We present the case of 53-year-old woman with a late diagnosis of an right pulmonary artery-left atrium fistula who underwent transcatheter device closure using multi-modality imaging for pre-procedural planning and procedural guidance.

Refractory chylothorax, a postoperative complication of CHD, is difficult to manage and sometimes fatal. Herein, we report the case of a 10-month-old infant with 22-mosaic trisomy and a coarctation complex, who developed refractory chylothorax after cardiac repairs and was successfully treated with midodrine, an oral alpha-1-adrenoreceptor agonist. Midodrine may be used as adjunctive therapy for postoperative refractory chylothorax.

Anomalous left coronary artery from the pulmonary artery is a congenital coronary artery anomaly comprising 0.5% of all CHDs. Stenosis in the left coronary artery implanted in the sinus valsalva is one of the complications of surgical repair.

Management of coronary stenosis after surgery is very critical and difficult, especially in newborns and infants. Transcatheter procedures can be lifesaving. Herein, the coronary stenting procedure applied to a 3-month-old patient with post-surgical coronary stenosis is presented.

Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein’s anomaly following Starnes procedure. The patient’s postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.

Unilateral absence of the pulmonary artery is a rare congenital cardiovascular anomaly that can lead to pulmonary hypertension and poor outcomes. We report the case of a 1-month-old infant with isolated unilateral absence of the pulmonary artery and severe pulmonary hypertension on the right and left sides, respectively. The patient was unresponsive to multiple medications for pulmonary hypertension, and surgical revascularisation was unfeasible. However, iloprost inhalation was effective.

This report outlines a 9-year-old Thai boy with acute myocarditis due to rhinovirus infection, manifesting complete heart block. Despite aggressive treatment, including pulse methylprednisolone and temporary pacing, his condition remained unstable. He gradually improved over five days in the paediatric cardiac ICU and continued recovery over six months. This underscores the need for comprehensive treatment and follow-up in paediatric myocarditis.

Arterial tortuosity syndrome is an extremely rare hereditary connective tissue disorder. We present a case of an incidentally diagnosed aneurysm of the aortic root and the ascending aorta caused by arterial tortuosity syndrome, which was confirmed genetically. The aneurysm was repaired surgically. One year after the procedure, there was no further dilation of the aorta or formation of new aneurysms.

We report on a 7-month-old boy (4.2 kg/60 cm) with severe immunodeficiency disorder and bacterial septicaemia who was referred for an infected atrial thrombus secondary to a jugular central line. The echocardiogram showed a teardrop-shaped thrombus with a wide base adherent to the interatrial wall and a flimsy tail moving freely in the right atrium. Chest CT scan showed multiple lesions in both lungs consistent with infected micro-thrombi. The thrombus increased in size despite 2 weeks of antibiotics and anticoagulation. We applied the Indigo® Lightning® 7 aspiration system from Penumbra® (Alameda, USA) and removed percutaneously the thrombus under transoesophageal ultrasound and biplane fluoroscopic guidance. At 6 weeks of follow-up, the patient is alive, under enoxaparin with no procedure-related complication.

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

Durable mechanical circulatory devices are commonly used to support children and adolescents in end-stage heart failure. However, these patients remain at high risk of acute medical complications, which may lead to significant impairment in functional capacity, altered quality of life, or death. We explore the incorporation of adolescent directives into medical decision-making in this scenario through a clinical case vignette.

A 12-year-old female with pulmonary artery stenosis developed pseudoaneurysms due to septic embolism, requiring close follow-up.

When the atrioventricular node is damaged, accessory pathways can perform primary atrioventricular conduction but may spontaneously degrade during childhood. After surgical atrial septal defect repair during infancy, an adolescent male presented with fatigue due to iatrogenic complete atrioventricular node block with a degrading antegrade accessory pathway resulting in symptomatic bradyarrhythmia.

Chromosomal 2q37 deletion syndrome, marked by developmental delays, distinctive facial features, and a spectrum of congenital anomalies, presents significant challenges in the cardiac management of affected individuals. This paper details the case of an 8-month-old male with 2q37 deletion syndrome, manifesting atrial and ventricular septal defects, patent ductus arteriosus, and right ventricular outflow tract stenosis, leading to a demanding postoperative course. Despite an initially stable post-surgery phase, the onset of junctional ectopic tachycardia necessitated prolonged veno-arterial extracorporeal membrane oxygenation support, highlighting the syndrome’s potential for intricate postoperative courses. The complexities encountered in this case, including extended renal replacement therapy and delayed thoracic closure, underscore the syndrome’s multisystem impact and the critical need for tailored, multidisciplinary care approaches. This report contributes to the growing body of knowledge on the cardiac implications of 2q37 deletion syndrome, emphasising the importance of individualised surgical strategies and the ongoing exploration of genotype–phenotype correlations in this rare genetic disorder.

We report a 20-year-old female patient (76 Kg/164 cm) with an extra-cardiac Fontan circulation who was referred to our institution for exertional dyspnoea and desaturation. The patient was diagnosed with a large calcified thrombus at the level of the Fontan fenestration, protruding inside the lumen of the conduit and reducing the diameter by half with a 3 mmHg pressure gradient. Transcatheter stent expansion of the obstructed extra-cardiac conduit was done with a 48 mm long XXL PTFE-covered Optimus-CVS® under temporary cerebral embolic protection with a TriGUARD-3™ deflection filter device (Keystone Heart). There was no procedural complication and the 3 months clinical outcomes are good.

Pulmonary vein stenosis continues to pose significant challenges in children, frequently requiring repeated cardiac catheterisation procedures. This report describes a successful application of a “triple kissing balloon” technique to treat complex pulmonary vein stenosis in two patients, all accomplished with the use of a single 6-French sheath.

Adolescents with severe cardiogenic shock can present to both paediatric and adult centres. We present six adolescent children who had extracorporeal membrane oxygenation consultation fast-tracked with clinical care input from the adult multidisciplinary team, including interhospital transfers on extracorporeal membrane oxygenation. After recovery on conventional cardiogenic shock care or extracorporeal membrane oxygenation, or bridge to transplant, all had favourable neurologic outcome.