Pregnant patients with Marfan syndrome (MFS) are at high risk of developing aortic dissection or rupture during the third trimester and early postpartum period. This increased likelihood is the consequence of the hyperdynamic and hypervolemic cardiocirculatory state and/or pregnancy-mediated structural changes of the arterial wall in response to hemodynamic and hormonal changes. In this article, we report on the case of a 26-year-old pregnant woman with MFS in the 30th gestation w

Kostadinovska, B. (Beti)

Milojevic, M. (Milan)

Nikolic, A. (Aleksandar)

Slaveski, D. (Dimche)

Pregnant patients with Marfan syndrome (MFS) are at high risk of developing aortic dissection or rupture during the third trimester and early postpartum period. This increased likelihood is the consequence of the hyperdynamic and hypervolemic cardiocirculatory state and/or pregnancy-mediated structural changes of the arterial wall in response to hemodynamic and hormonal changes. In this article, we report on the case of a 26-year-old pregnant woman with MFS in the 30th gestation week, who presented with type A aortic dissection at the emergency department. According to the multidisciplinary team decision, an urgent cesarean section was performed, followed by the Bentall procedure. The patient was discharged on the 10th postoperative day, and her premature child was discharged 6 weeks after birth

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J Card Surg. 2019;1–4. wileyonlinelibrary.com/journal/jocs © 2019 Wiley Periodicals, Inc. | 1DOI: 10.1111/jocs.14408CA S E R E POR TAcute aortic dissection in a patient with Marfan syndromeduring advanced pregnancyBeti Kostadinovska MD, MSc1 | Aleksandar Nikolic MD, PhD1 | Dimche Slaveski MD1 |Milan Milojevic MD, PhD1,21Department of Cardiac Surgery, AcibademSistina Hospital, Skopje, North Macedonia2Department of Cardiothoracic Surgery,Erasmus University Medical Center,Rotterdam, The NetherlandsCorrespondenceMilan Milojevic, MD, PhD, Department ofCardiothoracic Surgery, Erasmus UniversityMedical Center, Dr. Molewaterplein 40, 3015GD, P.O. Box 2040, 3000 CA, Rotterdam,The Netherlands.Email: m.milojevic@erasmusmc.nlAbstractPregnant patients with Marfan syndrome (MFS) are at high risk of developing aorticdissection or rupture during the third trimester and early postpartum period. Thisincreased likelihood is the consequence of the hyperdynamic and hypervolemiccardiocirculatory state and/or pregnancy‐mediated structural changes of the arterialwall in response to hemodynamic and hormonal changes. In this article, we report onthe case of a 26‐year‐old pregnant woman with MFS in the 30th gestation week, whopresented with type A aortic dissection at the emergency department. According tothe multidisciplinary team decision, an urgent cesarean section was performed,followed by the Bentall procedure. The patient was discharged on the 10thpostoperative day, and her premature child was discharged 6 weeks after birth.K E YWORD Saortic dissection, Marfan syndrome, pregnancy1 | INTRODUCTIONAortic dissection during pregnancy is unusual and particularlycatastrophic, with an estimated maternal prehospital mortality ofapproximately 50% and operative mortality of up to 30%. Theperioperative fetal mortality rate is even higher and depends on fetalviability.1,2 According to results from the Nationwide Inpatient SampleDatabase, aortic dissection has an average incidence of 0.44 cases per100 000 pregnant women and represents around 0.1% of aorticdissection cases.3 In addition to this, results from the 55 referralcenters participating in the International Registry of Acute AorticDissection4 showed higher rates of aortic dissection in pregnancy ofnearly 0.4% of all cases. Furthermore, the available data suggest thatroughly half of the aortic dissections in women younger than 45 yearsoccur during pregnancy, and more than 50% of these women wereaffected with the Marfan syndrome (MFS).5 Aortic dissection mayoccur at any time during pregnancy, but it is most frequent in the thirdtrimester (50%), followed by the postpartum period (20%) and duringlabor (15%).6 A review of the current literature shows a substantialdifference in the management of acute aortic syndrome duringpregnancy (Table 1). Therefore, we present the case of successfulsurgical treatment of a 26‐year‐old pregnant woman with an acutetype A aortic dissection at 30 weeks of gestation.2 | CASE REPORTA 26‐year‐old patient (gravida 1, para 0) in the 30th gestation weekwas referred to the emergency department with a sudden onset ofsevere anterior chest pain associated with dyspnea. According toinformation collected from the patient and a family member, she hadregular antenatal check‐ups and no history of severe illness oraccidents. At presentation, the patient had all the clinical featuresneeded to raise a suspicion of MFS. She had a positive