Transcatheter pulmonary valve replacement is the first choice to treat residual or recurrent right ventricular outflow tract dysfunction. Surgery is an effective option when anatomy is not permissive for transcatheter procedures. When surgical risk is too high, hybrid procedures might be considered.

In this paper, we describe the first use of Harmony valve in Europe in a 59 years old patient with a huge right ventricular outflow tract. The procedure was performed by a hybrid approach: before valve deployment, through an anterior mini-thoracotomy, the pulmonary artery was plicated to create a landing zone. The valve was deployed by trans-femoral venous approach. It was secured by putting a suture on the distal stent raw under fluoroscopic guidance. The procedure was uneventful and patient’s New York Heart Association class rapidly improved from III–IV to II.

In conclusion, hybrid strategies might represent an acceptable option for huge right ventricular outflow tract, to be less invasive and to minimise device embolisation risks. When a good match between patient’s anatomy and device can be achieved, a mini-invasive or micro-invasive surgical approach might be considered to minimise bleeding risks and shorten the hospital’s length of stay.

Desminopathy is a rare heritable cardiac and skeletal muscle disease caused by variants in the DES gene, which encodes the primary muscle-specific intermediate filament protein, known as desmin. Childhood-onset is commonly associated with severe early-onset myopathy and early death. Here, we reported an 11-year-old Chinese girl presenting with complete atrioventricular block and cardiomyopathy, without skeletal muscle involvement. Genetic analysis identified a de novo variant (c.152C > T/p.Ser51Phe) in the DES gene.

Kawasaki disease is an idiopathic vasculitis that involves the participation of various physiological systems and the inflammation of small-to medium-sized arteries, resulting in the formation of aneurysms. It most commonly affects children under 5 years. The development of coronary artery aneurysms is a significant concern in the context of Kawasaki disease, rendering it the prevailing acquired cardiac condition among children. It has been observed that 1–30% of Kawasaki illness patients experience neurological complications. However, facial nerve palsy is uncommon, with a prevalence of only 0.9%. Infants with Kawasaki disease and facial nerve palsy tend to be under 6 months of age and had a higher chance to develop coronary artery aneurysms compared to those without facial nerve palsy whose condition was never treated.

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.

A 15-year-old male presented with vasovagal syncope and troponin leak 4 days after his second COVID-19 vaccine. Based on initial diagnostic work-up, he was thought to have COVID-19 vaccine-associated myocarditis. His cardiac dysfunction persisted and further work-up including genetic evaluation and serial MRI studies later confirmed a diagnosis of arrhythmogenic cardiomyopathy. This is a unique case of an incorrect diagnosis based on timing and context of vaccine-related myocarditis. Reports of mild and self-limited myocarditis post-COVID-19 vaccination may cause vaccine hesitancy among the public, and so case reports such as this one show the importance of discerning underlying conditions amongst rare COVID-19 vaccination complications.

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.

Cardiac involvement of metastatic osteosarcoma is exceedingly rare and carries a dismal prognosis. Documentation of the surgical management of cardiac metastatic osteosarcoma is limited. We describe the successful resection of a ∼ 3.9 x 2.2 cm mass involving the right atrioventricular groove and right ventricular inferolateral wall, granting our patient 12 months of excellent cardiac function in the face of advanced metastatic disease.

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.

Pulmonary valve replacement in small children is rarely needed; when performed, it is usually done surgically. Herein, we describe a hybrid technique for pulmonary valve replacement through a small subxiphoid incision in a Toddler without utilising the cardiopulmonary bypass machine. The procedural technique is thoroughly explained. Collaboration between paediatric cardiology and cardiovascular surgery teams achieved optimal outcome with minimised invasiveness.

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.

Pulmonary arterial hypertension remains a progressive, life-threatening condition despite advances in medical treatments. We report the first case of the interventional creation of a reverse Potts shunt by stenting a closed ductus arteriosus in a four-year-old child with right ventricular failure due to suprasystemic pulmonary arterial hypertension associated with Von Hippel-Lindau disease, unresponsive to triple anti-pulmonary arterial hypertension therapy. Following the procedure, the patient’s clinical status and echocardiographic systolic and diastolic right ventricular function improved.

Pulmonary arteriovenous malformations are abnormal vascular connections between pulmonary arteries and veins, often causing right-to-left shunting. In this report, a 4-year-old boy with low oxygen saturation was diagnosed with a large, complex pulmonary arteriovenous malformation involving four feeding arteries. Percutaneous transcatheter closure was performed using four devices to occlude the major feeding arteries, resulting in increased arterial oxygen saturation from 72 to 98%. This report depicts the successful use of multiple devices for percutaneous closure of a complex pulmonary arteriovenous malformation in a child and highlights the minimally invasive and effective nature of this approach.

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.

Coronary artery fistulas are rare cardiac anomalies, often asymptomatic and frequently discovered incidentally during routine cardiac imaging. Transcatheter closure with coil embolisation is increasingly recognised as a preferable alternative to surgical intervention due to its lower complication rates. In this report, we present three paediatric cases in which coronary artery fistulas were successfully embolised using the Azur® CX Peripheral Coil System. This system demonstrates low-profile suitability for occluding highly tortuous vessels in children, enabling controlled coil delivery via a microcatheter. Its hydrogel-coated coils allow for expansion between gaps, making it highly effective as a minimally invasive treatment.

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.

Pulmonary vasodilators increased the systemic-to-pulmonary collateral flow in two single-ventricle cases, causing haemodynamic decline. Discontinuation reversed the collateral flow and improved heart failure, highlighting the need for careful monitoring. Serial cardiac MRI, as a non-invasive tool, may be essential to detect changes in collateral flow and guide vasodilator use for optimal management in this population.

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.