Primary vascular tumours of the heart are rare and heterogeneous in their presentation and classification. We present a primary intramuscular vascular malformation of the left ventricle in an asymptomatic 12-year-old girl. Characteristics on cardiac magnetic resonance imaging, specifically increased signal intensity on T2-weighted images, and marked contrast enhancement with gadolinium were suggestive of increased vascularity. Histologically, the mass was determined to be an intramuscular vascular malformation of the small vessel arteriovenous subtype. This represents one of a select few intramuscular vascular malformations of the left ventricle reported in children. Our patient remains completely asymptomatic and has had no change in the size and appearance of the mass after more than 30 months of follow-up.

Acute coronary syndrome is a cause of cardiac-induced chest pain and rarely seen among childhood. It should be kept in mind in patients presenting with typical chest pain with a history of CHD and/or surgery, as this will lead to life-threatening complications and death. We present an adolescent with a history of Bentall operation who had acute coronary syndrome owing to coronary thrombosis as a result of inappropriate drug use.

Seckel syndrome is a very rare autosomal recessive disorder also known as bird headed dwarfism”. It is characterised by proportional short stature, low birth weight, dysmorphic facial appearance, and mental retardation. In addition to its dysmorphic features, skeletal, endocrine, gastrointestinal, haematologic, genitourinary, and nervous system has been involved. Cardiovascular features very rarely associate with Seckel syndrome. We report two patients with Seckel syndrome, one with dilated cardiomyopathy and the other with multiple ventricular septal defects. Dilated cardiomyopathy and isolated ventricular septal defect have not been previously reported in Seckel syndrome. Cardiovascular evaluation should be performed in all patients with Seckel syndrome. Early diagnosis of congenital and acquired heart diseases will reduce morbidity and mortality in these patients.

Apical ventricular septal defects are a heterogeneous group of septal defects that need accurate anatomic characterisation for planning appropriate management. Left ventricular-infundibular apical septal defects are defects between the left ventricular septum and the infundibular apex of the right ventricle with distinctive morphological features. We describe two illustrative examples of this septal defect with focus on their therapeutic implications and long-term outcome.

Owing to increased life expectancy, patients with grown-up congenital heart disease nowadays present various types of arrhythmias. We report treatment of a 27-year-old patient with tricuspid and pulmonary atresia who was referred to our department with symptomatic tachycardia. During electrophysiologic study, a diagnosis of typical AV-nodal re-entrant tachycardia was made, and he was successfully treated despite the described anatomic malformation.

Coronary artery fistulas are relatively rare congenital or iatrogenic heart defects that can present with or without symptoms. Symptomatic patients manifest as myocardial ischaemia, arrhythmia, or heart failure. We present a asymptomatic child with a large left anterior descending coronary artery to right ventricular fistula.

The incidence of neonatal thrombosis has been increasing in recent times because of the use of a central venous catheter. A treatment modality consisting of fibrinolytic agents has been administered for this life-threatening condition. This case report presents the successful management of a pulmonary artery thrombosis with a recombinant tissue-type plasminogen activator in a patient with neonatal congenital nephrotic syndrome.

For the first time in 2017 adults with CHD was included in the World Congress of Pediatric Cardiology & Cardiac Surgery. With growing numbers and more complex patients reaching adulthood, there is a growing need for more attention to this subspecialty. Although survival is excellent and now over 90% of patients reach adulthood, many have residual problems and complications. Heart failure and arrhythmias are most commonly encountered. Life-expectancy is nearly normal for mild lesions, but remains reduced in moderate-complex lesions and re-interventions are often needed. As most patients want to live a normal life, sports participation and pregnancy become very important issues. Finally, although innovative treatments are being developed, including for end-stage heart failure, we have to refine strategies for optimal care, including during the end-stage lives of our patients. This article provides an overview of a selection of topics in the field of adults with CHD presented during the 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS) in Barcelona.

Surveillance of fetal arrhythmias in the outpatient setting remains limited by lack of monitoring modalities. Despite technological advances made in the field of obstetrics, existing devices are not currently suitable to monitor fetal arrhythmias. In this report, the author describes the current and developing fetal heart rate monitoring technologies including the recent introduction of hand-held Doppler monitors for outpatient surveillance of fetal arrhythmias.

Acute myocardial infarction is rarely reported in children. Most of the cases are secondary to congenital anomalies or Kawasaki disease. Coronary artery total occlusion caused by fibromuscular dysplasia has never been reported in young children. Here we report a case of a 12-year-old boy with fibromuscular dysplasia, who underwent successful coronary intervention for acute myocardial infarction.

Allergic reactions related to drug use is a common entity presenting often from minor urticaria to life-threatening anaphylactoid reactions. A common but easily overlooked diagnosis, Kounis syndrome, is an established hypersensitivity coronary disorder induced by drugs, foods, environmental factors, and coronary stents that can present in the same way as non-allergy-induced acute coronary syndrome. Here within, we present a unique case of dual presentation of Kounis syndrome and prolonged QTc in a young patient after a single dose of Domperidone and Lansoprazole.

Despite acceptable survival for Fontan operation, there are concerns about late complications affecting the major organs. We herein present two cases of adults after Fontan operation who developed focal segmental glomerulosclerosis. These cases suggest that focal segmental glomerulosclerosis is owing to haemodynamic incompetence associated with Fontan operation, including congestion, hypoxia, and hyperviscosity, which may be called Fontan-associated renal disease.

Mitral valve perforation is an uncommon aetiology of mitral regurgitation in the paediatric population. We present a case where 3-dimensional echocardiography assisted in the diagnosis of the source of mitral regurgitation and the surgical correlation.

Congenitally corrected transposition is a rare cardiac anomaly characterized by the combination of discordant atrioventricular and ventriculoarterial connections. Young patients with this lesion can present with congestive cardiac failure, usually secondary to a large ventricular septal defect or pulmonary stenosis. We report here our experience with a lady aged 79, admitted to our unit because of deterioration of her congestive cardiac failure as a consequence of uncorrected congenitally corrected transposition associated with degenerative severe aortic stenosis.