Available via license: CC BY-SA 4.0
Content may be subject to copyright.
Case Report
UNIVERSITAS PELITA HARAPAN 17
Anesthetic Challenges for Modified Bentall Procedure in a Pregnant
Marfan Patient with Acute Stanford Type A Dissection
Irma L. Tantri MD1, Theresa Menor MD2, DPBA, Nelia Laborte MD, DPBA2
1Department of Anesthesiology Faculty of Medicine, Pelita Harapan University
2Department of Anesthesiology, St. Luke’s Medical Center
Abstract
Aortic dissection is a life-threatening condition which requires immediate surgical intervention. It has
been estimated that half of aortic dissection and/or ruptures in women younger than 40 years of age
have been associated with pregnancy.19 The limited data on the coincidence of Marfan syndrome,
pregnancy and aortic dissection makes its anesthetic management a formidable challenge to any
anesthesiologist.
This is a case of a 33-year-old G1P0, 28 weeks age of gestation with Marfan syndrome, who had
Aortic Dissection Stanford type A and underwent an emergency Modified Bentall’s surgery with
cardiopulmonary bypass and deep hypothermic circulatory arrest.
This case illustrates the dilemma of managing this case since there are two patients involved, mother
and fetus. Wrong decision could result in demise of both. Although the aim is life for both, survival of
one without neurologic sequelae is already considered a success.
Keywords: Marfan syndrome, Modified Bentall Procedure, Pregnancy, Acute type A dissection
Abstrak
Diseksi aorta adalah kondisi yang mengancam jiwa yang memerlukan intervensi bedah segera.
Diperkirakan bahwa setengah kasus dari diseksi aorta dan / atau ruptur aorta pada wanita yang lebih
muda dari 40 tahun berhubungan erat dengan kehamilan. 19 Keterbatasan data mengenai manajemen
anestesi pada pasien hamil dengan sindrom Marfan yang mengalami diseksi aorta merupakan tantangan
tersendiri pada seorang ahli anestesi .
Berikut ini adalah laporan kasus seorang pasien hamil G1P0 berusia 33 tahun, usia kehamilan 28
minggu dengan sindrom Marfan, yang memiliki Diseksi aorta Stanford tipe A dan menjalani operasi
darurat Bentall's Modifikasi dengan bypass kardiopulmonari dan deep hypothermic circulatory arrest.
Laporan ini menggambarkan dilema yang timbul dalam pengelolaan kasus dimana terdapat dua pasien
yang terlibat, ibu dan janin. Keputusan yang salah bisa mengakibatkan kematian keduanya. Meskipun
tujuannya adalah untuk kelangsungan hidup ibu dan janin, kelangsungan hidup salah satu pasien tanpa
gejala sisa neurologis merupakan suatu keberhasilan.
Kata kunci : sindroma marfan, Prosedur Bentall, Kehamilan, Diseksi aorta tipe A akut
Introduction
Acute aortic dissection of the ascending aorta
(Stanford type A) is highly lethal. Forty percent
of patients who reach the emergency department
die immediately. This mortality rate is increased
by 1% per hour in the first 48 hours and between
5%-20% die during or shortly after the
surgery.2,24,32
-----------------------------------------------------------------------------
Irma. L. Tantri ( )
Faculty of Medicine Universitas Pelita Harapan
Jl. Boulevard Jend.Sudirman, Lippo Karawaci, Tangerang,
Indonesia. Tel: +62-21-54210130; Fax: +62-21-54210133;
Email: irma.tantri@uph.edu
Early death may occur as a result of malperfusion
syndromes (cerebrovascular, visceral, renal, or
peripheral ischemia), cardiac complications
(acute aortic insufficiency, coronary ischemia,
cardiac tamponade), or free rupture.17 Surgery,
which aims to prevent aortic rupture, cardiac
complications and other end-organs damage, is
the definitive treatment for patient with type A
acute aortic dissection.
Deep hypothermic circulatory arrest (DHCA) is
technique required in cases of acute aortic
dissection or aneurysms that extend into the
aortic arch.49
pISSN: 1978-3094 . Medicinus. 2018; 7 (1) : 17-26
ANESTHETIC CHALLENGES
18 UNIVERSITAS PELITA HARAPAN
Surgical replacement of the aortic valve and
surgery of the aortic aneurysm or dissection that
extend into the aortic arch carry risk of neurologic
injury from global ischemia or embolization of
atherosclerotic debris secondary to clamping of
the great vessels.5 Surgery of descending aorta is
associated with postoperative paraplegia
secondary to interruption of 13 to 17% of blood
supply of the spinal cord.5
This case report will show how neurologic
sequelae was prevented during anesthesia for
modified Bentall procedure to a pregnant Marfan
patient with acute type A dissection.
