. 2020 Oct;10(5):1625–1645. doi: 10.21037/cdt-20-370

Table 3. Summary of testing medical treatment for HF of sRV.

Reference	Design	Population	n	Intervention	Duration	Comparison	Outcome
Hechter, 2001 (106)	Observational retrospective	TGA Mustard	14	ACE-I	2 years	Before and after ACE-I	Positive: VO₂ max exercise duration, BP at max exercise
Hechter, 2001 (106)	Observational retrospective	TGA Mustard	14	ACE-I	2 years	Before and after ACE-I	Negative: exercise capacity and MRI measured EF
Lester, 2001 (107)	RCT/crossover	TGA atrial switch	7	Losartan	8 weeks	Placebo/crossover	Positive: exercise duration, TR, EF, systolic BP, exercise time
Lester, 2001 (107)	RCT/crossover	TGA atrial switch	7	Losartan	8 weeks	Placebo/crossover	Negative: no adverse effects
Robinson, 2002 (108)	Prospective cohort study	TGA atrial switch	9	Enalapril	12 months	Before and during ACE-I	Positive: resting HR
Robinson, 2002 (108)	Prospective cohort study	TGA atrial switch	9	Enalapril	12 months	Before and during ACE-I	Negative: exercise performance, cardiac index, VO₂ (rest, peak), SVR
Dore, 2005 (109)	Multi-center RCT/double blind/crossover	TGA atrial switch	29	Losartan	15 weeks	Placebo/crossover	Positive: none
Dore, 2005 (109)	Multi-center RCT/double blind/crossover	TGA atrial switch	29	Losartan	15 weeks	Placebo/crossover	Negative: exercise capacity, BNP
Josephson, 2006 (110)	Retrospective observational cohort	TGA atrial switch	8	Beta-blockers	36 months	Before and after beta-blockade	Positive: Trend towards improved symptoms, less TR, improved NYHA, improved RV function, exercise capacity
Josephson, 2006 (110)	Retrospective observational cohort	TGA atrial switch	8	Beta-blockers	36 months	Before and after beta-blockade	Negative: none
Giardini, 2007 (111)	Prospective cohort study	ccTGA or TGA atrial switch	8	Carvedilol	12 months	Before and after beta-blockade	Positive: RV and LV EF (MRI) biventricular remodeling, exercise duration
Giardini, 2007 (111)	Prospective cohort study	ccTGA or TGA atrial switch	8	Carvedilol	12 months	Before and after beta-blockade	Negative: peak oxygen uptake
Doughan, 2007 (112)	Retrospective cohort study	TGA atrial switch	60	Metoprolol Carvedilol	4 months	Beta-blockade/no treatment	Positive: NYHA after start beta-blockade (within group)
Doughan, 2007 (112)	Retrospective cohort study	TGA atrial switch	60	Metoprolol Carvedilol	4 months	Beta-blockade/no treatment	Negative: RVEF, RV size, TR
Khairy, 2008 (113)	Multi-center retrospective cohort study	TGA atrial switch	37	Beta-blockers	3 years	Beta-blockade/no treatment	Positive: Lack of betablockers predicts appropriate shocks in patients with AICD
Khairy, 2008 (113)	Multi-center retrospective cohort study	TGA atrial switch	37	Beta-blockers	3 years	Beta-blockade/no treatment	Negative: none
Therrien, 2008 (114)	RCT/double-blind/placebo	TGA atrial switch	17	Ramipril	1 year	Placebo	Positive: none
Therrien, 2008 (114)	RCT/double-blind/placebo	TGA atrial switch	17	Ramipril	1 year	Placebo	Negative: RV function (MRI), RV size (MRI), RV remodeling, exercise capacity, QoL
Bouallal, 2010 (115)	Prospective cohort study	ccTGA or TGA atrial switch	14	Bisoprolol Carvedilol	13 months	Before and after beta-blockade	Positive: NYHA, QoL, RVEF (radionuclide ventriculography)
Bouallal, 2010 (115)	Prospective cohort study	ccTGA or TGA atrial switch	14	Bisoprolol Carvedilol	13 months	Before and after beta-blockade	Negative: RVEF and size (MRI)
Tutarel, 2012 (116)	Retrospective observational control	TGA Mustard	14	Enalapril	13 months	Before and after ACE-I	Positive: NT-proBNP
Tutarel, 2012 (116)	Retrospective observational control	TGA Mustard	14	Enalapril	13 months	Before and after ACE-I	Negative: NYHA, exercise capacity
Van Der Bom, 2013 (117)	Multi-center RCT/double blind	ccTGA or TGA atrial switch	88	Valsartan	3 years	Valsartan/Placebo	Positive: RV volumes and mass (MRI), ventricular remodeling
Van Der Bom, 2013 (117)	Multi-center RCT/double blind	ccTGA or TGA atrial switch	88	Valsartan	3 years	Valsartan/Placebo	Negative: clinical event rate, RV function (MRI), exercise capacity, TR, QoL, neurohormonal activation, clinical outcome
Dos, 2013 (118)	RCT, double blind	TGA atrial switch	26	Eplerenone	12 months	Eplerenone/Placebo	Positive: Trend towards reduction in collagen turnover biomarkers (myocardial fibrosis)
Dos, 2013 (118)	RCT, double blind	TGA atrial switch	26	Eplerenone	12 months	Eplerenone/Placebo	Negative: RV mass and function, NT-proBNP
Sohdi (119), 2018	single-center prospective, randomized, open-label pilot study	TOF or TGA	17	Eplerenone	12 months	3 months before or after drug-free period	Positive: none
Sohdi (119), 2018		TOF or TGA	17	Eplerenone	12 months	3 months before or after drug-free period	Negative: serum levels of PINP, PIIINP or gal-3, 6MWD or QoL
van Dissel (120), 2019	Multi-center RCT	ccTGA or TGA atrial switch	88	Valsartan	3 years	Valsartan/placebo	Positive: decreased risk of events in symptomatic patients
van Dissel (120), 2019	Multi-center RCT	ccTGA or TGA atrial switch	88	Valsartan	3 years	Valsartan/placebo	Negative: survival

6MWD, six-minute walking distance; ACE-I, angiotensin-converting enzyme (ACE)-inhibitors; AICD, automated implantable cardioverter-defibrillator; BP, blood pressure; ccTGA, congenitally corrected TGA; EF, ejection fraction; Gal-3: galectine-3 ; HR, heart rate; LV, left ventricle; MRI, magnetic resonance imaging; NT-proBNP, N-Terminal pro-brain natriuretic peptide; NYHA, New York Heart Association; PINP, procollagen type I N propeptide; PIIINP, Amino-Terminal Propeptide of Type III Procollagen; RCT, randomized controlled trial; RV, right ventricle; SVR, systemic vascular resistance; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; TR, tricuspid regurgitation; QoL, quality of life.