Prevalence of Congenital Heart Disease-Related Pulmonary Arterial Hypertension Among The Children with Congenital Heart Disease Attending Follow-Up Clinic at Tash. Echocardiograph Diagnoses
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Date
2013-03
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Addis Ababa University
Abstract
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 < 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
Description
Keywords
Heart Disease, Pulmonary Arterial