Heart Stuff

This page is a work in progress. I’ve noticed that some people are finding our blog by searching for heart defects similar to Matthew’s, and I wanted to provide some more detailed information.

Matthew’s heart defect was called a Complete Atrioventricular Canal. This is basically a gigantic hole in the center of the heart. In medical terms, the defect has several components:

  • ASD or Atrial Septal defect. This is a hole between the top chambers of the heart
  • VSD or Ventricular Septal defect. This is a hole between the bottom chambers of the heart
  • Valvular defect. The two valves that normally separate the top and bottom chambers were fused into one common valve.

A Normal Heart:

Complete Atrioventricular Canal:

Normal Valves:

Valves with a Complete AV Canal:


The surgery to repair this defect involves patching the ASD and VSD, and separating the common valve into two separate, functional valves. This is a complicated surgery, but is done with a very high success rate.

Surgeons rebuild the walls of the heart with a patch, which is usually made out of a piece of tissue taken from the pericardium (sac around the outside of the heart). Then, the valves are reconstructed and attached to the patch. This is the tricky part of the surgery, as the one common valve can be complex to repair. The surgeon does his best to create valves that are tight enough not leak (medically, “regurgitation”), but loose enough to not become stiff and hard (“stenotic.”)

Repaired valves:

Surgical Risks:

This surgery really does have a very high success rate – “success” meaning the heart is effectively repaired and the child survives the surgery and goes on to have many years of a good quality of life. However, as with any surgery, there are risks involved.

  • The main risk is that years after the surgery, one of the repaired valves may become too stiff or too leaky to be effective, and may need to be replaced.
  • When the patch is placed in the ventricular septum, there is a 5-20% chance that the electrical conduction system of the heart will be damaged. This can lead to “heart block” and the possibility of the need for a permanent pacemaker. This is why we were so relieved that the VSD portion of Matthew’s heart defect healed spontaneously!
  • Children with Down Syndrome are at increased risk of post operative complications such as infections and lung problems – specifically pulmonary hypertension (increased blood pressures in the lungs, which can lead to fluid-overload in the lungs). In fact, pulmonary hypertension is more common in kids with Down Syndrome with or without the presence of other congenital heart defects.

6 thoughts on “Heart Stuff

  1. Hi “Matthew’s Mom”, my name is Lori and I am 22 weeks pregnant with a little girl who has been diagnosed with Down syndrome as well as heart defects which include: Hypoplastic left heart, COA, and VSD. I just had my 2nd fetal echo on friday, 2 days ago, and the prognosis is grim for our little girl.
    I am looking for words of support and your blog was forwarded to me. Your situation is almost exactly like ours and I feel so grateful to have read your success story!! I am not giving up on our baby even though so many are quick to give us her worst case scenario–death.
    We are going to call Seattle Children’s Hospital on Monday for a 2nd opinion/consultation as I don’t feel very supported by our current medical team. I appreciate any words of advice or encouragement you might have for us. I realize you are probably very busy–I am just overjoyed to hear that it is possible for a Ds baby to overcome these prenatal diagnosis’ and surprise everyone with their determination to thrive! Thank you so very much and best wishes of health and happiness to you and your amazing family.
    With thanks,
    Lori

  2. Lori,

    I sent you an email giving you our phone #. Please call! I would really love to talk with you more.

    Thank you for not giving up on your precious baby girl. I really do know how difficult this whole situation is, and how hard it is to hang on to any hope.

    I’m glad Matthew’s story can encourage you a little bit, and I would like to support you in any way I can. Please know that you, your family, and your little girl will be in our prayers!

    We saw the Seattle perinatologists 2 times when I was pregnant with Matthew. We actually didn’t feel very comfortable with them. They were very competent medically, of course, but we felt like just another “case” while we were there. They offered and encouraged abortion as an option, and when my husband Aaron left the room for a moment, they were quick to remind me that it was “my body, my decision” and I shouldn’t let my dear husband “influence” me! When we refused the abortion, they offered “early induction” as an option, meaning they could induce labor before Matthew was at a viable age, and he would die almost immediately thereafter.

    We transfered care to Spokane (Sacred Heart Children’s Hospital), and found their team (especially Dr. Anderson, also Dr. Burg, but they are all good) supportive, kind, empathetic, and every bit as competent medically as the Seattle team. Dr. Siwek did a fantastic job repairing Matthew’s heart surgically, and we will be forever indebted to him for that. The nurses and staff there were all incredibly kind and competent as well. It was nice to be in a Christian atmosphere and have the Center for Faith and Healing at a time like that, too.

    Also, please know that even when Matthew wasn’t expected to live – even when I thought any day could be his last – I was so very grateful for the time we had with him. Even if he had only spent a few days or weeks with us, his life would have touched us and made us better people. Even if we would have lost him, I wouldn’t have changed a thing – I would still have gone through it all just to be able to hold him, even if it was just for a little while.

    You will go through things that no parent should ever have to go through, and you will have to make decisions that no parent should ever have to make. But, just take things one day at a time – and just keep making the decisions that are right at each point in your journey. Right now, you have already made such a good decision in deciding to continue your pregnancy! All you can do is keep on doing what’s right for your little girl, and keep giving her the best chance you can.

    My heart is with you. Please keep in touch.

    Prayers,
    Amy Vawter (Matthew’s mom)

  3. I received your web link from a friend who used to go to our Church of the Nazarene where my husband is the pastor in Coeur d’Alene. [Check out Pastor Ron Hunter’s sermon in our church site. Click on the left side to his section.]
    Your Matthew was the same size as our son…who will be 41 on Billy Graham’s 90th Birthday on November 7th. Thank you SO much for sharing your stories and miracles!! If you have time to read our daughter’s stories on her blog, check it out at http://www.bewondered.blogspot.com. Her son had his cord wrapped around his neck and also a complete knot in the cord. She had to have a C-Section after trying to have him all day at KMC in Coeur d’Alene, Idaho. God bless you and yours!

  4. Amy,

    Reading your blog I see that you are trying to provide information for other families. WOuld you care to look at our website and see if you think it would be helpful and then perhaps link to it?

  5. Lori,

    Isn’t God amazing? Our little girl was diagnosed with a Tetralogy of Fallot at birth (along with Downs). At the time we didn’t know anything about Downs or heart conditions so it was all a big shock to us. We have had a similar experience – an outpouring of support and prayers by friends and family. It is very encouraging to know that other families have experienced some of the same emotions that we have – the fears about infection, of tet spells, etc. that others don’t really understand (can’t say how many times I get “don’t you think you’re being a bit overprotective?” from family members who just don’t understand the risks of infection). Our little girl had her surgery on June 23 and she has had a miraculous recovery – 6 days post op in the hospital (and we opted to stay an extra day “just in case”), and she is doing very well at home now. She is still on her meds, and still house-bound but hopefully we’ll be out and about (and off lasix) soon. Thanks for your encouraging story – I’ve linked to it from my blog AND my facebook page.

    -Amelia
    http://needlesandchard.blogspot.com

  6. I’m lovin’ reading your site as I sit here in the hospital with my baby girl recovering from her av heart surgery. This is so great. reading on..

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