The era of treatment of hypoplastic left heart syndrome (HLHS).


In Ukraine, the era of treatment of hypoplastic left heart syndrome (HLHS) has begun.
Summary: The hypoplastic left heart syndrome (HLHS) is the most difficult group of congenital heart defects, when any part of the left heart (valves, ventricle or aorta) is dramatically underdeveloped, which leads to heart failure in general to provide the systemic circulation , i.e. an adequate blood flow in the body of a child. Although HLHS occurs in about 3% of all congenital heart defects, this syndrome is the cause of 23% of the “heart” deaths in the first week of neonatal life. HLHS treatment is always surgical and usually consists of three stages, the first of which – Norwood operation, is performed in the neonatal period.
Then, at the age of 3-6 months, the next surgery is to come – the Glenn anastomosis, connecting the main vein to the pulmonary artery bypassing the heart. Further, usually between the ages of 5-6 years, the child has his/her third operation – the Fontan operation, connecting the inferior vena cava to the pulmonary artery, allowing venous blood completely drain into the pulmonary artery to bypass the right ventricle. Thus ends the cycle of operations to transform a single right ventricle to the system.
Although these operations are palliative, i.e., they do not correct an anatomical heart disease, at least 85% of children who have had all three stages, live 25 years after surgery, and even have their own children. Unfortunately, many of them in the future, may need a heart transplant. Such a poor long-term prognosis is related to the fact that the right ventricle is phylogenetically weaker and is unable to work for a long time in a high-pressure system. However, with all these difficulties, HLHS surgery is the only chance of survival for these children.
In addition, HLHS, is perhaps, the most “demanding” defect to the skill and experience of the team that does not forgive even minor errors in surgical technique, anesthesia, intensive care or perfusion. Therefore, HLHS treatment rightfully belongs to “aerobatics” in cardiac surgery.
We spent a lot of time getting to this day, and today we can say with confidence – the era of HLHS treatment in Ukraine has begun! Almost simultaneously the two children with HLHS have been successfully operated – Genghis in our center, and a 5-year-old Sofiyka in Amosov National Institute of Cardiovascular Surgery. Today, Genghis is already preparing for discharge, Sofiyka is still in intensive care department, but she is surely recovering.
We wish the children a speedy recovery, and congratulate our cardiac team with these achievements, we would also like to remind you that there is still a lot of work, because finally we got off the grounda transplant problem. And if at any time in the future Genghis or Sofia will need a heart transplant, we need to be ready for it.
We would like to share our success with the people, without whom Genghis would have no chance at life, with people who help us for many years, support in times of hopelessness and despair (this sometimes happens with us) make us move forward, sometimes simply because we can not fail their trust. These are our permanent sponsors and friends – Sapronov Yuri, Chernobyl Children International and Novick Cardiac Alliance. This is Ukrainian Society of Switzerland, school students of Boyko school, IT specialists from Brightgrove, LTD and Promodo, Olga Nonko, Leonid Filshtinsky and hundreds of ordinary people helping us, who believe in us and trust us! Heartfelt thank you!