Cardiology in the Young最新文献

Cardiac involvement of metastatic osteosarcoma is exceedingly rare and carries a dismal prognosis. Documentation of the surgical management of cardiac metastatic osteosarcoma is limited. We describe the successful resection of a ∼ 3.9 x 2.2 cm mass involving the right atrioventricular groove and right ventricular inferolateral wall, granting our patient 12 months of excellent cardiac function in the face of advanced metastatic disease.

Pulmonary arterial hypertension remains a progressive, life-threatening condition despite advances in medical treatments. We report the first case of the interventional creation of a reverse Potts shunt by stenting a closed ductus arteriosus in a four-year-old child with right ventricular failure due to suprasystemic pulmonary arterial hypertension associated with Von Hippel-Lindau disease, unresponsive to triple anti-pulmonary arterial hypertension therapy. Following the procedure, the patient's clinical status and echocardiographic systolic and diastolic right ventricular function improved.

Background: The American College of Cardiology has published clinical practice algorithms for common congenital heart lesions, including atrial septal defect, patent ductus arteriosus, valvar pulmonary stenosis, aortic coarctation, and ventricular septal defect. The purpose of this study was to define the current practice patterns in the management of these lesions and describe the impact of departure from these recommendations.

Methods: This was a retrospective analysis of the most recent 100 outpatient appointments for each lesion at our centre. Electronic medical records were queried to determine whether the scheduling, testing, and follow-up plan for each appointment were consistent with the published algorithms.

Results: A total of 500 visits were evaluated (150 new visits; 350 follow-up visits); 32% (n = 162) of encounters did not receive appropriate testing, 37% (n = 186) departed from recommended follow-up plans, and of the 350 follow-up visits, 45% (n = 156) departed from scheduling guidelines. Impact of these departures was quantified in reference to over- or under-expenditure of clinical resources. Of the aberrant testing encounters, 60% (n = 97) saw too few tests. Of the deviant follow-up plans created, 74% (n = 138) brought patients back to clinic too soon.

Conclusion: This study explores the deviation between current practice patterns and published clinical care guidelines. There is considerable variation across domain of analysis, diagnosis, and encounter type, resulting in uneven resource utilisation. Standardisation of care in these areas will improve utilisation and can be a starting point for improvement work.

Chylothorax, a postoperative complication of CHD surgery, involves chyle accumulation in the pleural cavity, posing challenges in diagnosis and management. This retrospective study investigates the prevalence, aetiology, management, and outcomes of postoperative chylothorax in paediatric patients undergoing cardiac corrective surgery at a tertiary care centre over 15 years. Medical records of paediatric patients who underwent cardiothoracic surgery at the Children's Heart Center at the American University of Beirut Medical Center between 2007 and 2022 were retrospectively reviewed. Data collection included demographic characteristics, blood parameters, chylous fluid characteristics, diagnostic criteria, treatment modalities, and hospitalisation details. Ethical approval was obtained, and descriptive statistics were employed using SAS 9.4. Among 2,997 children who underwent cardiothoracic surgery, nineteen cases of postoperative chylothorax were identified. The majority were females (63.2%) with a median age of 9 months. Glenn, Fontan, and Blalock-Taussig shunt-related surgeries were the most common operations associated with chylothorax. Single ventricle physiology was the predominant CHD observed (58%). Diagnosis relied primarily on clinical presentation, imaging studies, and triglyceride levels in pleural fluid. Treatment options included conservative dietary modifications, medical therapy such as octreotide, and surgical intervention if necessary. No mortalities were reported, and patients were adequately followed up. This study sheds light on postoperative chylothorax in paediatric cardiac patients, offering insights into its epidemiology, aetiology, clinical features, and treatment outcomes. While conservative and medical approaches effectively managed chylothorax in this group, larger studies are needed to develop standardised diagnostic and treatment protocols, improving outcomes in paediatric patients with postoperative chylothorax.

