Fontan-associated liver disease leads to liver cirrhosis and hepatocellular carcinoma, worsening patient prognosis. Although the Model of End-Stage Liver Disease Excluding International Normalized Ratio score correlates with disease severity, precise prediction methods remain elusive. The hepatic computed tomography (CT) attenuation is used to assess steatohepatitis, which may offer insight into disease progression. This study aimed to determine the usefulness of hepatic CT attenuation values in assessing Fontan-associated liver disease progression. All post-Fontan patients with >20 years’ follow-up data between 1980 and 2021 were reviewed. We set five regions of interest with a 20-mm-diameter circle in the peripheral region of the liver to obtain minimum, maximum, mean, and standard deviation of the CT attenuation values, which were adjusted to those of the aorta. The impact of hepatic CT attenuation values on cirrhosis development was evaluated. Cox regression revealed significant associations between adjusted minimum (hazard ratio: 0.01 [0.00–0.02, p < 0.01), adjusted maximum (hazard ratio: 159 [4.34–5831, p < 0.01]), and standard deviation (hazard ratio: 1.89 [1.29–2.76, p < 0.01]) and Model of End-Stage Liver Disease Excluding International Normalized Ratio score (hazard ratio: 1.2 [1.02–1.43, p = 0.03]) with cirrhosis. In cirrhosis cases, the adjusted minimum value exhibited significant longitudinal alteration prior to cirrhosis (p < 0.01), whereas Model of End-Stage Liver Disease Excluding International Normalized Ratio scores remained stable. The hepatic CT attenuation values were associated with cirrhosis development and the longitudinal changes are more sensitive than the Model of End-Stage Liver Disease Excluding International Normalized Ratio score.

Coronary ectasia is a very rare phenomenon seen in Noonan syndrome with only a few documented case reports. We describe a 14-year-old with Noonan syndrome and tetralogy of Fallot with described coronary artery ectasia since infancy who presented for possible transcatheter pulmonary valve placement and was found to have severe ectasia of bilateral coronary arteries.

For many patients with right ventricular outflow tract (RVOT) dysfunction, percutaneous pulmonary valve implantation (PPVI) offers a less-invasive alternative treatment option to surgery with a similar long-term result. However, the implantation of the valves recently available on the European market was only possible in patients with a limited maximal RVOT diameter. In patients with large RVOT, surgical treatment has remained the only possible treatment modality until recently. The self-expandable Harmony transcatheter pulmonary valve is a valve dedicated for patients with severe pulmonary regurgitation in the native and surgically repaired wide RVOT. The initial experience worldwide with this valve is very promising. However, it has not been implanted in Europe until recently. We report on four young adult patients with severe pulmonary valve regurgitation who underwent a successful PPVI with Harmony valve implantation. There were no serious complications in our small study group. In conclusion, the Harmony valve may offer a less-invasive and safe treatment alternative to surgery for patients in whom the available balloon-expandable valves offer only limited applicability. This valve has only recently become accessible on the European market.

A case of an adolescent male with hypoplastic coronary arteries and myocardial bridging who had a sudden cardiac death event playing soccer. This rare anomaly is not easily identified and may be missed during routine work-up. Treatment options are limited and there is a need for discussion of additional treatment considerations that may prevent sudden cardiac death in this population.

Hypoplastic left heart syndrome with a right aortic arch is rare, and performing the Norwood procedure in these cases is challenging. Here, we describe a 3-month-old patient with hypoplastic left heart syndrome having a right aortic arch accompanied by a 20q11.2 microdeletion. Because of cardiac dysfunction, ductal stenting was performed to avoid an early infantile Norwood procedure.

Not only has Wolff–Parkinson–White syndrome been associated with congenital cardiac abnormalities and cardiomyopathies, but familial clustering of Wolff–Parkinson–White syndrome has also been reported. Despite these well-known associations, direct genetic aetiology is rarely implicated in patients with Wolff–Parkinson–White syndrome. We report a 17-year-old girl with Wolff–Parkinson–White syndrome and left ventricular non-compaction cardiomyopathy due to a rare genetic variant in PR-domain containing protein 16. The report is supplemented by a comprehensive review of literature on association between PRDM16, left ventricular non-compaction and Wolff–Parkinson–White syndrome.

The anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital anomaly usually diagnosed in infancy. When diagnosed later during adolescence, it presents diagnostic and treatment challenges, especially in determining whether pulmonary vascular disease can be reversed. We report the case of an 11-year-old girl who experienced increasing fatigue and was diagnosed with anomalous origin of the right pulmonary artery from the ascending aorta through echocardiography, CT angiography, and cardiac catheterisation. Segmental pulmonary hypertension was noted, but the operability was uncertain. A new non-invasive test combining 2D phase-contrast cardiac MRI with inhaled iloprost was performed. Flow measurements revealed a 42% rise in right pulmonary artery flow, indicating preserved vasoreactivity. Lung biopsy confirmed pulmonary vascular changes consistent with Heath–Edwards Stage II–III. Based on these findings, surgical reimplantation of the anomalous artery into the main pulmonary artery was performed. The postoperative recovery after surgery was uneventful, and follow-up catheterisation demonstrated normalised haemodynamics without residual stenosis. This case highlights the potential value of cardiac MRI–based vasoreactivity testing with inhaled iloprost as an additional tool alongside standard diagnostics for evaluating operability in late-presenting anomalous origin of the right pulmonary artery from the ascending aorta. Combining imaging, histopathology, and haemodynamic data allowed a personalised and safe surgical approach.

We report the case of a 5-year-old boy with severe pulmonary arterial hypertension due to partial anomalous pulmonary venous connection and atrial septal defect. Sequential combination therapy using multiple pulmonary vasodilators enabled safe surgical repair. In this case of severe pulmonary arterial hypertension—associated with partial anomalous pulmonary venous connection and atrial septal defect—the treat-and-repair strategy proved effective.

We report a case of right ventricle to pulmonary artery conduit angioplasty in which a valvuloplasty balloon ruptured circumferentially and was retained within the conduit. A high-pressure balloon was used to relieve the obstruction and free the ruptured balloon. The procedure was further complicated when the distal part of the balloon broke away from the proximal part during an attempt to retrieve it back into the femoral sheath. This report highlights the inappropriate use of a soft balloon in a calcified xenograft conduit, which led to a series of complications, and discusses potential strategies for managing these complications.

A neonate with unbalanced right-dominant atrioventricular canal defect with intact atrial septum and abnormal pulmonary venous return developed bradycardic arrest and a subsequent diagnosis of tracheal rings and left lung aplasia. This report details the fatal nature of the triad of hypoplastic left heart, pulmonary venous obstruction, and airway anomalies, along with the role of advanced imaging in prognostication.

Sternal cleft is a rare congenital condition associated with unprotected viscera and respiratory failure. We present a case of a sternal cleft, absent right clavicle, and congenital diaphragmatic paresis in a neonate. The sternum was closed primarily, and the patient was taken back for plication of the diaphragm. At 3 years follow-up, the patient is free from complications.

We described a 14-year-old boy who underwent catheter ablation for atrial tachycardia that had difficulty in creating the whole circuit of 3D map due to widely spread scar after repeated surgery for multivalvular heart disease. The classical atrial entrainment method was very effective in planning the catheter ablation for the invisible circuit of the atrial tachycardia.

Unilateral absence of the pulmonary artery is a rare congenital defect requiring advanced imaging for diagnosis. We present two cases with a connection between a patent ductus arteriosus remnant and the left pulmonary artery. Therapeutic approaches, including imaging findings and management strategies, are discussed to highlight diagnostic and treatment considerations.

Patent ductal arteriosus stenting is an alternative procedure in patients with pulmonary-ductal-dependent circulation. Stent embolization is one of the major acute complications of ductal stenting procedure. We describe the case of a stent embolization into the abdominal aorta during the deployment of ductal stent in a premature low-weight infant (1.850 kg), affected by critical pulmonary stenosis and duct-dependent pulmonary circulation. The stent was successfully retrieved through a 4F Flexor catheter without vascular complication by using a new approach.