Early Impairment Left Ventricular Mechanics in Children With Mitral Valve Prolapse.

Early impairment of left ventricular (LV) function has been reported in some inherited connective tissue diseases such as Marfan syndrome or rheumatic heart disease in pediatric patients. However, there is no study about cardiac strain in pediatric patients with primary mitral valve prolapse (MVP). The aim of this study was to evaluate the LV functions in pediatric patients with primary MVP, mild or moderate mitral regurgitation (MR), and normal LV ejection fraction. The study group included 72 consecutive patients (40 female, mean age: 13.1 ± 5.2 with primary MVP who had mild or moderate MR, and normal systolic function (LV ejection fraction ≥60%) were compared with 40 healthy children using conventional echocardiography, tissue Doppler imaging, and 2-dimensional speckle tracking echocardiography. Patients were divided into subgroups according to the MR severity: mild (n:34), and moderate (n:38) and leaflet thickness: classical (n: 40), and nonclassical (n: 32). The children with MR had significantly lower values for global early diastolic strain rate ([SRe] patients: 1.40 ± 0.25 vs controls: 1.62 ± 0.54; p = 0.001), and E/SRe (patients: 72.7 ± 5.6 vs controls: 62.1 ± 4.9; p <0.04) when compared with the control group. In subgroup analysis, SRe (mild: 1.49 ± 0.38 vs moderate: 1.32 ± 0.31; p <0.001) was lower in MVP patients with moderate MR compared to mild MR, and E/SRe (mild: 69.4 ± 5.1 vs moderate: 75.1 ± 6.4; p <0.001) value was higher in MVP patients with moderate MR compared to mild MR. Diatolic strain parameters, SRe, and E/SRe are more sensitive markers of early subtle myocardial injury in pediatric patients with primary MVP.

The neutrophil-to-lymphocyte ratio and mean platelet volume can be associated with severity of valvular involvement in patients with acute rheumatic carditis.

OBJECTIVE: The aim of the study was to investigate the association between the severity of acute rheumatic carditis (ARC) and the neutrophil-lymphocyte ratio (NLR) and mean platelet volume (MPV). METHODS: Paediatric patients diagnosed with ARC between 2010 and 2016 and age- and gender-matched controls were retrospectively analysed. At the time of diagnosis, we reviewed the demographic features obtained: echocardiographic data, complete blood count reports, acute-phase reactants, including C-reactive protein, and erythrocyte sedimentation rate values. The patient group was further divided into two subgroups according to the degree of valvular regurgitation, which included those with severe and those with mild-to-moderate valvular regurgitation. RESULTS: The number of cases with ARC and age- and gender-matched controls were 120 and 50, respectively. The mean age of the patients was 12.25 ± 2.89 (range: 7-18) years. NLR, MPV, anti-streptolysin-O, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), haemoglobin level, and white blood cell (WBC) and neutrophil count were significantly higher in patients with acute carditis compared with the controls (p < 0.001). NLR was found to have a significantly positive correlation with CRP (r = 0.177, p = 0.001), ESR (r = 0.81, p = 0.03) and WBC count ( r = 0.47, p = 0.001). Moreover, we found a positive correlation between NLR and severity of valvular regurgitation (r = 0.34, p < 0.001), and a negative correlation between MPV and severity of valvular regurgitation ( r = -0.38, p < 0.05) in our patients. In multiple linear regression analysis, severe valvular regurgitation was associated with NLR (0.51; 95% CI: 0.32-0.68; p = 0.006) and MPV ( -0.78; 95% CI: -0.72 to -0.98; p = 0.008). CONCLUSIONS: NLR and MPV are novel inflammatory markers and simple, rapid and easily accessible prognostic parameters that can be associated with severity of valvular involvement in patients with ARC.

Rapid Improvement of Pulmonary Functions in Children After Transcatheter Closure of an Atrial Septal Defect.

