Univentricular atrioventricular connection. Review of the current therapeutical possibilities

Abstract

Unlike other congenital heart anomalies, univentricular atrioventricular connection is impossible to surgically repair most of the time.Only certain types of univentricular atrioventricular connection can undergo surgical repair. These are cases where a 50% overriding of the right atrioventricular valve in the interventricular septum is present, which, from the anatomical point of view, characterizes the double inlet of a left single ventricle. From the physiological point of view, conditions exist for good development of the right ventricle through adequate flow from the right atrium. This development may favor the possibility of separation of the two circulations through interventricular septation.This maneuver may be effective, even if the immediate postoperative evolution is somewhat unfavorable because the extension of the biological material used in the septation may reach up to 50% of the entire interventricular septum. Placement of such a large amount of material in the septum alters ventricular function and predisposes to interventricular residual defect.Nevertheless, hemodynamic characteristics may be controlled by clinical and surgical measures providing a satisfactory long-term evolution.On the other hand, most of the patients with univentricular atrioventricular connection undergo palliative surgeries depending on whether they have increased, normal, or decreased pulmonary blood flow.In the first situation, increased pulmonary blood flow, pulmonary artery banding or the Damus-Kaye-Stansel operation is suggested. In decreased pulmonary blood flow, the 3 following surgeries are recommended: the Blalock-Taussig operation, bidirectional cavopulmonary anastomosis, and total cavopulmonary anastomosis. In normal pulmonary flow, either due to mild pulmonary stenosis or pulmonary artery hypertension with a balance in systemic and pulmonary flows, the watchful waiting approach is advised.The main indication for surgery, either pulmonary artery banding or the Damus-Kaye-Stansel operation, is the presence of congestive heart failure without hypoxemia with an oxygen saturation of approximately 90% and consequent mild cyanosis, and hematometry within the normal range. The outcome of pulmonary artery banding is better in the absence of an abnormality in the left atrioventricular valve, either stenosis or even regurgitation, and also when the blood flow mixture is adequate at the atrial level due to the presence of an atrial septal defect.Therefore, establishing communication between the two atria prior to surgery or even expanding an already existing atrial septal defect through balloon atrioseptostomy is recommended.So reduction in the pulmonary venocapillary pressure prior to pulmonary artery banding makes this technique more effective in actually reducing pulmonary blood flow. Consequently, a greater restriction of the pulmonary flow is obtained, favoring a better immediate and late evolution. The lower pulmonary pressure protects pulmonary circulation as a whole, enabling the performance of the cavopulmonary t...

Atividade autonômica em uma adolescente com ventrículo único submetida à intervenção fisioterapêutica: relato de caso

“…Another factor to be taken into account was the possible progression of the disease, since the patient under study was already reaching the mean life expectancy age. 2 . In this aspect, according to Massin et al 10 , the greater the congenital cardiopathy, the higher is the degree of HRC harm.…”

“…Th e SV is usually associated with the transposition of the great vessels and to pulmonary stenosis, and the most eff ective treatment is surgery, carried out no later than in the fi rst year of life. In case this correction does not occur, the average life expectancy has been of around 14 years 2 .…”