Congenital Heart Defects: Author Martha Ramirez

Thank you, Jordyn, for having me on your blog. It’s great to be here! And thank you for celebrating my new children’s picture book, Broken Heart with me.
On March 11th of 2013, my life completely changed. Well, to be more precise, it was the year 2012 in October. In October, after undergoing Holter monitor testing and an echocardiogram (echo), my cardiologist discovered my heart was enlarged. After extensive testing and a conference held by the top specialists in San Francisco, they concluded open-heart surgery was needed to correct a congenital heart defect we never knew I had.
Wow. A congenital heart defect? Me?
Here I was, trying to digest the news. Trying to keep the faith. Striving to keep it together. I went from never having surgery in my life to full blown saw-through-the-sternum open-heart surgery.
If I hadn’t followed up with doctors, they never would’ve discovered it. It definitely pays to seek medical care, even when you’re well.
The doctors said I would’ve grown more tired each day and my heart would have enlarged until it stopped working. So here I am now, grateful to be sharing my story. And thankful God heard all the heartfelt prayers.
Photo by L.I.L.A Images
Inspired by my own heart story, my children’s book, Broken Heart, is about a brave girl who learns doctors have to mend her broken heart. Seven-year-old Julia goes on an unforgettable heart journey and takes her twin sister along for the ride. And because it’s very close to my heart, its debut is on the 13th month of my open-heart surgery anniversary. This book was inspired by my experience of open-heart surgery and having to stay strong and pray for the best.
I’ve learned a lot about the medical field in one short year. Lots of testing. Lots of patience. And lots of faith, that’s for sure. In the end, it was all about listening to my body and not ignoring my intuition that saved me. That and a whole lot of prayer.
Thank God for doctors and for new technology. I’m truly blessed that I had a team of top doctors looking out for me.

May you all stay heart healthy and heart strong.

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In addition to writing, Martha is a 2012 Genesis Semi-Finalist, a member of ACFW (American Christian Fiction Writers), YALITCHAT.ORG, the Muse Conference Board, CataNetwork Writers, and American Author’s Association. Her articles have appeared in various places including the Hot Moms Club and For Her Information (FHI) magazine. In 2012, her blog was nominated website of the week by Writer’s Digest. She looks forward to expanding her career and is hard at work on her next young adult novel. She currently resides in Northern California where she enjoys gardening and kickboxing (not simultaneously). Visit her blog at Martzbookz.blogspot.com.

What is Hypoplastic Left Heart Syndrome?

What is Hypoplastic Left Heart Syndrome (HLHS)? It is the most deadly type of cardiac congenital anomaly an infant can have.  It wasn’t more than 60 years ago when nothing could be done for these babies. It has a 100% mortality rate without intervention. Even as recently as the 90s the mortality was 40-50%. Now, over 90% of children are living and one year survival rates are around 68%.
The easiest way to think of HLHS is that the infant’s left ventricle is severely underdeveloped and blood isn’t oxygenated the normal way it should be. The left ventricle is the portion of your heart that is most responsible for propelling blood forward to the rest of your body. If that isn’t working well– you can see how this can prove difficult for the infant. 
Now, there are only two options for treatment of this rare but fatal heart condition and that is a three staged surgical repair (A BT shunt, a bi-directional Glenn, and a Fontan repair) done over the first couple of years of life or a heart transplant. Even if the infant goes to transplant they will likely need one lifesaving surgery after birth because their life depends on certain holes in the heart staying open. This is where the term “ductal dependant” lesion comes from. The obvious difficulty with offering transplant for these patients is the scarce number of infant hearts available for transplant.
What’s interesting and new in medicine is that they’re trying to predict, based on some blood testing, which infants will do better getting surgery and which will do better going to transplant. What they’re looking at is a micro RNA marker which is a biomarker that could predict the success of surgery for these patients. 
What might be some signs of a congenital heart defect (not necessarily this one) in an infant? Even with today’s ultrasound technologies– not ALL cardiac anomalies are detected prior to birth though a large majority are.
Look for:
1. Increased breathing rate above what would be normal for that age. A normal respiratory rate for an infant is between 40-60. Anything consistently over 60 breaths/minute is concerning. 
2. Increased heart rate.
3. Hepatomegaly. This means the liver is large. The liver sits on the right side of your abdomen housed just under the right rib cage. You should be able to feel the lower edge (maybe 1/2-1 finger breadth) but anything over that should be checked. The reason the liver will become palpable is due to blood congestion from a poor functioning heart.
4. Difference in blood pressure between the upper and lower extremities. As a screening test, blood pressure may be done on all extremities.
5. Difference in oxygen level between upper and lower extremities. 
Another way to pick up on a heart defect in a child is to give them oxygen and see if it changes their oxygen levels. If an infant’s heart is “shunting” where oxygen may not go directly to the lungs then giving oxygen will not change the patient’s oxygen levels or it will change them very little. 
Below are some steps we would use to stabilize the infant in the ED setting for a “ductal dependant” heart lesion: 
1. Give medication to keep the holes in the heart open. This would be a prostaglandin drip.
2. Prevent shock but don’t overload the heart. This is typically done through fluid boluses but we would give half the normal dose over a longer time period.
3. Use a vasopressor (this is a medication to keep the BP up) like Dopamine if needed.
4. If continued blood pressure support is required then add a medication like Milrinone which will decrease SVR (the resistance in the body the heart has to beat against) and increase circulation.
 
So, if you need a tough diagnosis for your fictional pregnant family to deal with–Hypoplastic Left Heart Syndrome might be the choice for you.