الفهرس 
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Abstract
Congenital heart disease (CHD) is a common disease that occurs in approximately 8 per 1000 of births with a wide range of clinical manifestations. Congenital heart defects are classified into 3 main groups: Left to right shunts, right to left shunts, and outflow tract obstructions. Pulmonary hypertension (PH) is a potential complication of CHD causing considerable morbidity and mortality. PH clearly affects right ventricle (RV) ending finally in RV hypertrophy and failure. Being non invasive and easy, several biomarkers are recently studied for diagnosis of PH and predicting its outcome. Adrenomedullin (ADM) is a vasodilator and a natriuretic peptide involved in angiogenesis and inflammation regulation , it acts as autocrine or paracrine substance. ADM is produced by different tissues and organs, including vessels, lungs and heart . It has also been shown to inhibit proliferation in pulmonary artery smooth muscle cells, and to alleviate pulmonary artery collagen accumulation in PH. Plasma levels of Mid-Regional pro Adrenomedullin(MR-proADM) accurately reflect that of ADM and have allowed the production of functional clinical assays to determine ADM concentrations.MR-pro ADM has been studied as a novel and promising biomarker in different diseases such as heart failure and PH. The objectives of this work are to evaluate the plasma levels of midregional pro-adrenomedullin (MR-pro ADM) in children with pulmonary hypertension associated with congenital heart disease (PAH-CHD); and to evaluate the diagnostic and prognostic value of MR-pro ADM in these patients, by correlation of its levels with clinical and echocardiographic data of PAHCHD. This study was conducted on (50) children with CHD. Patients were selected from those admitted at Pediatric Cardiology Unit, Pediatric Department, Tanta University Hospital from May 2021 to May 2022. They were 23 males and 27 females, and their ages ranged from 1month to 24 months. Patients were classified into two groups: group 1:.25 children with PAH-CHD (mPAP ≥ 25 mmHg). group 2:.25 children with CHD and no PH (mPAP< 25 mmHg). Twenty-five (25) healthy children of matched age and sex served as a control group. They were 11males and 14 females, and their ages ranged from 2 months to 22 months. All children included in the study were subjected to the following: I. Complete history taking. II. Thorough clinical examination. III. Investigations: a) Plain X-ray chest and heart. b) Elecrtocardiogram( ECG). c) Echocardiographic assessment: Doppler, Two-dimensional and Mmode Echocardiographic evaluation of these parameters: ▪ Type of congenital heart disease (CHD). ▪ Mean pulmonary artery pressure (mPAP). ▪ Right ventricular diameter. ▪ RV systolic function. ▪ RV diastolic function. ▪ LV systolic function. ▪ LV diastolic function. d) Plasma level of Mid-Regional pro-Adrenomedullin (MR-proADM): Using a sandwich enzyme-linked immunosorbent assay test (ELISA). The results of the present study revealed that: • There was no statistically significant difference between the three groups as regards age and gender. • There was significant family history of CHD in group I and group II as compared to control group . • There was significant decrease of weight and BSA in children with CHD either with or without PAH as compared to control group . • There was HF in 12 cases (48%) of CHD-PH patients as compared to other groups. • The mean HR in group I and group II was significantly higher than that of the control group . • VSD was the most common diagnosis in group I patients (24%) ,and PDA or ASD was the most common diagnosis in group II patients (16%). • Dilated RA and dilated RV were significantly higher in CHD-PH group as compared to CHD group ,whereas dilated LA and dilated LV were not significantly different betwen both groups. • As regards LV systolic function (FS%) or diastolic function (E/A ratio), there was no significant difference between the three studied groups. • As regards RV systolic function (FAC), there was significant decrease of (FAC) in CHD-PH patients as compared to CHD patients and control group. • As regards RV diastolic function ( E/A ratio),there was significant decrease of (E/A ratio) in CHD-PH patients as compared to CHD patients and control group. • There was significant increase of CTR (by chest X-ray :Cardiomegaly) in children with CHD-PH group as compared to CHD group. • ECG findings revealed that RA enlargment was significantly higher in CHD-PH group as compared to CHD group ,whereas LA enlargement was not significant between both groups. • Also, RV enlargement was significantly higher in CHD-PH group as compared to CHD group ,whereas LV enlargement was not significant between both groups. • There was statistically significant increase of mPAP in CHD-PH children as compared to CHD children and control group. • The mean plasma level of MR-pro ADM significantly increased in CHDPH children as compared to CHD group as compared to control group(p<0.05). • The best cut -off point of MR-pro ADM as diagnostic biomarker to discriminate CHD-PH patients from CHD patients was 199.22 nmol/l ,with 92% sensitivity, 96% specificity, 95.8% PPV ,92.3% NPV , and AUC was 0.980. • There was significant positive correlation between MR-pro ADM and mPAP in CHD-PH patients ,whereas there was significant negative correlation between MR-pro ADM and LV Systolic FS% ,RV Systolic (FAC),LV diastolic E/A mitral and RV diastolic E/A tricuspid in patients with CHD-PH. • MR-proADM significantly increased in patients with CHD-PH who died as compared to patients who survived.