We report on a patient with bicuspid aortic valve and anomalous right coronary artery from the opposite sinus without evidence of intramural course. Different authors support the universal presence of intramural course in patients with origin of the right coronary artery from the opposite sinus of Valsalva in normal heart. The occurrence of both bicuspid aortic valve and the absence of intramural course may not be accidental. This might suggest a developmental interaction between bicuspid aortic valve and anomalous coronary artery. Large observational study including characterisation by intravascular ultrasonography in patients with bicuspid aortic valve and anomalous coronary is needed.

Nicolaides–Baraitser syndrome is a rare, neuro-developmental disorder caused by heterozygous pathogenic variants in the SMARCA2 gene, involved with chromatin regulation. Cardinal features include intellectual disability, short stature, microcephaly, triangular facies, sparse hair, brachydactyly, prominent interphalangeal joints and seizures. Genetic testing demonstrated a loss within SMARCA2 at 9p24.3 inclusive of basepairs 2094861_2141830 (hg19) in our patient. This case highlights a child with Nicolaides–Baraiter syndrome, a SMARCA2 gene deletion and a novel association of hypertrophic obstructive cardiomyopathy.

We describe a 3-month-old infant with transposition of great arteries and restrictive foramen ovale who presented with severe cyanosis. Child had a large thrombus causing near-total occlusion of the inferior caval vein. An emergency atrial septoplasty was performed via internal jugular venous access. The case emphasises the technical challenges faced while performing this procedure through jugular approach and plausible solutions to overcome these challenges.

This article reported two cases of brucellosis in children on the waiting list for a cardiac transplant who have been referred with the diagnosis of Fever of Unknown Origin. The serology was positive and patients were treated with cardiac transplantation in the 6th week. They had no recurrence or rejection. Therefore, brucellosis should be considered in patients on the waiting list for cardiac transplants.

Although a high percentage of foetuses with supraventricular tachycardia respond to single or dual antiarrhythmic therapy, on occasion when there is no response to these combination regimens, direct intra-foetal therapy remains the only choice, albeit such an approach carries a potential risk to the foetus.

Data with regard to the safety and efficacy of triple antiarrhythmic combination have not been reported before. Here, we present a foetus with intractable tachycardia in whom arrhythmia termination was successfully achieved with triple oral antiarrhythmic therapy.

Balloon atrial septostomy is a palliative procedure that is performed in D-transposition of great arteries when surgery is not immediately available. Although D-TGA and left isomerism association are rare, it is an important condition as the BAS procedure approach is unique. In this case report, we present two cases of D-TGA with left isomerism where BAS was performed due to restrictive atrial septal defect and lack of immediate availability of the paediatric cardiac surgeon.

Cardiac amyloidosis presented with normal interventricular septum is an extremely rare entity, and diagnosis may be difficult. This report discusses a 44-year-old female who presented with worsening dyspnoea on exertion, orthopnoea, and lower-extremity oedema. Electrocardiogram depicted low voltage in limb leads and a pseudoinfarct pattern. Echocardiogram revealed biatrial dilatation without changes of ventricular chambers and restrictive filling physiology. A diagnosis of cardiac amyloidosis was considered. Cardiac MRI was pursued, showing delayed gadolinium enhancement, and this ultimately led to the myocardial biopsy confirming the diagnosis of cardiac amyloidosis. The case suggests that patients who present with heart failure of uncertain aetiology, amyloidosis could be a cause of cardiomyopathy despite the absence of “classical” echocardiographic features of amyloid deposition such as an increased interventricular septum thickness or“brilliant sparkled”appearance of the myocardium.

Seckel syndrome is a very rare autosomal recessive disorder also known as bird headed dwarfism”. It is characterised by proportional short stature, low birth weight, dysmorphic facial appearance, and mental retardation. In addition to its dysmorphic features, skeletal, endocrine, gastrointestinal, haematologic, genitourinary, and nervous system has been involved. Cardiovascular features very rarely associate with Seckel syndrome. We report two patients with Seckel syndrome, one with dilated cardiomyopathy and the other with multiple ventricular septal defects. Dilated cardiomyopathy and isolated ventricular septal defect have not been previously reported in Seckel syndrome. Cardiovascular evaluation should be performed in all patients with Seckel syndrome. Early diagnosis of congenital and acquired heart diseases will reduce morbidity and mortality in these patients.

