Plućna arterijska hipertenzija (PAH) teška je kronična, progresivna bolest. Pojavljuje se u dijela bolesnika s prirođenim srčanim bolestima (PSB), a izražen je primjer Eisenmengerov sindrom, PSB s najvećim mortalitetom i morbiditetom. Napredna terapija PAH-a u prirođenim srčanim bolestima odraslih (PSBO-om) znatno je poboljšala ishod i klinički tijek u ovakvih bolesnika, a kao terapija prve linije danas su u preporukama antagonisti endotelin receptora, prije svega bosentan. U svim studijama i registrima bosentan je poboljšao toleranciju napora, reducirao plućnu vaskularnu rezistenciju i smanjio smrtnost takvih bolesnika. Ostali lijekovi napredne terapije (inhibitori fosfodiesteraze i prostaglandini) dodaju se u slučaju progresije bolesti. Napredna terapija PAH-a može se primjenjivati dugoročno s dobrim rezultatom i malo nuspojava. Osim Eisenmengerova sindroma i drugi bolesnici iz grupe PAH u PSBO mogu biti liječeni naprednom terapijom. U liječenju operiranih bolesnika s PSBO-om koji imaju ili se u njih naknadno razvije PAH indiciran je i drugi dvostruki inhibitor endotelina, macitentan. Terapija kombinacijom lijekova nije dovela do daljnjega bitnog poboljšanja u usporedbi s liječenjem monoterapijom inhibitorima endotelinskih receptora. Moguće je da će i pacijenti s Fontanovom cirkulacijom biti ciljana skupina za liječenje tim lijekovima.Pulmonary arterial hypertension (PAH) is a severe chronic, progressive disease. It affects the group of patients with congenital heart diseases (CHD); a high-profile example is Eisenmenger’s syndrome, the CHD with the greatest mortality and morbidity. Advanced PAH treatment for adult congenital heart diseases (ACHD) has significantly improved the outcome and clinical course for these patients, and endothelin receptor antagonist, primarily bosentan, are recommended as first-line treatment today. All case studies
and registries show that bosentan improved the exercise tolerance, reduced pulmonary vascular resistance, and decreased the mortality of these patients. Other advanced therapy drugs (phosphodiesterase inhibitors and prostaglandins) are added in case of disease progression. Advanced PAH therapy can be administered
long-term with good results and few side effects. Apart from Eisenmenger’s syndrome, other patients from the PAH group in ACHD can be treated through advanced therapy. In the treatment of surgical patients with ACHD who have developed or are developing a subsequent PAH, a different double endothelin inhibitor is indicated, macitentan. Therapy through a combination of drugs has not led to any further significant improvements in relation to treatment by a monotherapy of endothelin receptor inhibitors. It is possible that patients with Fontan circulation will be the target group for the treatment with these drugs
