Coronary anomalies are a rare but potentially fatal congenital defect. Among high-risk anatomic features, totally anomalous origin of the coronary arteries from the pulmonary trunk is extremely rare, with only 57 cases reported in the literature as to our knowledge. We report a case of such a congenital anomaly with a successful surgical repair and good cardiac function recovery. We emphasise the need for awareness, since this anatomic presentation precludes collateral development and has, therefore, a higher mortality rate.

Double aortic arch is a congenital vascular ring with tracheal and oesophageal compression, potentially causing stridor and dysphagia. While some recommend early surgery, others favour observation. We present a 7-week-old female with mild symptoms and prenatally diagnosed double aortic arch who suffered cardiac arrest, highlighting the need for early surgery in patients with vascular rings and airway or oesophageal compression.

Protein-losing enteropathy associated with failing Fontan circulation remains a significant challenge in long-term, single-ventricle circulation management. Evidence is very limited regarding medical therapies associated with a reduction in disease severity and morbidity outside of cardiac transplantation. We report a novel experience with remission of recalcitrant protein-losing enteropathy after treatment of Cryptosporidium gastroenteritis with a combination of antimicrobials including nitazoxanide, paromomycin, and azithromycin.

Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect in young children. It is typically diagnosed through direct visualisation of the left coronary artery arising from the main pulmonary artery or observing retrograde flow from the left coronary artery to the main pulmonary artery at angiography or echocardiography. This case highlights the diagnostic challenges of ALCAPA in young kids, where antegrade left coronary artery flow due to pulmonary hypertension masked the anomalous origin on echocardiography. Despite the absence of congenital shunts, long-term severe mitral regurgitation led to pulmonary hypertension, presenting with atypical imaging features. The boy accepted a successful surgical reimplantation of the left coronary artery to the aorta with concomitant mitral valve repair and had significant resolution of symptoms.

Hyperlactatemia is a common and concerning finding in the paediatric cardiac ICU as it may signify tissue hypoperfusion and/or hypoxia. However, it is important to include other aetiologies for an elevated lactate in the differential, especially when the lactate is significantly elevated (> 8 mmol/L). We present the case of metabolic acidosis with severe hyperlactatemia secondary to Warburg effect and presumed thiamine deficiency in a paediatric heart transplant patient with post-transplant lymphoproliferative disorder.

Pseudoaneurysm after valve-sparing aortic root replacement is uncommon but potentially life-threatening. We report an unusual case of spontaneous resolution of an aortic root pseudoaneurysm that developed in a 14-year-old boy with Marfan syndrome following valve-sparing aortic root replacement. This case suggests that, in carefully selected situations, non-surgical management with intensive follow-up may be beneficial.

Percutaneous interventions have become significant in the management of congenital heart diseases, with transcatheter procedures being increasingly used for valve dysfunction, particularly for cases requiring repetitive surgeries. This abstract presents a successful transcatheter valve-in-valve implantation in a 16-year-old patient with severe tricuspid regurgitation following a bioprosthetic tricuspid valve replacement. The procedure involved transcatheter tricuspid valve implantation using the Mammoth 25x40 mm balloon catheter and the 26 mm Myval transcatheter heart valve system (Meril Life Sciences Pvt. Ltd, Vapi, Gujarat, India), resulting in immediate improvement in right atrial pressure and regurgitation. The patient underwent an electrophysiological assessment as part of the follow-up and was discharged with a normal sinus rhythm. Tricuspid valve interventions, although less common, are essential in congenital heart diseases, which necessitate prosthetic heart valve implantation due to long-term complications. The valve-in-valve procedure offers a safe alternative, especially in paediatric patients, for reducing risks caused by repetitive surgeries, providing a valuable treatment option in experienced centres.