family historyof aortic disease and sudden death, and her brother underwentgenetic testing, which confirmed the diagnosis of MFS.Emergency echocardiography showed a type A aortic dissectionwith an entry tear and intimal flap of 14 to 16mm above the rightcoronary artery ostium, resulting in a massive false lumen in theascending aorta (Figure 1). The diameter of the ascending aorta was51mm, left ventricular ejection fraction (LVEF) was preserved, andthere was severe aortic valve regurgitation with no sign ofTABLE1SummaryofcaseseriesStudyNo.ofpatientsPopulationGestationTreatmentreceivedMaternalmortalityFetalmortalityMatloffetal.1970250%(1/2)withMFS30thwkandpostpartum(MFS)●Onepatientunderwentaorticsurgerywithoutcesareansection0%50%●OnepatientunderwentaorticsurgerySniretal.198820%(0/2)withMFS34thwkandpostpartum●Onepatientunderwentaorticsurgerywiththefirstcesareansection0%0%●OnepatientunderwentaorticsurgeryWilliamsetal.198820%(0/2)withMFS36thwkand32ndwk●Twopatientsunderwentaorticsurgerywiththefirstcesareansection0%0%Rossiteretal.19952100%(2/2)withMFS17thwkand31stwk●Onepatientunderwentaorticsurgerywithoutcesareansection0%50%●OnepatientreceivedconservativetreatmentZeebregtsetal.1997633%(2/6)withMFSMFS:32ndwk,35thwk●Twopatientsunderwentaorticsurgerywiththefirstcesareansection0%50%Non‐MFS:24thwk,29thwk,37thwk,22ndwk●Threepatientsunderwentaorticsurgerywithoutcesareansection●OnepatientreceivedconservativetreatmentPacinietal.20097100%(7/7)withMFS5thmon,7thmon,3pts8thmon,2ptspostpartum●TwopatientsunderwentaorticsurgerywiththefirstcesareansectionNRNR●Threepatientsunderwentaorticsurgerywithoutcesareansection●TwopatientsreceivedconservativetreatmentYangetal.20151145%(5/11)withMFSMFS:10thwk,12thwk,33rdwk,36thwk,andpostpartum●Twopatientsunderwentaorticsurgerywiththefirstcesareansection73%82%●EightpatientsunderwentaorticsurgerywithoutcesareansectionNon‐MFS:37thwkand4ptspostpartum●OnepatientreceivedconservativetreatmentZhuetal.20172568%(17/25)withMFS1sttrimester2pts,2ndtrimester9pts,3rdtrimester9pts,andpostpartum5pts●16patients:aorticsurgerywiththefirstcesareansection16%40%●Sevenpatientsunderwentaorticsurgerywithoutcesareansection●TwopatientsreceivedconservativetreatmentAbbreviations:MFS,Marfansyndrome;NR,notreported;pts,patients;wk,week.2 | KOSTADINOVSKA ET AL.tamponade (Figure 2). Subsequently, the ultrasound examinationshowed a viable fetus with a crown‐rump length corresponding to the30th week of gestation, cephalic presentation, and weight around1700 g. Cardiotocography indicated irregular uterine contractionsand a fetal heart rate of 140 bpm with moderate variability. Theobstetric team reported that the fetus was stable and viable to adaptto extrauterine life in the neonatal intensive care unit.A multidisciplinary team including a cardiac surgeon, cardiacanesthesiologist, gynecologist, and pediatrician discussed the variousmodalities of treatment, and the decision was made to perform acesarean section followed by cardiac surgery. Immediately after obtaininginformed consent for both procedures, the patient was taken to theoperating theater. The emergency cesarean section was performed undergeneral anesthesia, using transesophageal echocardiography (TEE) forperioperative monitoring and fluid management (Figure 3). A male infantwas born and intubated after 2minutes of external stimulation due to afailure to initiate spontaneous breathing. He was transferred to theneonatal intensive care ward and placed on mechanical ventilation. Afterthe cesarean section, a modified Bentall procedure was performed usingcomposite aortic graft with a biological valve prosthesis utilizing Cabrolcoronary reimplantation. The decision was made to use a biologicalvalved conduit despite the age of the patient due to her wish to become aparent again. Once rewarming of the patient was completed, weaningfrom cardiopulmonary bypass (CPB) was accomplished uneventfully. TheTEE confirmed a well‐functioning aortic prosthesis without anyparavalvular leakage and good LVEF. On the sixth postoperative day, acomputerized tomography angiogram was performed to confirm the typeof aortic dissection (Stanford A, DeBakey II) (Figure 4). No otheraneurysmal changes were observed in the descending and abdominalaorta. The patient was discharged from the hospital on the 10thpostoperative day in a good general condition. The newborn wasdischarged home 6 weeks after birth.3 | DISCUSSIONThe reported rate of aortic dissections in pregnant patients with theMFS is around 5%, with a 50% chance of a baby being born with theMFS. The risk of aortic dissection is especially high in women with anaortic root diameter greater than 40mm before pregnancy. AlthoughF IGURE 1 Dilated aortic root and intimal flap. Ao, aorta; LV, leftventricleF IGURE 2 Intimal flap ends at the beginning of the truncusbrachiocephalicus. Ao, aortaF IGURE 3 Transesophageal echocardiographic