PMID- 36004220
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220826
IS  - 2666-2736 (Electronic)
IS  - 2666-2736 (Linking)
VI  - 10
DP  - 2022 Jun
TI  - Aortic root dilation in adult patients with Marfan syndrome: Does aortic root 
      stiffness matter?
PG  - 113-120
LID - 10.1016/j.xjon.2022.04.011 [doi]
AB  - OBJECTIVE: Aortic root (AoR) size remains an imperfect predictor of rate of 
      aortic dilation in Marfan syndrome (MFS). Indicators of vascular phenotype such 
      as aortic stiffness have been proposed as additional predictors. In this study we 
      assessed the rate of AoR dilation and stiffness in adult patients with MFS. 
      METHODS: We performed a retrospective chart review. We included adult patients 
      with MFS (aged 20-40 years) with at least 2 local echocardiograms 6 months apart 
      (no aortic surgery in-between). A blinded observer analyzed the echocardiograms. 
      AoR dilation rate and stiffness were calculated. RESULTS: Thirty-two patients 
      (53% women; median age, 21.1; interquartile range [IQR], 19-24 years at first 
      echocardiogram) were included. AoR dilation rate in the entire cohort was 0 to 
      8 mm/year (median, 0.465; IQR, 0.23-1.45 mm/year). Multiple linear regression 
      analysis showed that baseline AoR stiffness was associated with AoR dilation rate 
      (beta = 0.0004; P < .001 for elastic modulus), whereas baseline age and baseline AoR 
      dimension were not. Eighteen of these 32 patients (56%) eventually had AoR 
      surgery (Sx) and 14 did not have surgery (NSx). At baseline, Sx and NSx patients 
      were similar in age. AoR dimension was larger (Sx, 4.27 cm; IQR, 4.05-4.49 cm vs 
      NSx, 3.73 cm; IQR, 3.37-4.09 cm; P = .011) and AoR stiffness was higher in Sx 
      patients (beta stiffness index: median, 23.2; IQR, 17.8-28.6 vs median, 15.6; 
      IQR, 11.6-19.7; P = .024). AoR dilation rate was greater in Sx patients, 
      independent of baseline AoR dimension (1.63 +/- 0.41 mm/year vs 
      0.38 +/- 0.08 mm/year; P = .01). CONCLUSIONS: Our results showed that AoR dilation 
      rate varies among adult patients with MFS and is associated with baseline AoR 
      stiffness, measured by echocardiography. Further studies are warranted to 
      determine how aortic stiffness can be implemented clinically to refine management 
      in patients with MFS.
CI  - (c) 2022 The Author(s).
FAU - Cox, Kelly
AU  - Cox K
AD  - Division of Cardiology, Department of Pediatrics, Lucile Packard Children's 
      Hospital, Stanford University, Palo Alto, Calif.
FAU - Oquendo, Yousi A
AU  - Oquendo YA
AD  - School of Medicine, Stanford University, Palo Alto, Calif.
FAU - Liang, David
AU  - Liang D
AD  - Division of Cardiology, Department of Medicine, Stanford Hospital, Stanford 
      University, Palo Alto, Calif.
FAU - Selamet Tierney, Elif Seda
AU  - Selamet Tierney ES
AD  - Division of Cardiology, Department of Pediatrics, Lucile Packard Children's 
      Hospital, Stanford University, Palo Alto, Calif.
LA  - eng
PT  - Journal Article
DEP - 20220420
PL  - Netherlands
TA  - JTCVS Open
JT  - JTCVS open
JID - 101768541
PMC - PMC9390401
OTO - NOTNLM
OT  - AoR, aortic root
OT  - EM, elastic modulus
OT  - MFS, Marfan syndrome
OT  - Marfan syndrome
OT  - NSx, no surgery
OT  - SI, stiffness index
OT  - Sx, Surgery
OT  - aortic root
OT  - dilation
OT  - stiffness
EDAT- 2022/08/26 06:00
MHDA- 2022/08/26 06:01
PMCR- 2022/04/20
CRDT- 2022/08/25 02:32
PHST- 2021/04/05 00:00 [received]
PHST- 2022/03/04 00:00 [revised]
PHST- 2022/04/12 00:00 [accepted]
PHST- 2022/08/25 02:32 [entrez]
PHST- 2022/08/26 06:00 [pubmed]
PHST- 2022/08/26 06:01 [medline]
PHST- 2022/04/20 00:00 [pmc-release]
AID - S2666-2736(22)00163-2 [pii]
AID - 10.1016/j.xjon.2022.04.011 [doi]
PST - epublish
SO  - JTCVS Open. 2022 Apr 20;10:113-120. doi: 10.1016/j.xjon.2022.04.011. eCollection 
      2022 Jun.