We present the images of the first superior-type caval vein defect diagnosed in a foetal echocardiogram.

Development of dilated cardiomyopathy in patients with congenital complete atrioventricular block with or without pacemaker is well described. We report a case of dilated cardiomyopathy in a child with congenital complete atrioventricular block, long-QT syndrome, and VVI pacemaker. Temporary pacing in the right ventricular outflow tract demonstrated a 63% increase in cardiac output. After change to biventricular DDD pacing, left ventricular systolic function and diastolic dimensions normalised.

We report the use of levosimendan in two febrile, neutropenic children with cancer – one post bone marrow transplant – with acute heart failure following chemotherapy. Initial management with epinephrine, milrinone, and diuresis was unsuccessful. Infusion of levosimendan without a loading dose was added to the ongoing heart failure therapy, which resulted in persistent symptomatic and echocardiographic improvement without major side effects.

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.

We report the case of a novel mitochondrial DNA mutation in the MT-ATP8 gene in an infant with tetralogy of Fallot. Next-generation sequencing was applied to sequence whole mitochondrial DNA of the patient. A known Leber's hereditary optic neuropathy-associated mutation (G9804A), a heteroplasmic T7501C mutation (17%), and a novel C8481 T Pro > Leu missense mutation in the MT-ATP8 gene was identified.

We present a case of double aortic arch with a predominant right and a double arterial duct detected by echocardiogram in a 28-week gestation foetus. The first evaluation revealed that both arches were perfused; the 1-month postnatal echocardiogram showed the closure of both arterial ducts and the partial obliteration of the left aortic arch between the left subclavian artery and the dorsal aorta. In our case, the postnatal obliteration of the left arch in a double aortic arch was probably due to the closure of the left-sided arterial duct.

We present the case of percutaneous removal of a fractured stent fragment during cardiac catheterisation using a novel stretching method. The procedure was performed in a 9-month-old infant. The small distal fragment of a fractured stent in the aorta was stretched using a two-sided approach – that is, from the carotid and femoral artery. This manoeuvre allowed for the removal of the stretched, linear-shaped stent part through a 6 Fr sheath without any local and general complications.

We describe an atypical case of an atrioventricular septal defect with a common atrioventricular junction in which the right-sided component of the common atrioventricular valve was imperforate, producing tricuspid atresia with a severely hypoplastic right ventricle and an ostium primum defect. We discuss the implications of the anatomic findings with regard to concepts of cardiac development, drawing a comparison with similar cases previously reported.

Persistent pulmonary hypertension of the newborn is a source of considerable mortality and morbidity. Anomalous origin of one pulmonary artery, an uncommon congenital cardiac malformation, is a rare cause of persistent pulmonary hypertension. Here, we report the case of a patient with an anomalous origin of one pulmonary artery from the innominate artery who presented with persistent pulmonary hypertension refractory to treatment.

Cor triatriatum sinister is an uncommon congenital cardiac anomaly accounting for 0.1% of all congenital cardiac malformations, with an anomalous venous return being a frequently associated condition. We present the case of a 14-year-old girl misdiagnosed as having an atrial septal defect with pulmonary hypertension who actually had a cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein.

Shwachman–Diamond syndrome is an inherited bone marrow failure and cancer predisposition syndrome that affects multiple organ systems, including bone, pancreas, and, to a lesser extent, the heart. Myocardial fibrosis, necrosis, and a case of dilated cardiomyopathy have, so far, been described. We report the first case of atrioventricular septal defect in a patient with Shwachman–Diamond syndrome.

Myocardial bridging is usually seen in the setting of hypertrophic cardiomyopathy or left ventricular hypertrophy. It is rarely reported in an asymptomatic patient with an otherwise structurally normal heart. Familial subaortic stenosis is also a rare entity, and its mode of inheritance is still unknown. Here, we described the case of a 13-year-old asymptomatic girl with a positive family history of sudden cardiac death and subaortic stenosis who was diagnosed with severe myocardial bridging concomitant with familial subaortic stenosis.

Left atrial appendage aneurysm is an extremely rare anomaly and as such has been rarely imaged or seen intraoperatively with very little accumulated management experience. The available scant published literature stresses resection on cardiopulmonary bypass as the safest and by far the most commonly applied technique. We suggest a novel alternative imaging-guided management utilising an off-pump tourniquet snare technique under live transoesophageal echocardiography.

Adequate arch augmentation for interrupted aortic arch repair is quite important to avoid post-operative recoarctation and bronchial compression. We describe here two successful cases of aortic arch reconstruction using autologous materials such as a pulmonary artery patch and a reversed left subclavian artery flap in infants with an interrupted aortic arch type B complex.

Purulent pericarditis is a rare diagnosis to be made. It is exceedingly rare as a primary infection. We describe the case of an 18-month-old boy who presented with primary purulent pericarditis and developed a secondary endocarditis. Current literature on the subject is reviewed and discussed.

The authors describe a case of Kawasaki disease who presented fever, coronary artery aneurysm, and ischaemic intestinal obstruction requiring stage resections. A review of literature of such association is performed. Atypical Kawasaki disease is more complex to diagnose and in combination of persistent fever with no definite cause and surgical abdominal pain, Kawasaki disease should be considered in the differential diagnosis.