We report simultaneous transcatheter closure of a secundum atrial septal defect, a perimembranous ventricular septal defect, and a patent ductus arteriosus in a 3-year-old female wıth symptoms of heart failure. As it was not possible to determine the impact of each defect on the patient’s symptoms, all of them were closed with good results, for a tiny residual venrricular septal defect.

Coarctation of the aorta is a rare congenital cardiovascular anomaly. In some patients, one particular vessel in collateral circulation has achieved recognition as the artery of Hamilton and Abbott. This aberrant pathway can create local turbulence, elevating the risk of aneurysm formation. We report the clinical treatment of a 35-year-old patient with coarctation of the aorta combined with an aneurysm originating from the artery of Hamilton and Abbott to highlight the need for attention to the presence of this artery.

The initial and updated Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STAT and STAT 2020) and Risk Adjusted Classification for Congenital Heart Surgery-1 and Risk Adjusted Classification for Congenital Heart Surgery-2 scoring systems are validated to predict early postoperative mortality following congenital heart surgery in children; however, their ability to predict long-term mortality has not been examined. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium, a US-based registry of cardiac interventions in 47 participating centres between 1982 and 2011. Patients included in this cohort analysis had select congenital heart surgery representing the spectrum of severity as determined by STAT and Risk Adjusted Classification for Congenital Heart Surgery-1 and were less than 21 years of age. We applied STAT, STAT 2020, Risk Adjusted Classification for Congenital Heart Surgery-1, and Risk Adjusted Classification for Congenital Heart Surgery-2 for prediction of early mortality and long-term postoperative survival probability by surgical risk category. Long-term outcomes were obtained by matching Pediatric Cardiac Care Consortium patients with deaths reported in the National Death Index through 2021. Of 20,753 eligible patients, 18,755 survived the postoperative period and 2,058 deaths occurred over a median follow up of 24.4 years (Interquartile Range: 21–28.4). Each scoring system performed well for predicting early postoperative mortality with the following c-statistics: STAT: 0.7872, Risk Adjusted Classification for Congenital Heart Surgery-1: 0.7872, STAT 2020: 0.7724 and Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7668. The predictive ability for long-term risk of death was as follows: STAT: 0.6995, Risk Adjusted Classification for Congenital Heart Surgery-1 c = 0.6741, Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7156 and STAT 2020: c = 0.7156. Risk-adjusted score systems for congenital heart surgery maintain adequate but diminishing discriminative power to predict long-term mortality. Future efforts are warranted to develop a tool with improved long-term survival prediction.

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.

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.

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.

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.

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.

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.

A 50-year-old man presented with shortness of breath. The patient underwent ventricular septation for a single ventricle with L-malposition of the great arteries at 6 years of age. Examinations revealed that the calcified patch for septation was torn and perforated, resulting in a left-to-right shunt. We report a rare case of a single ventricle who survived after septation 40 years ago and underwent the reoperation due to a torn calcified patch.

In isolated subclavian artery, abnormal aortic arch development causes a loss of continuity with the aorta. Patent ductus arteriosus is a known cause of congestive heart failure. Herein, we present a rare case of congestive heart failure caused by isolated right subclavian artery and right patent ductus arteriosus associated with left-sided aortic arch treated by early closure.

Interruption of the inferior caval vein complicates device closure of atrial septal defects. We present a case where a simplified technique was used: from right jugular access the delivery system was directly engaged into the left atrium, where the entire septal occluder was deployed. Both discs were aligned with the interatrial septum, after which the right disc was recaptured and re-deployed in the right atrium under tension. This technique will allow device closure of atrial septal defects from the upper caval vein in selected cases.

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.