Cardiology in the Young最新文献

Background: Endomyocardial biopsy remains the gold standard for cardiac cellular rejection surveillance after heart transplantation. We studied a novel non-invasive index of left ventricular relaxation to detect cardiac cellular rejection in paediatric heart transplant patients.

Methods: This is a single-centre retrospective study of paediatric heart transplant patients who underwent endomyocardial biopsy from June 2014 to September 2021. Left ventricular relaxation index was calculated as the sum of diastolic tissue Doppler imaging velocities (E) of the left ventricular lateral, septal, and posterior walls divided by the percentage of the left ventricular posterior wall thinning by M-mode. Statistical analysis included t-tests and Mann-Whitney tests to compare means and medians between treatment and non-treatment groups. We used the cut-off with the maximum Youden index to compare the sensitivity and specificity of left ventricular relaxation index to detect rejection.

Results: The study included 65 patients who underwent 246 cardiac catheterizations and endomyocardial biopsies. Out of 246, 192 procedures were included and 54 were excluded due to recent transplants or lack of echocardiographic data. A total of 114 demonstrated Grade 0R, 68 Grade 1R, 8 Grade 2R, and 2 Grade 3R allograft rejection. The difference in mean left ventricular relaxation index between treatment versus non-treatment groups (2R, 3R vs. 0R, 1R) was not statistically significant (p = 0.917). A left ventricular relaxation index cut-off of 0.73 had the highest Youden index with good sensitivity (100%) and poor specificity (23%) for detecting rejections with grades 2R and 3R.

Conclusion: Left ventricular relaxation index, a novel index of left ventricular relaxation, was not a sensitive or specific predictor of cardiac cellular rejection in paediatric heart transplants.

The giant coronary artery aneurysm is a very rare finding (0.02% of the general population), in which the right coronary artery is most often affected by aneurysms. Herein, we present a rare case of a giant left coronary artery aneurysm involving multiple major coronary arteries and compressing the cardiac cavity. The giant coronary artery aneurysm was opened and explored under cardiopulmonary bypass, and many mixed thrombi and calcified tissue were exposed. The patient was discharged uneventfully 7 days after surgery. The best management strategy at present is based on case reports, small case series, and personal experience. Treatment must be individualized according to the aetiology, location, symptoms, size, disease progression, the existence of infection, and the degree of any coexisting atherosclerosis. Surgery is a good alternative, particularly if a giant coronary artery aneurysm has a high risk of rupture and compressing the cardiac cavity. Even today, the treatment strategy is still open to debate and a clear evidence-based management strategy has not been established.

Background: Coronary artery lesions are the most severe complications of Kawasaki disease. Despite recent advances, evidence of the association between risk factors and coronary artery lesion is lacking. In this study, we demonstrated the potential clinical indicators that could assist to evaluate the prevalence of coronary artery lesion among paediatric patients with Kawasaki disease.

Methods: We retrospectively enrolled 260 paediatric patients with Kawasaki disease. Patients with coronary dilation, coronary aneurysm, and intimal thickening of coronary arteries were included in this study. Medical records of each patient were collected. Logistic regression analysis was performed to explore risk factors and the occurrence of coronary artery lesion in patients with Kawasaki disease.

Results: Respectively, 64 (24.6%), 39 (15%), and 56 patients (21.5%) of the participants had coronary dilation, coronary aneurysm, and intimal thickening of coronary arteries. Univariate analysis revealed that age, gender, duration of fever, time of initial use of intravenous immunoglobulin, erythrocyte sedimentation rate, white blood cell counts, time of platelet increase, the maximum value of platelet, albumin, and immunoglobulin G level was associated with coronary artery lesion. In multivariable logistic analysis, those younger and mainly males were associated with all three outcomes of coronary artery lesion, lower serum albumin levels, and later initial use of intravenous immunoglobulin were linked to a higher risk of coronary dilation and coronary aneurysm.

