Search

The experience of diagnosis, decision-making and management in critical congenital heart disease is layered with complexity for both families and clinicians. We synthesise the current evidence regarding the family and healthcare provider experience of critical congenital heart disease diagnosis and management. A systematic integrative literature review was conducted by keyword search of online databases, MEDLINE (Ovid), PsycINFO, Cochrane, cumulative index to nursing and allied health literature (CINAHL Plus) and two journals, the Journal of Indigenous Research and Midwifery Journal from 1990. Inclusion and exclusion criteria were applied to search results with citation mining of final included papers to ensure completeness. Two researchers assessed study quality combining three tools. A third researcher reviewed papers where no consensus was reached. Data was coded and analysed in four phases resulting in final refined themes to summarise the findings. Of 1817 unique papers, 22 met the inclusion criteria. The overall quality of the included studies was generally good, apart from three of fair quality. There is little information on the experience of the healthcare provider. Thematic analysis identified three themes relating to the family experience: (1) The diagnosis and treatment of a critical congenital heart disease child significantly impacts parental health and wellbeing. (2) The way that healthcare and information is provided influences parental response and adaptation, and (3) parental responses and adaptation can be influenced by how and when support occurs. The experience of diagnosis and management of a critical congenital heart disease child is stressful and life-changing for families. Further research is needed into the experience of minority and socially deprived families, and of the healthcare provider, to inform potential interventions at the healthcare provider and institutional levels to improve family experience and support. [ABSTRACT FROM AUTHOR]

There is currently no clear consensus on screening techniques to evaluate the presence or severity of Fontan-associated liver disease (FALD). Cardiac MRI (CMR) is used routinely for post-Fontan surveillance, but CMR-derived measures that relate to the severity of FALD are not yet defined. This was a cross-sectional single-center study of post-Fontan patients who underwent a CMR. CMR exams were re-analyzed by a single pediatric cardiologist. Surrogates of FALD included Gamma-Glutamyl Transferase (GGT), Fibrosis-4 laboratory score (FIB-4), and imaging findings. Findings consistent with cirrhosis on liver ultrasound included increased liver echogenicity and/or nodularity. Statistical analyses were performed to investigate potential relationships between CMR parameters and markers of FALD. Sixty-one patients were included. A larger inferior vena cava cross-sectional area (IVC-CSA) indexed to height was significantly associated with a higher FIB-4 score (Spearman's ρ = 0.28, p = 0.04), a higher GGT level (Spearman's ρ = 0.40, p = 0.02), and findings consistent with cirrhosis on liver ultrasound (OR 1.17, 95% CI: (1.01, 1.35), p = 0.04). None of the other CMR parameters were associated with markers of FALD. A larger indexed IVC-CSA was associated with higher systemic ventricle end-diastolic pressure (EDP) on cardiac catheterization (Spearman's ρ = 0.39, p = 0.018) as well as older age (Spearman's ρ = 0.46, p = < 0.001). Indexed IVC-CSA was the only CMR parameter that was associated with markers of FALD. This measure has the potential to serve as an additional non-invasive tool to improve screening strategies for FALD. Visual abstract summarizing the primary findings of this paper [ABSTRACT FROM AUTHOR]

Parents of children with congenital heart disease experience unique stressors that affect their psychological well-being and health-related quality of life. A parent's ability to cope effectively influences how they adjust to stressful situations. The purpose of this review is to synthesize the factors influencing HRQoL in parents of children with congenital heart disease under the lens of the Transactional Model of Stress and Coping framework to identify areas for intervention and future research. An integrative literature review was conducted by keyword search of online databases PubMed, PsycINFO, Cumulative Index to Nursing and Allied Health Literature, and Medline from 2011 to 2022. Inclusion and exclusion criteria were applied to search results and studies were assessed for quality using the Mixed Method Appraisal Tool. The initial search yielded 1026 unique papers, and 24 were included based on the predefined criteria. The Transactional Model of Stress and Coping was applied to organize results and generate themes. Results showed a child's hospitalization is a time of uncertainty and distress for parents of children with congenital heart disease. Effective problem-focused and emotion-focused coping strategies can lead to reduced stress and improved health-related quality of life for parents. Additionally, multiple variables moderate the relationship between a parent's perceived stress and adjustment. Health care teams should focus on targeted education, faith, coping, and social support interventions. Further research is needed that examines the effect of mental health factors, cultural differences, and socioeconomic variables on the health-related quality of life of parents of children with congenital heart disease. [ABSTRACT FROM AUTHOR]

