Cardiology in the Young最新文献

Takotsubo cardiomyopathy can be triggered by stress. We report a 2-year-old boy with a single right ventricle after bidirectional Glenn surgery who developed Takotsubo cardiomyopathy following coil embolisation of collateral arteries and subsequently underwent successful Fontan completion.

We report an extremely low birth weight infant (494 g, 23 weeks + 1 day) with transient right ventricular hypertrophy, initially suspected as pulmonary atresia with ductus-dependent circulation. Patent ductus arteriosus management was complicated, requiring low-dose PGE1 and eventual surgical ligation. Serial echocardiography revealed progressive right ventricular dilation and normalisation of function, confirming transient hypertrophy. This case highlights diagnostic challenges and the importance of repeated cardiac assessment in extremely preterm infants.

Objective: This study aimed to evaluate the results of interstage home monitoring program and its impact on outcomes after initial univentricular palliation.

Methods: Patients with univentricular hearts who survived the initial palliation and were discharged from the hospital between 2013 and 2022 were included. The interstage outcomes in patients who received home monitoring program were evaluated, and survival was compared with those who did not.

Results: Among 207 patients included, initial palliation consisted of 117 Norwood procedures, 58 aortopulmonary shunts, 28 ductal stents, and 7 pulmonary artery bandings. Home monitoring program was feasible in 187 (90.3%) patients. Survival after hospital discharge was higher in patients with home monitoring program compared to those without (93.0 vs. 82.9% at 180 days, p = 0.012). In 187 home monitoring program patients, events occurred in 98 patients (52.4%), including 77 low oxygen saturations (41.2%), 22 infections (11.7%), 10 stagnations of weight gains (5.3%), 6 hypoxaemic attacks (3.2%), and 4 side effects of vaccinations (2.1%). Additionally, 62 patients (37.1%) needed an unplanned hospital readmission. They were more frequent after ductal stent than other procedures (22.6 vs. 10.5%, p = 0.034) and had a lower weight at the time of bidirectional cavopulmonary shunt (5.0 vs. 5.4 kg, p = 0.021). Among them, 32 patients (47.1%) needed catheter interventions, and 10 (14.7%) underwent additional surgical procedures prior to bidirectional cavopulmonary shunt. Survival after bidirectional cavopulmonary shunt was similar between the groups (94.4 vs. 96.6% at 360 days, p = 0.807).

Conclusions: Home monitoring program improved interstage survival between stages I and II. However, unanticipated readmissions were frequent during this period, and various catheter and surgical interventions were mandatory before bidirectional cavopulmonary shunt.

Acute myocardial infarction is rare in paediatric patients, with limited published data on incidence, diagnosis, management strategies, and outcomes. A systematic retrospective review was conducted on patients aged 1 day to 21 years who presented with concerns for ST-elevation myocardial infarction at a paediatric institution and an affiliated adult hospital (1/2013-12/2023). Out of 965 screened patients, 13 met the inclusion criteria. The cohort had a mean age of 15 years (standard deviation ± 4 years), with the youngest patient being 11 months old. The study population consisted of 12 males (92%) and 1 female (8%), with ethnic distribution as follows: Caucasian (69%), African American (15%), and Hispanic (15%). An electrocardiogram with ST-segment changes was observed in all patients. Echocardiography revealed a normal ejection fraction in 54% of patients. Most patients (54%) had CHD, with three patients (23%) being undiagnosed at the time of the initial presentation. Seven patients (54%) underwent cardiac catheterization at the adult hospital, while 2 patients (15%) had coronary evaluation at the paediatric facility. One percutaneous catheter intervention was performed in a patient with known coronary stenosis at the paediatric facility. Three patients (23%), all with congenital heart disease, had cardiac arrest during cardiac catheterisation, resulting in extracorporeal membrane oxygenation cannulation. These findings suggest evaluation of pediatric patients presenting with ST-segment elevation can be performed at a pediatric center to rule out congenital heart disease, especially prior to referral to an adult facility for coronary intervention.

Echocardiography and computed tomographic angiography in a three-month-old boy confirmed a right coronary artery fistula and a right coronary artery aneurysm. The patient was successfully operated via right axillary thoracotomy approach.

Cardiovascular MRI with magnetic resonance angiography supports clinical indication for transcatheter pulmonary valve replacement. We aimed to: (1) assess the feasibility of generating virtual right ventricular outflow tract models directly from magnetic resonance angiography datasets and (2) compare transcatheter pulmonary valve replacement candidacy based on visualisation of anatomic and valve models in virtual reality to outcomes and dispositions suggested by industry fit analysis derived from cardiac CT. Patients with native or surgically palliated right ventricular outflow tracts considered for transcatheter pulmonary valve replacement with temporally related magnetic resonance angiography and cardiac CT were included. Magnetic resonance angiography models were generated using commercial software; virtual valves were created using industry dimensions. A blinded interventional cardiologist determined pulmonary valve replacement candidacy using virtual reality review. A total of 16 patients (N = 7 males, 44%) with a median age 15.5 years (interquartile range [IQR] 13.9, 17.8) were identified. Median time for model generation was 20.6 minutes (IQR 18.5, 22.0). 11/16 (69%) patients passed industry screening fit analysis; 14/16 (88%) ultimately underwent transcatheter pulmonary valve replacement. Four patients who passed virtual reality screening failed industry screening but underwent successful transcatheter pulmonary valve replacement. One patient passed virtual reality and industry screening but did not undergo transcatheter pulmonary valve replacement. One patient passed virtual reality screening but failed industry screening and was not offered transcatheter pulmonary valve replacement. In conclusion, generating virtual models from clinical magnetic resonance angiography datasets is feasible. Modelling may help evaluate transcatheter pulmonary valve replacement candidacy, but must be used in conjunction with other data.

