Coronary arteriovenous fistula is an uncommon clinical entity. The right coronary artery is the most common site of origin, and the fistula commonly drains into the right-sided cardiac chambers. Very rarely it can arise from the left main coronary artery, and fistulas draining into the superior vena cavity are extremely rare. We report a 12-year-old asymptomatic boy with a large coronary arteriovenous fistula between the left main coronary artery and superior vena cava, with aneurysmal dilatation of the left main coronary artery. As the fistula was very large and to prevent its complications, it was planned to close the fistula percutaneously.

Infantile Pompe disease is a rare inborn error of metabolism characterized by severe hypertrophic cardiomyopathy and generalised hypotonia occurring in infancy. We present a case of an infant with severe hypertrophic cardiomyopathy that resolved after treatment with enzyme replacement therapy.

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.

Plastic bronchitis is a severe complication after a Fontan procedure, with an estimated incidence around 1–2% and poor prognosis. We present the cases of four patients with plastic bronchitis after a total cavopulmonary connection with a stenosis of the left pulmonary artery that was stented successfully. In three of the four patients, symptoms improved after catheter intervention in combination with pulmonary vasodilator and inhalative treatment.

Ascending aortic aneurysm is uncommon in the paediatric population, and because of the rarity the aetiology, natural progression, and prognosis of the disease remain unknown. A 7-year-old boy with ascending thoracic aortic aneurysm (60×67 mm) underwent graft anastomosis from the sinotubular junction to the undersurface of the arch. Analytical determinations including karyotyping and genetic mapping were all normal. To our knowledge, idiopathic aneurysm of the ascending aorta in children is very rare.

An intrapericardial vacuolated mass compressing and displacing the heart was diagnosed by echocardiography in a foetus of 22 weeks gestation. The birth was induced for early signs of foetal distress at 29 weeks and, after two initial pericardial evacuation procedures, the tumour was resected radically 7 days after birth at a weight of 1.55 kg. Mass histology showed teratoma associated with yolk sac tumour. We comment on the overall approach adopted after foetal diagnosis and the histopathological features of the tumour, and try to draw conclusions on patient outcome data.

We report a case of pulmonary atresia with intact ventricular septum, but in the setting of transposed great arteries, and thus the left rather than the right ventricle was hypoplastic.

Acute heart failure related to anaphylactic shock is often reversible and necessitates aggressive support to ensure full recovery. We report the case of a 15-year-old boy who developed severe ventricular dysfunction and haemodynamic instability after administration of amiodarone and required temporary mechanical circulatory support with a left ventricular assist device. He had full recovery of cardiac function and returned to baseline neurologic status. This is the first report of successful left ventricular assist device use for recovery from cardiovascular collapse due to anaphylaxis.

We describe a case of a baby girl born with hypoplastic left heart syndrome consisting of mitral atresia, aortic atresia, hypoplastic ascending aorta, and left ventricle. The pulmonary arteries were hypoplastic, measuring 3 mm. Fluorescence in situ hybridisation analysis demonstrated a microduplication of chromosome 22q11.2. Subsequent array comparative genomic hybridisation showed a gain of 2.3 Mb in one copy of chromosome 22q at band 22q11.21. The proband underwent a successful Norwood procedure with Sano shunt and subsequently underwent bi-directional Glenn shunt and Fontan procedure. This report highlights the association between hypoplastic left heart syndrome with hypoplastic pulmonary arteries and chromosome 22q11.21 microduplication.

The levoatriocardinal vein provides alternative egress from the left atrium to the systemic veins in left-sided obstructive lesions. Although rare, it has been described in association with hypoplastic left heart syndrome. We report a case of hypoplastic left heart syndrome with levoatriocardinal vein and aberrant right subclavian artery where cardiac magnetic resonance imaging/angiography proved to be a valuable imaging modality for pre-operative evaluation.

A female baby suffered from a rare association between histiocytoid cardiomyopathy, left ventricular non-compaction, and Wolff–Parkinson–White syndrome causing severe and recurrent arrhythmic storms. Antiarrhythmic drugs, radiofrequency ablation of Purkinje tissue, and sympathetic denervation were ineffective. The implant of a cardiac defibrillator allowed her to survive till heart transplant. Compound mutation of CACNA2D1 and RANGRF genes were found. To the best of our knowledge, this is the first comprehensive description of the concurrence of these two mutations and histiocytoid cardiomyopathy.

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.

We report a case of unroofed coronary sinus not associated with the persistent left superior vena cava. Definite diagnosis of the unroofed coronary sinus was obtained by trans-oesophageal echocardiography, which revealed the unroofed portion with left-to-right shunt. Real-time three-dimensional trans-oesophageal echocardiography could show the whole pictures of the defect, which was useful information for surgical repair.

Heart failure is a common cause of death in patients with muscular dystrophy. Mechanical support may be an important component of long-term heart failure therapy in these patients. We present a report of a child with muscular dystrophy successfully implanted with a Heartware HVAD.

We describe a case of influenza A myocarditis and transient left ventricular wall thickening in a 14-year-old girl presenting with acute heart failure. Admission echocardiogram revealed significant left ventricular hypertrophy with depressed left ventricular systolic function. The aetiology of the ventricular thickening was demonstrated to be myocardial oedema using cardiac magnetic resonance imaging. The natural course of this unusual clinical presentation of acute myocarditis and the importance of cardiac magnetic resonance imaging in this challenging clinical setting are discussed.

We report a unique case of compression of the superior caval vein by an aneurysmal right pulmonary artery in a patient with tetralogy of Fallot with absent pulmonary valve.