Thoracotomy-assisted arterial duct stenting is a palliative option for patent ductus arteriosus-dependent systemic circulation, typically reserved for infants at high risk for open-heart surgery, such as those with genetic syndromes or very low birth weight. We report the first documented case of thoracotomy-assisted arterial duct stenting in a patient with Kleefstra syndrome, resulting in systemic stabilisation and long-term survival.

Although inherited haemoglobinopathies may indicate low oxygen saturation measured by pulse oximetry (SpO2), the timing and mode of its decline after birth are unclear. Here, we present a patient with haemoglobin Köln disease who developed unexpected decline in SpO2 more than two years after heart surgery. Due to simultaneous identification of inferior vena cava stenosis, the differential diagnosis became complicated.

Wolff-Parkinson-White syndrome, characterised by accessory pathways, is rarely seen with dextrocardia. We present a case of situs inversus-dextrocardia with Wolff-Parkinson-White syndrome successfully treated via catheter ablation using 3D mapping (EnSite Precision®). Adjustments included reversed electrocardiogram (ECG) electrode placement, EnSite patch positioning, and fluoroscopic views. Coronary sinus access required counterclockwise manoeuvers. Mapping identified a left-sided accessory pathway, necessitating transseptal puncture with mirror-image adjustments. Ablation at the optimal site resulted in success. This case highlights the feasibility of catheter ablation in dextrocardia with tailored procedural modifications.

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.

Venous thrombosis in the Fontan circulation can be a devastating complication, and its management has traditionally been surgical. Here, we present two cases of extensive Fontan thrombosis that have both been successfully medically managed, with survival beyond two years.

Interrupted aortic arch is a rare congenital heart anomaly characterised by a complete discontinuity between the ascending and descending aorta, accounting for approximately 1–1.5% of CHDs. Its incidence in live births ranges from 3 to 20 per million. Survival into adulthood is only possible with the presence of a well-developed collateral circulation. In this case, a 49-year-old male patient presented with acute anterior myocardial infarction, and during emergency coronary angiography, discontinuity of the aortic arch was noticed. The procedure was successfully completed via radial access after femoral access failed. Subsequent thoracic computed tomography angiography confirmed the diagnosis of Type A interrupted aortic arch and revealed widespread collateral arterial circulation. No blood pressure difference was detected between the right-left arms and lower extremities, and the patient remained asymptomatic. This case demonstrates that this rare anomaly in adults can be incidentally detected during emergency procedures and that radial access is an effective alternative in overcoming anatomical obstacles.

This is a unique case of microbubbles incidentally found on echocardiogram in a child with unpalliated CHD. The microbubbles were found in both the venous and arterial circulations and spontaneously resolved. A detailed evaluation was done to identify the source of air in the circulation. The child did have concurrent necrotising enterocolitis, and it was felt that the bubbles were secondary to this and intestinal translocation of air.

This report describes a 41-year-old female with left isomerism, interrupted inferior caval vein with azygos continuation, dextrocardia, and repaired tetralogy of Fallot, who underwent percutaneous pulmonary valve implantation using the Venus P-valve system. Due to anatomical constraints, left jugular venous access was utilised. A Venus P-valve (30 by 25 mm) was successfully implanted in the right ventricular outflow tract using a simplified, one-curve trajectory directly on its delivery system without a delivery sheath. Contrast injections during valve implantation were not possible, and the pre-implanted duct occluder was our anatomical landmarks. This case highlights the adaptability of the Venus P-valve and the importance of individualised procedural strategies in addressing anatomical challenges and achieving optimal outcomes.

We report the case of a 10-month-old infant presenting with dilated cardiomyopathy caused by septal dyssynchrony linked to a right septal accessory pathway-mediated pre-excitation. No atrioventricular reciprocating tachycardia was reported, but this pre-excitation resulted in left septal dyssynchrony. After the introduction of flecainide therapy, the antegrade pre-excitation disappeared on ECG, leading to normalisation of left ventricular function.

We report the case of a 15-year-old male patient with severe pulmonary regurgitation who successfully underwent Alterra Adaptive Prestent implantation through the right jugular vein because of bilateral femoral vein occlusion.

The latent transforming growth factor-beta-binding protein 3 (LTBP3), which encodes one of the extracellular matrix proteins, plays an essential role in skeletal formation through both stimulatory and inhibitory effects on the growth of different cell types, as well as on the production and degradation of the extracellular matrix. Pathogenic variants in LTBP3 have been associated with genetic skeletal disorders that exhibit various cardiovascular features, including aortic root dilatation, aneurysm or dissection of the ascending and descending aorta, and mitral valve prolapse). Aortic root dilatation, an aortopathy that may have lifethreatening consequences, is among the clinical findings in various connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Aortic root dilatation, aneurysm, and aortic dissection should be carefully investigated by cardiologists. In this study, we describe three siblings with short stature and dental anomalies. A homozygous, novel, c.2726-1G > A pathogenic variant in LTBP3 was identified through exome sequencing. After the detection of the LTBP3 variant, the patients were evaluated for possible cardiac findings, which revealed mitral valve prolapse and aortic root dilatation despite the absence of clinical symptoms. In this study, we aimed to shed light on the diagnosis of dental anomalies and short tature syndrome in three siblings with a novel LTBP3 pathogenic variant revealed through exome sequencing. Additionally, we emphasise the importance of searching for cardiac findings, even in the absence of clinical symptoms. We highly suggest that cardiologists take note of cardiac findings in patients with dental anomalies and short stature syndrome.

Congenital extrahepatic portosystemic shunt (CEPS) is an uncommon condition in which the portal vascular system drains into the systemic veins. We describe an unusual case of CEPS with congenital heart disease, presenting with aneurysmal enlargement of the pulmonary artery and symptoms of airway compression, highlighting the importance of evaluating for additional pathology in case of unexplained oxygenation defect or pulmonary hypertension.

Interrupted aortic arch is an uncommon cardiac anomaly characterised by a lack of continuity between the ascending and descending aorta. The presence of interrupted aortic arch in adults is extremely rare, and there is limited documentation of such cases in the literature. In this article, we present a unique case of interrupted aortic arch in an adult diagnosed through angiography. This case falls under the anatomical classification of type B interruption, although the blood supply to the left subclavian artery originates from the ascending aorta. Its haemodynamic characteristics are completely different from those of the classical type B interruption.