Aortic events in pregnant patients with Marfan syndrome. Lessons from a multicenter study

Martín, Cristina; Evangelista, Arturo; Teixidó, Gisella; Villar, Susana; Serrano-Fiz, Santiago; Ospina, Victor; Mingo, Susana; Moñivas, Vanessa; Martı́nez, Daniel; Villarreal, Juan; Forteza, Alberto

doi:10.1016/j.rec.2021.07.012

Cited by 3 publications

(6 citation statements)

References 29 publications

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“…Retrospective and prospective studies are limited and primarily evaluate acute aortic complications during pregnancy, rather than changes in the aortic root dimensions. A prospective study of 89 pregnant females with MFS from Martín et al 18 reported an aortic dissection rate of 2.2% (two Type A and one Type B with aortic root dimensions at dissection 43, 37, and 45 mm, respectively), lower than most rates previously reported, which they speculated may be due to high rate of betablocker use (82% of their patients). Additionally, a study by Lind and Wallenburg 19 reported 78 pregnancies in 44 females who experienced five aortic dissections (four Type A and one Type B), none of whom were on a beta-blocker.…”

Section: Discussionmentioning

confidence: 79%

“…Manuscript respectively), lower than most rates previously reported, which they speculated may be due to high rate of beta-blocker use (82% of their patients). 18 Additionally, a study by Lind and Wallenburg reported 78 pregnancies in 44 females who experienced 5 aortic dissections (4 type A and 1 type B), none of whom were on a beta-blocker. 19 While some of these studies suggest beta-blockade may be effective in preventing aortic dissection, the data is conflicting and speculative.…”

Section: Manuscriptmentioning

confidence: 99%

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Beta-Blocker Use during Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan's Syndrome

et al. 2023

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Background Pregnant patients with Marfan syndrome (MFS) are at an increased risk for adverse aortic outcomes. While beta-blockers are used to slow aortic root dilatation in non-pregnant MFS patients, the benefit of such therapy in pregnant MFS patients remains controversial. The purpose of this study was to investigate the effect of beta-blockers on aortic root dilatation during pregnancy in MFS patients. Methods This was a longitudinal single-center retrospective cohort study of females with MFS who completed a pregnancy between 2004 and 2020. Clinical, fetal, and echocardiographic data were compared in patients on vs off beta-blockers during pregnancy. Results A total of 20 pregnancies completed by 19 patients were evaluated. Beta-blocker therapy was initiated or continued in 13 (65%) of the 20 pregnancies. Pregnancies on beta-blocker therapy experienced less aortic growth compared to those off beta-blockers (0.10cm [IQR: 0.10-0.20cm] vs 0.30cm [IQR: 0.25-0.35cm]; P=0.03). Using univariate linear regression, maximum systolic blood pressures (SBP), increase in SBP, and absence of beta-blocker use in pregnancy were found to be significantly associated with greater increase in aortic diameter during pregnancy. There were no differences in rates of fetal growth restriction between pregnancies on vs off beta-blockers. Conclusions This is the first study to evaluate changes in aortic dimensions in MFS pregnancies stratified by beta-blocker use. Beta-blocker therapy was found to be associated with less aortic root growth during pregnancy in MFS patients.

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Section: Discussionmentioning

confidence: 79%

Section: Manuscriptmentioning

confidence: 99%

Beta-Blocker Use during Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan's Syndrome

et al. 2023

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“…Although aortic events in pregnant women with MS and aortic dilation have been largely described in various papers,13–16 no evidence of this risk has been described in women with BAV, save a few case reports 17 18. Recently published results from the ROPAC, the largest published prospective dataset to date, found no complications in 42 women with BAV and no cases of aortic dissection 7.…”

