OR28. Diagnostic delay in Congenital Heart Disease Related Pulmonary Arterial Hypertension: Insights from the Single Tertiary Hospital Center of Patients With Pulmonary Hypertension Registry In East Java Indonesia

Abstract

Abstract Aims The aim of this study was to determine the time from the onset of symptoms to the diagnosis of PAH associated with CHD in Saiful Anwar general Hospital,Malang and to determine the factors with delayed definitive diagnosis. Methods The Registry enrolled 128 adult patients with CHD-PAH from January 2019 to December 2020. Patients were considered to have delayed disease recognition if . 2 years elapsed between symptom onset and the patient receiving a CHD-PAH diagnosis, starting on PAH-specific therapy, or receiving a diagnosis by right-sided heart catheterization. Results In 54.6% of patients, symptoms were experienced > 2 years before CHD-PAH was recognized. The mean diagnostic interval from symptom onset to diagnostic RHC was 3.18 ± 1.75 years, while Overall, 9.3%, 25% and 65.7% of patients were diagnosed after <1, 1–2 and > 2 years, respectively). There was significant proportion of delayed diagnosed > 2 years in Secundum ASD compare than ≤ 2 years (74.3% vs 51.7%. Respectively, ,p=0.014, OR 2.6 95%CI : 1.2-5.6). Patients who life in remote area (OR 1.82; 95%CI 1.48-2.26), and General practitioner as Physician consulted at symptom onset (OR 1.72;95%CI 1.64-2.46) were independent predictors of longer diagnostic interval. We found significant different level of MPAP and PVR in patients who was delayed time to diagnosed CHD related to PAH > 2 years compare than ≤ 2 years (55.32±8.8 vs 42.4±10.2, 10.6±1.28 vs 4.2±1.42 respectively ,P=0.014, P = 0.012). Conclusions We show’s that ASD secundum as subtype of CHD, Patients who life in remote area, and General practitioner as Physician consulted at symptom onset were independent predictors of longer diagnostic interval.