Heart “tube” completely misaligned “loophole” to maintain the life of a three-year-old child

  Recently, the Department of Thoracic Surgery of Jiangsu Provincial People’s Hospital successfully performed Warden radical surgery for a young child with a rare congenital heart disease – a special type of complete pulmonary vein ectopic drainage, saving the child’s life. This is a rare condition that has not been reported in China.  Ning Ning, a child from Lianyungang, was almost three years old and had a slightly greenish purple lip shortly after birth, which did not concern his parents. When Ning was more than two years old, he became dyspnea and cyanosis when he ran around a little. A heart ultrasound showed an enlarged right atrium and an atrial septal defect, which was diagnosed as congenital heart disease – atrial septal defect.  The parents of the child asked around and found out that Jiangsu Provincial People’s Hospital performs pediatric heart surgery, so they brought the child to the hospital. The director of pediatric cardiothoracic surgery, Gu Haitao, received the patient and found that Ningning’s lips and extremities were mildly cyanotic, and the left anterior chest wall was obviously bulging, so he asked the pediatric cardiac ultrasonographer to examine the heart with cardiac ultrasound and found that the child not only had a small defect in the atrial septum, but the more complicated malformation was that the left and right pulmonary veins were completely misaligned. This caused the right atrium to be huge and the left atrium to be stunted because it did not get enough blood, which also made the low-oxygen body venous blood and high-oxygen pulmonary venous blood, which were not in contact with each other, mix together, and a small part of it was transfused to the whole body through the atrial septal defect, which greatly reduced the oxygen content of the blood, resulting in symptoms such as purple lips and difficulty in breathing, while most of the blood flowed to the pulmonary artery, causing Severe pulmonary hypertension.  Dr. Gu Haitao, chief physician of thoracic surgery, organized a consultation with relevant personnel and clearly diagnosed this as a rare and special type of complete pulmonary vein ectopic drainage. The surgical approach was different from the previous pulmonary vein ectopic drainage. In common complete pulmonary vein ectopic drainage, the left and right pulmonary veins converge together to form a common trunk, which can be easily anastomosed to the left atrium. In this patient, the left and right pulmonary veins were not confluent but opened separately. A review of the foreign literature revealed that Dr. Warden had reported that a specific type of partial pulmonary vein ectopic drainage was perfectly suited for the correction of this patient’s malformation. The Warden method was used to correct the cardiac malformation and normalize the abnormal blood flow. Postoperative ultrasound examination of the right and left atria was normalized.  Dr. Gu Haitao, chief physician of the Department of Thoracic Surgery, said, “Normally, children with congenital heart disease show symptoms at a very young age, but the reason why Ningning only showed up at the age of 2 is because there is a 5mm hole in the middle of his left and right atria, a crucial “hole”. -The congenital atrial septal defect, which allows some of the blood that wrongly flows into the right atrium to flow into the normal left atrium, has kept him alive so far. However, as Ning Ning grows up, the amount of exercise increases, and the blood volume increases, the 5mm hole is no longer sufficient to meet the growth needs. If left untreated, it will lead to pulmonary hypertension and lost opportunity for surgery.  The operation for the rare and special type of complete pulmonary vein ectopic drainage in the Jiangsu Provincial People’s Hospital was successful, and Ning Ning recovered well without further medication and has returned home recently.