Case summary
This is a case of a 33-year-old primigravid, 28
weeks age of gestation (AOG) with Marfan
syndrome, who consulted due to sudden chest
discomfort with radiation to the left jaw
associated with burning substernal chest pain and
diaphoresis.
Past medical history showed that the patient had
hiatal hernia and gastroesophageal reflux disease
(GERD) and was on esomeprazole tab as needed.
She had no previous surgery. Prenatal check up
was not clear.
Family history showed that her mother and
brother have Marfanoid features. Physical
examination revealed a conscious, coherent
patient who arrived via stretcher. She weighs 60
kg and stands160 cm. Her vital signs were as
follows: blood pressure (BP) 106/69 mmHg on
both upper extremities, 110/71 on both lower
extremities, heart rate (HR) 97-100 bpm,
respiratory rate (RR) 20 per minute. Airway
examination revealed mallampati I classification
with good neck movement. Chest examination
revealed a pectus carinatum deformity. Her
abdomen was globular. Fetal heart tone was
appreciated at 150 beats per minute.
Cephalic presentation was palpable. No uterine
contractions were noted. Internal examination
revealed a soft and close cervix. Her arm span of
174 cm with positive wrist and thumb sign were
noted. Hindfoot deformity was also observed.
Good peripheral pulses were appreciated.
Neurologic examination revealed intact cranial
nerves, normal motor strength, no tingling nor
numbness, normal response of tendon reflexes
with no pathologic reflexes found.
Diagnostic workups revealed anemia with
leucocytosis, neutrocytosis and hypocalcemia
(see Appendix A). Carotid duplex scan showed >
50% stenosis in the left common carotid artery.
Positive troponin I indicates myocardial necrosis.
Transesophageal echocardiography (TEE)
showed a hypocontractile free walls of the right
ventricle. An echocardiography showed aortic
dissection confirmed by magnetic resonance
angiography (MRA) which showed aortic
dissection Stanford type A (Fig. 1A & 1B).
Patient was diagnosed to have aortic aneurysm
and dissection Stanford type A with carotid
stenosis and right coronary artery disease, Marfan
syndrome, G1P0 28 weeks AOG cephalic, not in
labor. She was classified as ASA class III-E.
Modified Bentall’s procedure with double set up
for possible obstetrical intervention was
contemplated. Risks and benefits were explained
to patient and relatives. The risk of demise for
both the patient and fetus was not eliminated.
Survival of patient but with neurologic and
nephrologic sequelae was also a possibility.
There was no immediate indication for an
emergency caesarian section and fetal demise can
occur. The risks were explained by all specialists
— obstetrician, cardiologist, thoracic,
cardiovascular surgeon, intensivist, neonatologist
and anesthesiologist. Patient and relatives
accepted risks. Urgent modified Bentall
procedure was scheduled.
MEDICINUS Vol. 7 No. 1 Oktober 2017 – Januari 2018
UNIVERSITAS PELITA HARAPAN 19
Figure 1A&B. MRA of the chest/thoracic (9/29/10) showed aortic aneurysm with dissection at the level of proximal
ascending aorta down to the visualized common iliac arteries.
Patient was given betamethasone 12 mg IM and
cefuroxime IV prior to the surgery. Packed RBC,
thawed fresh frozen plasma, platelet concentrate
and fresh whole blood were prepared for use
during surgery. Proton pump inhibitor and
dexamethasone 15 mg iv in a gauge 16 peripheral
IV line were administered during preinduction
period.
Monitors placed were as follows: temperature
probe on the nasopharynx, 5 leads-ECG, end tidal
CO2, arterial line at right radial artery and left
brachial artery, and pulmonary artery catheter
inserted in the right internal jugular vein. Urethral
catheter was inserted to monitor the urine output
hourly.