The classical Hamburger and Hamilton (HH) paper demonstrates the normal stages of development of the chick embryo that have been extensively used as the basis of understanding normal and abnormal development of the chick embryo heart. Careful examination of the series of images published in this seminal paper indicates that the cardiac images of stage 16 embryo shown in this article may reflect an abnormally developed heart. In this article, the argument is presented that the embryo depicted in the HH paper is not normal, but instead inflicted with a conotruncal heart defect. [ABSTRACT FROM AUTHOR]

At some point in their life, adolescent patients with a congenital heart disease (CHD) transition from paediatric services to adult care facilities. The process is not without any risks, as it is often linked with a significantly progressive deterioration in adolescents' health and loss of follow-up. In fact, transition patients often encounter troubles in finding a care giver who is comfortable managing their condition, or in re-establishing trust with the new care provider. Planning the rules of transition is pivotal in preventing these risks. Unfortunately, the American and European guidelines on CHD provide just generic statements about transition. In a recently published worldwide inter-societies consensus document, a hybrid model of transition, which should be adapted for use in high- and low- resource settings, has been suggested. Currently, in literature there are a few models of transition for CHD patients, but they are by far local models and cannot be generalized to other regions or countries. This paper describes the Irish model for transition of care of CHD patients. Due to the peculiarity of the healthcare organization in the Republic of Ireland, which is centralized with one main referral centre for paediatric cardiology (in Dublin, with a few smaller satellite centres all around, according to the "hub and spoke" model) and one centre for adult with CHD (in Dublin), the model can be considered as a national one and the first to be released in the old continent. [ABSTRACT FROM AUTHOR]

Increasingly non-cardiac tertiary neonatal intensive care units (NCTNs) manage newborns with CHD prior to planned transfer to specialist cardiac surgical centres (SCSC). It improves patient flow in SCSCs, enables families to be nearer home, and improves psychological well-being Parker et al. (Evaluating models of care closer to home for children and young people who are ill: a systematic review, 2011) . This practice has gradually increased as the number of SCSCs has decreased. This study examines the effectiveness of this expanding practice. The management provided, length of stay in the NCTN and outcomes are described for one UK NCTN situated at a significant distance from its SCSC. A retrospective observational study of cardiac-related admissions to a NCTN between January 2010 and December 2019 was conducted. 190 neonates were identified: 41 had critical CHD; 64 had major CHD. The cohort includes babies with a wide range of cardiac conditions and additional complexities. 23.7% (n = 45) required transfer to a specialist center after a period of stabilization and growth ranging from several hours to 132 days. 68% (n = 130) were discharged home or repatriated to a local NICU. Of the remaining 15 babies, 13 were transferred to other specialties including the hospice. Two died on NICU. The mortality was consistent with the medical complexity of the group Best and Rankin (J Am Heart Assoc 5:e002846, 2016), Laas et al. (BMC Pediatr 17:124, 2017). 8.9% (n = 17) died before age 2. Nine babies had care redirected due to an inoperable cardiac condition or life-limiting comorbidities. Our study demonstrates a complex neonatal cohort with CHD can be managed effectively in a NCTN, supporting the current model of care. The NCTN studied was well supported by pediatricians with expertise in cardiology alongside visiting pediatric cardiologists., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Kawasaki disease is an acute vasculitis affecting children, which can lead to coronary artery (CA) aneurysms. Optical coherence tomography (OCT) has identified CA wall damage in KD patients, but it is unclear if these findings correlate with any distensibility changes in the CA and how these changes evolve over time. This paper seeks to establish the link between OCT findings and vessel distensibility with a novel deep learning coronary artery segmentation system and use the segmentation framework to automatically analyze the temporal evolution of coronary stiffness over many years. 27 KD patients underwent catheterization with coronary angiography of the left coronary artery (LCA), followed by OCT of proximal and distal segments of the LCA. Changes in the CA caliber over the cardiac cycle were measured automatically and compared against OCT findings suggestive of KD-related vascular damage. In addition, 34 KD patients with regressed or persistent CA aneurysms were followed with serial CA angiography over an average of 14.5 years. Distensibility changes were calculated using a deep learning coronary artery segmentation framework and evaluated longitudinally. Distensibility in the coronary arteries after KD negatively correlated with increasing severity of OCT findings of KD-related vessel damage. KD patients have a significant increase in CA wall stiffness at 1 year after diagnosis, which then plateaus subsequently, compared to controls. Also, patients with persistent CA aneurysms have a statistically significant increase in wall stiffness over time in comparison to those with regressed CA aneurysms. Distensibility changes in the CA of KD patients calculated using our automated deep learning approach correlates with the severity of OCT findings of KD-related CA damage. This decreased distensibility peaks at 1 year in KD patients when following longitudinally and is more severe in those with persistent CA aneurysms. [ABSTRACT FROM AUTHOR]

