Marfan syndrome (MFS) is linked with adverse pregnancy events, one of the most significant being aortic dissection. We present a case of a woman with MFS with prior aortic root dilatation who opted for a Personalised External Aortic Root Support (PEARS). To date, she is only the fifth woman to have had this valve-sparing procedure prior to pregnancy. We outline her care in a tertiary centre with multidisciplinary expertise, from preconception through to the postpartum period.
WilliamsDLindleyKJRussoM,et al.Pregnancy after aortic root replacement in Marfan’s syndrome: a case series and review of the literature. AJP Rep2018; 8: E234–E240.
2.
CauldwellMSteerPJCurtisSL, et al.Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome. Heart2019; 105: 1725–1731.
3.
RomanMJPughNLHendershotTP, et al.Aortic complications associated with pregnancy in Marfan syndrome: the NHLBI national registry of genetically triggered thoracic aortic aneurysms and cardiovascular conditions (GenTAC). J Am Heart Assoc2016; 5: e004052.
4.
Regitz-ZagrosekVRoos-HesselinkJWBauersachsJ, et al.ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J2018; 2018: 3165–3241.
5.
KremerJFaragMZaradzkiM, et al.The reimplantation valve-sparing aortic root replacement technique for patients with Marfan syndrome: a single-center experience. Sci Rep2019; 9: 1–8.
IzgiCNewsomeSAlpenduradaF, et al.External aortic root support to prevent aortic dilatation in patients with Marfan syndrome. J Am Coll Cardiol2018; 72: 1095–1105.
PepeGGiustiBSticchiE,et al.Marfan syndrome: current perspectives. Appl Clin Genet2016; 9: 55–65.
10.
MehtaLSWarnesCABradleyE, et al.Cardiovascular considerations in caring for pregnant patients: a scientific statement from the American heart association. Circulation2020; 141: e884–e903.
11.
TreasureTTakkenbergJJMGolesworthyT, et al.Personalised external aortic root support (PEARS) in Marfan syndrome: analysis of 1–9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement. Heart2014; 100: 969–975.
12.
van HagenIMRoos-HesselinkJW. Pregnancy in congenital heart disease: risk prediction and counselling. Heart2020; 106: 1853–1861.
13.
SayamaS, et al.Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study. BJOG2017; 125: 487–493.
14.
TreasureTPetrouMRosendahlU, et al.Personalized external aortic root support: a review of the current status. Eur J Cardiothorac Surg2016; 50: 400–404.
15.
MilesJ. The gift of life. Courier Mail QWeekend 29-30 August 2020: 10–12.
16.
NienaberCAYuanXErnstS. PEARS Procedure and the difficulty to provide evidence for its benefits. Eur Heart J2020; 41: 4086–4088.
17.
GolandSElkayamU. Pregnancy and Marfan syndrome. Ann Cardiothorac Surg2017; 6: 642–653.
18.
CurryRAGelsonESwanL, et al.Marfan syndrome and pregnancy: maternal and neonatal outcomes. BJOG2014; 121: 610–617.
19.
CauldwellMSteerPSterrenburgM, et al.Birth weight in pregnancies complicated by maternal heart disease. Heart2019; 105: 391–398.
20.
LuHZangY. Interpretation of intrapartum care for women with existing medical conditions or obstetric complications and their babies (NICE 2019). Chinese Gen Pract2019; 22: 3910–3914.
21.
ErsbøllASHedegaardMSøndergaardL,et al.Treatment with oral beta-blockers during pregnancy complicated by maternal heart disease increases the risk of fetal growth restriction. BJOG2014; 121: 618–626.
22.
RamzyJNewGCheongA,et al.Iatrogenic anterior myocardial infarction secondary to ergometrine-induced coronary artery spasm during dilation and curettage for an incomplete miscarriage. Heart Lung Circ2015; 24: S189.
23.
CauldwellMCoxMGatzoulisM, et al.The management of labour in women with cardiac disease: need for more evidence?BJOG2017; 124: 1307–1309.
