Coronary artery fistula with giant coronary artery ectasia is a rare abnormal CHD. Multidetector CT is useful for the diagnosis. Early diagnosis and surgery are recommended.

We present the case of an infant with a single functional ventricle who developed ventricular dysfunction and heart failure due to an electrical activation delay and dyssynchrony. Earlier recognition of this potentially reversible aetiology may have changed her poor outcome.

We describe a case of multisystemic cystic echinococcosis exhibiting intracardiac involvement. A lesion inside the lateral wall of the left ventricle induced negative T waves on leads V5 and V6.

We report the prenatal diagnosis and the neonatal follow-up of a patient with isolated total abnormal systemic venous connection to the left atrium. Right-sided and left-sided superior caval veins and the inferior caval vein were all connected to the left atrium. Pulmonary venous return was normal. This was associated with some right ventricular underdevelopment. To our knowledge, this is the first fetal description of this very rare congenital cardiac malformation.

Williams syndrome is a well-recognised congenital disorder characterised by cardiovascular, connective tissue, and central nervous system abnormalities. Coronary artery abnormalities are seen in patients with supravalvar aortic stenosis, but end-stage ischaemic heart disease is rare. We report a case of end-stage ischaemic heart disease due to severe coronary arterial stenosis, highlighting how cardiovascular MRI contributed to the management.

A 4-month-old girl with Down syndrome showed unexpected deterioration of pulmonary hypertension. Despite aggressive pulmonary vasodilation therapy, the patient died at 5 months of age. Lung autopsy showed that the pulmonary veins were obliterated by intimal fibrous thickening, and the media of the veins was arterialised with an increase in elastic fibres. Pulmonary veno-occlusive disease should be considered in the management of individuals with Down syndrome.

Infective endocarditis is a serious complication occurring after percutaneous pulmonary valve replacement with a Melody valve; it is of uncertain incidence, and presents a challenging diagnosis. Vegetations may be difficult to visualise with transthoracic and transoesophageal echocardiography. We report the role of intracardiac echocardiography in this condition by confirming the prosthesis involvement.

Laboratory investigations have shown the role of inflammation in the pathogenesis of pulmonary hypertension and improvement after anti-inflammatory drugs. Despite these observations, reports on the use of steroids to treat pulmonary hypertension in humans are absent from the literature. In this article, we report the use of glucocorticoids in the treatment of two children with pulmonary hypertension, demonstrating its potential utility.

In this article, we present a case of a desaturated Fontan patient with an infra-diaphragmatic venous collateral to the pulmonary vein, which was too tortuous to attempt closure at the source. A trans-septal approach was successfully used to close the collateral in a retrograde manner.

We report the case of 24-year-old woman who presented with ductal aneurysm 7 years after a triple ligation of a patent ductus arteriosus. She underwent successful repair through median sternotomy and under moderate hypothermic circulatory arrest and selective cerebral perfusion.

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.

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.

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.

We report the case of a young boy with a history of total occlusion of the superior caval vein, diagnosed early after complex neonatal cardiac surgery, who developed severe protein-losing enteropathy. Protein-losing enteropathy was precipitated by a relatively mild stenosis at the junction of the inferior caval vein with the right atrium. Percutaneous stent dilation of the veno-atrial junction definitively relieved the stenosis, and the protein-losing enteropathy subsequently resolved.

Cystinosis is a rare, autosomal-recessive genetic disorder. The kidneys are commonly involved, as there is cystinosin protein malfunction, and nephropathic cystinosis ensues. Although cardiac and vascular involvements are rare, we describe a unique case of aortic dissection in a 25-year-old female with cystinosis. We discuss the possible aetiologies of aortic dissection in this condition.

Catheter closure of membranous ventricular septal defects is generally not considered feasible in small infants. We report the successful closure of a membranous ventricular septal defect in a 1.8-kg infant with bilateral femoral artery occlusion using Amplatzer Duct Occluder II additional size device. The ventricular septal defect was crossed from the right ventricle, and the device was deployed using transthoracic echocardiographic guidance.

Aortopulmonary window is a rare cardiac developmental anomaly characterised by a communication between the ascending aorta and the pulmonary artery. Aortopulmonary window may be isolated or associated with cardiac defects such as ventricular septal defect, atrial septal defect, interrupted aortic arch, and tetralogy of Fallot. We report a case of aortopulmonary window associated with aberrant subclavian artery based on fetal two-dimensional echocardiogram. The mother was referred for fetal echocardiography because of multiple fetal anomalies. Prenatal echocardiography at 30 weeks of gestation revealed a defect between the main and right pulmonary arteries and the ascending aorta (type III). The patient was born at 38 weeks of gestation via caesarean delivery, and was admitted to the neonatal intensive care unit because of respiratory failure and multiple congenital anomalies. Postnatal echocardiogram and cardiac MRI confirmed the prenatal findings. In addition, this patient had severe Dandy–Walker malformation and renal anomalies with poor prognosis. The family decided to withdraw respiratory care support on day of life 4, and the neonate passed away shortly after.