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Cardiac tumours are extremely rare in children. Although more than 90% are benign, children can develop obstructive or embolisation derived symptoms, arrhythmias, constitutional symptoms, cardiac tamponade, or even sudden death. Although the majority are asymptomatic, and some spontaneously regress, appropriate follow-up is needed on a case-by-case basis, as patients may develop late symptoms. Definitive diagnosis is only possible through histological analysis; however, it is possible to infer tumour type with a high grade of certainty based on imaging features, particularly cardiac magnetic resonance. Surgical resection is advocated for those causing symptoms, obstruction, cardiac dysfunction, and high risk of embolisation. The aim of this review is to present the state of the art related to cardiac tumours in the paediatric population, in the context of our own experience.

Cardiovascular complications are the most frequent cause of death in patients with the Hutchinson–Gilford progeria syndrome. However, due to its rarity, studying the course of cardiac abnormalities has been a challenge. The cardiovascular phenotype helps to provide greater insight into the natural history of these abnormalities.

Vena caval filters remain as a useful tool in patients with deep vein thrombosis and contraindications to anticoagulation. Although they are rarely used in paediatric patients, they have been shown to be safe and effective when used in the inferior vena cava. In this case report, we describe the off-label use of a retrievable vena caval filter in the superior vena cava in an adolescent with acute lymphoblastic leukaemia with extensive thrombosis of the right upper neck veins as a means to reduce the risk of pulmonary embolism.

We report on a 50-year-old female patient, with several severe comorbidities and high-surgical risk, in whom we successfully performed a simultaneous transcatheter pulmonary and tricuspid valve-in-ring implantation to treat both bioprosthetic pulmonary valve dysfunction and native torrential tricuspid valve regurgitation, the latter previously managed with a Carpentier annuloplasty ring.

Percutaneous pulmonary valve implantation is a less invasive procedure to treat right outflow tract dysfunction related to surgical procedures such as repair of Tetralogy of Fallot. Despite the lower risks, complications have been reported, namely embolisation of the pre-stent. We report a case of a 16-year-old boy, whose procedure was complicated by embolisation of the pre-stents and the strategy used to reimplant them, prior to the successful implantation of a pulmonary valve.

Background:The use of statins in children, although not frequent, is recommended in specific clinical contexts, namely, familial hypercholesterolaemia, conditions carrying a moderate-high cardiovascular risk and sub-optimal cholesterol levels after implementation of lifestyle modifications. The aim of this study is to characterise children with dyslipidaemia managed with statins, followed at a tertiary referral centre in central Portugal.Methods and results:The authors carried out a retrospective and descriptive study made up of 66 patients (50% males, mean age of therapy onset 11.9 years) followed up at the Cardiovascular Clinic of a tertiary referral centre between January, 2012, and May, 2018. Clinical, analytical, and echocardiographic parameters were analysed. About 60.6% had clinical and/or molecular diagnosis of familial hypercholesterolaemia. On average, each patient had three cardiovascular risk factors, obesity (31%) being most prevalent, followed by arterial hypertension (14%). Statin therapy showed a statistically significant reduction in the lipid profile, particularly in the total cholesterol (23%) and low-density lipoprotein cholesterol (30%) levels, as well as in the carotid intima-media thickness (p = 0.015). Hepatic and muscle integrity markers were within normal range.Conclusions:Statins are safe and efficient in the management of children with hypercholesterolaemia. Our study showed that apart from its lipid-lowering properties, it also reduced significantly the carotid intima-media thickness and, implicitly, the cardiovascular risk of these patients.

Ruptured sinus of Valsalva aneurysm is a rare entity in children. To our knowledge, this is the youngest child reported in the literature with a sinus of Valsalva ruptured aneurysm being successfully treated percutaneously using an AmplatzerTM Duct Occluder II Additional Sizes device.

Superior–inferior ventricular arrangement with double-outlet left ventricle and malposition of the great arteries is an extremely rare congenital cardiac anomaly. The authors present the case of an infant who presented with cyanosis and respiratory distress. To the best of our knowledge, this is the first case reported.

Congenital stenosis of the pulmonary veins is a rare condition whose outcome is guarded despite the available treatment options. We report a case of a 6-month-old infant with significant stenosis of all four pulmonary veins.

Coronary artery fistulae are uncommon but may be haemodynamically significant, being an incidental finding in 0.1–0.2% of coronary angiograms. Even rarer is the association between fistulae and non-atherosclerotic coronary artery aneurysms. They most frequently originate in the right coronary artery, and the right cardiac chambers are the most common draining chambers. Most children are asymptomatic, whereas those older than 20 years may present with signs of congestive heart failure, infective endocarditis, myocardial ischaemia, or aneurysm rupture. Management is either surgical or via percutaneous means. We report the case of a 5-year-old child referred for assessment of an asymptomatic cardiac murmur. The echocardiographic evaluation showed an enlarged right atrium, a fenestrated atrial septal defect, and a giant right coronary artery aneurysm with a fistulous tract that appeared to drain directly into the right atrium. Computed angiocardiac tomography and cardiac catherisation confirmed the presence of a large right coronary fistula originating from the right coronary aneurysm draining into the right atrium. The patient underwent surgical ligation of the fistula and the post-operative course has been uneventful. He is currently on double antiaggregation therapy.