Cardiac complications after haematopoietic cell transplantation in paediatric patients are significant yet under-recognised. Pericardial effusion has been associated with worse outcomes and transplant-related mortality.

We aimed to evaluate the incidence, risk factors, and clinical course of pericardial effusion after paediatric allogeneic haematopoietic cell transplantation. We identified transplantation recipients “at risk” for clinically significant pericardial effusion based on our definition, described our clinical experience and provided recommendations for screening and management.

Clinical data of children who underwent allogeneic haematopoietic cell transplantation at Texas Children’s Hospital from January 2010 to April 2021 were analyzed retrospectively. Factors potentially contributing to time to pericardial effusion, time to pericardial effusion resolution, and overall survival were evaluated.

We included 629 haematopoietic cell transplantation recipients with a median age at transplantation of 8.5 years (0.1–24.3). Seventy-three patients (11.6%) developed pericardial effusion within a median time of 102 days (1–403) post-haematopoietic cell transplantation, and 50 (68.5%) had resolution of pericardial effusion at the time of last evaluation. Older age at the time of haematopoietic cell transplantation, transplant-associated thrombotic microangiopathy, and cytomegalovirus diagnoses independently increased the risk of pericardial effusion development, while cytomegalovirus diagnosis decreased the likelihood of pericardial effusion resolution. Both non-significant pericardial effusion development and clinically significant effusion development were significantly associated with post-haematopoietic cell transplantation mortality, compared to no pericardial effusion development.

Paediatric haematopoietic cell transplantation recipients with malignant diseases, older age at the time of transplantation, cytomegalovirus infection, or transplant-associated thrombotic microangiopathy are at higher risk for pericardial effusion development, which in turn predicts worse outcomes with increased risk of death. We propose a model for improved detection, evaluation, and management of pericardial effusion post-haematopoietic cell transplantation.

Brain abscess is a rare but life-threatening complication in patients with cyanotic congenital heart disease (CHD), especially in those without prior corrective surgery.

We report the case of a 36-year-old woman with unoperated cyanotic CHD who presented with neurological deficits. Diagnostic evaluation included cranial magnetic resonance imaging (MRI).

Imaging revealed a brain abscess. Contributing factors included chronic hypoxia, polycythaemia, and right-to-left shunting facilitating systemic circulation of infectious agents. The patient was treated with intravenous antibiotics and surgical drainage.

This case highlights the elevated risk of brain abscess in uncorrected cyanotic CHD and underlines the importance of early recognition and timely management to avoid fatal outcomes.

Transcatheter pulmonary valve implantation has emerged as a minimally invasive and preferred therapeutic option for patients with dysfunction of previously repaired right ventricular outflow tracts. The Myval™ Octacor valve is a new device designed for this purpose, though limited reports exist regarding its use in the pulmonary position.

To report the immediate and short-term outcomes of percutaneous pulmonary valve implantation using the Myval™ Octacor valve in patients with severe right ventricular–pulmonary artery conduit or pulmonary valve bioprosthesis dysfunction.

This was a single-centre retrospective review of data obtained from case files.

The Myval™ Octacor valve was used in ten patients with a mean age of 34.5 ± 7.4 years. The median procedure duration and fluoroscopy time were 146 minutes and 30.5 minutes, respectively. The median Z-score for valves used was −0.5. The median right ventricular systolic pressure decreased from 68.5 mmHg pre-procedure to 33 mmHg post-procedure. The median peak instantaneous gradient across the right ventricular outflow tract or conduit decreased from 30 mmHg to 6.5 mmHg. There were no reported incidences of frame fracture, conduit rupture, device embolisation, or endocarditis.

This is the first UK experience of using the new-generation Myval™ Octacor valve in percutaneous pulmonary valve implantation. The results demonstrate the valve’s safety and clinical efficacy, with favourable outcomes in terms of procedural success, haemodynamic improvement, and echocardiographic findings.

The impact of CHD on safe driving for adolescents is currently unknown. A prospective, qualitative descriptive study was conducted among adolescents with CHD to describe perceived barriers, facilitators, and impacts of CHD on safe, independent driving among adolescents.

Twenty-eight adolescents aged 15–19 years with CHD participated in virtual, semi-structured interviews in 2023. Adolescent interview data were analysed with conventional content analysis refined by Theoretical Domains Framework in NVivo software.

Mean participant age was 16.4 ± 0.23 years (57% male). Single ventricle physiology (25%) and septal defects (32%) were prevalent diagnoses among the study population. Most participants (92%) did not have driving restrictions.

Driving as a normal rite of passage for adolescents with CHD; and confident—but curious—about the impacts of CHD on driving. Adolescents felt confident that driving is not impacted by CHD. They were curious about the likelihood of cardiovascular emergencies and related symptoms while driving. Perceived barriers and facilitators to safe, independent driving were like what has been described in published literature among adolescents without CHD.

