Cardiology in the Young最新文献

Aims: This study explored the prospective use of the Ages and Stages Questionnaires-3 in follow-up after cardiac surgery.

Materials and method: For children undergoing cardiac surgery at 5 United Kingdom centres, the Ages and Stages Questionnaires-3 were administered 6 months and 2 years later, with an outcome based on pre-defined cut-points: Red = 1 or more domain scores >2 standard deviations below the normative mean, Amber = 1 or more domain scores 1-2 standard deviations below the normal range based on the manual, Green = scores within the normal range based on the manual.

Results: From a cohort of 554 children <60 months old at surgery, 306 participated in the postoperative assessment: 117 (38.3%) were scored as Green, 57 (18.6%) as Amber, and 132 (43.1%) as Red. Children aged 6 months at first assessment (neonatal surgery) were likely to score Red (113/124, 85.6%) compared to older age groups (n = 32/182, 17.6%). Considering risk factors of congenital heart complexity, univentricular status, congenital comorbidity, and child age in a logistic regression model for the outcome of Ages and Stages score Red, only younger age was significant (p < 0.001). 87 children had surgery in infancy and were reassessed as toddlers. Of these, 43 (49.2%) improved, 30 (34.5%) stayed the same, and 13 (16.1%) worsened. Improved scores were predominantly in those who had a first assessment at 6 months old.

Discussion: The Ages and Stages Questionnaires results are most challenging to interpret in young babies of 6 months old who are affected by complex CHD.

Purpose: High takeoff of the right coronary artery suspected by echocardiography is widely considered a normal variant. However, in our experience, some patients initially thought to have a high takeoff of the coronary artery were later found to have an anomalous coronary origin with high-risk features. The aim of this study was to test the hypothesis that high takeoff of the right coronary artery suspected by echocardiography may indicate the presence of an anomalous coronary artery lesion with an intramural course requiring further investigation.

Methods: A retrospective chart review was performed of patients evaluated at the University of Florida Congenital Heart Center from January 2010 through September 2015. Charts of all 62 patients who were noted to have an anomalous coronary artery or concern for an anomalous coronary artery were reviewed to identify those who were initially identified as having simply a high takeoff of the right coronary artery by initial echocardiogram. A total of 24 patients met these criteria.

Results: Out of 24 patients identified as having high takeoff of the right coronary artery on their initial echocardiogram, 20 had confirmatory computerized tomographic angiography. On review of these patients, 9 had a right coronary origin from the left. This included 3 patients with an anomalous right coronary artery from the left coronary sinus and 6 with an anomalous right coronary artery origin just above the left coronary sinus. Six of these 9 patients had an intramural course. The remaining patients had high takeoff above the right coronary sinus or normal coronary origins.Additionally, on review of all patients with computerized tomographic angiographic confirmation of high takeoff of the coronary artery, those with high takeoff above the left coronary sinus were more likely to have an intramural course (6 out of 9). Meanwhile, none of the 6 patients with high takeoff above the right coronary sinus confirmed by computerized tomographic angiography had an intramural course.

Conclusion: Accurate identification of the coronary origin and course of the anomalous coronary artery is difficult by echocardiogram. Correct diagnosis of origin and course is important for appropriate risk stratification and treatment decisions. Therefore, patients with high takeoff of the right coronary artery suspected by echocardiography should undergo additional evaluation to assess for the presence of a potentially malignant course.

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.

Background: In North America, less than 30% of children with complex CHD receive recommended follow-up for neurodevelopmental and psychosocial care. While rates of follow-up care at surgical centres have been described, little is known about similar services outside of surgical centres.

Methods: This cohort study used Maine Health Data Organization's All Payer Claims Data from 2015 to 2019 to identify developmental and psychosocial-related encounters received by children 0-18 years of age with complex CHD. Encounters were classified as developmental, psychological, and neuropsychological testing, mental health assessment interventions, and health and behaviour assessments and interventions. We analysed the association of demographic and clinical characteristics of children and the receipt of any encounter.

