Emery-Dreifuss muscular dystrophy is a slowly progressive skeletal muscle and joint disorder associated with cardiac complications. Dilated cardiomyopathy was the initial manifestation of Emery-Dreifuss muscular dystrophy in an 8-year-old girl. Despite normal muscle and myocardial biopsies, genetic testing revealed LMNA mutations. As Emery-Dreifuss muscular dystrophy is associated with minimal skeletal muscle weakness, cardiac complications can facilitate its diagnosis.

We present the successful transcatheter closure of the perforation of the cardiac wall from atrial appendage in a 1-year-old girl. Although open-heart surgical repair has been the primary option, percutaneous device closure should always be considered before surgery.

Hyperthermia is defined as an elevated body temperature above the normal range due to a failure of heat regulatory mechanisms. In addition to its effects on other organ systems, hyperthermia is associated with profound cardiovascular effects. We report the sentinel case of a 6-year-old girl with structurally and electrically normal heart, who presented with life-threatening hyperpyrexia-induced ventricular tachycardia, which was refractory to cardioversion and anti-arrhythmics but responded promptly to cooling. We emphasise the lifesaving role of immediate and aggressive cooling in such patients.

A 4-day-old girl with Posterior fossa anomalies, Haemangiomas of the head and neck, Arterial, Cardiovascular, and Eye anomalies and ventral developmental defect syndrome comprising a facial haemangioma, aortic coarctation at the aortic arch, torturous aortic aneurysm distal to coarctation, and ductus arteriosus originating proximal to the coarctation is presented. The aortic arch was successfully reconstructed without cardiopulmonary bypass, and she is currently doing well after 4 years and 8 months.

We present a case of a boy with long QT syndrome type 2, who was admitted after an out of hospital cardiac arrest due to ventricular fibrillation. Subsequently, all treatments – intravenous magnesium, optimisation of electrolytes, an isoproterenol infusion – failed to terminate his electrical storm. As a last option before left-sided cardiac sympathetic denervation, mexiletine was started and the electrical storm resolved completely.

Neonatal aortic thrombus is a rare and critical condition that can present mimicking severe coarctation of the aorta or interrupted aortic arch. Transcatheter thrombectomy for this lesion has not been well described. We report a premature neonate with an occlusive proximal descending aorta thrombus, who underwent transcatheter mechanical thrombectomy using an Amplatzer Piccolo PDA occluder (Abbott, North Chicago, IL, USA). The procedure was successful with no subsequent distal thromboembolic events.

Myocarditis is an inflammation of the heart muscle. In this case, a previously healthy, 17-year-old adolescent with myocarditis after BNT162b2 mRNA vaccination was reported. He was admitted to the hospital with severe chest pain, changes in electrocardiography, and elevation in serum troponin level after fourth day of receiving first dose of vaccine. There was no coronary arterial disease in coronary angiogram. A diagnosis of vaccine-induced myocarditis was made, and supportive treatment was initiated.

An isolated carotid artery is a rare aortic arch anomaly. Instead of connecting to the aorta, the isolated carotid artery connects anomalously to the pulmonary artery. Chronically altered cerebral circulation poses the risk of cerebral hyperaemia following surgical reimplantation. We describe successful reimplantation of the isolated left common carotid artery in a child, highlighting the importance of a multidisciplinary approach for good clinical and neurological outcomes. We also briefly discuss the embryologic basis of this rare arch anomaly.

The use of an implantable cardioverter-defibrillator to prevent sudden cardiac death is an approved method. Experience is limited regarding implantation techniques in infants and low-weight children. We herein describe the implantation of an epicardial implantable cardioverter-defibrillator in a 15-month-old infant weighing 8 kg. We also briefly discuss implantable cardioverter-defibrillator implantation in paediatrics, particularly infants, hoping that our experience will be drawn upon in further such attempts.

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

A 6-year-old boy, born with hypoplastic left heart syndrome, underwent total cavopulmonary connection and later presented in a significantly deteriorated condition. A CT scan revealed multiple thrombi in the extracardiac conduit, although the patient was maintained on an effective anticoagulant therapy. Further examination revealed anamnestic antibodies suggesting that the patient had gone through a clinically inapparent COVID-19 infection, which we conclude most likely contributed to his hypercoagulable state and led to the formation of significant thrombi impairing the patient’s haemodynamics. The patient underwent a surgical thrombectomy; there were no post-operative thrombotic complications.

Complete thrombosis of a pulmonary artery interposition graft in an adolescent with complex repaired CHD was treated successfully with a combination of a novel mechanical thrombectomy system, stent implantation, and thrombolysis. This thrombectomy system used a flexible catheter with a built-in mechanism to attenuate blood loss, while providing effective recanalisation of a foreign graft.

Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.

Two adolescent males presented within 3 days after the first and second dose of the BNT162b2 vaccine with chest pain. Elevated troponin levels, ST segment elevation, and enhancement of the myocardium in cardiac MRI suggested myocarditis. Left ventricular function remained normal, symptoms resolved, and patients were discharged in 4 days. BNT162b2 vaccine may be associated with self-limited myocarditis in youth.

Tetralogy of Fallot with absent pulmonary valve syndrome is commonly associated with trachea-bronchial anomalies, possibly due to airway compression caused by massively dilated pulmonary arteries secondary to severe pulmonary regurgitation. This airway obstruction may persist post-operatively also. We report a case of an infant who required a series of management strategies including bronchial stenting to manage his obstructive symptoms.

Multisystem inflammatory syndrome in children is a new entity in association with SARS-CoV2. Clinical features of Kawasaki disease were noted from the first reported cases of MIS-C. Before the COVID-19 pandemic, Kawasaki disease shock syndrome was considered to be a distinct and unique form of KD. We present a representative case that prove the current difficulty in clearly distinguishing MIS-C from pre-COVID-19-KDSS and emphasie the overlap of the diagnostic criteria.

Ebstein malformation of tricuspid valve is a congenital disease of tricuspid valve with associated right ventricular cardiomyopathy. Hypertrophic cardiomyopathy is a form of inherited left ventricular cardiomyopathy caused by sarcomeric protein gene mutations with inherent risks of sudden cardiac death. Here we report a rare case with co-occurrence of Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy in a young patient.

Access to complex stenotic pulmonary arteries can be challenging due to their anatomy or secondary to prior multiple surgeries and interventions. Two techniques have been previously described to address this issue: the telescopic catheter-in-long sheath parallel to a stiff guidewire technique and the use of a microcatheter in a telescopic scope. We integrated and modified these techniques creating a super telescopic system with a SuperCross® microcatheter-in-catheter-in-long sheath, parallel to a contralateral stiff guidewire to access a previously repaired and stented left pulmonary artery. The stiff wire support and the 90° flexiblity of the Supercross® microcatheter assembled coaxial to the diagnostic catheter and the long sheath contributed to the successful ballooning and stenting-in-stent of the pulmonary artery.

Coronavirus disease 2019 causes respiratory and systemic disease and has led to a sudden epidemic affecting people of all ages. Patients with congenital heart disease represent a high-risk population. In this article, we present a newborn who required extracorporeal membrane oxygenation support for acute respiratory failure in the early postoperative period due to exposure to severe acute respiratory syndrome coronavirus 2 after aortic arch repair and ventricular septal defect closure. To the best of our knowledge, this patient represents the first neonatal case of severe acute respiratory syndrome coronavirus 2 infection after congenital heart surgery and is the youngest patient to need extracorporeal membrane oxygenation support.