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  • Title: Heart failure with preserved right ventricular ejection fraction in postoperative adults with congenital heart disease: A subtype of severe right ventricular pathophysiology.
    Author: Ohuchi H, Hayama Y, Negishi J, Noritake K, Iwasa T, Miyazaki A, Yamada O, Shiraishi I.
    Journal: Int J Cardiol; 2016 Jun 01; 212():223-31. PubMed ID: 27043063.
    Abstract:
    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 γ-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.
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