These findings celebrate the normalcy of driving during adolescence and reveal curiosities about the impacts of stress, anxiety, fatigue, and risks of heart attack and stroke on driving. Adolescents may look to CHD healthcare providers to help them learn about driving. These findings may inform the development of tools to facilitate meaningful conversations with adolescents regarding driving safety as part of the transition to adult CHD care.

To examine the added value of an elevated calculated peak right ventricular outflow tract pressure gradient as a surrogate for infective endocarditis diagnosis.

A cohort study included patients admitted between 2003 and 2020 at a tertiary children’s medical centre. Patients with surgically inserted exogenous right ventricular outflow tract conduits and infective endocarditis were included. Infective endocarditis was diagnosed using the revised Duke criteria (2023). Controls had right ventricular outflow tract conduits and febrile illness from other causes. Clinical, laboratory, and echocardiographic findings, including calculated peak right ventricular outflow tract pressure gradient, were collected.

Among 26 febrile episodes (11 with infective endocarditis, 15 controls), the infective endocarditis group had a higher peak right ventricular outflow tract pressure gradient during acute illness (70 vs. 23 mmHg, p < 0.05). On admission, 18% of infective endocarditis patients had a definite diagnosis by Duke’s criteria, 45% had a probable diagnosis, and 36% lacked confirmation. Including peak right ventricular outflow tract gradient as a major criterion would yield a 90% diagnosis rate upon admission (45% definite, 45% possible).

Increased right ventricular outflow tract pressure gradient in febrile patients with exogenous conduit in the right ventricular outflow tract is a potential marker for infective endocarditis. Including this gradient as a major Duke criterion enables earlier and more definitive diagnosis in debatable cases.

Heterotaxy patients after Kawashima surgery, with separately draining hepatic veins pose a complexity for Fontan completion. We describe a novel percutaneous technique for hepatic vein diversion and complete transcatheter Fontan.

A 6-year-old female with single ventricle physiology and bilateral hepatic vein drainage underwent Kawashima surgery. She later developed a complete heart block and elevated mean pulmonary pressures (17 mmHg). Permanent pacemaker implantation and left hepatic vein ligation were performed. Due to elevated pulmonary pressures, transcatheter Fontan completion was chosen over surgical options, and hepatic veins were included without prior surgical preparation as there were well-developed venous collaterals between the hepatic veins. The patient remained asymptomatic post-intervention with no complications and was discharged on anticoagulation therapy.

Pulmonary artery capacitance is a relatively novel measurement associated with adverse outcomes in pulmonary arterial hypertension. We sought to determine if preoperative indexed pulmonary artery capacitance was related to outcomes in paediatric heart transplant recipients, describe the changes in indexed pulmonary artery capacitance after transplantation, and compare its discriminatory ability to predict outcomes as compared to conventional predictors.

This was a retrospective study of paediatric patients who underwent heart transplant at our centre from July 2014 to May 2022. Variables from preoperative and postoperative clinical, catheterisation, and echo evaluations were recorded. The primary composite outcome measure included postoperative mortality, postoperative length of stay in the top quartile, and/or evidence of end organ dysfunction.

Of the 23 patients included in the analysis, 11 met the composite outcome. There was no statistical difference between indexed pulmonary artery capacitance values in patients who met the composite outcome [1.8 ml/mmHg/m2 (interquartile 0.8, 2.4)] and those who did not [1.4 (interquartile 0.9, 1.7)], p = 0.17. There were no significant signs of post-operative right heart failure in either group. There was no significant difference between pre-transplant and post-transplant indexed pulmonary artery capacitance or indexed pulmonary vascular resistance.

Preoperative pulmonary artery capacitance was not associated with our composite outcome in paediatric heart transplant recipients. It did not appear to be additive to pulmonary vascular resistance in paediatric heart transplant patients. Pulmonary vascular disease did not appear to drive outcomes in this group.

“Frailty” is associated with worse outcomes in adult cardiology. There is limited data on the associations between frailty and outcomes in paediatric cardiology. We aimed to define the prevalence of frailty and identify associations between frailty and neurodevelopmental and quality-of-life outcomes in high-risk paediatric cardiac populations.

This cross-sectional study included patients 4–18 years seen in a neurodevelopmental programme between 6/2017 and 11/2022. Demographic and clinical data were obtained from medical records. As part of the routine care, physical therapy assessment and neurocognitive, psychosocial, adaptive functioning, and quality-of-life surveys were administered. Social determinants of health were assessed by insurance status and Childhood Opportunity Index. Frailty was defined as the abnormality in 3 of 5 categories: body composition, weakness, slowness, physical activity, and exhaustion. Chi-Square, Student t, and Wilcoxon Rank Sum tests were used to assess differences between frail and non-frail groups.

