Double-chambered left ventricle is a rare CHD that is usually asymptomatic and managed conservatively but can present with ventricular arrhythmias or heart failure. It is important to differentiate from acquired diseases such as post-infarct pseudoaneurysm that need surgical treatment, and cardiac MRI offers an excellent diagnostic tool.

We encountered a paediatric case of graft failure due to antibody-mediated rejection after heart transplantation in which ivabradine was effective. Inappropriate sinus tachycardia in denervated transplanted hearts is a good indication for ivabradine administration as beta-blockers have a limited efficacy. To our knowledge, this is the first report on the effectiveness of ivabradine in a paediatric heart transplant rejection case.

Transposition of the great arteries is the most common cyanotic CHD in newborns. This CHD, in which the aorta arises from the right ventricle and the pulmonary artery from the left ventricle, is often accompanied by one or several defects such as atrial septal defect or patent foramen ovale, ventricular septal defect and patent ductus arteriosus, which allow the transition between both parallel circulations. Rarely, the disease may be accompanied by left ventricular outflow tract obstruction (subpulmonary obstruction) and coarctation of the aorta.

We present a highly complicated and unusual transposition of the great arteries patient with critical aortic coarctation and hypoplastic pulmonary arteries with abnormal outflow and course.

A baby with complete atrioventricular block was born with a birth weight of 1403 g. Isoproterenol was ineffective and emergency pacing was required. Since transcutaneous pacing was ineffective and transvenous pacing was untenable due to small body size, transesophageal pacing was performed for 3.5 hours until permanent pacemaker implantation. There were no complications. This is the first report of continuous transesophageal pacing in a very-low-birth-weight infant.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

The carotid artery is a valuable vascular access that can be used in patients who have undergone repetitive interventional and surgical procedures and premature babies. In the past, cut-down was used but nowadays, mostly the procedure is performed under ultrasonographic guidance. Complications such as bleeding, haematoma, and pseudoaneurysm may occur when the carotid artery is used as a vascular access for the procedures such as aortic balloon valvuloplasty, coarctation balloon angioplasty, or after interventional or surgical treatments to the carotid artery. Although pseudoaneurysm is very rare, prompt diagnosis and accurate treatment planning are life-saving. In this article, the diagnosis and treatment of pseudoaneurysm in the left common carotid after transcatheter coarctation balloon angioplasty in a 6-month-old infant will be presented.

Percutaneous atrial septal defect closure is widely used as an alternative to surgical repair in many hospitals. Infective endocarditis related to occluding devices is commonly known, but following that atrial septal defect closure with a device in a child is rare. This report describes an 11-year-old girl who developed late-stage bacterial endocarditis following incomplete endothelialisation after a percutaneous procedure.

Among Kawasaki disease patients with systemic artery aneurysms, the brachial and internal iliac arteries are the most commonly affected, and occlusions of both arteries are often found. However, the long-term fate of large common iliac artery aneurysms remains unknown, because their prevalence is very low. The long-term outcomes of common iliac artery aneurysms caused by Kawasaki disease in four patients (three females, one male) were investigated retrospectively based on their medical records and angiograms. Their ages ranged from 30 to 36 years-old. The onset age of Kawasaki disease ranged from 4 to 8 months, and the interval from the onset of Kawasaki disease to the latest angiogram ranged from 17 to 21 years. All patients had bilateral large coronary aneurysms and common iliac artery aneurysms with maximal diameters greater than 10 mm. Although all patients had multi-vessel coronary artery stenotic lesions and systemic artery aneurysms, they were asymptomatic. The three female patients underwent coronary artery bypass grafting, and the male patient underwent replacement of artificial vessels for large bilateral common iliac artery aneurysms at 3 years old of age. Over the long-term, common iliac artery aneurysms greater than 10 mm persisted as calcified aneurysms. However, they had no symptoms due to their common iliac artery aneurysms, and their ankle brachial pressure index was preserved, even if the stenosis of the common iliac artery developed as a late outcome, because the collateral arteries were well developed. The progression of stenosis of the common iliac artery after Kawasaki disease was slower.

This systematic review and meta-analysis were conducted to evaluate the prevalence of cardiac manifestations associated with multisystem inflammatory syndrome in children worldwide. We conducted electronic searches in Ovid MEDLINE, Ovid EMBASE, and the World Health Organization COVID-19 Literature Database from the inception of the SARS-CoV-2 pandemic to 1 January, 2022. Three authors independently screened the abstracts to determine eligibility, assessed methodology in the full texts, and extracted the data.

