General Thoracic and Cardiovascular Surgery (2021) 69:1004–1007
https://doi.org/10.1007/s11748-021-01596-3

CASE REPORT

A patient with Marfan’s syndrome who developed an acute aortic
dissection at 28 weeks of pregnancy treated with aortic root
replacement
Riki Sumiyoshi1 · Hideki Morita1 · Kento Fujii1 · Takehiro Shirasughi1 · Hiroyuki Kawaura1 · Masakazu Aoki1 ·Hiroshi Nagano1
Received: 18 July 2020 / Accepted: 14 January 2021 / Published online: 7 February 2021
© The Author(s) 2021

Abstract
The patient was a 31-year-old pregnant woman who gave birth to her first child by vaginal delivery 7 years ago. She wasdiagnosed with Marfan’s syndrome based on physical findings; however, the condition was not diagnosed before the onset.
The patient developed acute aortic dissection at 28 weeks of pregnancy. A cesarean section was first performed to save thepatient’s life; then, a total hysterectomy was performed to prevent the risk of postpartum hemorrhage. Furthermore, aortic
root replacement was performed using a temporary mechanical valve. The patient and her child have survived without any
complications.
Keywords Marfan’s syndrome · Pregnancy · Acute aortic dissection · Aortic root surgery

Introduction

Case reportPregnancy increases the circulating blood volume in thebody by 1.5 times compared with during non-pregnant state,
which increases cardiac output and heart rate [1]. In addition, when aortic tunica media elastic fibers become fragile
due to an increase in hormones during pregnancy [2, 3],aortic dilatation occurs and resistance decreases. These are
fit-for-purpose changes for normal pregnant women; however, in patients with connective tissue diseases, such as
Marfan’s syndrome, medical conditions may worsen, and if
aortic dissection develops during pregnancy, both the motherand child are at high risks.
We performed aortic root replacement on a patient with
Marfan’s syndrome who developed acute type A aortic dissection at 28 weeks of pregnancy, observed a good course,and hereby reported the case.

The patient was a 31-year-old woman in the 28th week ofgestation who had delivered her first child vaginally 7 years
earlier. During pregnancy, there were no abnormalities ineither the mother or infant. The patient presented at her regular obstetrics and gynecology department complaining of
sudden left chest pain. As the infant was confirmed to behealthy, the patient was referred to her regular physician. A
diagnosis of acute type A aortic dissection was made basedon echocardiography and the patient was transferred to ourinstitution by ambulance to undergo surgery.
The patient’s medical history was unremarkable. Her
family history revealed that her biological father had experienced aortic dissection. The patient was 173 cm tall,weighed 61 kg, and was lucid upon arrival at our institution. She had long hands and feet, arachnodactyly of thefingers and toes, limited development of the elbow joints,
skin striae, and pectus carinatum. Auscultation revealed
a to-and-fro murmur. The patient’s blood pressure was
147/80 mmHg, and her heart rate was 80 beats/min.
Electrocardiography showed a sinus rhythm and no ST
changes. A chest X-ray revealed a cardiothoracic ratio of
53% and enlargement of the left first arch. An ultrasoundcardiogram revealed an enlarged aortic root (65 mm) and aflap and false lumen just above the right coronary cusp. The* Riki Sumiyoshi
rikinista2120@gmail.com
1Department of Cardiovascular Surgery, Saitama Red
Cross Hospital, 1‑5 Shintoshin, Chuo‑ku, Saitama‑shi,
Saitama 330‑8553, Japan

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General Thoracic and Cardiovascular Surgery (2021) 69:1004–1007

aortic valve was tricuspid, and severe aortic valve regulation
was observed.
Contrast-enhanced chest and abdomen computed tomography showed a 65-mm-diameter ascending aorta and a69-mm-diameter sinus of Valsalva. There was an initial flap
limited to the ascending aorta and the false lumen was patent(Fig. 1a, b). No dissection was observed beyond the aortic
arch. Pericardial effusion was not observed. The fetus wasconfirmed in the abdominal cavity, and blood vessels werecompressed (Fig. 2).
DeBakey type 2 aortic dissection was diagnosed. Afterconsultation with the obstetrics and gynecology department
at our institution, a decision was made to perform cardiac
aortic surgery after cesarean section and hysterectomy.
Disinfection and draping, as well as an extracorporeal
circuit, were set up to allow for open chest surgery to be performed immediately if the patient’s hemodynamics became
unstable during the cesarean section or hysterectomy. The
cesarean section and total hysterectomy were performed
first. The infant weighed 1173 g with an Apgar score of
2/10 at 5 min. The infant was immediately transferred to theneonatal intensive-care unit. Aortic surgery was performed
after abdominal closure.
The aortic root was dilated in a pyriform manner. Blood
was delivered to the ascending aorta in the non-dissectedarea by the Seldinger method, and extracorporeal circulation
was established with blood removal from the right atrium
via two tubes. A left ventricular vent was inserted, and theascending aorta was occluded when ventricular fibrillationoccurred. The ascending aorta was dissected, and cardiac
arrest was caused by selective antegrade injection of cardioplegic solution. The central temperature was cooled to 20 °C