Over the past 50 years, pediatric and congenital electrophysiology has progressed from a rare subspecialty practiced at just a handful of centers to become an essential part of nearly every congenital heart program the world over. This paper traces the evolution of the subspecialty in North America by recognizing the individuals who dedicated their careers to this field and trained subsequent generations of practitioners, thereby establishing a legacy that insures continued progress in the treatment of arrhythmias for children and patients with congenital heart disease. Data were generated from a survey of the current membership of the Pediatric and Congenital Electrophysiology Society and were supplemented by review of archived Society records. Maps depicting the locations for clinicians and training centers over time provide a graphic display of progress in this field. [ABSTRACT FROM AUTHOR]

Optimal outcomes are as much influenced by critical decision making pathways as by the technical skill of the operator. The complexity and potential cognitive traps underlying critical decision making has long been recognized in the aviation and business communities, however, remains a largely subconscious, unexamined discipline amongst congenital cardiac interventionalists. Challenges to making good decisions in the catheterization laboratory include heuristics, biases, and cognitive traps. In this paper we discuss some of the more common decision making challenges encountered and we address potential solutions to such decision making with particular focus towards standardization. [ABSTRACT FROM AUTHOR]

Accordingly, the correct I p i value is 0.53 and not 0.33 in the sentence, "Absolute AVC width changed significantly over time in the total cohort ( I p i < 0.001), but with no significant difference between the three groups ( I p i = 0.53)." Furthermore, the correct I p i value is 0.09 and not 0.28 in the sentence, "The ventricular width also showed significant growth in the total cohort ( I p i < 0.001), but again no difference between the three diagnostic groups was seen ( I p i = 0.09).". [Extracted from the article]

Enteral feeds are often withheld from neonates with ductal dependent cardiac lesions who are receiving prostaglandins. This is despite positive benefits of enteral feeding. We describe a multicenter cohort of these neonates who were fed pre-operatively. We also give a granular description of vital sign measurements and other risk factors prior to feeding. A retrospective chart review was performed at seven centers. Inclusion criteria were full-term neonates under one month of age with ductal dependent lesions receiving prostaglandins. These neonates were fed for at least 24 h during the pre-operative period. Premature neonates were excluded. Using the inclusion criteria, 127 neonates were identified. While being fed, 20.5% of the neonates were intubated, 10.2% were on inotropes, and 55.9% had an umbilical arterial catheter in place. Median oxygen saturations in the six hours prior to feeding were 92.5% in patients with cyanotic lesions, median diastolic blood pressure was 38 mmHg and median somatic NIRS were 66.5%. The median peak daily feeding volume reached was 29 ml/kg/day (IQ range 15.5–96.8 ml/kg/day). One patient developed suspected necrotizing enterocolitis (NEC) in this cohort. Only one adverse event occurred, which was an aspiration thought to be related to feeding, but did not result in intubation or cessation of feeds. NEC was rare among neonates with ductal dependent lesions while receiving enteral nutrition pre-operatively. Umbilical arterial catheters were in place in the majority of these patients. Hemodynamic measures demonstrated a high median oxygen saturation prior to initiation of feeds. [ABSTRACT FROM AUTHOR]