The purpose of the study was to analyze the results of surgical treatment of total abnormal pulmonary vein drainage at the National Academy of Cardiovascular Surgery of the National Academy of Medical Sciences for the period from 2007 to 2019. Material and methods. During this period 34 patients were operated at the National Amosov Institute of Cardiovascular Surgery, 15 female (44.1%) and 19 male (55.8%). One man was operated twice. The criterion for selection for critical defect was the total abnormal drainage of the pulmonary veins, requiring emergency surgical treatment. On the whole, 36 operations were performed. On the day of surgery, the average age of the patients was 109.3 ± 101.4 days (from 2 to 505 days), and their average weight was 4.01 ± 1.1 kg. 17 patients had the defect diagnosed prenatally and confirmed by Echo-KG immediately after birth. In 10 cases it was diagnosed at birth, which allowed the immediate delivery to the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences for the provision of highly qualified medical care. It should be noted that 31 patients had an accurate anatomical diagnosis based on Echocardiography alone. This helped avoid angiocardiography, which had a significant negative impact on the condition of patients in the preoperative period. It was found that in 14.2% (5) patients there was a supracardial form of anomaly, in 57.1% (20) it was an intracardiac form, in 17.1% (6) patients it was infracardial and 11.4% (4) had a mixed form. Echocardiography revealed that 100% of pulmonary hypertension was observed in 57.1% (20) of cases. Results and discussion. . In all cases, surgery was performed to radically correct the total abnormal drainage of the pulmonary veins. In addition, in 4 cases, such a surgery was supplemented by the Rashkind procedure. After surgery, 32 out of 34 patients (94.1%) were discharged to inpatient hospitals for continuing treatment. Thus, the mortality rate was 5.9%. In one case of repeated operative intervention after 6 months, a total anomalous drainage of the pulmonary veins was performed, and after 2 months – RKTAPVD. After repeated surgical interventions, the patient experienced improvement, and he was discharged from the clinic in satisfactory condition. During surgery, the following complications were observed: hemolysis – 1 case, pulmonary edema – 1 case, pneumothorax – 1 case, perforation of the subclavian artery – 1 case, paresis of the left dome of the diaphragm – 1 case, acute heart failure – 4 cases which transferred into the postoperative complications. They were: acute ventricular failure, acute heart failure – 3 cases, respiratory failure – 1 case, small ejection syndrome with renal failure – 1 case, pneumothorax – 1 case, hemolysis – 1 case, atelectasis left th lung – supraventricular tachycardia – 2 cases, atrial flutter – 1 case. Later they became the cause of fatal cases – in the postoperative period 2 (5.9%) patients died. The causes of death among patients were: progressive heart failure (n=1), which was a consequence of small size of the left ventricle; pulmonary hypertensive crises (n=1), due to increased reactivity of the pulmonary vascular bed in the early postoperative period. There was also a respiratory failure – 1 case; perforation of the left atrium during the Rashkind procedure – 1 case; surgical bleeding – 1; supraventricular tachycardia with development of arrhythmogenic shock – 1, acute heart failure – 3 cases (among them there was 1 left ventricular failure due to myocardial infarction, 1 right ventricular failure due to thrombosis of the pulmonary artery, 1 lower thoracic artery). Thus, the results coincided with similar ones in other studies. Conclusion. Despite the high lethality, such surgical interventions should be performed, as this is the only chance to save these patients and prolong their life, and to prepare them for the next surgery. It should also be noted that the fatality rate is constantly decreasing.
Full text: PDF (Ukr) 210K