How is Marfan Syndrome treated?
People affected by the Marfan Syndrome should be treated by a
physician familiar with the condition and how it affects all body
systems. There is no cure for the disorder yet but careful medical
management can greatly improve the prognosis and lengthen
the life span.
Location
Treatment for cardiovascular problems is critical and the aorta
must be monitored for weakness. Drugs can help to reduce the
stress experienced by the aorta but surgery may be needed
eventually to replace the vessel.
Every affected person should work closely with his/her physician(s)
for their customised treatment plan. However, in general, treatment
includes the following:
1
Annual echocardiogram to monitor the size and function of
the heart and aorta.
2
Initial eye examination with a slit-lamp to detect lens
dislocation, with periodic follow-up with an ophthalmologist.
3
Atenolol/ Losartan may be prescribed to lower blood pressure
and consequently, reduce stress on the aorta.
4
Antibiotics may be prescribed prior to dental or genito-urinary
procedures to prevent bacteria from entering the blood
stream in people who have artificial heart valve.
5
People who have had aortic surgery must take blood thinning
medication.
Lifestyle and home remedies
You may need to avoid competitive sports and certain recreational
activities if you are at increased risk of aortic dissection or rupture.
Increases in blood pressure, common in activities such as weightlifting,
place extra strain on the aorta. Less intense activities — such as
brisk walking, bowling, doubles tennis or golf — are generally safer.
Regular assessment
Careful medical management can greatly improve prognosis
and lengthen life. Complications can be prevented if patients
are seen on a regular basis by their various specialists.
Emotional support
Marfan patients and their families may seek out information
and support to strengthen their coping strategies which are
crucial towards living a healthy and productive life.
National University Hospital
5 Lower Kent Ridge Road, Singapore 119074
Tel: 6779 5555 Fax: 6779 5678 Website: www.nuh.com.sg
Contact Information
National University Heart Centre, Singapore
1 Main Building of NUH.
Opening Hours: 8.30 am - 5.30 pm (Monday - Friday)
Closed on Weekend & Public Holidays
Website: www.nuhcs.com.sg
Getting to NUH
Circle Line Kent Ridge MRT Station
Marfan Syndrome
Commuters can transit at the Buona Vista MRT Interchange and alight two stops after
at the Kent Ridge Station. The station is served by three exit-entry points.
Exit A: Right at the doorstep of National University Heart Centre, Singapore.
Exit B: Along South Buona Vista Road, which links to Singapore Science Park 1.
Exit C: Leads to NUH Medical Centre.
Information in this brochure is given as a guide only and does not replace medical advice from your doctor.
Please seek the advice of your doctor if you have any questions related to the surgery, your health or medical
condition. Information is correct at time of printing (Apr 2015) and subject to revision without notice. Copyright©
is held by the publisher. All rights reserved. Reproduction in whole or in parts without permission is strictly
not allowed.
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What is Marfan Syndrome (MFS)?
How is Marfan Syndrome diagnosed?
The Marfan Syndrome is a heritable disorder of the connective
tissue that affects many organ systems, including the skeleton,
lungs, eyes, heart and blood vessels.
The Marfan Syndrome is difficult to diagnose because there is no
specific laboratory test for the condition. In addition, characteristics
of the disorder vary greatly among affected individuals. Most
affected people do not show all of the possible signs and
complications of the syndrome.
Connective tissue “connects”, provides structural support and
determines the elasticity of the body’s organs, bones and
ligaments. In Marfan Syndrome, the connective tissue in the heart,
lungs, eyes and skeletal systems can stretch and weaken.
Accurate diagnosis of the Marfan Syndrome can be assessed after
a complete physical examination that focuses on the systems
affected by the disorder. This includes:
What causes Marfan Syndrome?
People with Marfan Syndrome may have:
• A tall, thin build.
• Long arms, legs and fingers.
• Scoliosis, or curvature of the spine.
• A chest that sinks in or sticks out.
Marfan Syndrome is caused by a defect in the gene that enables
your body to produce a protein that helps give connective tissue
its elasticity and strength.
Most people with Marfan Syndrome inherit the abnormal gene
from a parent who has the disorder. Each child of an affected
parent has a 50-50 chance of inheriting the defective gene. In
about 25 percent of the people who have Marfan Syndrome, the
abnormal gene does not come from either parent. In these cases,
a new mutation develops spontaneously.
• Complete family history
• Genetic test
• Slit lamp eye examination by an ophthalmologist.
• Flat feet.
• Stretch marks on the skin not explained by pregnancy
or weight gain.
• Echocardiogram
Heart Disease and Marfan Syndrome
Scoliosis, or curvature
of the spine
One of the biggest threats of Marfan Syndrome is damage to the
aorta, the artery that carries blood from the heart to the rest of the
body. Marfan Syndrome can rupture the inner layers of the aorta,
causing dissection that leads to bleeding in the wall of the vessel.
Surgery may be required to replace the affected part of the aorta.
Some people with Marfan Syndrome also have mitral valve prolapse
- heart valves which may have malformed or are overly elastic,
resulting in irregular or rapid heart beats and shortness of breath. It
may require surgery.
Aortic Rupture
Physical Traits in People With Marfan
Syndrome
A chest that sinks in
or sticks out
Aortic Dissection
Outer layer
Tear
Vessel
Inner layer
Blood
Outer layer
Inner layer
Flat feet
Long arms, leg
and fingers