Development of the taksande's score: A new scoring system for the diagnosis of pulmonary arterial hypertension

Abstract

Background: In the pediatric population, pulmonary arterial hypertension (PAH) is associated with a number of underlying diseases which causes significant morbidity and mortality. PAH in children is mainly idiopathic or associated with congenital heart disease (CHD). No scoring systems have been developed to aid in the diagnosis of PAH. Aim: This study aims to develop a PAH scoring system that is more applicable to the children. Materials and Methods: This prospective diagnostic study was conducted on the 428 CHD children admitted to a tertiary referral hospital. The pediatricians had examined independently and used the palpation and auscultation to detect the study participants for PAH (Index text). Echocardiography for PAH (Reference standard) was performed after the clinical assessments of the children. For the development of the new scoring system, the data collected which include anthropometry, clinical signs (tachypnea, cyanosis, pedal edema, and hepatomegaly) and the cardiovascular examination (Loud second heart sound, palpable P2, ejection click, dullness in 2nd intercostal space, and murmur). All statistical analyses were performed using STATA statistical software (version 10.0). Results: The study population consisted of 428 children who had CHD. On examination, 71% children were wasted, 44.63% had tachypnea, 20% had cyanosis, 18.46% children had pedal edema, and 41% had hepatomegaly. Murmur was present in 53% of the study cases. The optimal cutoff threshold score derived from the receiver operating characteristic curve analysis was 5. Based on this optimal cutoff threshold, the calculated sensitivity and specificity were 85.5% (95% confidence interval [CI] 76.7%–91.8%) and 92.5% (95% CI 89.1%–95.1%), respectively. The positive predictive value and negative predictive value were 76.6% and 95.6%, respectively. The diagnostic accuracy of the new score was 89.71. Conclusions: This new PAH scoring system is easy and simple to apply as the majority of the parameters can be obtained from a routine history and clinical examination.