8.30.2010

Hypoplastic Left Heart Syndrome (HLHS)

Ever since meeting with the fetal cardiologist I've stayed away from researching HLHS, which is what Kaleb will have if his left side of his heart does not continue to grow. I've done a great job of this until now, at some point I needed information on it. Why are all of Kaleb's conditions so rare? When I heard he had a heart defect I almost felt hopeful because even though clearly the heart is one of the most important organs in the body, children are born with heart defects often and current medical science is so amazing in what they can do these days. I feel like I'm constantly pushing against something and there's no give, I'm getting tired. Like I said before, I just want someone to say to me, "Oh I see this a lot, we can fix it". 

Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle aortic valve, and aorta) do not develop completely. The condition is congenital (present at birth).
Initially, a newborn with hypoplastic left heart may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include:
  • Lethargy
  • Poor suckling and feeding
  • Shortness of breath
  • Rapid breathing
  • Cold extremities
  • Enlarged liver
  • Poor pulse
  • Pounding heart
  • Bluish (cyanosis) or poor skin color
  • Sudden death
In healthy newborns, bluish color occurs in hands and feet as a response to cold (this reaction is called peripheral cyanosis).
However, a bluish color observed in the chest or abdomen, lips, and tongue is abnormal (called central cyanosis) because it reflects lack of adequate levels of oxygen in the blood. This is secondary to the heart malformation and circulatory malfunction. Central cyanosis often increases with crying.
Once the diagnosis of hypoplastic left heart is made, the baby will be admitted to the neonatal intensive care unit. A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to maintain circulation of blood to the body.
These measures do not solve the problem. The condition always requires surgery.
The first surgery, called the Norwood operation, occurs within the first few days of life. Stage I of the Norwood procedure consists of building a new aorta, and an artificial shunt is inserted to maintain blood flow to the lungs.
Afterwards, the baby usually goes home. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be done.
Stage II of the operation is called the Glenn shunt or Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the body (the superior vena cava) directly to blood vessels to the lungs to get oxygen. The surgery is usually done when the child is 4 - 6 months of age.
During stage I and II, the child may still appear somewhat blue (cyanotic).
Stage III, the final step, is called the Fontan procedure. The remainder of the veins carrying blue blood from the body (the inferior vena cava) is connected directly to the blood vessels to the lungs, and the right ventricle now serves as the only pumping chamber for the heart. This surgery is usually performed between the ages of 18 months and 3 years of age. After this final step, the baby is no longer blue (cyanotic).
Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure.
In some hospitals, heart transplantation is considered a better choice than the 3-step surgery process. However, there are few donated hearts available for small infants.
Hypoplastic left heart is a rare type of congenital heart disease. It is more common in males than in females.
The problem develops before birth when there is not enough growth of the left ventricle and other structures (aortic and mitral valves that guard the exit and entrance of the ventricle and the aorta, the blood vessel that carries oxygenated blood from the left ventricle to the entire body).
In patients with this condition, the left side of the heart is unable to send enough blood to the body. As a result, the right side of the heart must maintain the circulation for both the lungs and the body. This extra workload eventually causes the heart to fail.
The only possibility of survival is a connection between the right and the left side of the heart its blood vessels, through which blood may pass. This is called a shunt. Babies are normally born with two of these connections (the foramen ovale and the ductus arteriosus), which normally close on their own a few days after birth.
If ductus arteriosus is allowed to close in a baby with hypoplastic left heart syndrome, however, the patient will quickly die because no blood will be pumped to the body.
As with most congenital heart defects, there is no known cause. Around 10% of patients with hypoplastic left heart syndrome will have other birth defects.
A physical exam may show signs of heart failure, liver enlargement, rapid breathing, and lethargy. Additionally, the pulse at various locations (wrist, groin, and others) is usually very weak. There are usually abnormal heart sounds when listening to the chest.
Tests may include:
  • ECG shows enlargement of the right ventricle of the heart
  • X-ray of the chest may show signs of other birth defects
  • Echocardiogram
  • Cardiac catheterization
If left untreated, hypoplastic left heart syndrome is fatal. Survival rates for the staged repair continue to rise as surgical technique and postoperative management improve. Survival after the first stage is more than 75%.
It is important to note that the size and function of the right ventricle are important determinants of outcome after surgery.
There is no known prevention for hypoplastic left heart syndrome. As with many congenital diseases, the causes of hypoplastic left heart syndrome are uncertain and have not been linked to a mother's disease or behavior.
Complications include:
  • Irregular, fast heart rhythms (arrhythmias)
  • Chronic diarrhea (from a disease called protein loosing enteropathy)
  • Heart failure
  • Fluid in the abdomen (ascites) and in the lungs (pleural effusion)
  • Blockage of the artificial shunt
  • Strokes and other neurological complications
  • Sudden death