1005Fig. 2  Compression of the interior vena cava by the uterus and fetus

and circulatory arrest was induced. The aorta was transected
at the center of the brachiocephalic trunk, a prosthetic graft(26 mm Triplex™; Terumo Corporation, Tokyo, Japan) was
anastomosed, and extracorporeal circulation was resumed.
The aortic valve was tricuspid, and the sinotubular junction and sinus of Valsalva were markedly enlarged. The valve
cusp became thin and fragile (Fig. 3); thus, root replacement
was performed. An artificial valve (25 mm SJM Regent;
Abbott, Abbott Park, IL) and a prosthetic graft (30 mmGelweave Valsalva; Vascutek Terumo Inc., Scotland, UK)
were sutured to the aortic annulus. Both the left and rightcoronary arteries were reconstructed using the Carrel patchtechnique.

Fig. 1  a Dilation and dissection of the aortic root. b Initial flap observed in the ascending aorta

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Fig. 3  A thin and fragile aortic valve

The duration of the cesarean section and total hysterectomy was 1 h and 30 min, and the time to transfer to aortic
surgery was 10 min. The duration of aortic surgery was 5 hand 58 min. The extracorporeal circulation time was 3 h and28 min, the aortic cross-clamp time was 2 h and 48 min, andthe circulatory arrest time was 33 min. A blood transfusion
was performed with two units of red blood cells, two unitsof fresh frozen plasma, and 25 units of platelet concentrates.
Postoperatively, the orotracheal intubation was extubatedthe day after the surgery. The patient was transferred to thegeneral ward from the coronary care unit on day 7 after surgery. The patient had an uneventful postoperative course
and was discharged on day 18. The patient is currently being
treated as an outpatient. The infant was discharged on day
86 after delivery without any disabilities.

Discussion
The incidence of aortic dissection in women younger than
40 years of age is 0.4 in 100,000, and approximately halfof these cases are said to be related to pregnancy [4]. The
mortality rate is high, with 21.4% of patients dying before
being seen at a hospital, and mortality rates of 60.7% and
75% on the first and second days after onset, respectively [5].
Between 2010 and 2017, 33 maternal deaths (376 cases) inJapan were attributed to cardiovascular system disorders,with acute aortic dissection accounting for approximately
half of the deaths (15 cases) [6]. In some reports, cesarean
section and aortic surgery were performed in two stages toreduce the risk of hemorrhage. If acute aortic dissection
occurs, the mortality rate within 2 days after onset is so highthat performing aortic surgery later is very risky. Thus, it is13

