Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications

Toprak, Betül; Szöcs, Katalin; Zengin-Sahm, Elvin; Sinning, Christoph; Hot, Amra; Bannas, Peter; Hecher, Kurt; Hüneke, B.; Mir, Thomas S.; Rybczynski, Meike; Girdauskas, Evaldas; Blankenberg, Stefan; Kodolitsch, Yskert Von

doi:10.3390/jcm9041124

Cited by 4 publications

(9 citation statements)

References 44 publications

(63 reference statements)

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“…With respect to those patients have received isolated aortic surgery in the available literature, aside from low rates of mortality and good freedom from reintervention, it should be noted that more conservative strategies of operating (i.e., with ascending diameters >5.5 cm, in the absence of high-risk factors) are not recommended based on the data ( 15 , 20 ). Limited discussion can be made regarding obstetric patients with BAV, other than that some data suggest that in the absence of connective tissue disorders, BAV patients fare no worse than the general population in relation to immediate aortic risk ( 23 ). Masri et al make comment of a small cohort of patients they identified which did see rapid increases in rate of aortic dilatation, though this did not translate to increased rates of peri-pregnancy dissection ( 18 ).…”

Section: Discussionmentioning

confidence: 99%

Incidence of bicuspid valve related aortic dissection: a systematic review and meta-analysis

Wilson-Smith¹,

Eranki²,

Muston³

et al. 2022

Ann Cardiothorac Surg

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Background: The true incidence of bicuspid valve-related aortic dissection (AD) is extremely difficult to ascertain. This review aimed to provide the reported cumulative incidence of bicuspid aortic valve (BAV)-related AD in actively monitored study populations.Methods: Four electronic databases were used to perform literature searches. A meta-analysis of proportions or means were performed for categorical and continuous variables, as appropriate. Survival data was calculated from the aggregation of Kaplan-Meier (KM) curves from the included studies, where reported.Results: A total of 4,330 patients were identified in eleven studies. A cumulative incidence of bicuspid valverelated AD of 0.6% across a median follow-up time of 9 years was identified. Actuarial survival across this monitored population at 1, 3, 5 and 10 years was 97.2%, 96.7%, 92.45%, and 81.1%, respectively.Conclusions: This systematic review and meta-analysis identified a low incidence of AD across the examined follow-up period. Large, prospective studies involving early identification of bicuspid valve pathology, recruitment, and follow-up of BAV cohorts with comparison to the baseline population are required to most accurately determine the outcomes of these patients.

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Section: Discussionmentioning

confidence: 99%

Incidence of bicuspid valve related aortic dissection: a systematic review and meta-analysis

Wilson-Smith¹,

Eranki²,

Muston³

et al. 2022

Ann Cardiothorac Surg

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“…Aortic disease is one of the important causes of maternal indirect death ( Cantwell et al, 2011 ). Most scholars believe that pregnancy is a major risk factor for adverse aortic events ( Yuan, 2013 ; Toprak et al, 2020 ). It may be that the hyperdynamic and hypervolemic cardiovascular state in pregnancy that increases the stress and shear force of the aortic wall has significant effects on the aorta ( Cox et al, 2014 ; Bons and Roos-Hesselink, 2016 ).…”

Section: Clinical Significancementioning

confidence: 99%

“…It should be noted that, in addition to pregnancy, connective tissue diseases such as BAV and MFS themselves, are also risk factors for aortic dissection ( Hiratzka et al, 2010a ; Michelena et al, 2011 ). Interestingly, a recent retrospective study showed that pregnancy only increases the immediate aortic risk of MFS patients, but not that of BAV patients ( Toprak et al, 2020 ). Further, pregnant women with MFS have a higher risk of aortic dissection or proximal aortic surgery than BAV alone ( Toprak et al, 2020 ).…”

Section: Clinical Significancementioning

confidence: 99%

“…Interestingly, a recent retrospective study showed that pregnancy only increases the immediate aortic risk of MFS patients, but not that of BAV patients ( Toprak et al, 2020 ). Further, pregnant women with MFS have a higher risk of aortic dissection or proximal aortic surgery than BAV alone ( Toprak et al, 2020 ). Therefore, whether this management strategy for women with MFS can be extrapolated to women with BAV is limited by the absence of reference research data.…”

Section: Clinical Significancementioning

confidence: 99%

“…Therefore, whether this management strategy for women with MFS can be extrapolated to women with BAV is limited by the absence of reference research data. Although previous pregnancies cannot increase long-term risk of adverse aortic events ( Toprak et al, 2020 ), pre-pregnancy evaluation and post-pregnancy management of pregnant women with cardiac disease by professionals is still necessary ( Hiratzka et al, 2010a ; Borger et al, 2018 ). For female patients with MFS who plan to become pregnant, ACCF/AHA guidelines recommend a surgical threshold of 40 mm for the aortic diameter ( Hiratzka et al, 2010a ), while the ESC guidelines recommend a threshold of 45 mm ( Erbel et al, 2014 ).…”

Section: Clinical Significancementioning

confidence: 99%

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Aortic Dilatation in Patients With Bicuspid Aortic Valve

et al. 2021

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Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. BAV aortic dilatation is associated with an increased risk of adverse aortic events and represents a potentially lethal disease and hence a considerable medical burden. BAV with aortic dilatation warrants frequent monitoring, and elective surgical intervention is the only effective method to prevent dissection or rupture. The predictive value of the aortic diameter is known to be limited. The aortic diameter is presently still the main reference standard for surgical intervention owing to the lack of a comprehensive understanding of BAV aortopathy progression. This article provides a brief comprehensive review of the current knowledge on BAV aortopathy regarding clinical definitions, epidemiology, natural course, and pathophysiology, as well as hemodynamic and clinically significant aspects on the basis of the limited data available.

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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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Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications

Cited by 4 publications

References 44 publications

Incidence of bicuspid valve related aortic dissection: a systematic review and meta-analysis

Incidence of bicuspid valve related aortic dissection: a systematic review and meta-analysis

Aortic Dilatation in Patients With Bicuspid Aortic Valve

Pregnancy-related aortic complications in women with bicuspid aortic valve

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