Abstract

SESSION TITLE: PAH with Complications SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/17/2022 12:15 pm - 1:15 pm PURPOSE: Congenital heart disease (CHD) serves as the second most commonly associated form of primary pulmonary arterial hypertension (PAH). The prognosis of PAH related to congenital heart disease differ from other types of PAH due to factors such as defect types and sizes. The aim of the study is to compare the outcomes of PAH secondary to CHD (PAH-CHD) and PAH due to other causes. METHODS: We extracted the data from the National Inpatient Sample (NIS) database for the years 2016 to 2019 and analyzed all hospital admissions for PAH-CHD versus primary PAH due to causes other than CHD (Other PAH). We selected CHDs commonly known to be associated with primary pulmonary hypertension: atrial septal defect (ASD), ventricular septal defect (VSD), aortopulmonary window, atrioventricular septal defect, tetralogy of Fallot, patent ductus arteriosus (PDA), truncus arteriosus, double inlet left ventricle and double outlet right ventricle. We compared baseline characteristics, outcomes, and healthcare utilization between the two groups. RESULTS: Of 37,739 patients admitted with PAH, 1,355 patients had concomitant CHD and were compared with 36,384 adult PAH patients without CHD. In the PAH-CHD group, ASD was the most common associated CHD (1,195, 88%) followed by VSD (125, 9.2%) and PDA (39,2, 9%). The PAH-CHD patients were relatively younger (mean age of 53.15 vs 65.7 years), less likely to be of white race (62.36% vs 66%) and had a lower proportion of comorbid coronary artery disease (14.3% vs 28.86%), obesity (15.13% vs 22.66%), dyslipidemia (44% vs 51%), diabetes (18.08% vs 34.48%) and chronic kidney disease (19.93% vs 33.15%) [all p<0.05*]. A total of 115 (8.5%) PAH-CHD patients and 1,844 (5.07%) other PAH patients died during hospitalization. PAH-CHD patients had a higher in-hospital mortality (aOR=1.73 ;95%CI 1.08-2.76, p=0.02*). The PAH-CHD group had higher odds of having chronic respiratory failure (aOR=1.13;95%CI 1.04-1.22, p=0.003*), right ventricular failure (aOR=1.72 ;95%CI 1.12-2.6, p<0.013*), cardiogenic shock (aOR=2.1;95%CI 1.15-3.84, p=0.015*) and acute ischemic stroke (aOR=25.18;95%CI 6.78-93.46, p<0.0001*). compared to the other PAH group. PAH-CHD patients also had longer length of stay (10.6 vs 7.10 days, p=0.0009*) and higher hospitalization costs per admission ($209,507 vs $90,599, p=0.007*). CONCLUSIONS: The PAH-CHD group had higher in-hospital mortality and healthcare resource utilization than the other PAH group. PAH-CHD group were more likely to have chronic respiratory failure, right ventricular failure, cardiogenic shock and ischemic stroke. CLINICAL IMPLICATIONS: PAH-CHD seem to have higher mortality and worse PAH related in-patient complications. The difference between two groups highlights the need for further research and trials to understand the unique natural history and appropriate management of PAH-CHD patients. DISCLOSURES: No relevant relationships by Cyra-Yoonsun Kang No relevant relationships by Sandhya shri Kannayiram No relevant relationships by parnia khamooshi No relevant relationships by Viviana Reyes Pinzon No relevant relationships by Sujitha Velagapudi

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