We identified 2848 citations; 94 studies (14,932 patients) were included. The prevalence of vasopressors was 48.2% (95% CI 45.1%, 51.3%), left ventricular systolic dysfunction occurred in 37.2% (95% CI 34.1%, 40.3%), myocarditis in 34.1% (95% CI 30.5%, 37.8%), electrocardiographic dysrhythmias and abnormalities detected in 23.1% (95% CI 18.8%, 27.6%), coronary abnormalities identified in 18% (95% CI 16%, 20%), extracorporeal membrane oxygenation deployed in 2.2% (95% CI 1.7%, 2.8%), and mortality rate of 2.2% (95% CI 1.7%, 2.7%). A sensitivity analysis was performed after removing eleven studies with high bias, and the adjusted prevalence was not different than the original evaluation.

In this meta-analysis of the largest cohort of multisystem inflammatory syndrome in children patients to date, we established the most accurate prevalence of the most common cardiac manifestations. Providers will subsequently have more precise data to anticipate patient outcomes and approach discussions concerning the frequency of monitoring outside the acute hospital period.

Oral propranolol therapy is commonly used for the prevention of tachyarrhythmias in infancy and childhood. Propranolol is commercially produced in four concentrations allowing varying volumes to be administered. However, quite often an alternative strength of propranolol liquid is issued without clear change in instructions or warning. This may lead to parents inadvertently administering the wrong dose.

Noonan syndrome is an inherited disorder caused by alterations in the RAS-MAPK pathway. There have been several identified genotype–phenotype associations made with respect to congenital cardiac lesions and Noonan syndrome variants, but limited data exist regarding single ventricle disease in this population. Here, we report two patients with PTPN11-related Noonan syndrome and hypoplastic left heart syndrome variants.

We report a rare case of aortic valve atresia and type C interrupted aortic arch with retrograde filling of the ascending aorta via the right common carotid artery through intracranial collateralisation and a presumed intact circle of Willis, who successfully underwent a complete biventricular repair in the neonatal period.

Takotsubo cardiomyopathy, a disease that causes transient contractile abnormalities mainly in the left ventricular apex, is rarely reported in children, especially in those with single-ventricle disease. A 4-year-old boy with a single right ventricle was transferred to our hospital following a severe seizure and was diagnosed with takotsubo cardiomyopathy by echocardiography. His cardiac function improved; however, he developed hypoxic-ischemic encephalopathy.

We present a case of possible vertical COVID-19 transmission-related paediatric inflammatory multisystem syndrome in a neonate with CHD. Myocarditis and supraventricular tachycardia along with hepatic injury and renal failure were diagnosed on a background of mild aortic valve stenosis; the patient was successfully treated with immunomodulation. Since paediatric inflammatory multisystem syndrome can affect the heart, we could consider neonates with haemodynamically insignificant CHD to be at a higher risk of fatal outcomes. Issues related to early diagnosis and management need to be addressed.

Vena caval filters remain as a useful tool in patients with deep vein thrombosis and contraindications to anticoagulation. Although they are rarely used in paediatric patients, they have been shown to be safe and effective when used in the inferior vena cava.

In this case report, we describe the off-label use of a retrievable vena caval filter in the superior vena cava in an adolescent with acute lymphoblastic leukaemia with extensive thrombosis of the right upper neck veins as a means to reduce the risk of pulmonary embolism.

Heart failure in tetralogy of Fallot in the newborn period is rare and is usually due to either large aortopulmonary collaterals or absent pulmonary valve syndrome with severe pulmonary regurgitation. Pink tetralogy of Fallot and tetralogy of Fallot with disconnected pulmonary arteries from the aorta may present beyond the newborn period with heart failure when Pulmonary vascular resistance (PVR) falls. We describe the diagnostic and therapeutic pathway in a rare case of heart failure in newborn with tetralogy of Fallot.

Tuberous sclerosis is a genetic multisystem disorder characterised by hamartomas in several organs. Cardiac rhabdomyomas are the main features of the disease but lipomas can very rarely be associated. Herein, we present a very rare association of tuberous sclerosis and cardiac lipoma detected by echocardiography and diagnosed as a lipoma via MRI and fat suppression technic, aim to report this very rare association, and emphasise usefulness of MRI in cardiac mass lesions.