. Author manuscript; available in PMC: 2022 Jul 7.

Published in final edited form as: Nat Rev Dis Primers. 2021 Sep 2;7(1):64. doi: 10.1038/s41572-021-00298-7

Table 2.

Randomized Clinical Trials of ARBs in Patients with Marfan Syndrome

Trial name	Trial type	Drugs tested	Patients age (years); number	Follow-up time	Primary outcome	Results	Notes
Pediatric Heart Network²¹	Double-blind, stratified	Atenolol and Losartan	0.5–25; 608	3.0±0.1 y	Rate of change in root Z-score per year	Primary outcome NS (−0.139 vs −-0.107 Z/y, p=0.08)	Pharmacogenomics substudy²⁶²; Aortic stiffness associated with aortic enlargement¹³³
COMPARE¹²³	Open-label	Losartan	>18; 233	3.1±0.4 y	Change in absolute root diameter	Primary outcome NS (0.77 with losartan vs 1.35 mm with no therapy, p=0.01)	Significant only for FBN1 haploinsufficiency²⁶³
Spanish²²¹	Double-blind	Atenolol And Losartan	5–60; 140	3.0 y	Change in absolute diameter or Z-score of root and asc	Primary outcome NS (−-0.04 vs −0.01 Z, P=0.193)	Ambulatory blood pressures not different between groups
Marfan Sartan²²⁰	Double-blind	Losartan And Placebo	>10; 299	3.5 y	Rate of change in root Z-score/y	Primary outcome NS (−-0.01 vs −-0.03 Z/y, p=0.68)	100% sequenced: 78% of cases had an FBN1 mutation
Taiwanese²¹⁹	Open-label	Losartan and a BB compared with a BB alone	None specified; 29	~3 y	Rate of change in absolute root diameter/y	Primary outcome significant (0.10 vs 0.89 mm/y, p=0.02)	None
Aortic Irbesartan Marfan Study¹²⁴	Double-blind	Irbesartan and Placebo	6–40; 490	5 y	Rate of change in absolute root diameter/y	Primary outcome significant (0.53 vs 0.74mm/y, p=0.03)	None

Abbreviations: asc, ascending aorta, BB, β-blocker; d, day; m, month; y, year; NS, not significant.