mid‐esophageallong‐axis images of the aortic valve showing aneurysmal dilatation ofthe aortic root and ascending aorta and intimal dissection flap in theascending aortaF IGURE 4 Computerized tomography angiography of a patientwith DeBakey II aortic dissectionKOSTADINOVSKA ET AL. | 3general risk factors for cardiovascular disease increase a woman’s riskfor developing aortic dissection during pregnancy, the hyperdynamic andhypervolemic cardiocirculatory state and/or pregnancy‐mediated struc-tural changes of the arterial wall in response to hemodynamic stressesand hormonal changes seem to be the main pathophysiological driversfor aortic dissection. Given this risk and that of other cardiovascularcomplications, American and European Guidelines on the managementof cardiovascular disease during pregnancy strongly recommend thatpregnant women with MFS should be monitored by echocardiography at4‐ to 12‐week intervals throughout pregnancy and at 6 monthspostpartum. The use of beta‐blockers to control heart rate and deliveryin‐hospital with the cardiac surgery department onsite is also highlyrecommended. Despite all physical features and positive family history,unfortunately, our patient was not diagnosed with MFS, and the aboveprevention measures were not followed. If a dissection occurs, it is apotentially fatal event for both the mother and the unborn child thatrequires urgent surgical intervention. Ideally, surgical interventions foraortic dissection should minimize fetal risk without compromising thesafety of the mother. Unfortunately, the fetoplacental response to CPBis complex, and fetal loss occurs in nearly 30% of cases. An appreciationof the risks related to gestational age, including the factors that mayinfluence the survival of the mother and fetus, and close cooperationbetween a maternal‐fetal team and a cardiac surgical team are keycomponents of any strategy used to achieve better outcomes.The survival rate without severe disabilities among prematurebabies has markedly increased during the past two decades.In contemporary practice, once the fetus reaches week 25 ofgestation, cesarean section, and other active neonatal andobstetric measures are widely used.7 Given the low risk of thecesarean section when the mother is in a clinically stablecondition, an urgent delivery followed by aortic surgery shouldbe the treatment of choice for all women with aortic dissectionin the third trimester of pregnancy (28 weeks). In somecircumstances, this is now achievable even earlier, dependingon the viability of the fetus. Certainly, this approach provides thebest chance of survival for the unborn child and the mother.4 | CONCLUSIONPregnancy in women with MFS is associated with a substantialrisk of maternal and fetal complications. Although aorticdissection is uncommon, it is not rare. When it does occur andthe fetus is viable, delivery by cesarean section followed byimmediate open surgical repair is the current treatment of choice.Timely diagnosis and a multidisciplinary approach are mandatoryto achieve satisfactory results in pregnant women affected byaortic dissection.CONFLICT OF INTERESTSThe authors declare that there are no conflict of interests.ORCIDMilan Milojevic http://orcid.org/0000-0003-1948-9179REFERENCES1. Thalmann M, Sodeck GH, Domanovits H, et al. Acute type A aorticdissection and pregnancy: a population‐based study. Eur J CardiothoracSurg. 2011;39(6):e159‐e163.2. Elassy SM, Elmidany AA, Elbawab HY. Urgent cardiac surgery duringpregnancy: a continuous challenge. Ann Thorac Surg. 2014;97(5):1624‐1629.3. Sawlani N, Shroff A, Vidovich MI. Aortic dissection and mortalityassociated with pregnancy in the United States. J Am Coll Cardiol.2015;65(15):1600‐1601.4. Braverman AC, Harris K, Pyeritz R, et al. Aortic dissection duringpregnancy: results from the International Registry of Acute AorticDissection (IRAD). J Am Coll Cardiol. 2012;59(13 suppl):E1903.5. Immer FF, Bansi AG, Immer‐Bansi AS, et al. Aortic dissection inpregnancy: analysis of risk factors and outcome. Ann Thorac Surg. 2003;76(1):309‐314.6. Wanga S, Silversides C, Dore A, de Waard V, Mulder B. Pregnancy andthoracic aortic disease: managing the risks. Can J Cardiol. 2016;32(1):78‐85.7. Patel RM, Rysavy MA, Bell EF, Tyson JE. Survival of infants born atperiviable gestational ages. Clin Perinatol. 2017;44(2):287‐303.How to cite this article: Kostadinovska B, Nikolic A,Slaveski D, Milojevic M. Acute aortic dissection in a patientwith Marfan syndrome during advanced pregnancy. J CardSurg. 2019;1–4. https://doi.org/10.1111/jocs.144084 | KOSTADINOVSKA ET AL.

Acute aortic dissection in a patient with Marfan syndrome during advanced pregnancy

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Acute aortic dissection in a patient with Marfan syndrome during advanced pregnancy

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