A defect in the veins leading from the lungs to the heart.
In TAPVC, the blood does not take the normal route from the lungs to the heart and out to the body. Instead, the veins from the lungs attach to the heart in abnormal positions and this problem means that oxygenated blood enters or leaks into the wrong chamber.In the right atrium, oxygen-rich (red) blood from the pulmonary veins mixes with low-oxygen (bluish) blood from the body. Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium. From there it goes into the left ventricle, then into the aorta and out to the body. The rest of the blood flows through the right ventricle, into the pulmonary artery and on to the lungs. The blood passing through the aorta to the body doesn’t have a normal amount of oxygen, which causes the child to look blue.
Symptoms may develop soon after birth. In other children, symptoms may be delayed. This partly depends on whether the lung veins are blocked as they drain toward the right atrium. Severe obstruction of the pulmonary veins tends to make infants breathe harder and look bluer (have lower oxygen levels) than infants with little obstruction. When surgical repair is done in early infancy, the long-term outlook is very good. However, your child will need regular follow-up with a pediatric cardiologist and, once your child reaches adulthood, lifelong regular follow-up with a cardiologist who’s had special training in congenital heart defects. Follow-up is needed to make certain that any remaining problems, such as an obstruction in the pulmonary veins or irregularities in heart rhythm, are treated. Some children may need medicines, heart catheterization or even more surgery.
Treatment for hypoplastic left heart syndrome requires either a three-step surgical procedure called staged palliation or a heart transplant. The first step is the Norwood operation.This procedure is performed shortly after birth. It converts the right ventricle into the main ventricle pumping blood to both the lungs and the body. The main pulmonary artery and the aorta are connected and the main pulmonary artery is cut off from the two branching pulmonary arteries that direct blood to each side of the lungs. Instead, a connection called a shunt is placed between the pulmonary arteries and the aorta to supply blood to the lungs.
The Norwood operation is the most complex and highest risk procedure in the sequence of staged reconstruction for hypoplastic left heart syndrome. Current management at major pediatric heart centers has resulted in survival rates of 75 percent or better. The recovery period in the hospital following the Norwood operation is often unpredictable and complicated, averaging about three to four weeks. A small percentage of patients who leave the hospital may continue to experience significant problems in the first months of life.
Occasionally, the right ventricle does not function well following the Norwood operation, and in some case, cardiac transplantation may need to be considered.
If a child with hypoplastic left heart syndrome reaches the time for the second stage (about 4 to 6 months of age) without major complications, the survival through the Glenn and Fontan operations are much better, exceeding 90 percent with current methods.
Almost all children with hypoplastic left heart syndrome will continue to need some cardiac medications to maximize the efficient function of their heart, and all will require regular periodic follow-up visits with their cardiologist to evaluate their cardiac function and detect late complications such as arrhythmias.
Congenital heart disease (CHD) is the most common congenital disorder in newborns. There are no published data on the occurrence of congenital heart disease in Kerala. However there is general agreement among health workers that it follows a similar pattern all over the world in different geographies. The reported prevalence of CHD at birth ranges from 6 to 13 per 1000 live births. On an average, 8 children are born with congenital heart defects for every 1000 live births in India. Variation is primarily due to the use of different methods to detect CHD (fetal echocardiography versus postnatal referral to a cardiac center). Deriving from the crude birth rate, it…
keep readingCongenital heart disease (congenital heart defect) is an abnormality in your heart’s structure that you’re born with. Although congenital heart disease is often considered a childhood condition, advances in surgical treatment mean most babies who once died of congenital heart disease survive well into adulthood. While medical advances have improved, many adults with congenital heart disease may not be getting proper follow-up care. If you had a congenital heart defect repaired as an infant, you likely still need care as an adult. Symptoms Symptoms or signs of congenital heart disease may not show up until later in life. They may recur years after you’ve had treatment for a heart defect.…
keep readingMinimally invasive heart surgery (also called keyhole surgery) is performed through small incisions, sometimes using specialized surgical instruments. The incision used for minimally invasive heart surgery is about 3 to 4 inches instead of the 6- to 8-inch incision required for traditional surgery. The traditional heart surgery procedure Open heart surgery is typically done through a vertical cut placed over the middle of the chest, including full division of the breastbone.While most patients tolerate this well, it can take around 12 weeks or more before the wound is completely healed. This can seriously delay a return to normal activities. These days, it is often possible to avoid such invasive options…
keep readingabout Dr sajan koshy
Pediatric Cardiac Surgeon
Dr Sajan Koshy MS MCh is currently heading the unit of Paediatric & Congenital heart surgery at Aster Medicity. Prior to joining Aster, he was working as senior consultant and head of the department of paediatric cardiac surgery at MIMS Calicut. He started his professional career from alleppey medical college in 1988. He also functioned as a co- convener in first and second symposia on perioperative care of congenital heart disease held in Kochi in the year 2002 and 2004 respectively & as the scientific committee chairman for the national CME of cardio thoracic surgery held at Calicut in September 2007. He has Conducted many lectures in all the districts of North Kerala for the benefit of medical professional bodies to create awareness about congenital heart disease.
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