Introduction: Dysphagia is common in infants born with critical CHD. Thickened liquids are often used to treat dysphagia, but associated risks limit widespread use among feeding specialists. This survey aims to assess dysphagia treatment patterns and thickened liquid use across paediatric cardiac surgical centres.

Methods: A 24-question, cross-sectional survey. Convenience and snowball sampling methods were used to engage 52 paediatric cardiac surgical centres affiliated with the Cardiac Newborn Neuroprotective Network. Descriptive statistics were used to analyse and compare responses.

Results: Twenty-six individual respondents represented 21 unique paediatric cardiac surgical centres. Most responses were from experienced, speech-language pathologists (78%) at medium size centres (88%). Ninety-three percent of responding centres used thickened liquids to treat dysphagia and 81% only after formal instrumental assessment of swallowing. Thickened oral feeding was used for single-ventricle patients by 85% versus 69% for two-ventricle patients. Barriers to recommending thickened oral feedings included the cost of thickening agents, parental non-adherence, and gastrointestinal concerns.

Conclusions: This is the first survey to report multi-institutional dysphagia treatment practice variation at United States congenital cardiac surgical centres. Thickened oral feedings are frequently used across centres in high-risk critical CHD patients but treatment benefit remains unclear. This survey highlights a broad scientific community poised to direct dysphagia research in critical CHD to address practice variation, short- and long-term impact of thickened oral feeding on feeding outcomes, and barriers to use and access of thickening agents.

Objective: Left bundle branch area pacing is a recent technique gaining rapid acceptance due to its broader target area and excellent electrical parameters. The aim of this study was to demonstrate the feasibility of left bundle branch area pacing in children and share short-term results.

Materials and methods: A retrospective study conducted at a single centre between December 2021 and April 2024 involved 19 children who underwent left bundle branch area pacing using Select Secure leads. The study included echocardiographic evaluations, pacing parameters, and follow-up outcomes.

Results: The cohort comprised 10 males and 9 females. Median age was seven years (range 2-18), and median weight was 38 kg (range 13-56). All patients had complete atrioventricular block, with seven having isolated congenital complete atrioventricular block and 12 postoperative complete atrioventricular block. In nine patients, transitioning from epicardial to endocardial pacing resulted in ventricular dysfunction due to chronic right ventricular pacing. The remaining patients received left bundle branch area pacing initially. One patient underwent implantation in a septal position close to the left bundle due to left bundle branch area pacing infeasibility. The median post-procedure QRS duration was 92 msec (range 80-117). Median R wave amplitude, threshold, and impedance values were 14.7 mV (range 13.3-16.8), 0.7 mV (range 0.5-1.1), and 728 ohms (range 640-762), respectively. Atrioventricular (DDD mode) leads were implanted in 10 patients, and ventricular leads (VVIR mode) were implanted in nine patients. Median fluoroscopy dose was 18.7 mGy (13.5-34.52).

Conclusion: Left bundle branch area pacing can be safely conducted in paediatric patients exhibiting a narrow QRS duration and stable pacing parameters.

This article presents the case of a 3-year-old boy whose primary symptom was hypoproteinemia. After evaluations at multiple hospitals, he was sequentially diagnosed with protein-losing enteropathy and constrictive pericarditis. Following pericardiectomy, his albumin levels returned to normal. Additionally, a review of several similar cases is provided, with the aim of aiding in the diagnosis of related conditions.

Introduction: Children with CHD are at increased risk for neurodevelopmental disabilities and neuropsychological impairments throughout their life span. The purpose of this report is to share our experience building a sustainable, novel, inpatient, interdisciplinary Neurocardiac Critical Care Program to mitigate risks and optimize outcomes during the ICU stay.

Material and methods: A descriptive review was chosen to identify meaningful characteristics, challenges and lessons learned related to the establishment, expansion of and sustainability of Neurocardiac Critical Care Program in a 26-bed pediatric cardiac ICU.

