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Introduction - my baby has HOCM

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  • Introduction - my baby has HOCM

    Hi everyone

    I'm new here. My son is 11 weeks old and was dx at 5 weeks with severe HOCM. Last week we found out this is because he has Noonan Syndrome. We are from uk and very lucky to have a fantastic free healthcare system and are being lookEd after at a world class cardiac hospital. In addition to his heart problems he is profoundly deaf. We have no family history of any of these conditions.

    Just wondering if you can give me your take on his echo reports - he had one at 5 weeks and one at 8 weeks and will have another in 2 weeks (he'll be 13 weeks).

    At 5 weeks the report says: situs solitus, av and va concordance, small pfo with bidirectional flow, severe lv and rv hypertrophy which is symmetrical and concentric. Pulmonary valve appears dysplastic with v max of 3.2m/s, 40mmHg, severe right ventricular outflow tract obstruction with a gradient of 75mmHg, moderate left ventricular outflow tract obstruction with v max 3m/s, pg 35mmHg. Mild to moderate mr, lvef course of the coronary arteries, normal aortic arch with no coarctation, confluent and normal size pulmonary arteries

    At 8 weeks the report says: echo continues to show severe concentric right and left ventricular hypertrophy with severe right ventricular outflow tract obstruction with a peak gradient of 85mmHg and moderate left ventricular outflow tract obstruction with peak gradient of 68mmHg. There did not appear to be any mild cavity obstruction. There was mild to moderate mitral regurgitation which appeared functional due to SAM. There was diastolic dysfunction and rather prominent coronary arteries.

    Owen is feeding well and putting on weight. He was having sweaty episodes and dusky lips on feeding before diagnosis but this improved once he started propanolol. However despite his dosage being increased (now on 0.75ml 3x per day) he is having sweaty episodes and dusky lips again.

    Please let me know your thoughts on the reports - even if you don't think it sounds good!

    Thanks xx
    Last edited by Moomin; 07-28-2011, 11:26 AM.

  • #2
    Re: Introduction - my baby has HOCM

    Welcome Moonin! You came to the right place for support and lots of information on HCM. It's good they were able to diagnosed your son at such a young age so that they can treat the problem early on. There are others here on this site that were diagnosed as infants who can possible give you insights as to how they were treated early on and their experiences.

    You should call the HCMA office and speak to Lisa about your son's readings and for further information on treatment. She has a wealth of knowledge on HCM. Do you know if the cardiac hospital you are using is a "Center of Excellence" which has specialists for HCM?? There are lots of things they can try and may recommend surgery at some point for your son, but I'm would think they want him to be older and stable otherwise.

    I had a son that had a heart problem (not HCM) among other problems diagnosed as Mohr-Majewski Syndrome. He had surgery at 5 days old ---they can do amazing things & that was 23 years ago!! He was going to need another heart surgery but they wanted to wait til he was about 2 yrs old. Unfortunately he did not make it due to his other problems. I remember the blue lips and sweaty episodes but having oxygen helped him. Call Lisa, HCM is a very specialized heart problem.
    Mer

    Diagnosed 09/2009 @51 yrs old- HCOM
    Myectomy 2/17/2011
    Son & Daughter no findings as of yet (echos)
    No family history determined yet -- 1 sister, 2 brothers & extended family.

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    • #3
      Re: Introduction - my baby has HOCM

      Hi Moonin,
      I'll second Mer's suggestion that you call the HCMA office and arrange to talk with Lisa. I see that you're in the UK -- you can arrange to call via Skype so it won't cost anything.

      Gordon
      Myectomy on Feb. 5, 2007.

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      • #4
        Re: Introduction - my baby has HOCM

        Moomin - I'm so sorry you are going thru so much with your dear little one - please keep us updated and know that we care very much. You will find many here willing to share experiences and support with you.

        Linda

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        • #5
          Re: Introduction - my baby has HOCM

          I'm sorry you have to deal with this (and for his sake too!). Its sounds like he's getting quality care and they are on top of things. I'm sure other parents will chime in and let you know that while this all seems for scary, they've adapted.
          Marc
          Diagnosed @ 48
          Saw Dr. Michael Debakey @ age 5 - "He's fine, just a little noisy"
          Father to 3 boys 22, 25, 29 (all currently clear - pending genetics)
          AICD - Valentines Day '08, Spark Plug replaced 11/14
          After much research, I had a Myectomy @ Mayo for my 50th Birthday '08
          Quietly going insane . . .

