Abstract 14059: Correlation Between Demographics, Clinical Characteristics, Hemolytic Markers, and Pulmonary Hypertension Confirmed by Invasive Hemodynamics in Sickle Cell Disease

Abstract

Introduction: Pulmonary hypertension (PH) is a common complication and cause of death in sickle cell disease (SCD). Prior study demonstrated poor correlation between the assessment of mean pulmonary artery pressures (mPAP) by echocardiogram (TTE) and right heart catheterization (RHC) in SCD. Yet, most PH studies in SCD have used TTE for the identification of PH. We sought to better characterize the clinical features of symptomatic SCD patients with/without PH confirmed on invasive testing. Methods: All symptomatic, defined as dyspnea or shortness of breath, SCD patients with RHC between 2008-2017 in a single large center in the Chicagoland area were recruited. PH was defined by mPAP > 20 mmHg. Based on World Health Organization PH classification, Group 2 PH was differentiated from Group 1 PH by a pulmonary capillary wedge pressure (PCWP) ≥16 mmHg. Diagnosis and etiology of Group 1 PH was confirmed by chart review. We analyzed categorical and continuous variables using Pearson’s χ2 test and the Student’s t test, respectively, with Bonferroni correction as appropriate. Results: 62 individuals with SCD and invasive hemodynamic testing were identified. All were African American, 37% were female, 90.3% were homozygous SS genotype, and 74.2% were on hydroxyurea ( Table 1 ). Only mean LDH concentration was significantly different in those with PH compared to without (361 vs 448 U/L; P =0.02). Compared to Group 1 PH, those with Group 2 PH were younger (34.5 yr vs. 49 yr.; P =0.01), had higher mPAP (38 mmHg vs. 28 mmHg; P =0.002), had a higher prevalence of hypertension ( P =0.03) and chronic kidney disease ( P =0.004), and were frequently on a diuretic, angiotensin pathway blocking medication, calcium channel blocker, β-blockers, and/or nitrates ( Table 1) . Conclusions: In symptomatic patients with SCD, PH diagnosed on invasive hemodynamics is associated with higher LDH. Those with Group 2 PH tend to be younger, have worse renovascular disease, and require more cardiovascular medications.