February 2, 2010


I’m not talking classical music 😉

alot is going on right now, I’ve been having some funky symptoms more than usual lately, palpitations, shortness of breath (different than the asthma kind) and tiredness, its kinda messing with things, could be from my over zealous night shift schedule, but it could be something going on with my heart. As an ACHD patient, I cant just ignore these symptoms, it could be something serious

after some thought and looking over my records from my current cardiologist, I’m not satisfied with the care I am getting locally, this this stems partially from the  fact that he mentioned some new diagnoses at the last appointment and never explained them, so I’m a little nervous about that, as well as the bedside manner he has shown (not giving me much time to ask questions, feeling sort of intimidated sometimes), this along with some issues I’ve found in my records, and the fact that there is no local 100% ACHD doctor or surgeon, they all see adults with acquired diseases like coronary artery disease as well as ACHD patients has made me look further.

I am going back to boston, where I originally had my surgeries as a baby. this is actually the #1 ACHD center in the country, so strangely enough I’m as excited as I am nervous. I’ve got alot of questions, if in fact, as Dr. VT ACHD cardio says, I do have shones syndrome, according to the bethesda guidelines I need to be seen every year anyways…

so, I’m in the process of coming up with a list of questions, its a little weird to be going back to boston for me on one hand, on the other I’m excited to get some real answers, and have someone listen to me and answer my questions. as it stands now, I’m really not sure what’s going on with that ticker of mine, and I’m not sure what “shones syndrome” and “parachute mitral valve” imply, so those are at the top of my list of questions. any other suggestions????

I just wanted to keep in touch and let you all know what was up….


6 Responses to “BACH”

  1. kerri said

    We’ve talked about all this stuff, so you know I think you’re doing the right thing :).

    I hope you get some answers going on in Boston. Keep us posted, and good luck!

  2. Shannon said

    My son has Shones Syndrome. He is 3. I think its important that you are seeing a cardiologist very experienced in treating adults with CHDs as the field is constantly evolving.

    I have a few articles you may be interested in. The first is an article on parachute mitral valves and should explain the morphology to you. The second is a the American Society of Cardio-thoracic surgeons guidelines for treatment of adults with CHDs. It includes section 6 which is on Shone’s related lesions (mitral, outflow tract obstructions, BAV etc).

    If you want either in PDF, email me. Totally, understand if you like to wiat to speak with cardiology.

    Hope Boston goes well.

    • trinityheart8891 said

      those PDFs would be great….I appreciate the help, I’m just frustrated it just seems like such a paradox, on one hand I’m “fine” and according to Dr VT ACHD guy its “not my heart” but on the other hand he starts throwing around terms like shones syndrome and parachute mitral valve, which are new to me, and not explaining them….dude, I just want someone to freaking listen, I know my body….really, I’ve only been in it for 23 years, and lived with this “shones” and parachute mitral valve for 23 years….trying to blow it off as something thats no big deal isnt going to gain my trust….its my levt ventricle and atrium and lungs that are going to pay the price

      ok, done with the rant
      thanks again

  3. Shannon said

    Well, just send me an email and I shall attach them. You’ve probably googled it by now but Shones is just a term for CHDs in which you have both inflow and outflow issues on the left side. The theory is that the ‘syndrome’ originates with some malformation in the mitral valve. This causes reduced or turbulent flow into the left side which leads to stenosis. Its considering to be on the continuum with HLHS. You must have a relatively mild-moderate case to have made it to adulthood without a valve replacement so don’t become too worried by some of the cases you read about.

    Its common for cardiologists to avoid using the term Shones – they just think it – because it doesn’t add anything informative for most patients. You still deal with the ‘issues’ as they come up.

    The thing that is often talked about with Shone’s that freaks people out is that its PROGRESSIVE. Some CHD’s are just like the heart being built wrong. Uh oh, this has a hole or this has an artery in the wrong place. Then the surgeons try and change the structure and its FIXED (well, kinda). With Shone’s you tend to get emergent problems as things are dealt with.

    Shone’s is about smaller and slightly abnormal structures on the left side (the mitral valve, the left ventricular outflow tract (LVOT). aortic valve (Bicuspid Aortic Valve – BAV) and aortic arch (coarc) all growing while a bit wonky. Valves typically worsen with age until replacement is required. Its really common for patients with shones (in my research) to present like this:

    1) Critical coarc in infancy – REPAIRED
    2) Subaortic stenosis worsens in early childhood – REPAIRED
    3) Aortic valve reaches severe level of gradient – REPAIRED (or ROSS replacement)
    4) Suddenly the MITRAL VALVE is leading to regurg and pulmonary pressures or the LV is hypertrophic….>
    5) Sometimes mitral valve repair or replacement (hard in small kids).

    Its a dance between these periods of stability and intervention.

    However, once the various sources of stenosis / turbulence / gradient are resolved kids thrive and the LV Hypertrophy resolves. You don’t want to leave the gradients to a point where the LV is damaged, that’s why ongoing care is important.

    OK, probably too much info. Going to get back to work now 😉

  4. trinityheart8891 said

    shannon, I dont know your email but mine is mtromblyrt (at) comcast . net, thanks for the explanations, it makes sense knowing the defects that I have, and this Dr. VT ACHD guy says it is a “mild” form of shones, we’ll just see what dr valente says…I wouldnt be surprised if it is, what gets me is that it is progressive….YUCK!!! I knew it was coming, but still, I def know now why they say every year for shones pt’s, I dont want to have to deal with any of the issues that come from letting any of this fly too long…..
    hopefully I’ll figure out whats going on

  5. Melissa said


    So is it too much admitting that when I went to BACH that it reminded me of going to Disney Land when I was a kid? Excited and nervous all at once…and then of course it is a way cool children’s hospital so there is this gigantic marble machine thing in the lobby. Too cool. I think you are absolutely doing the best thing. If nothing else, I’ve learned from getting my second opinions, that it at least clarifies what I think about my health (and heart), and that’s what is important. I also find peace of mind knowing that my doctors know more than anyone out there, and that they will take my symptoms seriously (at least most of the time, and definitely if I’m concerned b/c I know it’s not my normal).


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