This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein’s anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis “bidirectional Glenn”, was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.

The Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. As a result, patients with “Fontan physiology” may face a number of rare but potentially life-threatening complications including hepatic dysfunction, abnormalities of coagulation, protein-losing enteropathy, and plastic bronchitis. Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.

A 15-year-old girl gave a recent history of dyspnoea and “funny turns”. She had congenital aortic stenosis, previous valvotomies, a mechanical valve replacement, permanent pacemaker, atrial tachyarrhythmias, impaired ventricular function, systemic hypotension, pulmonary hypertension, and anxiety. The diagnosis of diaphragmatic flutter was delayed due to all the differential diagnoses and rarity of the condition. It was confirmed by observation, respiratory band monitoring, volume-time spirogram and fluoroscopy during an attack.

We describe a neonate who presented with an echogenic mass in the right atrium 8 weeks after closure of ventricular and atrial septal defects. On a routine post operative check up after discharge, a mass was detected in the right atrium on echocardiography. As a thrombotic formation was suggested, lysis was started, in combination with the administration of unfractioned heparin. As there was no change in echogenicity or size of the mass, it was surgically excised. Histopathological examination revealed a myofibroblastic inflammatory tumour.

Atrioventricular septal defect with common atrioventricular junction is a relatively common congenital cardiac malformation. It sometimes presents challenging surgical problems, especially when seen in combination with tetralogy of Fallot. We describe herein our current concepts regarding surgical repair of atrioventricular septal defect with and without tetralogy, discussing indications for surgery, timing, technical aspects, and outcomes.

We describe an infant of 8 months who presented with left ventricular dilation due to an extensive intralobar sequestration of the right lung. The pulmonary sequestration was associated with a patent arterial duct and a right aortic arch. Percutaneous closure of the anomalous aberrant artery feeding the sequestrated lung resulted in prompt regression of the left ventricular enlargement.

We report an infant with Ebstein anomaly of the tricuspid valve and severe branch pulmonary artery stenosis who was diagnosed with Williams–Beuren syndrome.

Loeys–Dietz syndrome is a newly described entity characterised by a constellation of arterial tortuosity, cranial malformations, and hypertelorism. We report a case of a 7-year old boy with confirmed Loeys–Dietz syndrome and discuss magnetic resonance imaging as a complete technique for assessment and follow-up of aggressive vascular pathology in the brain, thorax, and abdomen, which may dictate early surgical intervention.