Initial vital signs were as follows: BP 165/60
mmHg, HR 105 bpm and pulmonary arterial
pressure (PAP) 25/19 mmHg (normal PAP is 15-
28/ 5-16mm Hg).52
Initial arterial blood gas showed a metabolic
acidosis (see Appendix A) and was corrected
with the NaHCO3 administration. Nitroglycerin
drip was started during induction period to
achieve hemodynamic stability during intubation
and to improve coronary blood flow.
Anesthetics administered were as follows:
fentanyl 250µg followed by infusion, midazolam
1 mg, propofol infusion, rocuronium 50 mg
followed by infusion and sevoflurane titrated
between 1-3%. Patient was intubated with 7.5
size ETT under direct visualization of vocal
cords. She was hooked to ventilator with the
setting as follows: volume controlled ventilation,
tidal volume 450 ml, RR 14 per minute, IE ratio
1:2 and FiO2 1.
Transesophageal echocardiography (TEE) done
after induction showed a dilated aortic root of 44
mm x 38 mm (normal < 37mm)41 and proximal
ascending aorta 35 mm x 27 mm (normal is 30
mm)21 with dissection of the posterior aortic wall
from the aortic root to the descending thoracic
aorta of 32 mm (normal 18 – 22 mm)21 with
thrombus formation at the false lumen. There was
also moderate to severe aortic regurgitation with
mild mitral and tricuspid regurgitation.
Citicholine 1 gm, tranexamic acid 2 g were
administered. Nitroglicerine drip, low dose
dopamine drip and blood transfusion were
started. Median sternotomy commence 29 hours
from onset of symptoms or five hours from
arrival at the emergency department. Heparin at
3 mg/kg was administered. Activated coagulation
time (ACT) level of 375 was achieved before
cannulation of the axillary artery, femoral artery
and right atrium. ACT determination was done
every 30 minutes to maintain a level above 350.
Surgery proceeded with a period of cardiac arrest
by infusion of cardioplegic solution into the
aortic root. Deep hypothermia at 20°C was
achieved by infusion of cold blood using CPB,
application of blanket roll and ice pack over the
head. After the core temperature of 20C was
achieved, innominate artery was clamped.
ANESTHETIC CHALLENGES
20 UNIVERSITAS PELITA HARAPAN
Blood cardioplegic solution was given in
retrograde manner to facilitate the aortic root
replacement with a composite valve graft.
During this period of time, antegrade cerebral
perfusion technique was used to provide blood
flow to the brain. Perfusion to other organs was
also preserved through femoral artery
cannulation Rewarming of the temperature to
28°C was then initiated and maintained during
completion of the coronary artery bypass of the
right main coronary artery using a distal
saphenous vein graft.
All anesthetic agents were given by continuous
intravenous infusion. Total DHCA time,
clamped/ischemic time and CPB time were 24
minutes, 155 minutes and 190 minutes
respectively.
After rewarming to 37°C, the bypass was
gradually terminated. Heparin reversal with
protamine was administered intravenously.
The post cardiopulmonary bypass course was
uneventful. The PAP was maintained at 17-
30/15-20 mmHg, ejection fraction was 69%
compared to 63% preoperatively.
The modified Bentall surgery lasted 7 hours.
Total blood loss was 1000 cc (maximal allowable
blood loss was 252 cc) with total urine output of
1350 cc. Blood products transfused were PRBC
of 4 units, FFP of 3 units and platelet concentrate
of 5 units.
She was maintained on mechanical ventilator for
six hours at the post anesthesia care unit (PACU).
Postoperative fetal heart tones could not be
appreciated. Pelvic ultrasound showed fetal
demise. Expectant management for the
intrauterine fetal demise (IUFD) was the best
option at this time.
Patient was transferred to coronary care unit
sedated but arousable with pain controlled by
tramadol infusion and later by oral combination
of paracetamol 325 mg and tramadol 37.5 mg.
On 2nd post op day, left ventricular dysfunction
was noted on 2D echo with dyskinesia on the
entire interventricular septum and hypokinesia on
the anterior left ventricle (LV) free wall with best
contraction at the antrolateral and inferolateral
LV free wall (EF 33% by Tiechholz and 49% by
Simpson’s).
Dobutamine drip 5-15 mcg/kg/min was started
and titrated. Fondaparinux 2.5 mg was also
administered subcutaneously as an
antithromboembolic agent. Other management
includes general liquid diet progressive heart
delight, humulin R sliding scale, calcium 600 mg,
Vit D 400 IU tablet p.o. and potassium chloride
tab 750 mg p.o. Neurologic examinations
revealed same as preinduction.
