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.

Warfarin is used as anticoagulation for children for a wide range of cardiac indications but carries the disadvantage of requiring international normalised ratio monitoring and dose adjustment. Management of warfarin therapy is challenging due to its narrow therapeutic window and is further complicated in children by dietary changes, frequent illnesses, and developing systems of metabolism and haemostasis.

A retrospective review was performed of patients’ medical records to assess the indication for warfarin use, percentage of international normalised ratio values in target range (%ITR), and frequency of phlebotomy.

Twenty-six patients were identified. The most common indication for warfarin use was in patients post-total cavo-pulmonary connection (n = 19, 73%). We demonstrated a variability in duration of warfarin therapy following total cavo-pulmonary connection (median of 11.1 months). Nineteen (73%) patients had used the CoaguChek machine for home measurement of international normalised ratio. The median frequency of phlebotomy for all indications was once every 10 days, and the median %ITR was 55.4 % (29.7–86.4%). Of note, the percentage under target range in the patients with mechanical mitral (n = 2) and aortic valves (n = 1) was found to be 23% and 33%, respectively.

These data demonstrate a high frequency of international normalised ratio values outside of the target range as seen in previous studies of warfarin in children. This necessitates frequent phlebotomy and dose changes, which can have a significant effect on the quality of life of these patients and their families highlighting the need to focus on quality improvement in the area of anticoagulation in paediatric cardiac patients.

Long-term results of total aortic cusp replacement such as the Ozaki procedure in paediatric patients have rarely been reported. We describe the 13-year follow-up of a 9-year-old boy with aortic valve regurgitation who underwent total aortic cusp replacement without the need for reintervention. His aortic valve regurgitation is mild with peak pressure gradient of 22 mmHg at the last follow-up.

Transcatheter closure of patent ductus arteriosus has an established safety and efficacy in childhood using coils. However, complications and unexpected events are infrequently reported, such as haemolysis, embolisation, and stenosis of the left pulmonary artery or descending aorta. In the current work, we report an unusual obstacle that occurred upon the release of a patent ductus arteriosus coil in a child. Abnormal straightening of the coils on the pulmonary side was encountered, with failure of recoil of the patent ductus arteriosus windings noticed upon the coil release. However, the coil was successfully snared, and another was deployed with an uneventful recovery.

Clinicians in paediatric cardiology have unique exposure to different pathologies and treatment based on geographic location and resource availability. “Cardiology Across Continents” is a virtual, case-based discussion series aimed at knowledge sharing across a variety of practice and resource settings. We report on a recent case discussion highlighting a rare cause of new-onset heart failure in a teenager, which is more frequently seen among African and Asian populations.

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.

Electrocardiogram interpretation is critical in neonatal care, as artefacts may mimic life-threatening arrhythmias. We report the case of a preterm newborn girl, delivered at 31 weeks of gestation due to acute fetal distress, who developed tachycardia shortly after birth. The initial ECG showed chaotic electrical activity, initially raising suspicion of artifact; however, a subsequent tracing confirmed a life-threatening arrhythmia with wide QRS complexes, prolonged QTc, and torsades de pointes. As the patient remained haemodynamically stable, intravenous lidocaine and magnesium sulphate were administered, achieving pharmacological cardioversion. Propranolol was subsequently initiated, and the lidocaine infusion was tapered and replaced with oral mexiletine. Serial electrocardiograms showed QTc interval improvement, left ventricular function normalised, and the patient was discharged on the 39th postnatal day following stabilisation and supportive care for prematurity. Genetic testing identified a de novo SCN5A c.796C > G (p.L266V) mutation associated with Long QT syndrome type 3. At 1 year of age, the patient remains stable and asymptomatic while receiving combined therapy with propranolol and mexiletine.

Recommendations for immunisation practices in children with single ventricle CHD are lacking. A survey of 53 heart centres received responses from 40 centres (33 complete and 7 partial) revealing variability in immunisation recommendations. Only 11% have a written protocol. Immunisations were delayed before cardiopulmonary bypass in 94% (32/34) and after cardiopulmonary bypass in 97% (30/31), with 34% (13/38) re-dosing some immunisations post cardiopulmonary bypass. Further research is needed to develop guidelines.