15 thoughts:

justine

I can't even imagine what this feels like. But I do know that there is such thing, sometimes, as too much information. Sometimes, all you can do is love that little life in you ... and pray, if you can find it in you to do so. I'll be thinking of you. *hug*

rebecca

I'm so sorry, I cannot begin to imagine what you are going through. All I can offer are prayers & support. A book that I read recently after losing my daughter brought me a lot of comfort...I Will Carry You by Angie Smith, it's an incredible book & one that may bring you strength in the moments when you need it most. Thinking of you & sending many prayers for you and your family.
~LFCA

Baby bump bound

Here by way of LFCA- just wanted to say I am so sorry and my thoughts are with you and your beautiful family.

Sassy Gal

Hello,

I don't know you but, I have some friends and there son has this same thing. If you would like I can take your information and pass it on to them. I know that they are always willing to help a couple that recieve this diagnosis. There son is 5. So there is a positive outcome even when what all the doctors tell you sounds really bad. Anyway, if you want leave your info on my blog givingupadream.blogspot.com and I will get that out to them right away.

Praying for you,
Pez

Suzy

I can't imagine what you are going through, my heart just aches for you. I know a number of people who have lost a child because of undiagnosed HLHS. Hopefully at least because you know what the problem is, they will be able to fix it. The surgeries success rates are going up and up.
I will be thinking of you, and praying for your little one. xxx

Katie

I am so very sorry. I can't imagine how difficult this must be. I will be praying for you and your baby.

Hanen

Here from LFCA and sending lots of good wishes. As worrying as all the research is, I hope it means you feel a bit forewarned, and better able to talk to the doctors. xxx

cgd

Here from LFCA. I am so sorry you are going through this. I just wanted you to know that I went through a poor fetal diagnosis in March/April. It was one of the darkest moments of my entire life. I had no idea how I was going to breath, walk, be a person in this world with such a sick baby. My heart breaks for you and your family. Please reach out of support, we are here for you.

Melody

Here from LFCA. My daughter also had congenital heart defects-- not the same ones but serious ones. Her heart was swiss cheese. She had surgery to repair the worst of the holes (3 of them) when she was 4 months. She was growth restricted in utero and had a bad umbilical cord so I was closely monitored. She had some brachycardia in utero, so I did see a fetal cardiologist, but her real heart problems did not become apparent until a few days after her birth.

Anyway, it was rough, but she survived surgery and follow-up procedures (there were complications), and she's now a thriving 19-month old-- walking, talking, and beautiful.

It IS hard, and you DO need to talk to people who have gone through/are going through the same thing. I know my daughter had different congenital defects, but feel free to e-mail me if you have questions or want to talk about what the process was like for me. I do have advice for parents whose children are having surgery and will be spending an extended amount of time in the hospital.

Jenn

Also here from LFCA and I just wanted to say how sorry I am that you and your family are having to deal with all of this. I can't even imagine. I'll be praying for you and your precious baby. (hugs) to you.

cowgirltn

I will start my prayers for his heart to grow and form completly.

Anonymous

Here from LFCA. Have you heard of the blog It's Either Sadness or Euphoria? I think it's sadnessoreuphoria.wordpress.com. She has a heart baby who has had the same surgeries your child will need. You should look her up. Good luck to you and Kaleb, you will be in my thoughts.

.daily.amos.

Hi. I too, like many others, am here by way of LFCA. I've read Kaleb's story and my heart goes out to you and your family.

I understand what it is like to have a poor prenatal diagnosis given to you and how it rocks your world. It is a very lonely experience.

I hope that you get some good news soon and Kaleb's heart will continue to grow.

-Brianna

Erin

I thank all of you wonderful people for these sweet comments, ;) Pardon my dumbness centered around acronyms but what is LFCA? I feel so dumb asking that question but I am really confused, lol. It's so great though to know we have so many people thinking about us and giving us their stories. THANK YOU!!

Searching

I am so sorry about your tiny Kaleb! I would encourage you to connect with other parents who have kiddos with the same diagnosis. I am sure the high risk OB/perinatal cardiologist will give you more information as time goes on and more is known and he is closer to being born. It is a very serious condition and very scary!! Sometimes it's just too hard for the little ones to overcome and sometimes they have surgeries that are able to give them a good shot at life. I am very glad you are getting good care so everyone on your medical team can be prepared when he arrives! There ARE babies who survive with HLHS. Praying for you and your baby!

Angie who wrote the book "I Will Carry You" also has a blog http://audreycaroline.blogspot.com/

*LFCA- http://lostandfoundandconnectionsabound.blogspot.com/ done up by Mel from Stirrup Queens, she is amazing!!

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