Modern approaches to medical treatment of heart failure in patients with Fontan circulation
ORIGINAL PAPERS
Abstract
Introduction.Fontan surgery is a key method of palliative care for patients with a functionally single ventricle heart. Despite the high 10-year survival rate (up to 95%), 30% of patients develop heart failure, which remains the leading cause of death. Fontan’s circulation features require an individual approach to medical therapy for heart failure, but evidence-based recommendations are limited due to anatomical heterogeneity and small cohort size.The aim of the studyis to systematize modern approaches to heart failure therapy in Fontan circulation, to present our own experience in managing this group of patients.Materials and methods.The study is retrospective. The study included 25 patients with Fontan circulation, 16 (64%) boys and 9 (36%) girls. The average age at the time of the first comprehensive examination in our clinic was 11.2±4.4 years. Systemic right single ventricle was observed in 9 (36%) patients, systemic left single ventricle in 12 (48%) patients, and undifferentiated in 4 (16%) patients. In our group of patients, there were no cases of stenosis of cavopulmonary anastomosis, as well as deformities or narrowing of the pulmonary arteries and veins. All patients underwent an assessment of their medical history, laboratory parameters, and the results of instrumental examination methods.Results.22 patients (88%) received medication therapy for chronic heart failure:angiotensin-converting enzyme inhibitors— 13 children (52%), aldosterone antagonists — 11 patients (44%), diuretic therapy — 7 patients (28%), drugsfrom the angiotensin II receptor antagonist group — 1 patient (4%), beta adrenoblockers — 13 (52%), 2 people (8%) received sodium — dependent glucose transporter type 2 inhibitors, and 2 people (8%) received combined angiotensin receptor blockers with non-lysine inhibitors. 11 people (44%) receivedspecific therapy for pulmonary arterial hypertension, of which 3 received combined PAH-specific therapy. The follow-up period was 2 years. Stabilization of the condition on the background of multicomponent medical therapy was achieved in 64% of patients (n=16), which was confirmed by clinical and instrumental data. Positive dynamics were noted in 24% of patients (n=6). The mortality rate was 4% (1 case) in a patient with decompensated pulmonary hypertension and multiple organ failure. Following the addition of Hemodynamicsdition of Dapagliflozin or Valsartan + Sacubitril to the basic therapy ofchronic heart failurein patients with Fontan circulation, no side effects were noted. Moreover, according to clinical data, clinical stabilization was achieved, with patients reporting good drug tolerance.This work is devoted exclusively to the analysis of medical therapy of chronic heart failure in patients with Fontan circulation, without affecting the aspects of treatment of concomitant pathologies and complications in this category of patients. We have summarized the current data on the strategy of heart failure in patients after Fontan surgery. Particular attention is paid to the use of drugs from the group of sodium-glucose cotransporter type 2 inhibitors and combined angiotensin receptor blockers with non-lysine inhibitors. An individual approach to therapy, the use of modern pharmacological drugs and regular monitoring of patients’ condition form the basis for successful treatment of this complex clinical group.
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