A male infant with fetal hydrops was diagnosed with congenital dilated cardiomyopathy and died at 2 months despite intensive treatment. Trio-exome sequencing identified as de novo FHL2 variant (p.Arg131Cys). He also presented with non-cardiogenic cholestatic liver injury and hyperferritinemia, which may represent novel FHL2-related phenotypes based on supporting evidence from experimental models.

We report a 7-year-old boy with tetralogy of Fallot who developed a right ventricular pseudoaneurysm one year after right ventricular outflow tract reconstruction with a 17 mm pulmonary homograft. Urgent reoperation revealed proximal homograft dehiscence. Repair was performed using an Invengenx® pericardial patch. This rare complication highlights the importance of long-term surveillance following homograft implantation in congenital heart surgery.

Total anomalous pulmonary venous return is a rare CHD that may present in the neonatal period with signs of congestive cardiac failure. First-line treatment is corrective surgery in the first months of life. The authors report the case of a 2-month-old infant with non-obstructive total anomalous pulmonary venous return submitted to a two-stage hybrid repair, involving cardiac surgery and interventional cardiology.

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.

Giant left atrium is extremely rare in the paediatric population, especially in asymptomatic cases. We report a 5-year-old child with a giant left atrium due to “non-rheumatic” mitral valve regurgitation that presented as a stroke secondary to atrial tachycardia. This case is noteworthy for the unusual “silent” manifestation of a giant left atrium.

Innominate vein redirection to the pulmonary venous atrium has been used in single ventricle patients in order to relieve lymphatic complications resulting from systemic venous hypertension. This has been achieved both surgically, known as the Hraska procedure and via transcatheter approach, known as the Rome procedure. Determining the pathway from the innominate vein to the pulmonary venous atrium is challenging with important intra- and extra-cardiac structures close-by. We present two cases; one surgical and one transcatheter approach, where 3D-printed cardiac models were used to assist in the pre-procedural planning of this relatively novel and challenging strategy.

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.

Aberrant subclavian artery arising from the pulmonary artery is an extremely rare anomaly of the aortic arch and is often associated with CHD. It can remain asymptomatic or result in subclavian or pulmonary steal syndrome. We present three cases with aberrant subclavian arteries (two left-sided, one right-sided) and reviewed 44 case reports through an extensive PubMed research to contextualise our findings to the existing literature. Fifty-five per cent of patients had a left, 45% had a right aberrant subclavian artery. The majority of patients (86%) had associated CHD. Blood pressure discrepancies and imaging—particularly transthoracic echocardiography, CT, and cardiac catheterisation—were pivotal in diagnosis. Aberrant subclavian arteries are a rare vascular anomaly, understanding of the embryology and anatomy is essential for the understanding of complex congenital heart variations. Early detection and surgical intervention are crucial to prevent complications.

Multisystem inflammatory syndrome in children is a systemic disorder that involves incessant fever, extreme inflammation, and organ dysfunction, which is at first associated with exposure to COVID-19. This case, however, presents a unique twist. A 7-year-old paediatric patient, initially presenting with fever and rash, exhibited a polymorphous rash varying from macular to maculopapular, with a cutis marmorata-like appearance, particularly on the extremities. What followed was a rapid deterioration, with the patient developing tachypnoeic respiration and moderate hypotension (80/35 mm Hg). Elevated D-dimer levels, troponin, ferritin, a low platelet count, a low albumin level, and lymphopenia were detected, further complicating the case. This unique case challenges our understanding of multisystem inflammatory syndrome in children and prompts further investigation.

Despite the severity of the case, the patient’s condition improved significantly with treatment. The patient’s COVID-19 polymerase chain reaction test was negative, but the COVID-19 immunoglobulin G test was positive. Transthoracic echocardiography demonstrated a mild/moderate systolic dysfunction of the left ventricular (Ejection fraction 52%, SF 25%), echogenicity of coronary vessels, and a first-degree mitral insufficiency was detected. Thorax CT showed pulmonary oedema. Milrinone and noradrenaline were promptly started for hemodynamic support. Intravenous immunoglobulin, methylprednisolone, favipiravir, and anakinra were started for his treatment. On the second day of hospitalisation, the patient’s condition was ameliorated. On the 10th day of hospitalisation, the patient was discharged from the hospital, marking a successful recovery from a complex and severe case. This successful recovery serves as a beacon of hope in the management of multisystem inflammatory syndrome in children.

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.