Repeat 2D echo on 4th post op day showed an
improvement in LV function (EF 51% by
Simpson’s) and wall motion (hypokinesia of
entire septum).
She stayed at the coronary care unit for 6 days.
No evidence of neurologic, nephrologic deficit,
pulmonary and cardiac problems was noted. .
Patient was discharged with improvement and
preferred to be transferred to another hospital for
delivery of the fetus.
Discussion
We are presented with a patient with aortic
aneurysm with dissection type A. This requires
immediate surgical intervention because of 50%
risk of rupture.2,24,32
The presence of aortic dissection and systemic
features with score ≥7 made our patient fulfill
criteria of having Marfan syndrome following the
2010 Revised Ghent nosology criteria (see
Appendix B). The aortic dissection in Marfan’s
syndrome patient usually manifests during
pregnancy which happened in this case.
Type A dissection, meaning dissection involving
the ascending aorta (Fig. 2) present in the patient,
carries the risk of neurologic sequelae when the
aorta is clamped.
MEDICINUS Vol. 7 No. 1 Oktober 2017 – Januari 2018
UNIVERSITAS PELITA HARAPAN 21
Figure 2. Type A aortic aneurysm and dissection at the level of the proximal ascending down to the common iliac
arteries (left) and intimal flap from proximal ascending aorta (right)
In addition, the presence of more than 50 %
stenosis of the left carotid artery, though not yet
an indication for carotid endarterectomy, also
added to the risk of cerebral ischemia. Thus,
cardiopulmonary bypass and deep hypothermic
circulatory arrest techniques, defined as the use
of systemic hypothermia (core temperature of 14-
20°C)3 and the intentional cessation of the
circulation for periods up to 60 minutes30, were
employed by placing the patient at 20°C to reduce
the cerebral metabolic rate and oxygen
consumption, and thus minimize the
complication of cerebral ischemia.
Recurrence of aortic dissection in this patient is
likely to happen especially during subsequent
pregnancy.19,23 Thus, grafting of the aortic
dissection will not be enough. There should also
be an aortic valve replacement and reimplantation
of the left coronary ostium with a long interposed
graft wrapping behind the grafting of the aortic
dissection.38
The type A aortic dissection which also disrupted
the right coronary artery blood flow (Fig. 3)
required the patient to undergo coronary artery
bypass graft in addition to the aortic dissection
surgery and aortic valve replacement. Thus, the
extension of the duration of the surgery is
unavoidable.
Figure 3. Disruption of the right coronary blood flow (left) and injury to the aortic valve due to aortic dissection
causing moderate-severe aortic regurgitation (right)
ANESTHETIC CHALLENGES
22 UNIVERSITAS PELITA HARAPAN
However, the problems did not end there. The
patient was also a primi gravid at 28 weeks AOG.
Her prenatal check up and work-ups were not
available. Referral to the Obstetric department
could not find any indication for elective
caesarian section at that time. The patient was
not in labor. The fetal lung was expected to be
immature as surfactant would be released at 30
weeks AOG, at which time could be detected in
amniotic fluid.42 Forty eight hours of steroids
(dexamethasone every 12 hours for 4 doses or
bethametasone every 24 hours for 2 doses) should
be administered to accelerate fetal lung
maturation.11 However, the emergent nature of
the surgical condition could not afford to wait 48
hours. Aspiration of amniotic fluid to detect the
presence of surfactant at 28 weeks AOG seemed
to have no beneficial effect.42
If elective caesarian section was done in a
preterm patient who is not in labor and with very
low birth weight fetus with immature lung, the
chance of survival of the fetus would be less than
50%.10 Bleeding could be expected from the
uterus during the caesarean section in full term
pregnancy with blood loss as much as 500 ml –
1000 ml in 75% of women having a caesarean
section in the Philippines and more than 1000 ml
in 4% of that population.18 Furthermore, the life
of the post partum patient would be greatly
endangered if the patient would undergo aortic
surgery wherein anticoagulants would be
administered.
The patient’s condition would have more than
45% risk of mortality if not immediately
operated.24 An increase of 1-2% mortality was
expected for every hour delay of surgery from
onset of symptoms.24
The risks and benefits were explained to the
patient and the relatives. The family and the
physicians agreed to do the aortic surgery to save
the life of the pregnant patient without any
neurologic sequelae with the hope that the fetus
would be able to survive the insult caused by the
medical and surgical conditions.
