Stručni rad

https://doi.org/10.15836/ccar2016.43

Advanced Therapy in the Treatment of Pulmonary Arterial Hypertension in Adult Congenital Heart Disease

Maja Strozzi ; Medicinski fakultet Sveučilišta u Zagrebu, Klinički bolnički centar Zagreb, Zagreb, Hrvatska

Sažetak

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.

Ključne riječi

pulmonary arterial hypertension; adult congenital heart defects; Eisenmenger’s syndrome; advanced pulmonary hypertension treatment; endothelin receptor antagonist

Hrčak ID:

153654

URI

https://hrcak.srce.hr/153654

Datum izdavanja:

23.2.2016.

Podaci na drugim jezicima: hrvatski

Posjeta: 2.608 *