Background: Dilated cardiomyopathy (DCM) is characterized by impaired systolic and diastolic function of one or both ventricles. Aim of the Work:To assess left ventricular (LV) regional systolic as well as global systolic, diastolic functions and desynchrony in children with idiopathic DCM using tissue Doppler imaging. Materials and Methods:The study comprised 30 patients with idiopathic DCM (4.4+3.3 years) and 10 healthy children as a control group. Electrocardiography (ECG)-gated echocardiography was done using m-mode and conventional Doppler as well as tissue Doppler imaging to obtain modified Tei index, mitral annular wave velocities (S', E' and A') as well as offline analysis of color tissue velocity imaging to obtain the peak systolic velocities of 12 LV points in apical four chamber, two chamber and long axis views. Results:The peak systolic velocities of mitral annulus as well as the different LV segments of patients were significantly lower than that of the control group. The E/E' ratio of patients was (11.39+2.4). S' and E' wave of lateral and septal aspects of mitral annulus were correlated. The Tei index of LV of patients (0.568+0.1136) was significantly higher than that of the control group. The averaged mid LV segments showed significantly lower peak systolic velocity (3.018+0.777cm/sec). There was significant difference between different LV segments as regards the peak systolic velocities (P<0.05). When comparing the time to peak standard deviation (TP-SD) of lateral wall (±36.071) to septal wall (±33.020), no marked difference was detected (P>0.05). Conclusion:The diastolic dysfunction among children with idiopathic DCM lies in the borderline zone. Systolic and diastolic dysfunctions correlate. There is regional heterogeneity of left ventricular systolic velocities being in general lower in mid segments. No marked left ventricular systolic desynchrony in DCM was found in pediatric age group.
Background: Wilson disease (WD) is an autosomal recessive disease with copper overload. Its clinical picture depends on specific tissue/system damage by the excess copper. Aim of the work:We aimed to study prospectively the phenotypic spectrum of structural and functional cardiac changes among children with WD. Methods: 16 children with confirmed WD underwent electrocardiography (ECG), conventional and tissue Doppler echocardiography. Results: ECG was normal in 11 patients (68.7%), inverted T was detected in 2 (12.5%), ST elevation in 2 (12.5%) while P-pulmonale and inverted T were detected in 1 (6.25%). Five patients (31.25%) had mild and one (6.25%) had severe tricuspid regurgitation. Two girls (12.5%) with WD had underlying congenital heart defects, one had atrial septal defect (ASD) and another had double inlet left ventricle (DILV), malposed great vessels and severe pulmonary stenosis. There was a positive correlation between LV mass and duration of treatment (r=0.559, p=0.030), and a negative correlation between age of onset and LV mass index (r=0.600, p=0.018). There was no significant correlation between age of onset and duration of treatment with myocardial perfusion imaging (MPI) or tissue Doppler parameters. Conclusion: WD in children is associated with cardiac structural and functional changes including congenital structural heart malformations; ASD and DILV. Future research is needed to verify if ASD and DILV in WD are embryonic presentations of copper overload in WD.
Kawasaki disease (KD) is an acute systemic vascular disease that affects mostly medium sized and small vessels. The most serious of which is coronary artery disease. Vasculitis involves proliferative granulomatous inflammation and resolves by cicatrization if not treated. It is generally a self-limited disease and its highest incidence is in children under five years. The diagnostic criteria include the presence of fever for at least 5 days along with four of five other clinical features (rash, mucositis, conjunctival injection, cervical lymphadenopathy or extremity changes). Atypical Kawasaki disease includes patients who meet only 2 or 3 of the 5 criteria for diagnosis. We here report a 4-year old male patient who presented with fever, mucositis, perineal rash and evidence of de novo femoral vasculitis leading to spontaneously leaking femoral arteritis. His complete blood count revealed thrombocytopenia. The patient fulfilled the criteria for the diagnosis of atypical Kawasaki Disease.He received intravenous immunoglobulins and aspirin in anti-inflammatory doses and his condition improved. We report this unusual presentation of Kawasaki disease with spontaneous femoral artery leak, rash and thrombocytopenia. Diagnosis of atypical Kawasaki disease is very challenging but essential as initiation of specific management is life-saving. Level of Evidence of Study: IV (1).
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