Heart failure with preserved right ventricular ejection fraction in postoperative adults with congenital heart disease: A subtype of severe right ventricular pathophysiology

Background: Right ventricle (RV) may determine heart failure (HF) severity in adults with congenital heart disease (ACHD). However, the association of RV properties with clinical profiles remains unclear. Purpose: To clarify the associations of RV properties with biomarkers, exercise capacity, and unscheduled hospitalization (USH) in postoperative ACHD patients. Methods and results: We evaluated determinants of RV end-diastolic volume (EDVI, ml/m(2)) and pressure (EDP, mmHg) in 260 patients who were divided into 4 groups, i.e., those with RVEDVI < 100 and EDP >= 10 (A, n = 49, 19%), those with RVEDVI >= 100 with EDP >= 10 (B, n = 22), those with RVEDVI < 100 and EDP < 10 (C, n = 134), and those with RVEDVI >= 100 and EDP < 10 (D, n = 55). EDVI, EDP, and ejection fraction (EF, %) of the RV were independently associated with the corresponding value of the left ventricle (LV) (p < 0.0001 for all). Group A had a RV-EF of 53 +/- 8 and was defined HF with preserved RV-EF (RV-HFpEF). Younger age, lower platelet count, and elevated plasma gamma-glutamyltransferase independently predicted the RV-HFpEF. When the presence of pulmonary hypertension and LV-HFpEF was included in the analysis, these two factors independently predicted the presence of RV-HFpEF (p < 0.05-0.001). On multivariate analysis, older age, number of surgeries, cardiac index, and RV systolic pressure independently determined peak oxygen uptake (PVO2, p < 0.05) and RV outflow reconstruction independently predicted USH. The RV-HFpEF group showed the lowest PVO2 and highest incidence of USH among the 4 groups (p < 0.05). Conclusions: Impaired RV characteristics, especially RV-HFpEF, with liver dysfunction and lower platelet count predicted lower exercise capacity with a poorer prognosis in postoperative ACHD patients. (C ) 2016 Elsevier Ireland Ltd. All rights reserved.