There are very few studies in the literature on respiratory system functions and complications of children with an atrial septal defect (ASD). The aim of this study is to investigate the pulmonary functions and pulmonary complications before and after transcatheter closure in children with an ASD. In this study, pulmonary function test parameters of 30 ASD patients between 5 and 18 years of age who were eligible to be treated by transcatheter ASD closure were compared with 30 healthy children. The patients undergoing transcatheter ASD closure received pulmonary function tests (PFT) at baseline (1 day before ASD closure), and 3 months after the procedure. Forced vital capacity (FVC), forced expired volume in 1 s (FEV1), peak expiratory flow, and mean forced expiratory flow during the middle half of FVC were measured. The mean age of the 30 ASD patients was 9.59 ± 3.1 years; and 20 (66.6%) were female and 10 (33.3%) were male. The mean age of the control group was 10.15 ± 2.21 years, and 19 (63.3%) were girls and 11 (36.6%) were males. ASD patients had significantly reduced FVC (73.11 ± 24.6%; 86.05 ± 26.1; p = 0.001, respectively), and FEV1 (81.34 ± 26.2% and 99.2 ± 19.6%; p = 0.001; respectively) at baseline. But significant improvement was observed in FVC values in the 3rd-month post-closure comparison of the patient group with the control group (73.11 ± 24.6%; and 88.36 ± 14.5%; p = 0.01, respectively); FEV1 values (81.34 ± 26% and 99.54 ± 18.2%; p = 0.005, respectively) and mean forced expiratory flow between 25 and 75% of vital capacity (MEF25-75) values (94.6 ± 33.4% and 124.2 ± -24.1%; p = 0.01, respectively) were also improved. There was no statistically significant relationship between the PFT measurements at baseline and after closure of the defect and age at transcatheter closure, gender, body height, body weight, ASD diameter, Q p/Q s, right ventricle systolic pressure, or mean pulmonary artery pressure values. At the 3rd month of ASD closure, there was no significant difference in the comparison of the PFT values of the patient and control group. Disturbance in the significant flow limitation of the peripheral airway of ASD patients was observed with PFT. Better pulmonary outcomes were observed in ASD patients after transcatheter closure.

Congenital Hemophilia A Presenting With Hashimoto's Encephalopathy and Myocarditis: The First Reported Case.

Here, we report a case of hemophilia A with myocarditis, encephalopathy, and spontaneous intramedullary hemorrhage. A 14-month-old male infant presented with loss of consciousness, generalized tonic-clonic convulsions, and cardiac failure. The neurological examination was normal. Myocarditis was diagnosed. After administration of fresh frozen plasma, the aPTT did not return to normal. The factor VIII (FVIII) level was 10.2% the normal level, and the patient was diagnosed with hemophilia A. The cerebrospinal fluid (CSF) evaluation was unremarkable, with the exception of elevated CSF protein levels. An electroencephalogram revealed diffuse slowing of background activity. The spinal MRI revealed chronic bleeding in areas of the medulla spinalis. Tests for the antibodies associated with autoimmune encephalitis were negative. Anti-thyroglobulin (THG) (11.8 U/mL [normal, 0 to 4 U/mL]) and anti-thyroid peroxidase (TPO) (53.9 U/mL [normal, 0 to 9 U/mL]) antibodies were present in the serum. The patient received 1 g/kg intravenous immunoglobulin G (IVIG) for 2 days based on a diagnosis of myocarditis and Hashimoto's encephalopathy. After the first month of treatment his clinical findings were negative. He developed an inhibitor to FVIII 1 month after initiation of FVIII replacement therapy. Hemophilia A has various clinical presentations. Autoimmunity may lead to early inhibitor development in patients with hemophilia.

Subclinical diastolic dysfunction in children with Wilson's disease assessed by tissue Doppler echocardiography: a possible early predictor of cardiac involvement.

BACKGROUND: The aim of this study was to evaluate the left ventricular systolic and diastolic functions and cardiac rhythm problems for the early detection of myocardial dysfunction in children with Wilson's disease. METHODS: We compared patients who had Wilson's disease (n = 22) witl Wilson's disease was based on clinical symptoms and laboratory tests (serum ce cardiographic examination, as well as pulsed-wave Doppler, tissue Doppler ech Holter monitoring was also performed in all subjects. RESULTS: All patients were asymptomatic on cardiological examination an tion, fractional shortening, wall thickness and left ventricular mass were similar disease had significantly lower mitral E velocity, mitral E/A ratio (P= 0.046, P= 0.0 as estimated by pulsed wave Doppler echocardiography. Wilson patients had lo pler echocardiography (P=0.006) compared to the controls. On 24-hour ECG cardiac arrhythmia. CONCLUSION: Our study showed results that might be consistent with disease children which probably represents an early stage of cardiac involvem the patients.