A Patient in Labor with
Marfan Syndrome
Aubrey Ballard, SRNA
Missouri State University
Objectives
 Presentation of Case Study
 Marfan Syndrome
 Marfan Syndrome and Pregnancy
 Marfan Syndrome and Anesthesia
 Answer Questions
Patient Information
 20-something year old, female
 Transported via EMS in labor, 175/115
 Limited prenatal care; preeclampsia
 History of methamphetamine use (quite 4 months
prior). Negative urine drug screen
 “Writhing in pain”, noncompliant with instructions, not
allowing monitoring or additional lab work
 Anesthesia consulted for labor epidural
Lab Values
WBC
13.8 H
RBC
4.22 L
Hgb
12.0
Hct
36
Plt
281
PT
14.2
INR
1.1
PTT
26
Lab Values
Sodium
138
Total Bilirubin
0.3
Potassium
3.3
AST
16
Chloride
103
ALT
24
CO2
22
Alk Phos
232
BUN
4
Lactate
Dehydrogenase
231
Creatinine
0.6
Total Protein
6.4
Est. GFR
>60
Albumin
2.9
Glucose
104
Uric Acid
7.3
Calcium
8.6
Magnesium
1.3
Urinalysis
Color
Yellow
Clarity
Slightly Cloudy
pH
8
Specific Gravity
1.015
Protein
3+
RBC
2-5
WBC
2-5
Crystals
Present
Bacteria
Few
Casts
None
Mucus
Small
Patient Information
 Allergies: Codeine
 Current medications: multi-vitamin, magnesium gtt
 Anesthesia interview conducted
Marfan Syndrome (MFS)
 Autosomal dominate genetic defect in fibrillin-1 or
fibrillin-2 gene
 Fibrillin is a major component of microfibrils and also
help store TGF-β
 Inherited or de novo germline mutations
 Diagnosis made from physical features & heredity
Changes associated with
MFS
 Cardiovascular




Aortic dilation/dissection – especially at the root
Aortic and mitral valve prolapse/regurgitation
Pulmonary artery dilation
Arrhythmias, cardiomyopathy, congestive heart failure
 Skeletal





Arachnodactyly with hyperextensibility
Chest wall deformities
Scoliosis/kyphosis
Osteoporosis
High arched pallet, retrognathia, micrognathia
Changes associated with
MFS
 Ocular





Lens subluxation
Myopia
Cataracts
Retinal detachment
Glaucoma
 Pneumothorax/pulmonary blebs, asthma, emphysema
 Inguinal and incisional hernias
 Dilation of dural sac
 Dissection of cranial arteries and aneurysmal subarachnoid
hemorrhage
Marfan & Pregnancy
 Pregnancy increases the likelihood of cardiovascular
complications in patients with MFS
 Approximately 15% of MFS patients develop a major CV
manifestation with pregnancy & 40% have obstetric
complications
 Prophylactic treatment with beta-blockers or internal
defibrillators has been suggested before conception
 Increased CO during labor and after delivery increases risk
of aortic rupture
 Vaginal delivery vs. Cesarean section
 Prolonged maternal bleeding
Marfan & Anesthesia
 In depth history and physical
 Hemodynamic management of cardiovascular conditions;
Arterial line
 General vs regional (no epinephrine for blocks)
 Possible difficult intubation; minimize laryngoscopic
response
 Careful positioning – joint laxity
 Dural ectasia
 Recognize and treat aortic dissection
S/S of Aortic Dissection
 Important to distinguish dissection from ischemia
 Abrupt onset of severe chest or back pain
 Does not respond to nitrates or beta-blockers acutely but
may decreased with decrease in blood pressure
 New aortic diasolic murmur, unequal peripheral pulses
or BP, absence of rales with chest pain
 Ischemia may occur with aortic root involvement
Treatment of dissection
 Lowering BP & decreasing myocardial contractility
 X-ray, echo, MRI, CT, TEE
 Anticoagulation/thrombolytic therapy contraindicated
 Need for surgery based on size and location
 MFS aneurysm >4.5 cm indicates need for operative
repair
Case Study
 Emergent Cesarean Section with RSI
 In room at 0936 with BP – 155/105
 Incision at 0943 with deliver of baby at 0944
 BP decreased to 92/68
 Extubated without difficulty and transported to PACU
 CT
CT findings
 A dissection that starts in the distal aortic arch between
the takeoff of the left subclavian and the aberrant right
subclavian artery that extends throughout the thoracic
aorta, ending just above the level of the celiac trunk
and upper abdominal aorta. The takeoff of the aberrant
right subclavian artery is ectatic measuring 2 cm in
diameter. The distal aortic arch is ectatic measuring 3.2
cm in diameter.
Case Study
 Taken to ICU
 Cardiology consult
 Received 2 units PRBC for H&H of 8.1/24
 Next day was transferred to larger facility
References
 Elisha, S. (2011). Aortic Dissection. Case Studies in Nurse Anesthesia. 282285.
 Nagelhout, J.J., Plaus, K.L. & Rieker, M. (2014). Respiratory, Anatomy,
Physiology, Pathophysiology & Anesthetic Management. Nurse Anesthesia.
653
 Kasper, Dennis L. (2005). Harrison's Principles of Internal Medicine.
Revised/Expanded ed. New York: McGraw-Hill, Medical Pub. Division. 23292330
 Andropoulos, D., Stephen S., Isobel R., and Emad M. (2010). Anesthesia for
Congenital Heart Disease. 2nd ed. Oxford: Blackwell.
 Coté and Lerman's a Practice of Anesthesia for Infants and Children.
Revised/Expanded ed. Philadelphia, PA: Elsevier/Saunders, 2013.
 Bongard, F.S., and Darryl Y.S. (2008) Current Diagnosis & Treatment Critical
Care. Revised/Expanded ed. New York: McGraw-Hill Medical.
References
 Hall, J. (2005). Principles of critical care (Revised/Expanded ed.).
New York: McGraw-Hill, Medical Pub. Division.
 Tintinalli, J. (2011). Tintinalli's emergency medicine: A comprehensive
study guide (Revised/Expanded ed.). New York: McGraw-Hill.
 Marfan Foundation. (2013). What is Marfan Syndrome. Retrieved
August 19, 2014, from http://www.marfan.org/about/marfan
 FBN1 gene. (2014, August 12).Genetics Home Refernce. Retrieved
August 17, 2014, from http://ghr.nlm.nih.gov/gene/FBN1
 Goland, S., & Elkayam, U. (2009). Cardiovascular Problems in
Pregnant Women With Marfan Syndrome. Circulation, 619-623.
References
 Hanumanthaiah, D. & Sudhir, V. (2013). Neuraxial
block in a patient with dural ectasia. Indian Journal of
Anaesthesia. 624-625
 Bagirzadeh, L., Guglielminotti, J. & Loeys, B. (2013).
Anesthesia recommendations for patients suffering
from Marfan syndrome. Orphan Anesthesia. 2-8.