Pulmonary arterial hypertension is a rare disease with a poor prognosis. Globally, pulmonaryrnvascular disease associated with congenital heart disease may represent the most preventablerncause of pulmonary artery hypertension and its morbid and fatal squeals. Epidemiological datarnare scarce, particularly in the pediatric population of developing countries. This is the firstrndescription of the pediatric data in our setup too.rnOBJECTIVESrnTo determine magnitude, common associated CHD ,co morbid conditions and treatmentrnpractices of patient with PAH with CHD age o to 18 years old children in TASH.rnMETHODS:rnThe study was retrospective chart review conducted from Jan 2011 to Dec 2012 with informedrnconsent from research committee. Pediatrics patients aged 0-18 years with the diagnosis of CHDrnwith moderate to large left to right shunt and others CHD that have potential to cause PAH werernincluded. Patient characteristics, type CHD, type PAH, symptoms at presentation, durationrnsymptoms before presentation, echo, x-ray, ECG, associated complication, co morbid condition,rnand treatments were among the most important data collected. Diagnosis was made withrnechocardiography in all the cases.rnEisenmenger syndrome was defined when there was arnreversed (pulmonary-to-systemic) or bidirectional shunt. The clinical characteristics between thernpatients with/without PAH and Eisenmenger syndrome were compared and their risk factorsrnanalyzed with a multivariate Logistic model, chi-square test and odd ratio.rnRESULTS:rnA total of 157 patients (78 male,79 female) was included in the study. Mean age at time ofrndiagnosis CHD was 33 months (range 1 month-18 years) and mean at diagnosis of PAH was 40rnmonths(range 1 month-12 years). PAH â€“CHD are diagnosed in 67(42.3) with fixed PAH beingrn33 (21%). Types of congenital heart diseases seen were VSD (26.8%), PDA (22.3%), ASDrn(6.4%), combined simple left to right shunt lesion (75.2%), tetralogy of Fallot (15.3%), D-TGA(rn5.7%), other complex congenital heart 3.8 per cent. Among the PAH-CHD patients, 49.2% ofrnthem had Eisenmenger syndrome. The patients with large shunts were at an elevated risk ofrnPAH. Compared with isolated arterial septal defect ,ventricular septal defect and patent ductusrnarteriosus patients with multiple different defects are at increased risks of PAH ( OR =rn8.348,95%CI(3.34-20.13) P < 0.004) .Only 19(12.1%) patients had surgical or device for theirrnCHD. Overall 105(68.9%) have congenital heart disease that will require immediate interventionrnincluding cardiac catheterization and surgical intervention. 136(86.6%) and 23(14.6%) had onernor more complication and co morbid conditions respectively at diagnosis. 77(49%) of the studyrnsubjects were on one or more forms of treatment for the associated complication. One (3%) ofrnpatient was on specific drug (Sildenafil) for PAH among those with fixed PAH. Death occurredrnin one patient during the study period.rnCONCLUSION:rnPAH(fixed) is a common (21%) complication in CHD patients in the clinic and ventricular septalrndefect is the most common pathogenic type of CHD. Patients with multiple types of defect are atrnincreased risk of developing PAH. Patients with others complications( like CHF, recurrent chestrninfections) at presentations are at increased risk PAH. Nutritional deficiencies of different typesrnare the common complication and/ or co morbidity identified. Only nineteen (12%) patients werernoperated for underling cardiac condition (CHD) and one patient is on specific drug for PAH