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          • #6
            Re: Introduction - my baby has HOCM

            Noonan's kids are a little different then the typical "HCM" kid so it is important you have a specialist who is keen on syndromes and HCM. We have many families with both conditions yet most are not big message board people - so be patient others will find you here soon. Propranolol is a great option for this little one - and doing your best to keep him well hydrated will help his heart too! Its a long journey ahead of you... these children can have lots of little bumps in the road but can live very long and productive lives.
            Best wishes,
            Lisa
            Knowledge is power ... Stay informed!
            YOU can make a difference - all you have to do is try!

            Dx age 12 current age 46 and counting!
            lost: 5 family members to HCM (SCD, Stroke, CHF)
            Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
            Therapy - ICD (implanted 97, 01, 04 and 11, medication
            Currently not obstructed
            Complications - unnecessary pacemaker and stroke (unrelated to each other)

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            • #7
              Re: Introduction - my baby has HOCM

              I just wanted to say Hello . . . and Welcome. My heart goes out to you right now. As others have said, please call the office and talk with Lisa. It was the best call I ever made. This time of transitioning into the idea of having a child with a special heart is SO HARD, but I am here to tell you that it does get easier. Life is never the same but it does get easier and becomes a routine part of your life. There is also another forum specifically for parents with children who have been diganosed with cardiomyopathy (all forms). But there are several moms on there with children who were diagnosed with HCM (even Noonan's) as infants. It is through the Children's Cardiomyopathy Foundation which you can find online as well. It may be another avenue to pursue in reaching out to other families - all in very silmilar situations. I can think of a least one or two other moms from England on that forum as well- so you may be able to specifically ask about doctors/facilities/etc. It sounds as though you are getting your baby the medical care he needs and being followed very closely, which is important with pediatric HCM cases. Although I cannot comment on any of the stats you provided, know that in my experience (seeing two different HCM specialists) that the numbers are only one factor. They really pay attention to symptoms - and in the case of your child, he is growing which is a really great sign. So take a little comfort in that. My son's reports have always said "severe" but in really talking with the doctors they would consider him more moderate. He did have hypertropy during his first echo at 37 days old (although we were never told of this) so we know that it is something he was born with. He is doing really well, no symptoms and no medicine for now. Just know that you are doing everything you can right now, keep the faith, enjoy your baby and reach out to others when you need some support. We are here for you!

              Sending you thoughts and prayers during this difficult time!
              Angie & my boys
              Gavin diagnosed at 3 1/2 yrs old - Mar 2009 w/HCM, Gene mutation identified 7/09
              Younger brother (Cullen) - born 7/08, gene negative but still being screened on occasion due to his brother's "unique presentation" - meaning there could be another gene involved
              Husband (Chad) - clear
              Myself - Positive for gene mutation (identified 10/09), echo negative but diagnosed with mild regurgitation of the mitral, tricuspid & pulmonary valves

              Comment


              • #8
                Re: Introduction - my baby has HOCM

                I can help some to help you understand the echos. As an anesthetist I read them prior to surgery. I am no means an expert but at least I can help with understanding the verbage.
                I suggest you check out this website to understand the complex function of the heart. It is awesome at explaning what things mean and shows pictures.

                http://www.cvphysiology.com/index.html

                Also, no matter how long it takes with the doctor have them go over every word and explain everything until you understand it. You have to speak up with your physician and tell them you don't understand or want to understand. I always take the time to explain the anesthesia I give in detail. Because how can you make an informed decision if the decision you are making is on information you do not understand. So please make your physicians discuss everything with you untill you think you understand it. Thats what you are paying them for. Also if you have questions write them down before the visit so you don't forget them when you see the doctor.


                situs solitus: this means that the position of the chest and abdomen organs are in normal position.

                av and va concordance: av=atrioventricular va=ventriculoarterial. Is stating the va that the vessels going to and from the heart such as the pulmonary vein and the aorta are on the correct side of the heart (and not reversed which is bad). It also mentions the av which means the left is connected to the right side. So your child's heart anatomy is normal in that respect.

