Clinical and Paraclinical Findings of Primary Pulmonary Hypertension in Children

Background: Given the significant morbidity and mortality of juvenile pulmonary arterial hypertension (PAH), understanding the clinical and paraclinical findings of this condition can aid in the early diagnosis and identification of helpful factors in its treatment. Objectives: This study aimed to evaluate the clinical and paraclinical findings of idiopathic pulmonary arterial hypertension (iPAH) in children. Methods: This cross-sectional study was carried out on 20 children with iPAH who were assessed in terms of pulmonary hypertension or hospitalized at the cardiac ward of Imam Reza hospital, Mashhad, Iran, during January 2018 to October 2001. The clinical data, chest X-ray (CXR), electrocardiographic, echocardiographic, cardiac catheterization findings, and mortality results were extracted from the medical records and recorded in a researcher-made questionnaire. Results: The current study’s findings revealed a higher prevalence of iPAH in females (70%) than men, as well as in term neonates compared to preterm newborns. The most common clinical findings were active dyspnea, heart murmur, and cyanosis. Prominent pulmonary conus, right atrial enlargement (RAE), and right ventricular hypertrophy (RVH) were the most common CXR findings. The right axis deviation, RVH, and RAE were the most common abnormal findings of electrocardiography, respectively. Based on echocardiographic findings, dilated right atrium and dilated right ventricle were reported in all subjects. The mean of tricuspid regurgitation pressure gradient (TRPG) and peak systolic pressure gradient (PSPG) were 76.33 ± 22.8 and 44.2 ± 13.18 mmHg, respectively. The mean ratios of systolic pulmonary artery pressure (PAP), diastolic PAP, and mean pulmonary artery pressure (mPAP) to aortic systolic pressure were estimated at 0.92, 0.78, and 0.88, respectively. The results confirmed a significant relationship between mPAP and consanguinity (P = 0.03). In addition, the findings demonstrated a correlation between echocardiographic findings (i.e., TRPG and PSPG) and systolic PAP, diastolic PAP, and mPAP (P < 0.05). In total, 56.3% of the patients expired, most of whom (37.5%) were the younger children. Conclusions: The findings suggest that IPAH should be considered as a possible diagnosis in various clinical manifestations, including respiratory distress, hepatomegaly, ascites and even neurological symptoms. Since there are no specific symptoms, therefore a set of history, physical examination, laboratory findings, and clinical suspicion should be taken into consideration.