-sponsored by The American Association for Thoracic Surgery (AATS) and had as its special focus “Diseases of the Cardiac Valves from the Fetus to the Adult”. We acknowledge the tremendous contributions made to paediatric and congenital cardiac care by Duke Cameron and Joel Brenner, and therefore we dedicate this December, 2014 HeartWeek Issue of Cardiology in the Young to them. Duke Cameron is Professor of Surgery at Johns Hopkins University and Cardiac Surgeon-in-Charge at The Johns Hopkins Hospital. Joel Brenner is Professor of Pediatrics at Johns Hopkins University and Director of the Taussig Heart Center at Bloomberg Children’s Center, The Johns Hopkins Hospital. Together, Joel and Duke lead the proud paediatric and congenital cardiac programme at The Johns Hopkins Hospital.

Advances made in the understanding of the molecular biology of the cardiac valves have been truly spectacular. Not all of those investigating these aspects, however, have an appropriate understanding of the underlying anatomy. Partly, this reflects problems in describing the components of the various valves, a difficulty also emphasised by surgeons who repair or replace the valves. In this review, we describe briefly the overall anatomy of the cardiac valves, pointing to their similarities and differences. We then suggest that uniform terms can be developed to account for the components of the valves, treating them as complexes that guard the atrioventricular and ventriculo-arterial junctions. The atrioventricular valvar complex is made up of an annulus, leaflets, tendinous cords, and papillary muscles. The tension apparatus is required to hold the leaflets together against the force of ventricular systole. The ventriculo-arterial complex is also based on the leaflets, but supported within the valvar sinuses, and limited distally by the sinutubular junction. It is the semilunar nature of the leaflets that underscores their snug closure during ventricular diastole. The complexes thus defined can be separated to produce paired valves in the normal arrangement, or to produce common valves in the congenitally malformed hearts. Knowledge of development now permits accurate inferences to be made regarding the origin of the various components, and their relevance to valvar disease. The valvar leaflets are developed from the endocardial cushions formed in the atrioventricular canal and the outflow tract by a process of endothelial-to-mesenchymal transformation. The papillary muscles of the atrioventricular valves are then derived from the trabecular layer of the developing ventricular walls, whereas the sinuses of the ventriculo-arterial valves are formed by additional growth of the non-myocardial tissues, concomitant with excavation of the outflow cushions to form the leaflets.

Objective: Symptomatic neonates with Ebstein’s anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. Methods: A total of 32 consecutive symptomatic neonates with Ebstein’s anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients’ weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein’s anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes’ palliation and 2 Blalock–Taussig shunts. Six recent patients with Ebstein’s anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. Results: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein’s anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). Conclusions: Surgical management of neonates with Ebstein’s anomaly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Between February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

Tricuspid valve endocarditis on a normal valve has been rarely documented in children. In all five previous cases the infecting organism was Staphylococcus aureus. We report five infants and children with tricuspid valve endocarditis, whose ages ranged from four months to four years. In all of them large vegetations were identified by two-dimensional echocardiography. Positive blood cultures were found in four of five patients. Staphylococcus aureus, Corynebacterium species, Haemophilus influenzae and Candida albicans were the respective isolates. There were three deaths, all relatively sudden, 10–33 days after commencement of antibiotic therapy. At the only post mortem carried out in the patient with Candida albicans endocarditis and Down syndrome, there were two large fungating masses straddling the tricuspid valve. They appeared to have obstructed the circulation. Microscopic examination of a lymph node and the thymus showed features consistent with an immune deficiency syndrome. One child has been lost to follow-up. In the remaining survivor, progressively severe tricuspid regurgitation with congestive cardiac failure, necessitated open heart surgery 13 months later. A homograft replacement of the destroyed septal leaflet was carried out. Tricuspid valve endocarditis in an infant or child is a serious and potentially lethal condition. Early surgical intervention to remove large obstructive vegetations appears to be warranted.

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.

Ebstein's malformation is a rare congenital cardiac malformation, accounting for about 0.5% of all congenital cardiac lesions. We report our experience with the anomaly as encountered at the Sudan Heart Centre from July 2004 to April 2005. Diagnosis was based on the echocardiographic demonstration of displacement of the septal leaflet of the tricuspid valve towards the ventricular apex by greater than 8 millimetres per metre squared. Results: In a period of 10 months, we identified 12 patients with the malformation, this number constituting 2% of all patients seen with congenital cardiac disease. The age ranged from 2 weeks to 35 years. Of the patients, half were asymptomatic, while the other half presented with congestive cardiac failure, 4 of these having cyanosis in addition to heart failure. Associated diseases included chronic renal failure, impaired hearing, stunted growth, and developmental delay. Electrocardiographic abnormalities included peaked and tall P waves, seen in four-fifths, an Rsr pattern, first degree atrioventricular block, atrial fibrillation, and Wolf–Parkinson–White syndrome, including the Mahaim pattern of pre-excitation. Associated abnormalities diagnosed echocardiographically included atrial septal defects, prolapse of the leaflets of the mitral valve, left ventricular dysfunction, atrial septal aneurysm, pulmonary valvar stenosis, and pericardial effusion. Conclusions: Ebstein's malformation was seen four times more frequently at the Sudan Heart Centre when compared to the average frequency reported in the Western literature. Many of the associated diseases encountered in Sudan had not previously been reported.

As far as we are aware, right-sided bacterial endocarditis has not previously been described as presenting with systemic illness and testicular swelling. We report a teenager who presented with this unusual combination as a consequence of right-sided endocarditis. He presented with high fever, with chills and rigor, along with painful enlargement of the left testicle, a productive cough with progressive breathlessness, and joint pains. His blood culture was positive for Staphylococcus aureus, and a computerised tomographic scan of the chest revealed multiple pulmonary emboluses. Ultrasound of the testicles showed features of inflammation, and an echocardiogram revealed a vegetation on the tricuspid valve.

We report a patient with Down's syndrome referred to our Department of Echocardiography for evaluation after an episode of tachyarrhythmia. We diagnosed Ebstein's malformation. As far as we know, this association has been reported previously on only three occasions. We review these previous cases, describe our echocardiographic techniques, and discuss our experience.