Prevalence of Congenital Heart Disease-Related Pulmonary Arterial Hypertension Among The Children with Congenital Heart Disease Attending Follow-Up Clinic at Tash. Echocardiograph Diagnoses
Creators: name: Gebi, Lencho
Publisher: name: National Academic Digital Repository of Ethiopia
Year: 2013

description: Pulmonary arterial hypertension is a rare disease with a poor prognosis. Globally, pulmonary vascular disease associated with congenital heart disease may represent the most preventable cause of pulmonary artery hypertension and its morbid and fatal squeals. Epidemiological data are scarce, particularly in the pediatric population of developing countries. This is the first description of the pediatric data in our setup too. OBJECTIVES To determine magnitude, common associated CHD ,co morbid conditions and treatment practices of patient with PAH with CHD age o to 18 years old children in TASH. METHODS: The study was retrospective chart review conducted from Jan 2011 to Dec 2012 with informed consent from research committee. Pediatrics patients aged 0-18 years with the diagnosis of CHD with moderate to large left to right shunt and others CHD that have potential to cause PAH were included. Patient characteristics, type CHD, type PAH, symptoms at presentation, duration symptoms before presentation, echo, x-ray, ECG, associated complication, co morbid condition, and treatments were among the most important data collected. Diagnosis was made with echocardiography in all the cases. Eisenmenger syndrome was defined when there was a reversed (pulmonary-to-systemic) or bidirectional shunt. The clinical characteristics between the patients with/without PAH and Eisenmenger syndrome were compared and their risk factors analyzed with a multivariate Logistic model, chi-square test and odd ratio. RESULTS: A total of 157 patients (78 male,79 female) was included in the study. Mean age at time of diagnosis CHD was 33 months (range 1 month-18 years) and mean at diagnosis of PAH was 40 months(range 1 month-12 years). PAH –CHD are diagnosed in 67(42.3) with fixed PAH being 33 (21%). Types of congenital heart diseases seen were VSD (26.8%), PDA (22.3%), ASD (6.4%), combined simple left to right shunt lesion (75.2%), tetralogy of Fallot (15.3%), D-TGA( 5.7%), other complex congenital heart 3.8 per cent. Among the PAH-CHD patients, 49.2% of them had Eisenmenger syndrome. The patients with large shunts were at an elevated risk of PAH. Compared with isolated arterial septal defect ,ventricular septal defect and patent ductus arteriosus patients with multiple different defects are at increased risks of PAH ( OR = 8.348,95%CI(3.34-20.13) P &lt; 0.004) .Only 19(12.1%) patients had surgical or device for their CHD. Overall 105(68.9%) have congenital heart disease that will require immediate intervention including cardiac catheterization and surgical intervention. 136(86.6%) and 23(14.6%) had one or more complication and co morbid conditions respectively at diagnosis. 77(49%) of the study subjects were on one or more forms of treatment for the associated complication. One (3%) of patient was on specific drug (Sildenafil) for PAH among those with fixed PAH. Death occurred in one patient during the study period. CONCLUSION: PAH(fixed) is a common (21%) complication in CHD patients in the clinic and ventricular septal defect is the most common pathogenic type of CHD. Patients with multiple types of defect are at increased risk of developing PAH. Patients with others complications( like CHF, recurrent chest infections) at presentations are at increased risk PAH. Nutritional deficiencies of different types are the common complication and/ or co morbidity identified. Only nineteen (12%) patients were operated for underling cardiac condition (CHD) and one patient is on specific drug for PAH