Cardiology in the Young最新文献

Background: Ventricular perforation is a serious complication of percutaneous myocardial biopsy. Accurate orientation of the biopsy forceps toward the ventricular septum under fluoroscopy is essential to minimise this risk. However, in paediatric patients, variations in ventricular septal orientation complicate proper forceps alignment. No previous studies have addressed this variability in children.

Methods: We retrospectively analysed paediatric patients who underwent myocardial biopsy between January 2019 and June 2023. The angle of the ventricular septum relative to the sagittal plane was measured using cardiac CT. Biopsies were performed under fluoroscopic guidance, with individualised angles adjusted to align with the septum. Fluoroscopic images were used to calculate the angle between the biopsy forceps and the septum and the combined angle relative to the sagittal plane.

Results: Sixteen patients underwent a total of 85 biopsies; only the first biopsy per patient was included in the analysis. The mean angle of the septum was 54.3° (range: 30.0-75.0°), the mean angle between forceps and septum was 21.3° (range: 3.5-53.4°), and the combined angle relative to the sagittal plane was 75.6° (range: 45.0-115.7°). No cases of ventricular perforation were observed.

Conclusion: Ventricular septal orientation varies significantly in paediatric patients. Individualised adjustment of fluoroscopic angles based on pre-procedural CT measurements allows accurate positioning of biopsy forceps and may reduce the risk of severe complications.

Background: Late complications following surgical repair of aortic coarctation may include hypertension, aortic valve disease, coronary artery disease, aneurysm formation, and recoarctation. However, preprocedural aneurysmal dilatation is rarely reported.

Case presentation: We report the case of an 11-year-old male with severe aortic coarctation and a giant aneurysm identified prior to any surgical or interventional treatment. Takayasu's arteritis was ruled out, and the patient underwent successful percutaneous placement of a covered stent. Post-procedure, he showed no residual gradient or aneurysmal lesion.

Conclusion: This case highlights a rare presentation of aneurysmal dilatation occurring prior to any intervention for aortic coarctation and supports the efficacy of covered stent placement as a safe and effective therapeutic option.

Objective: To examine the accuracy of pulse oximetry in neonates by race and ethnicity.

Study design: We performed a single-centre retrospective cross-sectional study in neonates aged ≤ 7 days at Children's Healthcare of Atlanta in the neonatal and cardiac ICU between 2010 and 2022. Eligible patients had pulse oximetry with arterial oxygen saturation measurements taken within 10 minutes. We evaluated the mean saturation bias, accuracy root mean square, and the frequency of occult hypoxaemia by race and ethnicity.

Results: Of 1645 infants, there were 639 non-Hispanic Black infants (38.8%), 651 non-Hispanic White infants (39.6%), and 215 Hispanic infants (13.1%). The majority had no CHD (71.4%), required respiratory support (81.1%), and had oxygen saturation levels = > 90% (87.6%). The mean pulse oximetry and arterial oxygen measurement time difference was 0.8 minutes. The mean bias and accuracy root mean square were 8.7% and 13.7%, respectively, with no significant difference between the groups (p = 0.91). Occult hypoxaemia was found in 20.5% of infants. Compared to White infants, there were no differences in likelihood of occult hypoxaemia for either Black (adjusted prevalence ratio 1.13 (0.92-1.4), p = 0.24) or Hispanic (1.06 (0.77-1.45, p = 0.72) infants.

Conclusion: There was not a systemic discrepancy in pulse oximetry between racial and ethnic groups as previously described in older children and adults. However, pulse oximetry significantly overestimated arterial oxygen saturation. Future prospective studies that objectively measure skin pigmentation may be able to overcome some of the limitations of our study.

Primary pulmonary malignancies in single-ventricle patients are rare. Improved survival following surgical palliation has resulted in a growing Fontan population whose malignancy risk remains undefined. Standard oncologic approaches, especially for adenocarcinoma of the lung, need to be considered within the context of Fontan physiology. We present our management of primary lung adenocarcinoma in an adult with tricuspid atresia status postlateral tunnel Fontan palliation, highlighting considerations for this unique scenario.

Background: Noonan syndrome is characterised by typical facial features, short stature, CHDs, and other comorbidities, which are caused by germline mutations in genes coding for components of the Ras-mitogen-activated protein kinase pathway. Noonan syndrome is an inherited disease involving multiple systems, but ventricular arrhythmia in Noonan syndrome is rarely reported.

Case summary: Here we report a 15-year-old patient with leucine zipperlike transcription regulator 1-associated Noonan syndrome, who has CHD (left ventricular hypertrophy with left ventricular outflow tract obstruction), ventricular arrhythmia, bundle branch block, pectus excavatum, costal eversion, scoliosis, myopia, growth retardation, hearing loss, chest tightness, and fatigue. Chest tightness and fatigue are the main reasons for admission of the patient. The patient was treated with spironolactone, empagliflozin, tolasemide, potassium chloride, and bisoprolol. One month after treatment, the patient has no more chest tightness or fatigue. Genetic testing revealed that the patient had a novel heterozygous variant c.313delT (p.trp105Glyfs * 42) mutation in the leucine zipperlike transcription regulator 1. We provide a review of the literature of leucine zipperlike transcription regulator 1 mutations and find that ventricular arrhythmias have been reported in leucine zipperlike transcription regulator 1-related Noonan syndrome.

