Table 3:

Heart failure medication use in congenital heart disease patients

Diagnosis	Medications (Reference)	Important Findings
TGA, systemic RV	Beta blockers: Giardini et a I,47 2007; Shaddyetal,48 2007; Doughan et aI,49 2007; Bouallal et al,50 2010; Khairy et al,41 2017	Positive RV remodeling47 vs neutral or possible worsening function,48 improved exercise duration,47 improved NYHA functional class49, 50 and QOL,50 protective against appropriate shocks41
	ACE inhibitors: Therrien et al,51  2008; Hechteret al,52 2001;  Robinson et al,53 2001; Tutarel  etal,542012	No effect on RVEF, RVEDV and RVESV,51 no improvement in exercise performance52,53 Decreases NT-pro (BNP) levels54
	Angiotensin receptor blockers: Van der Bom et al,55 2013; Dore et al,56 2005; Lester et al,57 2001	No effect on RVEF, QOL,55 and exercise capacity55,56 During active treatment, systolic BP and degree of TR decreased and duration of exercise increased57
	Aldosterone receptor blockers: Dos etal,582013	Improvement of an altered baseline CTB profile suggesting reduction in myocardial fibrosis58
Tetralogy of Fa Not, subpulmonic RV	Beta blockers: Norozi et al,59 2007	No effect on peak Vo2 or ventricular function in patients with BNP >100 pg/mL and peak Vo2 <25 mL/kg/min59
	ACE inhibitors: Babu-Narayan etal,602012	No improvement in RVEF60
	Angiotensin receptor blockers: Bokma etal (REDEFINE),61 2018	No significant effect on RVEF, and secondary outcomes such as LVEF, peak aerobic exercise capacity, and NT-pro (BNP)61
Single ventricle circulation	Beta blockers: Ishibashi et al,62 2011	Cardiothoracic ratio improved, dosage of furosemide reduced, and ejection fraction improved62
	ACE inhibitors: Hsu et al,63 2010; Kouatli etal,64 1997	In infants, no improvement in somatic growth, ventricular function or HF severity; incidence of death and transplantation did not differ between groups63 No change in exercise capacity diastolic function, resting cardiac index, and SVR64
	Aldosterone receptor blockers: Mahle etal,65 2009	Did not improve endothelial function or alter most serum cytokine levels65
	Phosphodiesterase inhibitors: Goldberg etal,66 2011 Endothelin receptor blockers: Hebert et al (TEMPO),67 2014; Schuuring etal,68 2013	Increase in peak Vo2 and improved exercise time and functional class67 No effect on peak Vo2, NT-pro (BNP) level, and mental QOL68 Improved ventilator efficiency during peak and submaximal exercise but no effect on peakVo266

Abbreviations: ACE, angiotensin converting enzyme; APPROPRIATE, Ace Inhibitors for Potential Prevention of the Deleterious Effects of Pulmonary Regurgitation in Adults with Repaired Tetralogy of Fallot; BNP, brain natriuretic peptide; CTB, collagen turnover biomarker; LVEF, left ventricular ejection fraction; NT-pro (BNP), N-terminal pro (brain natriuretic peptide); NYHA, New York Heart Association; QOL, quality of life; RVEDV, right ventricular end diastolic volume; RVEF, right ventricular ejection fraction; RVESV, right ventricular systolic; SVR, systemic vascular resistance; TGA, transposition of the great arteries (here includes DTGA with atrial switch and CCTGA); TR, tricuspid regurgitation.