Understanding Marfan Syndrome
Simply stated, Marfan syndrome is a disorder of the connective tissues. Connective tissues are the body components that give support and substance to the ligaments, tendons, cartilage, blood vessel walls, heart valves, and similar structures. When a person is diagnosed with Marfan syndrome, it would means that his connective tissues are not normal in the sense that their chemical components are irregular. And because of that, the body structures that the tissue supports are not as firm as it should be.
In general, Marfan syndrome is a genetic disease. This means that it can be inherited from parents or grandparents. And it may affect several parts of the body in a given time because connective tissues are not really concentrated in one location alone.
The only indication that a person has Marfan syndrome is the presence of a particular heart problem in them. Physically speaking, they usually grow thin and tall. They could also develop lengthy legs and arms, as well as long, slender fingers. Health wise, mild cases of Marfan syndrome are asymptomatic, meaning they do not show any symptoms at all.
But for those patients who got it severe, they would experience problems mostly in their blood vessels. Almost all of their arteries, the major ones and the aorta, are generally weak. And if they attempt to do strenuous physical activities, the walls of these vessels would break. Blood will then leak through the tears and aortic dissection happens next. Aside from that, aneurysms may also form.
And because heart problems are a given to people with Marfan syndome, the heart structure usually affected are the valves. More particularly, it’s either the mitral valve or the aortic valve. If one of these valves is affected, the blood’s tendency is to flow backward instead of forward. And to keep that from happening, the heart has to work harder. Over time, it will be enlarged, causing other complications. In other case, it is observed that people with Marfan syndrome have unusually big mitral valve, which causes abnormal heart sounds. Doctors usually detect it thorough their stethoscopes.
To diagnose Marfan syndrome, physicians usually perform chest x-rays and echocardiogram with Doppler. Other tests may also be requested but more often than not, these two tests are sufficient. Due to the known effects of this disease in the body, diagnosing it early on is important. And once the patient is found out to be suffering from it, regular consultations with the cardiologist are necessary.
Again, Marfan syndrome can come mild or severe. The milder cases of this disease may not require any regular treatment at all. But for those who have accelerated cases of the disease, a medical treatment method as provided by a heart expert has to be followed.
Most of the time, the therapy starts with the administration of drugs to control the symptoms. Medications that can lower the person’s heart rate, as well as his blood pressure, are given. But for those who have their aortic valves already affected, there’s no other option but to do surgery.
And just like with any other heart diseases, patients with Marfan syndrome need to take a certain dosage of antibiotics before undergoing surgery or even dental procedures. This is because they are prone to endocarditis, the condition wherein an infection of the heart may arise through the entry of bacteria in the bloodstream. Invasive procedures as stated previously may cause this.
It is then mandatory for people with Marfan syndrome to watch their health at all times. Their physical activities have to be restricted, depending upon the development of the disease in them. Better ask your cardiologist about it before going back to your usual exercise regimes.