Of the 270 patients, 101 (37%) met the frailty criteria. Frailty was not associated with social determinants of health, cardiac diagnosis, genetic syndrome, number of cardiac surgeries, or history of clinical complications. Frail patients were more likely to be older (p = 0.004) and have neurocognitive (p = 0.024), emotional (p = 0.003), social (p < 0.001), motor (p < 0.001), and adaptive dysfunction (p < 0.001) and lower quality of life (p = 0.029).

Frailty is common in school-aged patients with cardiac disease and is associated with adverse neurocognitive, psychosocial, motor, and adaptive outcomes and worse quality of life. Risk stratification for frailty may be a critical evaluation and screening element of high-risk cardiac patients in neurodevelopmental programmes.

Haemolysis is developing prominence in the setting of supporting increasingly complex children with heart failure with a ventricular assist device. The goal of this study is to better characterise haemolysis and its implications in children supported with pulsatile ventricular assist devices.

This is a single-centre retrospective review of 44 children who were supported by Berlin Heart EXCOR between January 2006 and June 2020. Patients were divided into major haemolysers and non-major haemolysers. Major haemolysers were defined as patients with lactate dehydrogenase > 500U/L (2.5x the upper limits of normal) with either total bilirubin > 2mg/dL (with predominantly indirect hyperbilirubinemia) or anaemia out of proportion to the clinical scenario more than three days following implantation of the ventricular assist device(s). Patient demographics, ventricular assist device factors, and outcomes, including end-organ function and mortality, were compared between major haemolysers and non-major haemolysers.

Forty-four patients supported by the Berlin EXCOR were included in the analytic cohort of the study: 27 major haemolysers and 17 non-major haemolysers. Major haemolysis was more common in those supported with single-ventricle ventricular assist device (i.e., VAD in the context of functionally univentricular anatomy) compared to those with biventricular hearts, p = 0.01. There were no patients with an isolated left ventricular assist device or isolated right ventricular assist device in our analytic cohort of 44 patients. Of the 19 patients with single-ventricle ventricular assist device, 84% (16/19) were major haemolysers. Of the 25 patients with a biventricular assist device, 44% (11/25) were major haemolysers. Major haemolysers and non-major haemolysers had a body surface area of 0.28 and 0.40, respectively (p = 0.01). Overall, survival to discharge from the hospital was 66% (n = 29/44). Survival to discharge from the hospital was 52% (14/27) in major haemolysers versus 88% (15/17) in non-major haemolysers, p = 0.02. Only 3 of the 27 with major haemolysis had severe haemolysis, that is, lactate dehydrogenase > 2000 and bilirubin above 10. Non-major haemolysers had a better improvement in creatinine clearance during ventricular assist device support, p < 0.0001. (During the same era of this study, 22 patients who were supported with Berlin Heart were excluded from the analytic cohort because they did not have any recorded measurement of lactate dehydrogenase. Seventeen of these 22 patients had no clinical evidence of haemolysis. Survival to discharge from the hospital in this excluded cohort was 86% [19/22].)

Major haemolysis in patients with pulsatile ventricular assist device is more likely with single-ventricle ventricular assist device support and smaller body surface area.

To assess the feasibility of transcatheter closure in patients with dextrocardia and isolated atrial septal defect or ventricular septal defect.

A retrospective analysis was performed on the clinical data of 10 patients with dextrocardia and atrial septal defect or ventricular septal defect from June 2013 to January 2023 and successfully underwent transcatheter closure. Patient data were meticulously collected.

The study cohort comprised 10 patients, with three males, aged between 3 and 38 years. Intraoperative right heart catheterisation revealed the following measurements: the mean pulmonary arterial pressure of 20.5(18,24,3) mmHg, and a pulmonary-to-systemic flow ratio of 1.80(1.58,2.15). There were five atrial septal defects, with defect diameters of 26(20,30) mm, comprising three dextroversion and two mirror-image dextrocardia. Intraoperative echocardiography confirmed the absence of any residual shunt. Among them, a patient with mirror-image dextrocardia, atrial septal defects, and interrupted inferior vena cava required an alternative approach due to femoral vein limitations. The right internal jugular vein was punctured, and a 22 mm atrial septal occluder was successfully deployed via this route. The study identified five ventricular septal defects, with defect diameters of 4(3.5,5.5) mm, including two dextroversion and three mirror-image dextrocardia. Upon post-operative repeat left ventriculography, no residual shunt was detected in all but one case, which exhibited a minimal residual shunt. Throughout the perioperative period and subsequent post-operative follow-up, no severe complications were observed.

For patients with dextrocardia accompanied by simple CHD, transcatheter closure is a viable option when interventional treatment is indicated. Although transcatheter closure of atrial septal defects or ventricular septal defect in patients with dextrocardia presents unique challenges, it is safe and effective when the anatomical nuances of dextrocardia are thoroughly understood.