Section: Discussionmentioning

confidence: 99%

Pregnancy-related aortic complications in women with bicuspid aortic valve

Galián-Gay

Pijuan-Domenech

Cantalapiedra-Romero

et al. 2023

Heart

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ObjectivesTo describe the aortic-related risks associated with pregnancy in women with bicuspid aortic valve (BAV) and to evaluate changes in aortic diameter in pregnancy.MethodsProspective observational study of patients with BAV from a single-site registry of pregnant women with structural heart disease between 2013 and 2020. Cardiac, obstetric and neonatal outcomes were studied. An assessment of aortic dimensions was performed during pregnancy by two-dimensional echocardiography. Aortic diameters were measured at the annulus, root, sinotubular junction and maximum ascending aorta diameter, and the largest diameter was used. Measurements of the aorta were made using the end-diastolic leading edge-to-leading edge convention.ResultsForty-three women (32.9 years, IQR 29.6–35.3) with BAV were included: 9 (20.9%) had repaired aortic coarctation; 23 (53.5%) had moderate or severe aortic valve disease; 5 (11.6%) had a bioprosthetic aortic valve; and 2 (4.7%) had a mechanical prosthetic aortic valve. Twenty (47.0%) were nulliparous. The mean aortic diameter in the first trimester was 38.5 (SD 4.9) mm, and that in the third trimester was 38.4 (SD 4.8) mm. Forty (93.0%) women had an aortic diameter of <45 mm; 3 (7.0%) had 45–50 mm; and none had >50 mm. Three women (6.9%) with BAV presented cardiovascular complications during pregnancy or the postpartum period (two prosthetic thrombosis and one heart failure). No aortic complications were reported. There was a small but significant increase in aortic diameter during pregnancy (third trimester vs first trimester, 0.52 (SD 1.08) mm; p=0.03). Obstetric complications appeared in seven (16.3%) of pregnancies, and there were no maternal deaths. Vaginal non-instrumental delivery was performed in 21 (51.2%) out of 41 cases. There were no neonatal deaths, and the mean newborn weight was 3130 g (95% CI 2652 to 3380).ConclusionsPregnancy in BAV women had a low rate of cardiac complications with no aortic complications observed in a small study group. Neither aortic dissection nor need for aortic surgery was reported. A low but significant aortic growth was observed during pregnancy. Although requiring follow-up, the risk of aortic complications in pregnant women with BAV and aortic diameters of <45 mm at baseline is low.

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“…A median follow-up of 6 [2][3][4][5][6][7][8][9][10][11][12] years (maximum =47) was observed. The Kaplan-Meier estimates of the 5-year survival rate after treatment were 78% (95% CI: 62-87%) for the surgically treated group and 90% (95% CI: 50-98%) for the group treated with an endovascular interventionist procedure.…”

Section: Survival In Patients With Tamentioning

confidence: 99%

“…Aortic and cardiovascular damage occurs in a high percentage of so-called rare or orphan diseases, regardless of etiology as in Marfan syndrome (MS) or similar phenotypes such as Loeys-Dietz syndrome (6,7) and also in patients with vasculitis, such as Takayasu arteritis (TA) (8); patients with Takayasu's arteritis may have complicated or catastrophic cardiac outcomes (9)(10)(11).…”

Section: Introductionmentioning

confidence: 99%

Cardiothoracic surgery and peripheral endovascular intervention in cardiovascular damage from a cohort of orphan rheumatological diseases—epidemiological and survival analysis

Soto¹,

Saucedo-Orozco²,

Ochoa‐Hein³

et al. 2022

J Thorac Dis

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Background: Aortic diseases in some orphan rheumatological diseases require medical, surgical or peripheral endovascular intervention because they can be catastrophic. Objectives: to analyze the main clinical and epidemiological characteristics of patients with Takayasu arteritis (TA), Marfan syndrome (MS) and similar conditions that were treated with cardiothoracic surgery and peripheral endovascular intervention.Methods: Retrospective and descriptive cohort study that included patients of any age and gender with TA (as per the criteria of the American College of Rheumatology and EULAR/PRINTO), MS (according to Ghent criteria), and similar conditions who underwent cardiothoracic surgery or peripheral endovascular intervention. Data were collected from electronic charts.Results: A total of 77 patients with TA and 135 patients with MS and similar conditions were included.The frequency of surgical or interventional requirements in patients with TA and MS/similar conditions was 77/364 (21.2%) and 135/300 (45%), respectively; such patients were followed for a median of 6 [2-12] and 3.29 (0.42-6.62) years, with (maximum follow-up range of 47 and 21.37 years, respectively).Aneurysms were present in 11 (14.3%) and 66 (48.9%) in patients with TA and MS/similar conditions, respectively. Aortic, mitral and tricuspid valve damage occurred in 8 (10.4%) patients, 4 (5.2%) patients and 1 (1.3%) patient with TA, respectively; corresponding frequencies in patients with MS/similar conditions were 98 (72.6%), 50 (37.0%) and 20 (14.8%). We identified that 20% of patients with TA died after 5.08 years (95% CI: 0.23-25.42 years) and 20 % of the patients with MS and other similar conditions died after 7.52 years (95% CI: 1.10-9.02 years).

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Aortic events in pregnant patients with Marfan syndrome. Lessons from a multicenter study

Cited by 3 publications

References 29 publications

Beta-Blocker Use during Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan's Syndrome

Beta-Blocker Use during Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan's Syndrome

Pregnancy-related aortic complications in women with bicuspid aortic valve

Cardiothoracic surgery and peripheral endovascular intervention in cardiovascular damage from a cohort of orphan rheumatological diseases—epidemiological and survival analysis

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