We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.

Neonatal cardiac lupus is a rare, passively acquired autoimmune disease. We report a case of in utero myocarditis, confirmed postnatally, with papillary muscle rupture and severe tricuspid regurgitation after birth in the absence of conduction disturbances. Tricuspid repair was successfully performed with polytetrafluoroethylene neochordae. In this article, we discuss the pathophysiology, medical and surgical management, and implications at follow-up in this unique scenario.

To date, there are no reported cases of the Amplatzer Duct Occluder II: Additional Sizes’ use in percutaneous closure of an aortopulmonary window. We report a case of percutaneous closure of an aortopulmonary window in a 4.5-month-old, 6 kg child. Owing to the patient’s low weight, high risk of damage to the pulmonary valve, as well as the possibility of aortic and pulmonary artery obstruction, classic implants were deemed unsuitable and a decision was made to use the Amplatzer Duct Occluder II: Additional Sizes. The implant performed very well – the soft waist filled the aortopulmonary connection and both retention discs were properly shaped and pressed against vessel walls. Echocardiogram performed 12 hours after the procedure confirmed a correct occluder position. Low-profile retention discs had no impact on pulmonary valve function, despite the defect’s proximity to the valve. No obstruction of the aortic or pulmonary artery lumen was noted. The Amplatzer Duct Occluder II: Additional Size implant is a safe and useful device for percutaneous closure of an aortopulmonary window in a carefully selected group of patients.

We present the use of pulmonary vasodilators in three adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension. Patients improved by 1–2 NYHA classes with modest exercise-tolerance increase, and remained stable without side effects during 2.5, 10, and 14 years. Literature review revealed five studies with pulmonary vasodilators in heterogeneous, mostly repaired patient populations.

Mitral-aortic intervalvular fibrosa aneurysm is a rare disease with a few cases documented in children. We present the case of a 12-year-old boy, without a history of infection or previous surgery, diagnosed with MRI.

We describe three cases of newborns with complex CHD characterised by communication challenges. These communication challenges were categorised as patient, family, or system-related red flags. Strategies for addressing these red flags were proposed, for the goal of optimising care and improving quality of life in this vulnerable population.

A 15-day-old premature patient with ventricular septal defect and interrupted aortic arch type B underwent “hybrid” initial treatment consisting of bilateral pulmonary artery banding followed by stenting of the ductus arteriosus. A pre-registered CT scan was re-purposed with a new three-dimensional image fusion software (VesselNavigator) to create a roadmap for stent delivery.

We present an interesting and rare case of traumatic Gerbode ventricular septal defect and complete heart block. The multimodality images illustrate the diagnosis well. This case is an excellent demonstration of the diagnostic utility of multimodality imaging.

Some patients with pulmonary valve stenosis do not respond to balloon valvuloplasty and must undergo surgical repair. We report the case of a 12-year-old child with pulmonary valve stenosis and Noonan syndrome in whom we performed transcatheter Melody pulmonary valve implantation after balloon dilation failed. The result was excellent. This technique can be proposed as an alternative to surgery in such cases.

In this study, we describe a fetus with double-outlet atrium associated with complex arrangement of the ventricles and the great vessels. Various presentations of this malformation not described antenatally are discussed.

Disseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in <0.1% of Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

We report the case of a 12-year-old boy who presented with acute heart failure due to newly developed mitral valve regurgitation. The boy’s history combined with raised levels of inflammatory markers was suggestive of paediatric eosinophilic granuloma with polyangiitis. The echocardiographic course and rapid response to therapy are presented.

Double-chambered left ventricle is a rare cardiac anomaly. We report a case of double-chambered left ventricle in a one-and-half-year-old asymptomatic boy. We depict the use of three-dimensional echocardiography in the demonstration and diagnosis of the condition.

In this study, we describe the case of a 36-year-old woman who was diagnosed with hepatocellular carcinoma on a background of Fontan procedure for tricuspid atresia. She had worsening heart failure in the months before presentation, and early investigations noted derangement in liver enzymes and hepatomegaly. Liver biopsy confirmed a hepatocellular carcinoma. Hepatocellular carcinoma is a rare but recognised consequence of cardiac cirrhosis in Fontan patients.

Gaucher disease is an autosomal-recessive lysosomal storage disease characterised by the accumulation of glucocerebroside in macrophages; it is caused by mutations in glucocerebrosidase gene-1 in many organ tissues such as the liver, spleen, and bone marrow. Its different clinical subtypes, according to the presence and severity of neurological symptoms, are as follows: type I, non-neuronopathic (95%); type II, acute neuronopathic; and type III, chronic neuronopathic. Type IIIC is a rare subgroup characterised by cardiovascular involvement as well as eye-movement disorders and late-onset neurological symptoms. In such cases, homozygous D409H is the most frequently detected mutation. In this article, we report the case of a patient, aged 15 years and 8 months, with complaints of syncope and a diagnosis of type IIIC Gaucher disease.

Right pulmonary artery–left atrial fistula is a rare congenitalcardiac anomaly, and only a few articles published so far have reported the diagnosis and treatment of right pulmonary artery–left atrial fistula. In this study, we report three cases of successful transcatheter closure or surgical repair of right pulmonary artery–left atrial fistula.

We report the simultaneous use of cardiac re-synchronisation therapy and total cavopulmonary connection in a patient with dyssynchrony, wide QRS, and cardiac failure. To our knowledge, this simultaneous approach has not been reported previously. On follow-up, we noted that QRS width and brain natriuretic peptide levels improved. In addition, speckle tracking revealed improved synchronisation of ventricular wall motion.

Isolated right superior caval vein drainage into the left atrium is a rare congenital cardiac anomaly usually presenting with hypoxaemia and cyanosis. Surgical repair is the definitive treatment for this condition. In this report, we present the case of a 72-year-old patient with uncorrected anomalous drainage of a right superior caval vein into the left atrium, first diagnosed in the 1960s. To the best of our knowledge, this is the oldest reported unrepaired case.