                small pfo with bidirectional flow[B]: this is patent foramen ovale, which means it is a small hole in the septum of the atrium(the upper chambers of the heart). It communicates between the unoxygenated(right) and oxygenated (left) side of the heart. According to the echo blood flow is bidirectional which means blood flows from the right to the left mixing deoxygenated blood into the arterial system. The other way is better when oxygenated blood from the left is forced into the right side. Many people live with this condition and actually 1 in 4 adults have this when their fetal circulation which has a pfo doesn't close normally.


                severe lv and rv hypertrophy which is symmetrical and concentric: This is talking about the muscle of the right and left ventricle. It is saying it is hypertrophied, by either thick cells or abundant cardiac tissue cells. Concentric refers to pressure being the cause of the hypertrophy. Eccentric is also another term you may see but refers to volume being the cause of the thickening of the cardiac muscle.


                Pulmonary valve appears dysplastic with v max of 3.2m/s, 40mmHg: This refers to the valves apperance and movement, dysplastic doesn't move freely that it may be stiff and even cause it to block the blood from leaving the right ventricle to enter the pulmonary artery. The numbers are talking force and pressure, not sure what values are normal of the top of my head.

                severe right ventricular outflow tract obstruction with a gradient of 75mmHg: This is due to the pulmonary valve being dysplastic and RV hypertrophy which can cause a blockage of blood leaving the right ventricle and travel into the pulmonary artery which then travels to the lungs.

                [B]moderate left ventricular outflow tract obstruction with v max 3m/s, pg 35mmHg: This obstruction is usually caused by the left ventricle and septum of the left side of the heart thickening/hypertrophy. This is usually seen in HOCM and a usual cause for fainting.


                Mild to moderate mr: This means mild to moderate mitral valve regurgitation. The mitral valve is the valve between the upper and lower chambers of the left side of the heart. When the heart beats and blood is ejected from the left ventricle to the aorta, blood backflows into the left atrium (upper chamber) essentially flowing backwards.

                lvef course of the coronary arteries: This is discussing the anatomy of the coronary's. Like where is originates from. There is no wrong or right way.

                normal aortic arch with no coarctation: This is good. The aorta arches normally and is not narrowed (coarctation).

                confluent and normal size pulmonary arteries: This is good and normal.


                There did not appear to be any mild cavity obstruction. : This is good.

                There was diastolic dysfunction:: This means the left side of the heart cannot fill as well as it should during diastole(at rest when the heart doesn't pump). Systole is when the heart is in motion or pumping. When the heart cannot fill properly during diastole it doesn't pump very efficiently.


                His dusky lips could be from the pfo and the outflow obstructions. Usually a multitude of things.

                Hope this helps. And I wish you the best of luck. My husband has HCM and we are trying for kids and i know our children will have a 50:50 chance of having HCM. Best of luck.
                I have WPW and I am a Nurse Anesthetist.
                My husband has HCM.
                He was diagnosed in 2002 at 25 years old after a syncopal episode.
                AICD was placed immediately after diagnosis. He takes CCB's.
                I fear my husband doesn't take his condition seriously and "forgets" to take his medication.
                His mother was diagnosed with HCM after him.
                I am afraid our future children will have HCM and WPW.

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                • #9
                  Re: Introduction - my baby has HOCM

                  Hello and welcome. Your post kind of hit me because my daughter was diagnosed with Severe Concentric Bi-Ventricular Hypertophy. She was diagnosed at 6 years of age and her lift was not an easy one. She ended up with a transplant and is now in chronic heart failure. Reading through these posts there is one thing I would like to correct and that is that Concentric refers to ALL of the muscle being thickened: I have never heard any of my daughter's doctors say that concentric refers to pressures in the heart causing the hypertrophy. High blood pressure can cause hypertrophy, but then the hypertrophy is a secondary issue, not a primary issue. My daughter never had HBP and I have never had HBP yet she had a very severe form of this disease; all of the walls of both ventricles were extremely thick 4+cms. I was recently diagnosed with left ventricular hypertrophy and again, I have never had HBP. You have a hard road ahead of you, but you will become stronger for it. One of the places we were evaluated at was Great Ormond Street; I would caution you....we were told there was nothing wrong with our daughter's heart!!! Right.......!!

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