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

Int J Cardiol. 2016 Jun 1:212:223-31. doi: 10.1016/j.ijcard.2016.03.011. Epub 2016 Mar 19.

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.

Keywords: Adult congenital heart disease; Heart failure; Preserved ejection fraction; Right ventricle.

MeSH terms

  • Adolescent
  • Adult
  • Female
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery*
  • Heart Failure / diagnosis*
  • Heart Failure / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Postoperative Period
  • Retrospective Studies
  • Stroke Volume
  • Ventricular Dysfunction, Right / physiopathology*
  • Young Adult