Hypo-Plastic Left Heart Syndrome
New-born baby Joshua Tinworth needs open-heart surgery to give him a chance for life, but the risks are high. Luke and Alex Tinworth are looking forward to the arrival of a new baby brother. For mum and dad, eager anticipation of the new arrival is mixed with anxiety. In Karen Tinworth's 21st week of pregnancy, a routine ultrasound examination picked up a serious heart defect. Karen's unborn baby had a condition known as Hypo-Plastic Left Heart Syndrome One side of the heart is severely underdeveloped. Early diagnosis means treatment is possible, and Karen's baby is now under the care of Great Ormond Street's cardiologist Ian Sullivan.
After the baby is born, the left ventricle will not be strong enough to pump the blood around the body as it should. Left untreated, he would die soon after birth. Survival is made possible by some highly complex surgery which changes the way blood flows in the heart. It carries a high risk and the survival rate is not very good.
The baby will be induced at the Elizabeth Garrett Anderson and Obstetric Hospital and at nearby Great Ormond Street a team will be ready to perform surgery on Joshua's heart as soon as possible after he's born. After the birth, Joshua's skin starts to turn blue indicating a lack of oxygen in his blood. He'll need to be stabilised in intensive care before the short journey to Great Ormond Street.
Joshua is rushed to Great Ormond Street, but there's a complication and surgery can't go ahead as planned; Joshua's been having trouble with his breathing. He has a chest infection and needs to be out on a ventilator and a course of anti-biotics. They discover some fluid in his lungs which they don't think is related to his heart condition. Despite his chest problem, Joshua is stable and safe in intensive care.
Six days later, Joshua is well to go in to surgery, for the first of three operations he'll need. There will be a further surgery in six months time and then again when he is two years old. These surgeries are known as The Norwood Procedure, after the surgeon who developed them. The first surgery carries the greatest risk to Joshua's life.
Consultant Cardiothoracic Surgeon, Victor Tsang will be conducting the operation. He tells us "We have made a lot of advances in small baby open-heart surgery. The technology, the understanding and the experience have provided us with confidence to deal with very difficult problems, like Joshua's".
The operation is expected to last three hours, and will completely alter the way blood flows around Joshua's heart. Mr Tsang is a world-renowned specialist in this field, but even for him, working on such a tiny heart is a massive challenge. He explains the procedure "We use a lot of human valve tissue in complex baby heart surgery. In a lot of these surgeries we need to reconstruct many defects, and the human heart tissue is very accommodating".
For much of th operation, Joshua will be on a heart lung bypass machine, but for the most complex part of the surgery, his heart and circulation will be stopped altogether. Joshua's body temperature is lowered to 18°, normal body temperature is 37°. The low body temperature will minimise damage to the vital organs while the heart and circulation are stopped, but it's only safe to keep Joshua in this state for a maximum of 45 minutes.
The underdeveloped left ventricle, of Joshua's heart, is not strong enough to do it's job of pumping blood around the body. Mr Tsang will create a new pathway, bypassing the left side of the heart, connecting the pulmonary artery to the aorta, so that the right ventricle pumps blood to the lungs and the rest of the body.
The Surgery in Progress
Mr Tsang's reconstruction of the heart has to ensure blood supply to the lungs and to the body is evenly balanced. Too much, or too little could be fatal when Joshua is taken off the bypass machine.
Finally, Joshua's newly reconstructed heart takes control of his blood circulation. This is the moment of truth and the operation seems to have gone well. The chest will be left open, for now, to minimise the pressure on the heart after a very long operation. This operation was the most hazardous of the surgeries Joshua will face, and this is a major step forward.
Joshua has had a series of chest infections and some blood pressure problems in the two weeks following hi open-heart surgery. Now though, he seems to have pulled through the worst of his difficulties.
Sadly that did not remain the case, many more infections led him back to Great Ormond Street to have the second Norwood procedure. Complications arose and he was not well enough to leave intensive care. He died, in his mother's arms, six weeks later.
CREDITS: All of this information came from the UK Channel 5 "Child in a Million" documentary series