Primary Pulmonary Hypertension in Children

Objective: The aim of this study was to evaluate the clinical and para-clinical findings of primary pulmonary artery hypertension in children. Methods & Materials: This cross-sectional study was performed on the records of all children with primary pulmonary hypertension referred to pediatric clinic and cardiology department of Imam Reza Hospital during 2001-2016. The variables studied in this study were demographic and findings, clinical signs and clinical examination, CXR findings, echocardiographic findings, electrocardiographic findings, cardiac catheterization and mortality results. Results: The findings of the present study show the greater frequency of primary pulmonary hypertension in females (70%). The mean age of patients was 6.49 ± 4.82 years and the highest number of patients was in the age group of 0 to 5 years. The means birth weight of the studied patients was 3.24 0. 0.69 kg. Most of the patients were vaginal delivery, term, and their parents were related. The most common clinical findings were active dyspnea, heart murmur, cyanosis, shortness of breath, chest pain, and palpitations. The most common CXR findings were Prominent pulmonary conus, RAE and RVH, respectively. The most common abnormal findings in the ECG were RAD, RVH and RAE, respectively. The most common findings in echocardiography were Dilated RV and Dilated RA (in all cases) and then PFO or ASD. The mean TRPG was 76.33 22 22.80 and the mean PRPG was 44.20 13 13.18. In the evaluation of cardiac catheterization, mean systolic PAP was 89.77 31 31.77, diastolic PAP was 47.21 ± 20.57 and mean mean PAP was 66.15 ± 21.79. With increasing Diastolic PAP and mean PAP, PRPG levels increased. Of the sixteen patients who were followed up, 9 died (56.3%), with the highest mortality in the 0- to 5-year subgroup. Conclusion: According to the high morbidity and mortality of this disease, awareness of the clinical and paraclinical findings of this disease can help us to early diagnosis of this disease and identify the factors that influence its control.

کلیدواژه ها:

Primary pulmonary hypertension ، Echocardiography ، Cardiac catheterization ، children