The worst possible scenarios were also explained
to the relatives. The worst possibility was that the
patient and the fetus might not be able to survive
the surgery. Another possibility was the patient
might survive the surgery but with neurologic
sequelae or with kidney problem or with lower
extremity paralysis secondary to the clamping of
the aorta, carotid stenosis and prolonged surgery.
Yet, the more expected possibility was the patient
might survive but the fetus might not.
Since the patient was brought to the emergency
department 24 hours from onset of symptoms, the
patient was immediately operated five hours after
admission or 29 hours after onset of symptoms.
TEE was done after induction of GEA to
determine the final surgery. Ascending aortic
replacement, total arch replacement with
reattachment of brachiocephalic branches with
modified Bentall procedure (the replacement the
aortic root and reimplantation the left coronary
artery) were chosen to minimize the risk of the
recurrence of the later-onset aneurysms and
dissection of the aorta which commonly
encountered in Marfan syndrome.23
The modified Bentall technique, which was
reported by Svensson in 1992, is an aortic root’s
replacement using a composite valve graft
(bileaflet mechanical valve attached to a
polyester tube graft) involving a left coronary
ostium’s reimplantation using Kochoucous’s
button modification by leaving button’s of aortic
wall surrounding both coronary arteries, which
are then mobilized and sutured to the aortic graft
(Fig 4).38, 48
Standard cardiopulmonary bypass (heart lung
machine) and deep hypothermic circulatory arrest
were achieved to facilitate the aortic dissection
repair, valve replacement and CABG. Usual
cannulation was impossible to use due to the
location of the aortic dissection, thus axillary and
femoral arteries were used for site of cannulation
to maintain blood flow to the brain and other
organs.
The patient was extubated six hours post surgery
without any neurologic sequelae.
MEDICINUS Vol. 7 No. 1 Oktober 2017 – Januari 2018
UNIVERSITAS PELITA HARAPAN 23
Figure 4. Illustration of the modified Bentall procedure for aortic root replacement, during which buttons of aortic
wall surrounding coronary arteries are mobilized and sutured to the aortic graft. (Reprinted from Schwartz’s
Principles of surgery, 8th edition, 2004)
However, fetal heart tone could no longer be
appreciated post operatively. Thus, though the
difficult CABG and aortic surgery can be
considered a success, the demise of the fetus
intrautero made its success not in toto.
Aware of the all the consequences that might
occur, the patient and the relatives appreciated the
effort of the team who was able to prevent the
worst possibilities that might occur to the patient.
Conclusion
The anesthetic management for modified bentall
procedure in a pregnant patient with Acute Type
A dissection is very challenging since there are
two patients involved, mother and child. The
additional risk for higher maternal morbidity
which related to Marfan syndrome also made the
nature of the event more challenging for any
anesthesiologist.
Although the aim is life for both, survival of one
without neurologic sequelae is already
considered a success.
References
1. Akashi H, Tayama K, Fujino T, Onitsuka S, Sakashita H, Aoyagi S. Surgical treatment for acute type
A aortic dissection in pregnancy: a case of aortic root replacement just after cesarean section. Jpn Circ
J. 2000;64:729–730.
2. Anagnostopoulos CE, Prabhakar MJ, Kittle CF. Aortic dissections and dissecting aneurysms. Am J
Cardiol. 1972;30:263–73.
3. Andropoulos D et. al. Anesthesia for congenital heart disease 2nd ed. 2010: Chap 2; p 168
4. Assad RS, Lee FY, Bergner K, Hanley FL. Extracorporeal circulation in the isolated in situ lamb
placenta: hemodynamic characteristics. J Appl Physiol 1992;72:2176–80.
5. Barash P, et al. Clinical Anesthesia 6th edition, 2009 : 1073-1105
6. Baydin A, Nargis C, Nural MS, et al. Painless, acute aortic dissection `presenting as an acute stroke.
Mt Sinai J Med. 2006;73:1129 –31.
7. Blackburn S. Maternal, Fetal & Neonatal Physiology. A Clinical perspective. 2007 : Chap 10. P 336
ANESTHETIC CHALLENGES
24 UNIVERSITAS PELITA HARAPAN
8. Chow SL. Acute aortic dissection in a patient with Marfan's syndrome complicated by gestational
hypertension. Med J Aust. 1993 Dec 6-20;159(11-12):760-2.
