P144 USE OF SACUBITRIL/VALSARTAN IN A GUCH PATIENT POPULATION. PRELIMINARY STUDY

Abstract

Abstract Sacubitril/Valsartan (S/V) is used with optimal results in the therapy of heart failure (HF); reduces mortality and hospitalizations by 20% compared to ACE inhibitor therapy. There is a little data on the use of S/V in adult patients with congenital or acquired pediatric heart disease (GUCH). In these patients, HF is a real challenge for the cardiologist. HF in GUCH often has a different pathophysiology than usual, and interesting univentricular hearts in Fontan circulation, systemic right ventricle and patients undergoing multiple cardiac surgical procedures. The literature is poor in information and the guidelines do not give clear indications. We report our experience on a GUCH population. Starting from November 2019 we started S/V therapy in 13 GUCH patients with severe ventricular dysfunction, all in NYHA class III and already in optimal therapy for HF. Median age was 42.7 years (27–79 years); 8 males and 5 females. Basic heart disease: 4 patients with Fontan circulation, 3 patients with systemic right ventricle (TGA, operated according to Mustard), n. 2 complete CAV operated, n. 1 Ebstein‘s disease, n.1 Patent ductus arteriosus, n. 2 pediatric onset dilated cardiomyopathy. Median duration of follow–up 11.6 months (2–26 months). In n. 1 patient it was necessary to suspend the S/V due to skin itching, which resolved on diuscontinuation. The dosage was reduced in tree patients due to symptomatic hypotension (1 of these was taking Sildenafil). In n. In 1 patient there was a reduction in the glomerular filtration rate, which did not require discontinuation of the drug. After treatment: 6 patients are in NYHA class I, n. 3 pts in NYHA II class and 4 pts remained in NYHA III class. In the year preceding the start of S/V therapy, all patients cumulatively had n. 15 hospitalizations; in the year following the first six months of treatment, the number of hospitalizations decreased to no. 5 (4 for HF and 1 for severe anemia) The ejection fraction, evaluated by Simpson‘s method, remained stable. However, the limitations of the echocardiographic method in patients with such complex anatomy must be considered Conclusions The use of S/V is safe and effective in GUCH patients. However, larger studies and longer follow–up are needed.