Discussion: Our findings expand on the Noonan syndrome phenotype and suggest that mutations in the leucine zipperlike transcription regulator 1 gene are involved in ventricular arrhythmia.

A 15-year-old female presented with exertional chest pain and near-syncope. Imaging revealed a large left sinus of Valsalva aneurysm compressing the left coronary artery. She underwent successful surgical repair. Intraoperative and pathologic findings confirmed Takayasu arteritis. This case highlights a rare aetiology of cardiac chest pain in adolescents and underscores the importance of evaluating for an underlying vasculitis when a sinus of Valsalva aneurysm is identified.

As staged palliative surgery for patients with hypoplastic left heart syndrome ages to greater than 50 years in practice, providers are increasingly faced with the challenges of managing patients with "failed Fontan" physiology. In this brief case report, we discuss different ways paediatric palliative care can support this population.

Background: Impaired muscle function, aerobic capacity, and fatigue are common in individuals with Fontan circulation. Knowledge regarding the effects of strength training in this population is limited. Therefore, the study aimed to investigate the effects of strength training on dynamic muscle function, aerobic capacity, and fatigue in adults with Fontan circulation compared to matched controls.

Methods: In this pilot non-randomised controlled trial, nine patients with Fontan circulation (median age 28.9 years [IQR: 23.4-35.0], 44.4% women) and nine age- and sex-matched controls completed a 10-week strength training intervention. Dynamic muscle function was assessed through shoulder flexion, heel rise, elbow flexion, and knee extension tests. Aerobic capacity was evaluated using cardiopulmonary exercise testing, and fatigue using the questionnaire Multidimensional Fatigue Inventory. All assessments were conducted pre- and post-intervention. Within-group changes were analysed using the Wilcoxon signed rank test and between-group differences using the Mann-Whitney U test.

Results: Patients showed improvements in all muscle function tests post-intervention (shoulder flexions 39.3% [IQR: 18.9-69.7], p = 0.008; heel rise 26.7% [IQR:17.5-58.1], p = 0.008; elbow flexions 57.1% [IQR: 50.0-173.8], p = 0.007; knee extensions 66.7% [24.3-92.9], p = 0.008). The improvements were at comparable levels to controls. Only controls reported reduced fatigue (-19.4% [IQR: -28.7, -10.5], p = 0.01), while patients showed no change (-5.9% [IQR: -25.5, 3.2], p = 0.1). Aerobic capacity remained unchanged. No severe adverse events occurred.

Conclusion: Strength training is safe and improves dynamic muscle function in patients with Fontan circulation, with changes comparable to those of healthy controls. However, the effect of strength training on fatigue and aerobic capacity requires further investigation.ClinicalTrials.gov, ID: NCT05454254, https://clinicaltrials.gov.

Background: Dynamic, data-driven predictors of perioperative mortality risks in preterm/early-term neonates with CHD undergoing cardiac surgery in the first 24 months of life are limited.

Aims: To identify risk factors of mortality in the first 24 months of life for pre/early-term neonates with CHD.

Methods: Retrospective cohort study of patients <39 weeks of gestation undergoing cardiac surgery within 24 months of life from 2013-2020 at a tertiary care centre. Independent risk factors of mortality within 24 months of life were determined by multivariable Cox regression analysis.

Results: Among the 205 neonates, 33 (16.1%) died within 24 months. Multivariable analysis revealed that high-frequency ventilation (hazard ratio = 5.15; 95% confidence interval): 2.51, 10.6; p < 0.001), extracorporeal membrane oxygenation support (hazard ratio = 5.77; 95% confidence interval: 2.67, 12.5; p < 0.001), and CHD with a palliated circulation (hazard ratio = 6.07; 95% confidence interval: 2.84, 13; p < 0.001) were significant independent risk factors of mortality at any time during the index hospitalisation or the first 24 months of life.

Conclusions: Identifying and re-evaluating risk factors of mortality for preterm/early-term neonates with CHD at any time during the index hospitalisation or the first 24 months of life may guide resource allocation and therapeutic interventions.Trial registration number and date of registration: IRB P00028833 5/2/2018. Retrospectively registered.

This case report details the diagnosis and surgical management of a 7 cm aortic root aneurysm occurring in a 12-year-old with cutis laxa type B, an extremely rare connective tissue disorder. Our patient underwent a Bentall procedure as primary treatment for the aneurysm and had a successful interventional treatment of a postoperative pseudoaneurysm.