Middle aortic syndrome is a rare vascular disorder characterised by segmental narrowing of the distal thoracic aorta and/or proximal abdominal aorta, often accompanied by renal artery involvement. The condition may arise from genetic abnormalities, acquired factors, or idiopathic causes. In this study, we report the case of a 9-year-old boy who was found to have hypertension during a routine school health examination. Computed tomography angiography confirmed the diagnosis of middle aortic syndrome, revealing long-segment stenosis of the distal thoracic aorta. Following a comprehensive assessment, the patient underwent descending aortic reconstruction with a 14 mm Intergard knitted vascular graft. At the 6-month follow-up, imaging demonstrated satisfactory graft patency, and the patient's blood pressure remained well-controlled without the need for antihypertensive medications.

Objective: Right ventricular outflow tract reconstruction frequently leads to long-term pulmonary failure and ventricular dilation. This study aims to investigate the dynamics of matrix metalloproteinase-2, matrix metalloproteinase-9, and procollagen type-1 N-terminal propeptide in predicting postoperative remodelling and to evaluate the "protective" potential of restrictive right ventricular physiology.

Methods: This prospective comparative cohort study included 20 patients undergoing right ventricular outflow tract reconstruction (Research Group) and 20 patients undergoing atrial or ventricular septal defect repair via transatrial approach (Control Group). Biomarker levels were measured using ELISA at preoperative, early postoperative (24-48 hours), and 6-month intervals. Right ventricular geometry was assessed with cross-sectional snapshots at 6 months and 10 years. Right ventricular dilation was defined as right ventricular end-diastolic volume index > 150 ml/m² or diameter z-score > 2, while preserved geometry was defined as right ventricular end-diastolic volume index < 100 ml/m² or diameter z-score < 1.5.

Results: Early postoperative biomarker increases were similar (p = 0.310) but differed significantly at 6 months. In the study group, matrix metalloproteinase-2 (p < 0.001) and procollagen type-1 N-terminal propeptide (p = 0.001) levels remained significantly elevated, independent of pulmonary regurgitation severity (p > 0.05). Ten years later, restrictive physiology was observed in 10 patients (50%), representing a progression compared to the 8 patients identified at 6 months.

Conclusion: Persistent elevation of matrix metalloproteinase-2 and procollagen type-1 N-terminal propeptide reflects an active "remodeling" process. Restrictive physiology is associated with the "Biological Banding" mechanism. By increasing myocardial stiffness, this adaptive process is linked to protecting the right ventricle from irreversible dilation under chronic volume overload.

Background and objectives: Burnout and adverse mental health outcomes are increasingly reported by health professionals, affecting work engagement or collaboration, with negative effects on staff retention and healthcare quality. This cross-sectional study assessed the prevalence and correlates of work-related emotional exhaustion among health professionals in paediatric cardiac care.

Methods: Health professionals (153 nurses, 37 medical doctors, 22 allied and mental health professionals, 17 research/administrative staff; 55% response rate, 85% women) at a large quaternary paediatric hospital in Australia completed validated measures within the WithCare Health Professional Survey (June 2020-February 2021). Emotional exhaustion, depersonalisation, and personal accomplishment at work were measured using the Maslach Burnout Inventory. Hierarchical linear regression was used to identify correlates of tested emotional exhaustion, with calculation of 95% confidence intervals (CI).

Results: Two-thirds (68%) of participants endorsed feeling "used up at the end of the workday," while 65% felt "emotionally drained from work" at least a few times a month. Correlates of emotional exhaustion included higher anxiety (ß = 1.41, CI: 0.46, 2.35), greater avoidance-based coping (ß = 4.15, CI: 0.22,8.08), greater work-family conflict (ß = 0.55, CI: 0.38, 0.71), lower compassion satisfaction (ß = -0.55, CI: -0.81, -0.30), and lower approach-based coping (e.g., positive reframing or acceptance, ß = -3.44, CI: -6.24, -0.65). Demographics, clinical role characteristics, physical health, and psychosocial factors accounted for 62% of the variance in emotional exhaustion (p < 0.0001).

Conclusions: Health professionals providing paediatric cardiac care report emotional exhaustion, which can adversely affect both personal and professional well-being. Identification of correlates can inform the design of targeted initiatives to address mental health needs.