Heart, Aortic and blood vessel involvement in Marfan Syndromefan

Cardiovascular System and Marfan Syndrome

Heart Picture with aorta and central arteries The cardiovascular system is made up of the heart, blood and blood vessels and it’s one of the most important systems in the body. It’s also called the circulatory system because blood from the heart circulates around the body delivering oxygen and nutrients and picking up waste products.

Weak Aorta

The aorta is a large artery in the heart that carries blood to all other parts of the body. Individuals with Marfan Syndrome tend to have a wider and more fragile aorta than people without the disorder. The widening of the aorta is progressive meaning the condition becomes worse over time.

Aortic Dilation

People diagnosed with Marfan Syndrome have damaged connective tissue that often weakens or tears. Consequently, the walls of the aorta often become weakened or stretched over time. This process is called aortic dilation.

Aortic Dissection

The inner lining of the aorta may become so stretched and weak that it eventually tears or ruptures. The tear allows blood to leak out creating a flow of blood that separates the middle and outer layer of the aorta. This process is called aortic dissection and the condition is fatal if left untreated. Symptoms include a sudden severe pain that usually occurs across the chest or in the back between the shoulder blades.

Leaky Heart Valves

Many individuals with Marfan Syndrome tend to have leaky heart valves. These small valves keep blood flowing in one direction throughout the body. If the leaks are minor, the patient experiences what is known as a heart murmur. This is an extra or unusual whooshing or whistling sound heard between heartbeats. Murmurs are graded from 1 to 6 with 1 being the softest and 6 being the loudest.

Severe leaks can lead to a very fast or uneven heart rate which is referred to as heart palpitations. Symptoms include fatigue and shortness of breath. It is normal for people who are exercising or who feel excited or scared to feel their heart beat more rapidly or even skip a beat. However, people with heart palpitations experience the same sensations even when they’re resting or moving slowly.

Mitral Valve Prolapse

The mitral valve is located between the left atrium or upper chamber of the heart and the left ventricle or lower chamber. The mitral valve in people with Marfan Syndrome is often floppy, billows out and doesn’t close properly. This common heart condition is called mitral valve prolapse and it can be harmless depending on the severity. Symptoms of mitral valve prolapse include shortness of breath and a rapid or irregular heartbeat.

When the mitral valve doesn’t remain closed, it can allow blood to flow backwards during a heartbeat. This process is known as regurgitation and it can be serious. Depending on the severity of the condition, surgery may be necessary to repair the valve.

Last updated: Feb 03, 2010

Cardiovascular Involvement in Marfan Syndrome

Most individuals diagnosed with Marfan Syndrome experience cardiovascular problems caused by an abnormal heart and blood vessels. The complications can range from very minor to fatal such as sudden heart failure.