Radiofrequency ablation is the first-line treatment for arrhythmias with high success and low complication rates. Skin burns have been reported rarely after electrophysiological procedures, especially procedures in which higher-power energy is used and multiple ablations are performed. Here, we report a case of skin burn that developed after radiofrequency ablation for ventricular tachycardia originating from the right ventricular outflow tract.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited cardiomyopathy characterised by ventricular arrhythmias and an increased risk of sudden cardiac death. Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnosis is based on criteria that take into account electrical and structural cardiac abnormalities, as well as mutation analysis. Appropriate pharmacological therapy and the prevention of sudden death with implantable defibrillators are important in the management of these patients. Exercise is considered an important environmental factor for the development and progression of the disease.

We recently diagnosed two cases of isolated unilateral absence of the pulmonary artery just after birth. Through the therapy, we could not prevent obstruction of the pulmonary artery and lead to complications. There have been no symptoms attributed to isolated unilateral absence of the pulmonary artery so far. We should carefully consider the strategy for therapeutic intervention for asymptomatic cases.

Dr. Jennifer Li is the focus of our second in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Li was born in Boston, Massachusetts, United States of America, and moved to Indianapolis, Indiana where she completed her secondary education. She then attended Stanford University, majoring in Chemistry and English and graduating with distinction in 1983. Dr. Li then attended Duke University School of Medicine, graduating in 1987. She then completed her internship at Children’s Hospital of Philadelphia in 1987–1989, returning to Duke University Medical Center to complete both her residency in general paediatrics in 1989–1990 followed by her fellowship in paediatric cardiology in 1990–1993. She would later complete her Master’s Degree in Health Sciences at Duke University in 2005.

Dr. Li has spent her entire career as a paediatric cardiologist at Duke University Medical Center, where she was appointed a Professor of Pediatrics and Professor of Medicine in 2008 and has held the position as Beverly C. Morgan Endowed Professor of Pediatrics since 2012. She has served as the Chief of Paediatric Cardiology at Duke University Medical Center since 2006. She also was the Director of Paediatric Research at Duke Clinical Research Institute from 2001-2015. Dr. Li has played an instrumental role in evaluating the safety and efficacy of drugs in children, as well as in analysing and linking large multicentric databases to evaluate the outcomes, quality, and cost of paediatric and congenital cardiac care. Dr. Li has received funding from the National Institute of Health of the United States of America, as well as from industry and foundation grants. This article presents our interview with Dr. Li, an interview that covers her experience collaborating with governmental organizations and industry in the pursuit of common interests to design clinical drug trials, link and analyse large, multicentric databases, and improve paediatric health care.

Idiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation. Antiplatelet therapy is prescribed in most cases reported in the literature, and reduction plasty is indicated when there is rapid growth of the right atrium, compression of adjacent structures, or refractory arrhythmias. We report four cases of idiopathic dilatation of the right atrium diagnosed during prenatal screening. We describe the intrauterine course and management in postnatal life until early childhood.

Twenty cases of aortopulmonary window are reported with special reference to some unusual modes of presentation and hemodynamics. Patients over 15 years of age accounted for 35% of the group. This delayed presentation may relate to referral patterns and socioeconomic conditions prevalent in India. Curiously, this elderly subset had two modes of presentation—either as large left-to-right shunts with low pulmonary vascular resistance (71.4%), or as Eisenmenger's syndrome (28.6%). The reasons for the low pulmonary vascular resistance in the majority of these older patients remain unclear. The echocardiographic features of these defects can be missed in presence of significant associated cardiac lesions. A careful search should, therefore, be made for an additional aortopulmonary window in all cases with evidence of intercirculatory shunting.

Aneurysms of one of the aortic sinuses of Valsalva are rare congenital or acquired lesions. Here we present the case of an adolescent with Down syndrome with ruptured aneurysm of the right coronary sinus into the right atrium. All sinuses of Valsalva were normal during cardiological screening owing to Down syndrome at the age of 2 weeks. Paediatricians should have a low threshold for referring patients with Down syndrome for cardiac re-evaluation because of the new onset of cardiac symptoms or cardiac physical findings, even in the situation in which there are normal echocardiographic findings in the past.

Percutaneous closure is the gold standard treatment for atrial septal defects, but the procedure can be complex in case of femoral thrombosis. Although unusual for congenital interventionists, transhepatic atrial septal defect closure is an attractive alternative to the internal jugular vein, especially when approaching the interatrial septum. Herein, we report the case of an adult patient with significant co-morbidities who had successful transhepatic atrial septal defect closure after a failed transjugular attempt. We describe the use of an absorbable haemostatic gelatin sponge to efficiently and safely achieve haemostasis after the use of a large vascular sheath with combined anticoagulation and antiplatelet therapy.