General Thoracic and Cardiovascular Surgery (2021) 69:1004–1007

preferable to perform surgery for aortic dissection as soon as
possible after cesarean section, unless the patient can safely
wait for surgery.
During pregnancy, approximately 12% of the cardiac
output flows through the uterus and placenta [7, 8], andchildbirth can remove the compression on large vessels
and increase the cardiac output by 60–80% [9]. The uterus
in pregnant women is generally thought to place pressure
on the large blood vessels, thereby adversely affecting
maternal circulation at 20 weeks of gestation or later [10].
At 28 weeks of gestation, the mean fetal weight is 1100 g,
the mean placental weight is 300–400 g [11], and the meanamniotic fluid is approximately 800 g. Aortic surgery is
typically performed first if the patient is hemodynamicallyunstable. However, during hypothermic extracorporeal circulation, the presence of an infant compressing the large
blood vessels and requiring a large volume of circulating
blood could compromise the survival of the mother. The
fetal demise rate under an artificial heart–lung machineis 20% [12]; however, hypothermic circulatory arrest has
been reported to cause increased fetal demise and severe
brain complications [12, 13]. In addition, the neonatal survival rate in Japan is > 80% after 24 weeks [14]. In the caseof this patient, the hemodynamics was steady and there
was time to perform cesarean section first; thus, the fetus
was delivered by cesarean section with maternal lifesaving
as the priority.
Postpartum hemorrhage occurs in 2–11% of all deliveries, and 75% of cases of abnormal bleeding within thefirst 24 h postpartum have been shown to be caused byatonic bleeding [15]. Treatments such as blood transfusion, oxytocin administration, intrauterine balloons, anduterine bimanual compression are usually used. However,if this occurs during open-heart surgery, it can be fatal
as manual treatment cannot be performed and heparin isbeing administered, thereby promoting bleeding. Thus, wedecided that the safest option would be to perform total
hysterectomy to prevent atonic bleeding. In this case, thebaby was the second child and there was a good chance forthe baby to be saved after delivery; therefore, the patient
did not wish for future pregnancies and agreed to undergo
hysterectomy. Even if the patient hoped for future pregnancies, as it is extremely difficult to save lives if atonic
hemorrhage occurs during an open-heart surgery, it wassafer to gain the family’s understanding and perform a
total hysterectomy for saving the mother’s life.

General Thoracic and Cardiovascular Surgery (2021) 69:1004–1007

Conclusion
We performed a cesarean section, a total hysterectomy, andan aortic root replacement in one go on a pregnant patientwith Marfan’s syndrome who developed acute aortic dissection at 28 weeks of pregnancy. Both the mother and childwere saved and had a good course.
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References
1. Greutmann M, Pieper PG. Pregnancy in women with congenital
heart disease. Eur Heart J. 2015;36:2491–9.
2. Shinji K, Keiko U, Kaoru Y, et al. Pregnancy-associated aortic
dilation or dissection in Japanese women with marfan syndrome.
Jpn Circ J. 2011;75:2546–51.
3. Perrucci GL, Rurali E, Gowran A, et al. Vascular smooth muscle
cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall. Cell Mol Life Sci. 2017;74:267–77.
4. Thailmann M, Sodeck GH, Domanovits H, et al. Acute type A
aortic dissection and pregnancy: a population-based study. Eur J
Cardiothorac Surg. 2011;39:e159-163.

1007
5. Lei Y, Jiang Z, Chen S, et al. Type A aortic dissection in pregnant
patients with fibrillin-1 gene mutation: two case reports and aliterature review. Exp Ther Med. 2018;16:4407–14.
6. Sekizawa A, Hasegawa J, et al. Saving mothers’ living in Japan
2020; p. 262.
7. Flo K, Wilsgaard T, Vartun A, et al. A longitudinal study of therelationship between material cardiac output measured by impedance cardiology and uterine artery blood flow in the second halfof pregnancy. BJOG Int J Obstet Gynaecol. 2010;117:837–44.
8. Thaler I, Manor D, Itskoviyz J, et al. Changes in uterine blood flow
during human pregnancy. Am J Obstet Gynecol. 1990;162:121–5.
9. Christina CH, Jennifer P. Trauma and surgical emergencies in theobstetric patient. Surg Clin North Am. 2008;88(2):421–40 (viii).
10. Rossi A, Cornette J, Johnson MR, et al. Quantitative cardiovascular magnetic resonance in a pregnant woman: cross-sectional
analysis of physiological parameters throughout pregnancy andthe impact of the supine position. J Cardiovasc Magn Reson.
2011;13:31.
11. Nakai A, Ogawa M, Sato M, et al. Subcommittee on the comprehensive review of factors associated with low-wide-wage infants.
Acta obstet et gynaecol Japonica. 2015;67(6):1527–35.
12. Marumoto A, Nakamura Y, Harada S, et al. Acute aortic dissection at 33 weeks of gestation with fetal distress syndrome.
GTCVS. 2011;59:566–8.
13. Sakaguchi M, Kitahara H, Seto T, et al. Surgery for acute A aortic dissection in pregnant patients with Marfan syndrome. Eur J
Cardiothorac Surg. 2005;28:280–5.
14. Yamabi H, Inui A, Matsuoka T, et al. Acute type A aortic dissection during late pregnancy period in a patient with Marfan’ssyndrome. J Jpn Soc Cardiovas Surg. 2019;48:425–7.
15. Montufar-Rueda C, Rodriguez L, Douglas-Jarquin J, et al. Severe
postpartum hemorrhage from uterine atony: a multicentric study.
J Pregnancy. 2013;2013:525914.
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