Confused whether marfan or marfanoid habitus

[venkatasu]

I am diagnosed with MVPS with no MR 20 years age and now i am 40 years of age. For the past 1 year i have been suffering from chest pain on a daily basis with no confirmed diagnosis (vague diagnosis as GERD, Musculo skeletal, even MVPs related or doubtful variant angina). TMT taken an year ago was negative. Recently one doctor suggested for marfan evaluation but the cardiologist refused to do that saying "Do you think all tall and thin built are marfans?".
I am tall and thin built (height 177cm weight 49 kgs). I don't have EL, No carinatums of the chest other thin thin built chest cavity, No thumb sign, No wrist sign, Arm span 179 cm (arm span to height ratio = 1.03), Lower segment = 95 cm (US/LS ration = .86), No pes planus, No highly arched palate (but crowed teeth), Scoliosis very mild < 20 degree,
Recent aortic root dia = 3.3 cm (old 2 years back 2.8 cm), aortic sinus = 30 mm, st junction 22mm, arch = 24mm, proximal descending = 20mm, distal descending = 18mm.
My dr says with out evaluating the entire GHENT or Modified GHENT that i don't have marfans but marfanoid habitus. Does tall and thin built with MVPS are treated with marfanoid habitus with the known parameters given above? If your answer is yes that there is chance of Mafanoid habitus or marfan syndrome or any other connective tissue disorder do I need routine echos and avoiding mild sports like badmintton. Please guide me on the above said two issues (1) the cause of chest pain and its evaluation (2) having marfan syndrome or marfanoid habitus or any other like MASS phenotype etc or simple tall and thin built with MVPS such it I can participate in mild sports.

Your valuable suggestion awaited at the earliest. Thank you

[venkatasu]

Thank you for your response. Here in our country the cut off value is taken as 3.7cm (aortic root) given my BSA = 1.6. My doctor advises to take ECG during pain to rule out any ischemic changes especially if it is triggered due to spasm. But the pain lasts for a few mts and some time there is only discomfort. I do have long lasting pain exactly at manubrium sterni which my GP says may be rib pain. So far no improvement in my symptoms. They also suggested Costo but I don't have tenderness while pressing. Above all I get pains in the back and chest even while lying back in the chair for a few mts especially when my shoulder blade and spine between it are rested.

[CCFHeartMD20]

Hi raghu500,

If I understand your question correctly, you are asking if your stents can move while you are driving.  The stents should not move from the place they are positioned.  It is firmly against the walls of the artery in which it was placed, and over time, the body coats it with substances that further make it stay in place.  I would not worry about driving.  

You should discuss a safe exercise program with your cardiologist, quit smoking if you smoke, keep your blood pressure under control, and have your cholesterol checked.  You should also make sure to take the medicines they have given you every single day and never miss a dose.  If you miss a dose of the special medicines given to keep the stent from clotting up, then you are at risk for another heart attack.  

Hope this helps!
CCFHeartMD20

[CCFHeartMD20]

Hi Venkatasu,

This is an extremely difficult question to answer without seeing you, examining you, and seeing your tests myself.  I also can not give you direct medical advice on whether or not you can exercise.  You will need to discuss that with your cardiologist.  That being said, I will give you some thoughts.  Given it seems you are very well read on the subject, I will try to go through the details of what you have written.

As you know, the GHENT criteria requires involvement of 2 major organ systems and involvement of a third.  Your aortic root is very, very mildly dilated (the cutoff is 3.2cm), so I'm not sure I would even count that as fulfilling one of the major criteria.  If you do not have a family history of Marfan's or individuals in your family that died from cardiac issues that could be related to Marfan's, I don't think genetic testing will be useful.  Therefore, you do not meet the GHENT criteria.  Of course, if you have a family member that you suspect had Marfan's, that would change things.  If you have Marfanoid features, we do not usually treat you in the same way as those with actual Marfan's.  I expect you could continue to exercise, but I would discuss that with your cardiologist.

Assuming you do have mitral valve prolapse with no MR, it sounds like it is very mild.  Given you had a negative treadmill test, you are probably ok to exercise.  Again, discuss this with your cardiologist.

As for your chest pain, your doctors are right that it could be related to something other than your heart.  Given that your MVP is mild, that shouldn't explain your chest pain.  If you have a family history of heart disease, that would change things a bit.  This would prompt your cardiologist to be more aggressive.  I would make sure to have your lipid panel, Hemoglobin A1C, and blood pressure checked.  This will help your doctors better understand your risk for heart disease.   If you smoke, you should stop and be evaluating for lung issues that could explain your chest pain.  Sometimes, we will order an exercise echocardiogram.  That way we can better see what is going on with your heart when you exercise.  You could consider discussing this with your cardiologist.  

Hope this helps!
CCFHeartMD20  

[raghu500]

I am Ravi, aged about 46 year. Last week I hurdled with chest pain, and minor heart attack. After that i admitted to Hospital and Treated and taken Angeogram. In angeogram drs. found that my heart having two blocks: One with 90% and other with 50%. After that I undergone for Angeoplasty for 90% block. After that I taken Three days rest. Now I am doing my regularly work. But avoid weightage  works. But I am driving a Car for two to three hours.
Now I am doubtful that whether the stent which is in my heart can be moved. If it is moved then the hearttack will remain persists. Gave suggestions.