Allergic reactions related to drug use is a common entity presenting often from minor urticaria to life-threatening anaphylactoid reactions. A common but easily overlooked diagnosis, Kounis syndrome, is an established hypersensitivity coronary disorder induced by drugs, foods, environmental factors, and coronary stents that can present in the same way as non-allergy-induced acute coronary syndrome. Here within, we present a unique case of dual presentation of Kounis syndrome and prolonged QTc in a young patient after a single dose of Domperidone and Lansoprazole.

We report a 25-year-old woman who was diagnosed with atrial septal defect (ASD). An ECG showed only first-degree atrioventricular block and incomplete right bundle branch block. One day after the percutaneous ASD closure, she had a slight fever and an ECG showed a type 1 Brugada pattern. ECG characteristics of ASD are similar to those of a Brugada ECG. This case is rare combination of Brugada syndrome with ASD.

A 27-year-old man applied for routine control due to aortic coarctation stent. During routine control, a late in-stent thrombus was detected. The patient was asymptomatic. Thrombus resolution was achieved with anticoagulant therapy. Anticoagulant therapy is one of the options in the treatment of aortic stent graft-associated asymptomatic thrombus. Surgical intervention might be required in case of thromboembolism, haemodynamic deterioration, and intra-aortic gradient with high blood pressure in the upper extremity.

In latent Wolff–Parkinson–White syndrome, ventricular pre-excitation is inapparent during sinus rhythm but carries the same possibility of sudden cardiac death and palpitations as overt Wolff–Parkinson–White syndrome. It is difficult to diagnose latent Wolff–Parkinson–White syndrome when a patient does not have syncope or palpitations. We report the case of an asymptomatic patient with latent Wolff–Parkinson–White syndrome detected on school heart screening using subtle electrocardiography findings.

Balloon dilatation of coarctation of aorta is a standard of care for the patients presenting with severe left ventricular dysfunction. It can be performed through femoral, carotid, and axillary arterial access. Very few case series were available in the literature through axillary arterial access, despite being its advantage as non-end artery and easily palpable in coarctation of aorta. We present our experience with five cases of neonatal coarctation of aorta with severe left ventricular dysfunction where successful balloon dilatation of coarctation of aorta was performed via axillary approach without adverse events.

Tricuspid valvulopathy has gained a lot of attention in recent years, especially due to the advances in percutaneous management. CHD can present with primary or secondary malfunction of the tricuspid valve, often not addressed due to high surgical risk after several interventions. We present two cases of adults with complex congenital heart malformations and borderline clinical situations who successfully underwent percutaneous tricuspid replacement.

We describe the case of a 17-year-old boy who developed acute myopericarditis associated with transient ventricular wall thickening. This is a unique teaching case illustrating that acute myocarditis can be associated with significant oedema, secondary to inflammation, causing marked wall thickening that is apparent on echocardiography. This oedema resolves completely with anti-inflammatory drug treatment.

Dilated cardiomyopathy is the most common presentation of cardiomyopathy in children with 20–35% of patients having an identified genetic component. There are more than 30 genes implicated in the pathogenesis of dilated cardiomyopathy. We present the first report of a female infant with dilated cardiomyopathy with a genetic variant in the dispatched RND transporter family member 1 gene.

Coronary sinus abnormalities are usually associated with arrhythmia disorders when symptomatic. We report a 5-year-old 14 kg patient with a giant diverticulum of coronary sinus and Wolff-Parkinson-White syndrome. Catheter ablation therapy was decided during follow-up due to inadequate response to multidrug therapy. Posteroseptal and left posterolateral accessory pathways were established and radiofrequency ablation was performed successfully through coronary sinus.

We report on a 7-month-old male with transient phrenic nerve palsy induced by diagnostic cardiac catheterisation. The phrenic nerve palsy, which is a rare complication, was due to extravascular bleeding from a branch of the internal mammary artery.

Cardiac dysfunction commonly occurs in congenital heart block associated with maternal anti-SSA antibodies, especially after pacemaker implantation. We report the case of a 4-year-old girl with antibody-associated congenital heart block and a large secundum atrial septal defect who presented with significant cardiac dysfunction 4 years after pacemaker implantation. Histological findings were useful for determining the course of treatment and perioperative risk of intracardiac repair.