9. Chirillo F, Salvador L, Bacchion F, et al. Clinical and anatomical characteristics of subtle-discrete
dissection of the ascending aorta. Am J Cardiol. 2007;100:1314 –9.
10. Committee to Study the Prevention of Low Birthweight and Division of Health Promotion and Disease
Prevention. Preventing Low birth weight. 1985: p 28
11. Creasy R et al. Maternal-fetal medicine:principles and practice.5th ed. 2004:p460
12. Datta S. Anesthetic and Obstetric management of high risk pregnancy: Cardiovascular disease in
pregnancy. p166
13. Dietz HC, Pyeritz RE. Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and
related disorders. Hum Mol Genet.1995;4 Spec No:1799–809.
14. Elkayam U, Gleicher N. Hemodynamics and cardiac function during normal pregnancy and the
puerperium. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy.New York, NY: Wiley-
Liss; 1998:23–32.
15. Elkayam U, Ostrzega E, Shotan A, Mehra A. Cardiovascular problems in pregnant women with the
Marfan syndrome. Ann Intern Med 1995;123:117–22
16. Espinoza S C, Selman A R, Pauchard T F, Rivera F J, Iturra U S, Montecinos R F, Eliash D H,
Undurraga H F. Acute type A aortic dissection in pregnant patient with Marfan syndrome. Report of
one case. Rev Med Chil. 2009 Jan;137(1):98-100. Epub 2009 Apr 23. Spanish
17. Estrera AL, Huynh TT, Porat EE, et al: Is acute type A aortic dissection a true surgical
emergency?. Semin Vasc Surg 2002; 15:75-82.
18. Festin et al. Caesarean section in four South East Asian countries: reason for, rates, associated care
practices and health outcomes. BMC Pregnancy childbirth v. 9. 2009: 9-17
19. Goland S, Elkayan U. Cardiovascular Problem in Pregnant Women with Marfan Syndrome. Circulation
2009;119;619-623
20. Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ, Dietz HC. Aortic root
replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg. 2002;73:438–443
21. Harrison’s Principles of Internal Medicine 16th edition, 2005 : pp 34, pp 2329-2330, p 1481
22. Hagan PG, Nienaber CA, Isselbacher EM,Bruckman D, Karavite DJ, Russman PL,Evangelista A,
Fattori R, Suzuki T, Oh JK, Moore AG, Malouf JF, Pape LA, Gaca C, Sechtem U, Lenferink S, Deutsch
HJ, Diedrichs H, Marcos y Robles J, Llovet A, Gilon D, Das SK, Armstrong WF, Deeb GM,Eagle KA.
International Registry of Acute Aortic Dissection (IRAD): new insights from an old disease. JAMA.
2000;283:897–903.
23. Hiratzka et al. Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease.
JACC Vol. 55, No. 14, 2010 :e27–129
24. Hirst Jr AE, Johns Jr VJ, Kime Jr SW: Dissecting aneurysm of the aorta: A review of 505
cases. Medicine 1958; 37:217-219.
25. Immer FF, Bansi AG, Immer-Bansi AS, McDougall J, Zehr KJ, Schaff HV, Carrel TP. Aortic dissection
in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg. 2003;76:309 –314.
MEDICINUS Vol. 7 No. 1 Oktober 2017 – Januari 2018
UNIVERSITAS PELITA HARAPAN 25
26. Jondeau G, Nataf P, Belarbi A, Farcot JC, Iung B, Delorme G, Gandjbakhch I, Bourdarias JP. Aortic
dissection at 6 months gestation in women with Marfan’s syndrome: simultaneous Bentall intervention
and cesarean section [in French]. Arch Mal Coeur Vaiss. 2000;93:185–187
27. Kirklin J, Barratt-Boyes et al. Hypothermia, Circulatory Arrest, and Cardiopulmonary Bypass. Cardiac
surgery. New York: Churchill Livingstone; 1993:61-127
28. Lachandani S, Wingfield M. Pregnancy inwomen with Marfan’s syndrome. Eur J Obstet Gynecol.
2003;110:125–130
29. Loeyz, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010;47:476-485
30. Mackay J, Arrowsmith J. Core topics in cardiac anesthesia. 1st ed, 2004; p279
31. Maruyama Y, Oguma F, Kosuge T, Yokosawa T, Eguchi S. Successful repair of an acute type A
dissection during pregnancy. Nippon Kyobu Geka Gakkai Zasshi. 1990 Nov;38(11):2296-9.