We describe a trisomy 21 patient with postnatal diagnosis of atrioventricular septal defect with isolated ventricular component who had a complicated post-surgical course following complete repair. Clinical outcomes included moderate-severe residual atrioventricular valve regurgitation needing re-operation, complete heart block leading to pacemaker insertion, seizures secondary to subdural haemorrhages, and chylothorax. We describe the surgical considerations specific to this cardiac morphology.

Primary intracardiac repair during the neonatal or early infantile period is challenging in low-birth-weight infants with coarctation of the aorta and a ventricular septal defect. We performed a staged repair in a 1020 g infant, beginning with stent angioplasty followed by surgical repair with direct anastomosis.

Coarctation of the aorta is characterised by narrowing of the descending aorta and is a rare cause of secondary hypertension in children and young adults. The aortic stenosis lesion is in a special location with severe consequences, and long-term survival is very low, with high rates of disability and mortality, and can be challenging to detect due to its few clinical manifestations. We report a case of a young patient with atrial fibrillation, renal infarction, and acute cerebral infarction, which are consequences of untreated hypertension due to coarctation of the aorta. The purpose of this report is to emphasize the importance of early diagnosis and management of coarctation of the aorta as a cause of secondary hypertension in children and young adults.

Mutations in genes encoding sarcomeric proteins account for 50–60% of familial hypertrophic cardiomyopathy cases. However, the molecular pathogenesis in approximately one-third of patients remains unidentified. We describe the case of a 15-year-old female who presented with intermittent palpitations and had a significant paternal cardiovascular history. She was diagnosed with hypertrophic cardiomyopathy, confirmed by echocardiogram and cardiac MRI. Genetic testing revealed a variant of unknown significance in exon 7 of the FHL1 gene and exon 43 of the ANK2 gene.

Atrial myxoma in children is rare, with vision loss and cerebral metastasis seldom reported. We present the case of a 12-year-old girl who experienced sudden vision loss and acute infarction, with a left atrial myxoma identified and surgically resected. One year later, she was readmitted with severe neurological symptoms. Imaging revealed multiple hypometabolic brain lesions, which were confirmed as metastatic myxoma through surgical resection and pathology. This case underscores the critical need for long-term follow-up in patients with cardiac myxoma to monitor potential delayed neurological complications.

With the passing of Richard Van Praagh in September of 2025, paediatric cardiology has lost one of its giants. With one of his greatest contributions, namely the segmental approach, he revolutionised the practice of paediatric cardiology. He and Stella, his wife and closest collaborator, developed the Cardiac Registry at Boston Children’s Hospital and published more than 300 articles. Together, they trained many fellows, with endless generosity and dedication, and gave conferences all over the world, spreading their ideas and concepts. Richard will be sorely missed, but he has transmitted to us the desire and the tools to continue to build on his firm foundations.

Premature atrial complexes are frequent among patients after heart transplantation. We herein report on the successful use of ivabradine in a 15-year old patient who exhibited marked palpitations caused by atrial premature complexes after orthotopic bicaval heart transplantation.

After right ventricular outflow tract obstruction reconstruction for CHD, surgical pulmonary valve replacement, or transcatheter pulmonary valve replacement may be performed if complicated by moderately severe or severe right ventricular outflow tract dysfunction. However, for patients whose anatomy is not suitable for transcatheter pulmonary valve replacement, surgical pulmonary valve replacement is the only option, but it has a higher rate of perioperative complications and longer hospitalisation for patients. In two cases of severe transcatheter pulmonary valve replacement in which percutaneous pulmonary valve implantation could not be performed directly due to the presence of a large right ventricle, a significant decrease in right ventricular function, and significant widening of the pulmonary arteries, we used a new hybridisation procedure to partially fold and reduce the diameter of the significantly widened pulmonary artery trunk without cardiopulmonary bypass by surgically opening the thorax in a median way, and then successfully implanted a percutaneous self-expanding pulmonary valve valve, with good immediate results in the postoperative period. The postoperative recovery was rapid and the recent results were excellent.