Results: Of 799 unique children with complex CHD (57% male, 56% Medicaid, and 64% rural), 185 (23%) had at least one developmental or psychosocial encounter. Only 13 children (1.6%) received such care at a surgical centre. Developmental testing took place at a mix of community clinics/private practices (39%), state-based programmes (31%), and hospital-affiliated clinics (28%) with most encounters billing Medicaid (86%). Health and behavioural assessments occurred exclusively at hospital-affiliated clinics, predominately with Medicaid claims (82%). Encounters for mental health interventions, however, occurred in mostly community clinics/private practices (80%) with the majority of encounters billing commercial insurance (64%).

Conclusion: Children with complex CHD in Maine access developmental and psychosocial services in locations beyond surgical centres. To better support the neurodevelopmental outcomes of their patients, CHD centres should build partnerships with these external providers.

Hypertensive heart disease and hypertrophic cardiomyopathy both lead to left ventricular hypertrophy despite differing in aetiology. Elucidating the correct aetiology of the presenting hypertrophy can be a challenge for clinicians, especially in patients with overlapping risk factors. Furthermore, drugs typically used to combat hypertensive heart disease may be contraindicated for the treatment of hypertrophic cardiomyopathy, making the correct diagnosis imperative. In this review, we discuss characteristics of both hypertensive heart disease and hypertrophic cardiomyopathy that may enable clinicians to discriminate the two as causes of left ventricular hypertrophy. We summarise the current literature, which is primarily focused on adult populations, containing discriminative techniques available via diagnostic modalities such as electrocardiography, echocardiography, and cardiac MRI, noting strategies yet to be applied in paediatric populations. Finally, we review pharmacotherapy strategies for each disease with regard to pathophysiology.

Objectives: The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Methods: Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed.

Results: Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16-26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4-42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m²) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test.

Conclusions: In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

Undetected respiratory infections may adversely affect the intrapulmonary resistance after Stage 2 or Stage 3 Fontan palliation. A few studies describe a higher risk for viral pneumonia during respiratory virus season, but none of them have focused on the effect of symptomatic viral pneumonia on in-hospital clinical course after bidirectional Glenn shunt. We analysed 77 patients who underwent bidirectional Glenn shunt surgery. Six patients were detected with pneumonia and proof of viral ribonucleic acid in tracheal mucus in the very early postoperative time. We compared them retrospectively to the remaining 71 patients regarding preoperative inflammatory signs, mortality, paediatric ICU length of stay, and ventilation time. The infection rate was not seasonal dependent. Ventilation time was significantly elongated in the pneumonia group (558 h ± 634 vs. 8.7 h ± 1.9; p < 0.0001) and so was the paediatric ICU length of stay (29 days ± 26 vs. 3 days±1; p = 0.007). Significantly more patients in the pneumonia group required extracorporeal cardiac life support postoperatively. The mortality was significantly increased in patients with pneumonia. Even subclinical viral pneumonia may cause ventilation-to-perfusion mismatch by raising intrapulmonary resistance. Recorded parameters of postoperative paediatric ICU therapy showed a significant impact of a viral pneumonia on patients after bidirectional Glenn shunt. The respiratory syncytial virus vaccination does not protect these patients from infection with other respiratory viruses. The focus should be put on preoperative diagnosis of pulmonary infections in the vulnerable group of patients with univentricular hearts.

A 16-year-old male with newly diagnosed granulomatosis with polyangiitis presented to the emergency room with chest pain. He was found to have a myocardial infarction involving the right coronary artery and the left circumflex artery. He underwent mechanical thrombectomy and stent placement without significant sequelae. This is a rare complication associated with granulomatosis with polyangiitis.

Several metrics exist for matching the size of donor to recipient in paediatric cardiac transplantation. Different centres employ different metrics for size-matching to determine the viability of donor hearts. Inconsistent evidence exists, with limited consensus as to the metric for size-matching that is most predictive of outcomes after cardiac transplantation. Furthermore, any metric must function within the tight timeline available for the assessment of the suitability of the donor. At the time of the writing of this paper, the most commonly used metric for size-matching in paediatric cardiac transplantation is the donor-to-recipient body weight ratio. In this article, we review published literature evaluating commonly used metrics for size-matching in paediatric cardiac transplantation, including weight, height, body surface area, and imaging parameters.

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.