Our heart is like the engine of a car. Normally, the left and right ventricles work together, which is equivalent to a two-cylinder engine with strong power. But there is a type of heart malformation where the heart works with only one ventricle, which is equivalent to a single-cylinder engine. The child operated on today, a 3-year-old boy, is lively, cute and exceptionally smart. But his heart has only one ventricle. It turned out that the tricuspid valve in the fetal heart did not develop during his mother’s pregnancy due to some disturbance, and the right ventricle did not develop either, so after birth, the left ventricle assumed the work of body and lung circulation. However, soon after birth, the baby’s lips and fingernails and toenails became purple, he was short of breath, and he always had to stop sucking milk, so in 2012, the parents brought their 5-month-old baby to the Department of Pediatric Cardiothoracic Surgery of Jiangsu Provincial People’s Hospital Maternal and Child Branch. If such a heart malformation is not treated surgically, firstly, the lack of oxygen will seriously affect the brain development, and secondly, one ventricle taking up two circulations will definitely lead to heart failure and death in childhood. Therefore, in 2012, we performed the first surgery on this baby, connecting his superior vena cava to the right pulmonary artery, which means that the venous blood of his upper body is directly returned to the pulmonary artery to exchange oxygen with the lungs. This time we connected his lower venous blood to the pulmonary artery through an artificial vessel as well, thus completing the correction of the cardiac malformation, the cyanosis disappeared and the left ventricle returned to its proper job of assuming the body circulation. In fact, this type of surgery has been carried out in the United States and other developed countries as early as the 1950s, and the Fontan procedure, named after the famous cardiac surgeon Fontan, has been developed and improved over the decades to form the current procedure with the best results – the extracardiac conduit for total vena cava pulmonary artery connection. Long-term outcome studies have shown that there is no significant difference in cardiac function from normal in the quiet state.