A 16-year-old male with newly diagnosed granulomatosis with polyangiitis presented to the emergency room with chest pain. He was found to have a myocardial infarction involving the right coronary artery and the left circumflex artery. He underwent mechanical thrombectomy and stent placement without significant sequelae. This is a rare complication associated with granulomatosis with polyangiitis.

CHDs affecting the right ventricular outflow tract often require repeated surgical or transcatheter interventions due to pulmonary insufficiency or stenosis. This study presents percutaneous implantation of large self-expanding valves in patients with complex right ventricular outflow tract anatomy after prior pulmonary branch stenting.

We present the case of a 31-year-old female with Fontan circulation who developed signs of protein-losing enteropathy 10 days after second COVID-19 vaccination. After standard investigations for identification of potential triggers for protein-losing enteropathy, we concluded that coronavirus disease 2019 (COVID-19) booster vaccination could have been the most probable underlying trigger. Prompt investigation of new symptoms post-vaccination in high-risk patients is necessary.

This report describes the first long-term survival following a heart transplant for Williams syndrome-associated cardiac pathologies. An 11-year-old patient with severe global left ventricular dysfunction presented with heart failure and underwent heart transplantation. Her peri- and post-operative courses were complicated by hypertension related to underlying vascular pathology.

A 69-year-old man had an abnormal intracardiac course of a pacemaker lead. CT angiography demonstrated a window between the right upper pulmonary vein and the superior caval vein. The window was treated with covered stents in the superior caval vein, which was complicated by a chronic pericardial effusion that was treated with a pericardial window 6 months later.

Sudden cardiac death poses a significant risk in patients with surgically repaired tetralogy of Fallot. Despite extensive research, risk stratification practices vary. This study surveyed the Pediatric and Adult Congenital Electrophysiology Society to identify these differences. Results showed diverse practices in indications, methods, and interpretation of electrophysiology studies, highlighting a need for standardised algorithms to improve patient outcomes.

Hybrid procedure of hypoplastic left heart syndrome, comprising ductus arteriosus stenting and bilateral pulmonary artery banding, is a good surgical option for initial palliative procedure for high-risk patients for Norwood procedure. However, ductal stenting may cause retrograde aortic blood flow obstruction. Furthermore, complete removal of stent while performing the Norwood procedure make the operation more difficult. We report a case that overcame these problems using a novel surgical technique.

We describe a 12-year-old boy with agenesis of the intrahepatic segment of the inferior vena cava (a rare congenital anomaly) in whom an electrified coronary guidewire with electrocautery was used percutaneously to perforate and open the atretic segment, followed by stent implantation. This technique may be a safe and feasible option for paediatric patients, offering a therapeutic alternative for complex cardiac anatomies.

Mitochondrial trifunctional protein deficiency is a long-chain fatty acid disorder that may include manifestations of severe cardiomyopathy and arrhythmias. The pathophysiology for the severe presentation is unclear but is an indicator for worse outcomes. Triheptanoin, a synthetic medium chain triglyceride, has been reported to reverse cardiomyopathy in some individuals, but there is limited literature in severe cases. We describe a neonatal onset of severe disease whose clinical course was not improved despite mechanical support and triheptanoin.

A patient with borderline left ventricle successfully underwent biventricular repair following a staged surgical approach to promote left ventricular growth. Despite initial concerns about left ventricle size, apex formation and adequate size of atrioventricular valve indicated potential for future growth. The patient demonstrated significant left ventricular growth, resulting in stable biventricular circulation and a favourable outcome over a three-year postoperative follow-up period.

Clinical Registration Number: Institutional Review Board of Osaka City General Hospital, number 1902139.

Biallelic pathogenic variants in the inorganic pyrophosphatase 2 (PPA2) gene are a rare but established cause of sudden infant death, which may be precipitated by a pyrexial or viral illness. It has also been associated with sudden death secondary to alcohol ingestion in young adults. We describe the case of a thirteen-month-old female who presented following out-of- hospital cardiac arrest and was subsequently diagnosed with compound, heterozygous pathogenic variants of PPA2. She survived this episode and made excellent neurological recovery despite her prolonged out-of-hospital resuscitation. This case describes her initial presentation, consideration of PPA2-related cardiac arrest in the differential diagnosis, and the subsequent secondary preventative strategies. These included insertion of an implantable cardiac defibrillator, avoidance of alcohol in her medication preparations and administration of childhood vaccinations in a hospital environment due to risk of pyrexia-induced arrhythmias.

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.