Multistage treatment of newborns and infants with hypoplastic left heart syndrome: a new approach to the condition assessment throughout the hospital and interstage periods (a case series)

Introduction: Hypoplastic left heart syndrome is a critical congenital heart disease that takes a leading place in the structure of mortality index, caused by congenital heart disorder, despite its low degree of incidence (up to 0.2 per 1,000 births). Objective: The study aimed to present a new approach to the arranging of highly specialized medical care for newborns and infants with hypoplastic left heart syndrome, which includes a complex of surgical, medicamentous and outpatient preventive care for children of the first year of life, both at inpatient and interstage treatment periods. Methods: The methodology consists of 5 stages. Stage 1 is aimed at bilateral pulmonary artery banding; Stage 2 is associated with prolonged infusion of prostaglandin E1, and symptomatic therapy including, if necessary, the Rashkind procedure, for at least 18  days; Stage 3 is Norwood operation; Stage 4 is dynamic inpatient and outpatient observation and treatment; Stage 5 is operation of bidirectional cavopulmonary anastomosis. Results: 8 patients were treated using the proposed methodology. All of them underwent bilateral pulmonary artery banding followed by Norwood’s operation, with no deaths. During dynamic observation, three patients required stenting of the aorta’s isthmus after the Norwood operation. One patient died later at his place of residence. Six patients underwent bidirectional cavopulmonary anastomosis, with no deaths. Conclusion: The proposed approach of multi-stage treatment of patients with hypoplastic left heart syndrome allows improving immediate results after the Norwood operation and reducing inter-stage mortality through early detecting and eliminating of possible complications.