Results: We successfully launched, expanded, and sustained an interdisciplinary Neurocardiac Critical Care Program. Here, we share the foundation, framework, challenges, and lessons learned as we established and sustained the Neurocardiac Critical Care Program. The key elements of our program are (1) consistent engagement by pediatric neurologists in the cardiac ICU, (2) comprehensive education initiatives, (3) evidence-based clinical practice changes, and (4) quality improvement and research projects.

Discussion: The development of a pediatric Neurocardiac Critical Care Program is feasible and sustainable. This program was informed by recent research related to perioperative and psychosocial risk factors that impact brain development and neurodevelopmental outcomes in this vulnerable population. By aligning our efforts, our multidisciplinary team is helping shift the paradigm in pediatric cardiac critical care to actively manage complex heart disease, while simultaneously and proactively mitigating risks to the developing brain and family unit.

Introduction: Vitamin D is crucial for normal organ function, vascular health and exercise performance, yet its deficiency is widespread. Patients with CHD often exhibit reduced exercise capacity. Limited research exists on vitamin D in CHD.

Methods: This study investigates serum 25-hydroxy vitamin D levels in 55 adult CHD patients (median age 31 years) compared to 55 age- and gender-matched controls without cardiac disease and examines associations with exercise capacity, peripheral microvascular function, muscle strength and biventricular function in CHD. Therefore, patients underwent fingertip arterial tonometry, transthoracic echocardiography, muscle strength measurements and cardiopulmonary exercise testing.

Results: Results indicated that 93% of CHD patients and 91% of controls had 25-hydroxy vitamin D levels <30 ng/ml, with both groups showing varying values depending on the season in which the studies were conducted. No significant difference in 25-hydroxy vitamin D levels was found between patients and controls. While vitamin D levels in CHD patients did not significantly correlate with age, body mass index, blood pressure, peripheral microvascular function, high-sensitivity C-reactive protein, cholesterol levels, N-terminal-pro hormone B-type natriuretic peptide, ventricular function or muscle strength, a significant correlation was found with percent-predicted peak oxygen consumption (ρ=0.41, p = 0.005 and ρ=0.34, p = 0.02 for reference values following Wasserman and the LowLands registry, respectively), even after adjusting for season (p = 0.03 and 0.05, respectively).

Conclusions: In conclusion, vitamin D levels were similar between CHD patients and controls, but vitamin D insufficiency is common and linked to reduced exercise capacity in CHD. Further research is needed to determine whether vitamin D supplementation combined with exercise could be beneficial in CHD with vitamin D insufficiency.

This study aimed to assess Well-Being Index scores in paediatric cardiac ICU (PCICU) registered nurses and advanced practice providers. Secondary objectives included identifying factors correlating with at-risk Well-Being Index scores and exploring predictors of these scores, with attention to the impact of the coronavirus disease 2019 pandemic. A multicentre electronic survey was conducted between October 2021 and January 2022. Registered nurses and advanced practice providers working in PCICUs at US centres participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society were included.The survey included the nine-item Well-Being Index and questions about demographics and factors influencing well-being, such as coronavirus disease 2019. The Well-Being Index is a validated tool to predict workforce distress and well-being. Out of 218 participants (180 registered nurses, 38 advanced practice providers), 137 registered nurses (76%) and 15 advanced practice providers (39%) had at-risk Well-Being Index scores. A total of 61% of nurses and 34% of advanced practice providers reported an intent to leave. Intent to leave was significantly linked to lower well-being for registered nurses (p = 0.002). Leadership support reduced registered nurses' distress risk by 68% compared to no support (p = 0.04). Increased stress since coronavirus disease 2019 raised registered nurses' poor well-being risk by four times (p = 0.001). PCICU registered nurses and advanced practice providers are at risk for poor well-being, exacerbated by the pandemic. Those with poor well-being may be more likely to leave. Leadership support is vital for nurses' well-being. Further research is needed to establish baseline well-being.