Get Adobe Flash player
    SUPPORTING CHILDREN WITH CONGENITAL HEART DISEASE

    April 2013

     


    Double Inlet left ventricle &
     Mali Story

     

     

    Double inlet left ventricle (DILV) is a congenital heart defect appearing in 5 in 100,000 newborns, where both the left atrium and the right atrium feed into the left ventricle. The right ventricle is hypoplastic or doesn't exist.

     

    In the normal heart, the right and left lower chambers (ventricles) receive blood from the right and left upper chambers (atria). Oxygen-poor blood returning from the body flows to the right atrium and right ventricle. The right ventricle then pumps blood to the pulmonary artery, which carries blood to the lungs to pick up oxygen.
    The blood, now oxygen rich, returns to the left atrium and left ventricle. The aorta then carries oxygen-rich blood to the rest of the body from the left ventricle.

     

    However, in those with DILV, only the left lower heart chamber is developed, and both upper chambers carry blood into this ventricle. This means that oxygen-rich blood mixes with oxygen-poor blood. The mixture is then pumped into both the body and the lungs.

     

    Symptoms of DILV may include:

     

    • Becoming tired easily, especially during feeding
    • Bluish skin color, called cyanosis (the lips may also be blue), due to low oxygen in the blood flowing to the body
    • Failure to gain weight and grow
    • Pale skin (pallor)
    • Poor feeding
    • Sweating
    • Swollen legs or abdomen
    • Trouble breathing

     

    Infants born with DILV cannot feed normally (breathlessness) and have difficulty gaining weight. The mixed blood in systemic circulation leads to hypoxia (lack of oxygen to the body and organs), so infants develop cyanosis and breathlessness early. Mortality is very high in the first 2 years, 85%, but after it decreases and between 2 and 15 years old the mortality is only around 9%. Diagnosis must be made within few days or even hours to prevent death.

     

    Surgery for Dilv

     

    The surgery needed for DILV is a three stage surgery. The operations are all pallative surgery as the heart can never be repaired or fixed and eventually the person will normally require a Heart transplant.

     

    STAGE 1–NORWOOD

     

    This surgery is normally performed in the first week of life. A repair is made to allow the right ventricle to pump blood to both the lungs and the body. Because blood flow must be forceful for the immature lungs, the surgeon creates a tube from a branch of the aorta to the pulmonary artery known as the modified Blalock-Taussing shunt. The baby will still appear blue (cyanotic) after this surgery.

     

    STAGE 2 - bi-directional Glenn or Hemi-Fontan

     

    This surgery is typically performed at about 6 months of life when the blood starts to flow more easily and the ventricular force is not needed. The surgeon creates a direct connection between the superior vena cava and the pulmonary artery thus diverting half of the blood flow without the assistance of ventricular force. This reduces the work on the right ventricle by allowing it to only pump blood to the body and allowing all blood from the upper body to go straight to the lungs. This will hopefully stabilise the hearts function until stage 3 can be completed.

     

    STAGE 3 - FONTAN


    This surgery is often performed between 18 - 48 months. The surgeon connects the inferior vena cava to the pulmonary artery. This will allow the remaining blood coming back from the body to go directly to the lungs. The final result will be no mixing of blood in the heart with more oxygen rich blood going to the body. This will significantly improve the child's health and growth, and hopefully give them more energy and stamina.

     


     

    Mali Story

     

    We found out at our 20 week scan that Mali had a congenital heart defect. All our local hospital could see was that she had a hole between her bottom chambers. We had to wait a further two weeks before we could see a foetal cardiologist in Cardiff. It was here we found out the full extent of mali diagnosis. Being told our unborn daughter had a heart condition feltluike the end of the world. We had so many questions, but no one could answer them.would she survive? How will this affect her life in the future?

     

     

    The next few months of pregnancy were hard. Some days we would feel positive and then other days we would feel alone and scared of what the future held for our daughter. We carried on going to monthly scans up in Cardiff to see how Mali was doing. Luckily every time we went they were pleased with her and decided we could be induced in Cardiff as she wouldn’t require immediate surgery.

     

    I was given a date for induction for the 19th June 2012 at Cardiff University Hospital. After a very quick labour Mali arrived at 9.27am on 20th june 2012 weighing a healthy 7lb 14oz. We were allowed to hold Mali for a few minutes before they took her to neonatal for scans and an ecg to check her heart now she was born. After two hours of being away from Mali we got to see her again and we were given Mali full diagnosis. She had Double Inlet Left Ventricle, VSD, ASD, Pulmonary Stenosis and an abnormal tricuspid valve. They told us her heart was a univentricular heart, which ment she only had one pumping chamber, whereas the normal heart would have two.

     

    After a week on the maternity ward, the Drs were happy to discharge Mali as she was stable and her oxygen saturations were in the 80s. (a normal persons oxygen saturations are around 100%).

     

    At 5 weeks old it was decided Mali should have a cardiac catheter at Bristol childrens hospital to stent Mali pulmonary artery to try and get her oxygen saturations higher. On 23rd July 2012 Mali went down to the cath lab at 1pm. We were told it would take around an hour and a half, after three hours we started to worry as we had not heard anything from the Drs. An hour later Mali consultant came and told us that the stenting of the pulmonary artery had not gone to plan and unfortunately she was on her way to peadiatric intensive care. They explained that due to the way Mali heart was formed they could not get the stent into the artery so had to bring the stent back down the blood vessel it was in. As they did this the stent unravelled near the groin and cut the blood vessel it was in.They had never seen this happen before. She spent a couple of days ventilated to help her body recover, a week in PICU and one night on ward 32 then we were home. She recovered really well after this and showed us she was our little fighter.

     

    Our time at home with Mali was short lived as 3 weeks later Mali oxygen saturations dropped to 55-60%. Cardiff decided they wanted to admit her for the night to monitor her. That night though Mali continued to get worse and her saturations continued to fall into the 40% even on oxygen. Mali was then blue lighted up to Bristol childrens hospital with my mum and I following in the car. Over the next few days Mali still didn’t improve and she kept de satting very low. We were told she would need her first open heart surgery. This surgery was called the Glenn Shunt. We were warned by her surgeons that normally this operation was done between 4-6 months, Mali was only 10 weeks old. They said they weren’t sure how she would cope with the surgery and how long her recovery would be due to her age.

     

    Unfortunatly this was Mali only chance of survival as without it she would eventually die. We trusted the surgeons and had faith she would show us she would be a little fighter all over again. Taking her down for surgery was the hardest and most emotional thing we have ever had to do. We stayed with her until she fell asleep, then we left her with the amazing surgeons and staff.

     

    Mali spent 7 hrs having surgery, we were told after 7 hours all had gone as planned and she had survived surgery. Now she just needed to get through the recovery. Mali recovery was slower than normal but after 12 days in PICU, 8 of those days ventilated and 2 days on ward Mali was allowed home. She had surprised all the doctors at how well she had recovered and proved to us all she was a little fighter.

     

    We are now 7 months post op and she is doing fantastic. She has learnt to sit and is now attempting to stand with help. We are so proud of how far she has come. She may still have more operations to come but at the moment we are enjoying everyday with our beautiful little fighter Mali, Our hero.

     

     

    Follow Us

    Amelia in Hospital

    twitter

    Amelia in Hospital

    linkedin