Bidirectional Cavopulmonary Anastomosis as a Stage in the Hemodynamic Correction of Double Outlet Right Ventricle

Double outlet right ventricle (DORV) is a complex congenital heart defect with an abnormal ventriculoarterial connection, in which the pulmonary artery and aorta arise predominantly from the right ventricle. This condition encompasses a wide range of anatomical variants and associated malformations. A subset of patients with DORV requires a univentricular approach to defect correction due to the impossibility of performing anatomical repair with a bidirectional cavopulmonary anastomosis. This cohort of patients presents specific perioperative management challenges, which are the focus of this article. Aim. To evaluate the preoperative characteristics, immediate and long-term results after performing a bidirectional cavopulmonary anastomosis as a stage of hemodynamic correction in patients with double abduction of the great vessels from the right ventricle. Materials and Methods. In the period from 1996 to 2024, 483 patients with double branching of the great vessels from the right ventricle were surgically corrected at the State Institution “NIACC named after M.M. Amosov of the National Academy of Medical Sciences of Ukraine”. Radical correction of the defect was performed in 446 (92.4 %) patients, in 5 (1 %) patients a one-and-a-half-ventricle repair, and in the remaining 32 (6.6 %) patients, hemodynamic correction was chosen due to the hemodynamic characteristics typical of the physiology of a single ventricle heart, so they underwent bidirectional cavopulmonary anastomosis as a stage of the single ventricle approach. On echocardiography, among the study group of patients (n=32) who underwent bidirectional cavopulmonary anastomosis, 24 (75 %) had transposition of the great arteries, in 4 (12.5 %) a tetralogy type, and 4 (12.5 %) patients had a type of interventricular septal defect. Results. Hospital mortality was 3.1 % (n=1). Uncomplicated course in the early postoperative period was observed in 26 (81.2 %) patients. At discharge, 12 patients with II functional class of heart failure transitioned to I functional class, among the other 20 patients with III functional class, 15 transitioned to I and 5 to II. An increase in oxygen saturation from 74 [50; 92] % to 86 [77; 92] % was also noted after the intervention. Patient survival was 96.7 % (n=30) at 6 months and 93.5 % (n=29) at 1 year and remained stable until the final stage of hemodynamic correction. The next, or final, stage of hemodynamic correction in the form of total cavopulmonary connection was performed in 22 (71 %) patients who were discharged from the hospital (n=31). An assessment of all operated patients (n=32) shows that the final stage of hemodynamic correction, or the Fontan procedure, was achieved in 68.7 % of patients. Conclusions. Double outlet right ventricle is a complex congenital heart disease that includes a wide range of anatomical variants and requires a careful approach to choosing the tactics of surgical treatment. Bidirectional cavapulmonary anastomosis as a stage of hemodynamic demonstrates favorable immediate and long-term outcomes in patients who cannot undergo radical correction.