32. Masuda Y, Yamada Z, Morooka N, et al. Prognosis of patients with medically treated aortic dissections.
Circulation. 1991;84:III7–13
33. Meijboom LJ, Drenthen W, Pieper PG, Groenink M, van der Post JA, Timmermans J, Voors AA, Roos-
Hesselink JW, van Veldhuisen DJ, Mulder BJ. Obstetric complications in Marfan syndrome. Int J
Cardiol. 2006;110:53–59.
34. Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome.
Circulation. 2005;111:e150 –7.
35. Mul TF, van Herwerden LA, Cohen-Overbeck TE, Catsman-Berrevoets CE, Lotgering FK. Hypoxic-
ischemic fetal insult resulting from maternal aortic root replacement, with normal fetal heart rate at
term. Am J Obstet Gynecol 1998;179(3Pt 1):825–7.
36. Murray CA, Edwards JE. Spontaneous laceration of ascending aorta.Circulation. 1973;47:848 –58.
37. Naito H, Naito H, Tada K. Open heart operation for a pregnant patient with Marfan syndrome [in
Japanese]. Mastui. 2005;54:525–529
38. Nakahira A, et al. Outcome after the modified Bentall technique with a long interposed graft to the left
coronary artery. Ann Thorac Surg. 2009 Jan: 87(1):109-15.
39. Pomini F, et al. Cardiopulmonary bypass in pregnancy. Ann Thorac Surg 1996;61:259-68
40. Preiss M, Hösli I, Holzgreve W, Zerkowski HR Aortic dissection in pregnancy in Marfan syndrome--
case report and treatment concept. Z Geburtshilfe Neonatol. 2001 May-Jun;205(3):110-3.
41. Robinson P N, Godfrey M. Marfan syndrome: a primer for clinicians and scientist. 2004: p 47
42. Rodeck C, Whittle M. Fetal medicine: basic science and clinical practice.2nd ed. 2009: p 143
43. Sakaguchi M, Kitahara H, Seto T, Furusawa T, Fukui D, Yanagiya N, Nishimura K, Amano J. Surgery
for acute type A aortic dissection in pregnant patients with Marfan syndrome. Eur J Cardiothorac Surg.
2005 Aug ;28:280–283.
44. Sakaguchi M, Kitahara H, Watanabe T, Kono T, Fukui D, Amano J. Successful surgical treatment for
acute aortic dissection in pregnancy with Marfan's syndrome. Jpn J Thorac Cardiovasc Surg. 2005
Apr;53(4):220-2.
45. Stecker MM, Cheung AT, Pochettino A, et al. Deep hypothermic circulatory arrest: I. Changes in
electroencephalogram and evoked potentials. Ann Thorac Surg. 2001; 71:14-21
ANESTHETIC CHALLENGES
26 UNIVERSITAS PELITA HARAPAN
46. Stecker MM, Cheung AT, Pochettino A, et al. Deep hypothermic circulatory arrest: II. Changes in
electroencephalogram and evoked potentials during rewarming. Ann Thorac Surg. 2001; 71:22-28
47. Svensson LG, Labib SB, Eisenhauer AC, et al. Intimal tear without hematoma: an important variant of
aortic dissection that can elude current imaging techniques. Circulation. 1999;99:1331– 6.
48. Schwartz’s principles of surgery, 8th edition, 2004.
49. Towsend. Sabiston Textbook of Surgery 18th edition, 2007
50. Tritapepe L, Voci P. Anesthesia for Caesarean section in a Marfan patient with recurrent aortic
dissection. Can J Anesth 1996/43:11/pp 1153-5
51. Van Karnebeek CD, Naeff MS, Mulder BJ, et al. Natural history of cardiovascular manifestations in
Marfan syndrome. Arch Dis Child.2001;84:129 –37.
52. White G. Basic Clinical Lab Competencies for Respiratory Care : An Integrated Approach. 2003. 4th
ed : p 152
53. Winn H, Hobbins J. Clinical Maternal-Fetal Medicine. 2000 : 687
54. Zeebregts CJ, Schepens MA, Hameeteman TM, Morshuis WJ, de la Riviere AB. Acute aortic dissection
complicating pregnancy. Ann Thorac Surg.1997;64:1345–134