We report a case of acute myocardial infarction in a nephrotic male patient. A 22-year-old man with a 1-year history of nephrotic syndrome due to membranous nephropathy presented with acute chest pain and was admitted to our emergency room. An electrocardiogram showed ST elevation in leads consistent with anterior and inferior myocardial infarction. Subsequent cardiac catheterisation showed evidence of thrombotic occlusion of the anterior descending coronary artery. The patient had no long history of hypercholesterolaemia or hypertriglyceridaemia. The case suggests that young patients with a short-term nephrotic syndrome may be at an increased risk for acute coronary syndrome owing to hypercoagulability state.

We sought to define the prevalence of echocardiographic abnormalities in long-term survivors of paediatric hematopoietic stem cell transplantation and determine the utility of screening in asymptomatic patients. We analysed echocardiograms performed on survivors who underwent hematopoietic stem cell transplantation from 1982 to 2006. A total of 389 patients were alive in 2017, with 114 having an echocardiogram obtained ⩾5 years post-infusion. A total of 95 patients had echocardiogram performed for routine surveillance. The mean time post-hematopoietic stem cell transplantation was 13 years. Of 95 patients, 77 (82.1%) had ejection fraction measured, and 10/77 (13.0%) had ejection fraction z-scores ⩽−2.0, which is abnormally low. Those patients with abnormal ejection fraction were significantly more likely to have been exposed to anthracyclines or total body irradiation. Among individuals who received neither anthracyclines nor total body irradiation, only 1/31 (3.2%) was found to have an abnormal ejection fraction of 51.4%, z-score −2.73. In the cohort of 77 patients, the negative predictive value of having a normal ejection fraction given no exposure to total body irradiation or anthracyclines was 96.7% at 95% confidence interval (83.3–99.8%). Systolic dysfunction is relatively common in long-term survivors of paediatric hematopoietic stem cell transplantation who have received anthracyclines or total body irradiation. Survivors who are asymptomatic and did not receive radiation or anthracyclines likely do not require surveillance echocardiograms, unless otherwise indicated.

Aortopulmonary septal defect and tetralogy of Fallot is a rare combination. We report a case of tetralogy of Fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization.

We used pulsed Doppler echocardiography to study the right ventricular diastolic function of29 normal babies and 12 neonates with pulmonary atresia and intact ventricular septum. Eleven patients underwent staged operations of closed pulmonaryvalvotomy followed by either rightventricular outflow reconstruction (n=5) or balloon angioplasty of the pulmonary valve (n=3). In normal babies, the Doppler wave form showed dominant early (E) and separate late active (A) waves of activity, when the heart rate was slow (<100/min). The two waves gradually merged with increasing heart rate, to form a monophasic active wave. Prior to any intervention, all except one of our patients had only a monophasic active right ventricular filling wave over the entire range of heart rates recorded. To quantitate these differences in ventricular filling, we derived 4 diastolic indices from the ratio of: the peak velocity of the early versus the late active wave (EIA); the integral with time of these waves (E/Aarea); the time of diastolic filling relative to the cardiac cycle (TIRR); and the peak diastolic filling velocity relative to the mean filling velocity over the cardiac cycle (Velocity Index). Profiles of the indices against heart rate for both normal controls and patients indicated that only the index of the time of diastolic filling (T/RR) and the Velocity Index were appropriate for our serial comparisons. Thus, for neonates with pulmonary atresia, the index of the time (TIRR) was significantly lower (0.29±0.03 vs 0.43±0.04, p<0.01) and the Velocity Index higher (5.98±0.79 vs 3.98 ±0.31, p<0.001) than those of normal. After valvotomy, but with a poor surgical result, babies continued to have a predominantly monophasic right ventricular filling pattern without significant improvement (p>0.05) of the index of time (T/RR=0.29±0.05) or velocity (5.88±1.17). Babies who underwent a second stage procedure and achieved a final good result had predominantly biphasic right ventricular diastolic filling waves with significant progression (p<0.001) in the index of time (T/RR=0.42±0.03) and velocity (4.09±0.49).

Stenting of the arterial duct (PDA) has become a standard palliation for ductal-dependent pulmonary circulation. Carotid arterial access provides a direct route for stenting vertical ducts. We evaluated our early results of hybrid ductal stenting via surgical carotid cutdown.

Long QT syndromes encompass the most prevalent group of ion channelopathies. Long QT syndromes are predominantly familial and predispose the affected individual to ventricular arrhythmias and sudden death. Permanent pacemaker insertion for long QT syndrome is discouraged apart from younger patients exhibiting 2:1 atrioventricular block. However, permanent pacemaker insertion is a relatively common procedure in neonates with atrioventricular block, and dual-chamber permanent pacemaker insertion in low birth weight infants is challenging. We describe the management of long QT syndrome − type 2 − presenting in an extremely preterm neonate including epicardial, dual-chamber permanent pacemaker insertion.