Annals of Pediatric Cardiology最新文献

We present the echocardiography images in a 6 year old girl who presented with a history of scalp swelling after trivial trauma which was subsequently diagnosed as metastases from primary intracardiac germ cell tumour.

Background: Aortico right atrial tunnel (ARAT) is a rare extracardiac communication between the aorta and the right atrium with two anatomical types. A recent global review identified 59 patients.

Methods: Patients with ARAT from two centers were analyzed for their demographics, symptoms, morphology, management, and follow-up thromboprophylaxis.

Results: Among 21 patients including 8 males with a median age of 3 years (18 days-72 years) diagnosed as ARAT, 12 (57%) had posterior tunnels and 9 had anterior tunnels. Four patients had multiple exits. Eighteen tunnels were closed after arteriovenous circuit formation. Six patients (29%) weighing <10 kg presented early with heart failure. Transcatheter closure normalized the hemodynamics including in one infant after failed surgery. Two elderly patients (10%) above 60 years presented with angina and atrial fibrillation. The rest were asymptomatic. Occluders were positioned in the narrow proximal aortic end of the tunnel in all except two patients, where the distal atrial end was closed. All procedures were successful without complications. There was one late death after 1 year from subarachnoid hemorrhage. At a median follow-up of 96 months, all survivors were asymptomatic. Thromboprophylaxis with dual antiplatelets for 1-2 years followed earlier was recently changed to aspirin with Coumadin. Complete remodeling occurred when the proximal aortic end was closed, but partial persistence of the track was noted after distal closure.

Conclusions: This largest cohort of ARAT showed the safety and efficacy of transcatheter closure even in neonates. The narrow proximal aortic end should be the target for closure rather than the distal atrial end to achieve complete remodeling.

Background: Echocardiographic quantification of left ventricular (LV) volume and ejection fraction (EF) is widely used in the pediatric population. However, there is no consensus on the most accurate method of quantifying ventricular volumes and systolic function.

Purpose: The purpose of this study is to compare two commonly used echocardiographic methods for the evaluation of LV volume and quantification of EF, the five-sixth area-length (5/6 AL) and the modified biplane Simpson (BS), to cardiac magnetic resonance (CMR) imaging in children.

Methods: CMR studies were paired with echocardiograms and retrospectively analyzed in children 18 years of age and younger. Studies performed more than 3 months between modalities, patients with congenital heart disease, and patients who had changes in medication regimen between corresponding CMR and echocardiograms were excluded. LV volumes and EF were calculated using the 5/6 AL and BS methods and compared to volumes and EF measured on corresponding CMR studies. Subgroup analyses were conducted based on LV function, pathology, and weight.

Results: We retrospectively analyzed 53 CMR and corresponding echocardiogram studies (23 studies for myocarditis and 30 studies for cardiomyopathy) in 46 patients. LVEF derived by both echocardiographic methods showed a good correlation to CMR (5/6 AL r = 0.85 and BS r = 0.82). However, both echocardiographic methods overestimated LVEF and underestimated LV volumes when compared to CMR.

Conclusion: Left ventricular volumes and EF, as measured by echocardiography, correlate well with CMR measurements. Echocardiography underestimates LV systolic and diastolic volumes and overestimates LVEF. While echocardiography is a good surrogate for estimating LVEF, CMR should be considered in patients for whom accurate measurements are needed for critical clinical decision-making.

Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy associated with fibrofatty tissue replacement of the ventricular tissue. The disease can cause ventricular dysfunction and arrhythmias and can increase the risk of sudden cardiac death. This cardiomyopathy can have variable clinical presentations, especially in the pediatric and young adult populations. In this report, we describe the case of an 18-year-old female with myocarditis as the initial presentation of ACM. She presented following a resuscitated cardiac arrest due to ventricular arrhythmia. On arrival, myocardial edema and delayed gadolinium enhancement were present on cardiac magnetic resonance imaging, with no ventricular changes observed, making the diagnosis consistent with myocarditis. Genetic testing revealed a pathogenic mutation in the desmoplakin gene consistent with ACM. Given the unconventional initial presentation of this patient's disease, early consideration of genetic testing may be beneficial to aid in the early diagnosis and management of ACM in young patients.

Noonan syndrome (NS) is a pleomorphic genetic disorder. Up to 50-80% of individuals have associated congenital heart disease. The scope of cardiac disease in NS is quite variable depending on the gene mutation. The most common forms of cardiac defects include pulmonary stenosis, hypertrophic cardiomyopathy (HCM), atrial septal defect and left-sided lesions. Amongst the rare vascular abnormalities few case reports have been mentioned about coronary artery lesions apart from sinus of Valsalva aneurysm, aortic dissection, intracranial aneurysm. This is a case report a rare case of asymptomatic coronary artery aneurysm in a young male with NS. There is lack of unified protocol for the screening, diagnosis, treatment, and follow-up of coronary artery disease in patients with NS. We conclude, echocardiography is sufficient in most cases in children. But a CT scan is appropriate in adults or when other lesions are suspected.

A full-term male neonate presented on the 11^th day of life with late-onset multisystem inflammatory syndrome-neonate (MIS-N) (cardioneurological compromise). Immediate anti-inflammatory modulation led to a gradual recovery of neurological and coronary lesions. However, temporal evaluation unmasked silent myocardial dysfunction in echocardiography validated further by elevated biomarkers, myocardial fibrosis in cardiac magnetic resonance imaging, and abnormal strain study persisting till 16 months of follow-up. This revealed a hitherto unknown and rare progression of MIS-N into dilated cardiomyopathy.

Total anomalous pulmonary venous connection (TAPVC) and anomalous pulmonary venous drainage are not synonymous. This has been described in the setting of right isomerism (bilateral right sidedness) where the pulmonary veins are connected anomalously but drain normally to the left-sided morphological right atrium. We describe another situation in right isomerism where normal pulmonary venous drainage is present in the setting of TAPVC.

Children with postpericardiotomy syndrome may develop hemodynamically significant pericardial effusions warranting drainage by surgical pericardial window or pericardiocentesis. The optimal approach is unknown. We performed a retrospective observational study at two pediatric cardiac centers. We included 42 children aged <18 years who developed postpericardiotomy syndrome following cardiac surgery between 2014 and 2021. Thirty-two patients underwent pericardial window and 10 underwent pericardiocentesis. Patients in the pericardial window group presented with postpericardiotomy syndrome sooner than those who underwent pericardiocentesis (median 7.5 days vs. 14.5 days, P = 0.03) and tended to undergo earlier intervention (median 8 days vs. 16 days, P = 0.16). No patient required subsequent drainage. There were no differences between groups in days of pericardial tube duration (median 4 days), complications, and subsequent days of intensive care or hospitalization. For children with postpericardiotomy syndrome with a pericardial effusion warranting drainage, these data suggest that pericardial window and pericardiocentesis have similar efficacy, safety, and resource utilization.