Conclusions: The potential risk factors that could be used to estimate the occurrence of coronary artery lesion in Kawasaki disease patients are young age, male, lower serum albumin lever, and later initial use of intravenous immunoglobulin. However, long-term follow-up and multi-centre studies are required to verify our findings in the future.

This case is a 4-month-old patient with Kawasaki disease who showed coronary artery lesions after a disease relapse. Following the guidelines from the Randomised Controlled Trial to Assess Immunoglobulin plus Steroid Efficacy, the patient initially saw an improvement in symptoms. However, a relapse occurred despite low levels of C-reactive protein, leading to significant coronary artery growth. Treatment with a third dose of intravenous immunoglobulin and cyclosporine led to a slow reduction in the size of the coronary artery abnormalities. This case highlights the difficulty in monitoring Kawasaki disease progression through clinical symptoms and C-reactive protein levels alone. We also emphasise the necessity of echocardiographic monitoring in patients receiving anti-inflammatory treatments, including steroids, because coronary artery problems can arise or continue without the usual signs of Kawasaki disease or increased C-reactive protein levels.

A six-month-old boy with Down syndrome, complete atrioventricular septal defect with intact atrial septum, and moderate left-sided atrioventricular valve regurgitation underwent surgery. The presence of a common atrioventricular junction could not be diagnosed preoperatively. Postoperative left-sided atrioventricular valve regurgitation remained mild by complete cleft closure, direct closure of incised atrial septum, and patch augmentation of the underside of septum primum.

Malnutrition is common among paediatric heart failure patients, with nutritional rehabilitation critical for survival and optimal health outcomes. Ventricular assist devices have been associated with improved growth, though additional nutritional support may be needed. Here, we report the use of human milk-based fortifiers to avoid severe malnutrition in a neonate supported on a ventricular assist device until transplantation.

The soft nitinol KONAR-MF™ ventricular septal defect (VSD) Multifunctional Occluder (MFO) device is increasingly used for transcatheter perimembranous ventricular septal defect closure. We report for the first time a case of delayed complete atrioventricular block with pacemaker implantation 20 months post-procedure. Through a systematic review, the overall rate of persistent complete atrioventricular block was 0.6% with this device, but follow-up duration was limited.

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.

Background: Adult Kawasaki patients may require intervention for occlusive coronary artery disease. Some adverse effects of first-generation drug-eluting stent implantation with sirolimus have been reported in this population.

Methods: A total of nine lesions in eight (seven males, one female) patients who underwent stent implantations in this population between 2000 and 2021 were reviewed.

Results: The age at stent implantation ranged from 31 to 47 years, with a median of 37 years. There were six lesions treated by primary percutaneous transluminal coronary interventions, and three by elective procedures. A coronary aneurysm was found in two lesions, and coronary artery calcification was found in all culprit lesions. The numbers of everolimus-eluting stents, sirolimus-eluting stents and bare metal stents were six, two, and one, respectively. As anti- thrombotic therapy, aspirin, clopidogrel, and prasugrel were given to four, three, and one, respectively. Warfarin was given to five patients. The follow-up ranged from 2 to 12 years, with a median of 4 years. Follow-up angiograms were performed for eight lesions, at 2 to 38 months, with a median of 11 months. The patency of the target vessel was confirmed in all eight vessels. Slight malapposition, and peri-stent contrast staining were found in two lesions each.

Conclusion: Acute coronary syndrome due to coronary artery lesions caused by Kawasaki disease occurred, even in lesions without any apparent coronary artery aneurysms. In our study, we show safe and efficacious placement of second-generation stent without adverse effects during the short-term follow-up, but long-term follow-up is needed to determine the efficacy and complication.

Ebstein's anomaly is rarely accompanied by coarctation of the aorta, although patients with Ebstein's anomaly have a relatively small left ventricle. Here, we report a rare case of Ebstein's anomaly with coarctation of the aorta and a bicuspid aortic valve. We compared the foetal echocardiographic parameters of five previous cases with Ebstein's anomaly without left heart obstruction to explore the association between left ventricular volume, Ebstein's anomaly severity, and left heart obstruction.