A 4-month-old male infant presented with recurrent cough for 2 months. He had a shortened right upper limb with absent right thumb and continuous murmur in the left parasternal area. The X-ray showed an absent radius and the first metacarpal and phalyngeal bones on the right side. Echocardiogram revealed aortopulmonary window and small secundum atrial septal defect. Aortopulmonary window was successfully treated by device closure. Holt–Oram syndrome with aortopulmonary window is an extremely rare association.

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.

Hepatoblastoma is the most common malignant liver tumour in early childhood. The metastatic extension of hepatoblastoma into the left atrium via the pulmonary vein is rare. Reported lesions almost always involve a right-sided approach. Here we report the case of a 3-year-old girl with a recurrent hepatoblastoma at multiple sites, including the left atrium, brain, and lung. The patient was treated surgically for the prevention of further embolic complications and cardiac failure.

Intrapericardial teratomas are extremely rare and most often benign tumours. In this paper, we have described a case of intrapericardial teratoma diagnosed prenatally and successfully operated. The presented case is noteworthy as an example of potentially catastrophic cardiorespiratory distress caused by the space-occupying nature of the tumour. A multi-disciplinary approach is mandatory because the tumour most often arises from the ascending aorta and in some cases may require the use of cardiopulmonary bypass.

Improving population-level cardiovascular health is a public health priority. Guided by the American Heart Association's metrics for ideal heart health, we examined the prevalence of behavioural heart health indicators using a representative sample of urban adolescents. Less than 1% (0.6%) of the sample had ideal heart health. The alarmingly low percentage of ideal behavioural heart health suggests that primary prevention approaches to address cardiovascular health in youth are warranted.

Ascending aortic pseudoaneurysms are a rare complication following cardiac surgery. Their traditional surgical management carries high morbidity and mortality, especially in patients with multiple comorbidities. Transcatheter closure of these pseudoaneurysms using a septal occluder device has been previously reported in adults as an alternative approach with fewer complications. We report the first case of a novel percutaneous femoral arterial-transthoracic approach for exclusion of an ascending aortic pseudoaneurysm with an Amplatzer atrial septal occluder device in a 9-year-old child with Takayasu's arteritis.

Dilated cardiomyopathy is characterised by dilation and impaired systolic function. We present the case of a child with dilated cardiomyopathy caused by a 624 kb duplication of 6q22.31, which includes the phospholamban gene. The patient also has failure to thrive and developmental delay due to complex cytogenetic abnormalities including a 5p15 deletion associated with Cri du Chat and an 11p15 duplication associated with Russell–Silver syndrome.

We describe aortic root dilatation, severe aortic regurgitation, and pulmonary artery stenosis that were accidentally diagnosed 23 years after the arterial switch operation for transposition of the great arteries in situs inversus. We successfully performed the modified Bentall procedure and pulmonary artery reconstruction. The pathology of the dilated aortic root revealed intimal atherosclerosis and linear necrosis of the tunica media, suggesting the vulnerability of the pulmonary artery to systemic pressure.

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.

Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

The neuro-cutaneous syndrome tuberous sclerosis is commonly associated with rhabdomyomas in various organs including the heart. We are reporting a rare case of a 7-month old male child with congenitally corrected transposition of the great arteries associated with tuberous sclerosis and cardiac rhabdomyomas. To our knowledge, this rare association has not been reported so far.

A newborn baby girl was diagnosed with tetralogy of Fallot and an aortopulmonary window. At 3 weeks of age, the coexistent diagnosis of an anomalous left coronary artery from the pulmonary artery was made. Initial surgical intervention included a Takeuchi baffle of the left coronary artery, closure of the aortopulmonary window, and placement of a right Blalock–Taussig shunt. Complete repair was undertaken at 12 months of age. Close pre-operative assessment of the coronary arterial anomaly is crucial in patients with an aortopulmonary window and tetralogy of Fallot.

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.

Cardiac involvement is a rare initial presentation of systemic lupus erythematosus. An 11-year-old girl was described to have massive haemorrhagic pericardial effusion and cardiac tamponade, which was later diagnosed as systemic lupus erythematosus. Therefore, in children presenting with cardiac tamponade, systemic lupus erythematosus should be considered as one of the differential diagnoses, as morbidity and mortality associated with cardiac tamponade can be dramatically reduced with early diagnosis and use of steroids.

In this case report, we present a 5-month-old girl diagnosed with a unique combination of transposition of the great arteries and cor triatriatum sinistra. A 1-day-old female patient presented to our hospital with cyanosis since the early neonatal period. We confirmed transposition of the great arteries by echocardiography. The patient underwent arterial switch operation on day 8 and was discharged on day 35. After 5 months of the operation, the patient had a lower respiratory tract infection and was unable to gain weight. Echocardiography revealed mild neopulmonary regurgitation, minimal neoaortic regurgitation, and pulmonary arterial hypertension. In addition, a fibrous membrane was also seen dividing the left atrium. The patient was diagnosed with cor triatriatum and underwent successful resection of the membrane.

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.

Transposition of the great arteries with intact ventricular septum and aortopulmonary window is an extremely rare anatomic combination associated with high morbidity and mortality. We report a case of a 3-month-old baby with d-transposition of the great arteries with intact ventricular septum and a large aortopulmonary window as a mechanism of inter-circulatory mixing.

A 12-year-old girl presented with a protein-losing enteropathy. Symptoms started 4 weeks after undergoing the Fontan procedure at the age of 1.5 years for mitral atresia, ventricular septal defect, and double-outlet right ventricle. Upon referral for 3 weeks of rehabilitation after multiple interventional measures and drug treatments, she appeared in a dystrophic state, with decreased plasma protein and electrolyte levels along with occasional tetanic convulsions. Blood glucose levels after a lactose tolerance test were markedly reduced. The introduction of a lactose-free diet was quickly effective, with plasma protein and electrolyte levels raised to normal levels, and the girl's body weight increased without ascites or oedema. Molecular genetic examination revealed a homozygous C/C13910 polymorphism in the LCT gene. Conclusion: Protein-losing enteropathy in the Fontan circulation may be provoked by lactase deficiency and should therefore be ruled out to exclude this rather common condition.

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.