Background
Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vascular bed and causes right heart failure and death. Combination therapy which targeted at 3 different pathways is necessary due to progressive nature of the disease. In PAH patients there are two approaches in combination therapy; ‘first line, up-front’ and ‘sequential add-on’ treatment. In first line, up-front treatment, from the beginning the patients are receiving double or triple drug therapy. In ‘sequential add-on’ approach initially a single drug is started and then according to the patient’s requirements, a second or third drug is added. There is no sufficient evidence about the efficiency and safety of treatment approaches. In our study, we aimed to evaluate our treatment approach in patients with PAH patients as a tertiary center.
Methods
PAH was diagnosed according to clinical, echocardiographic and right heart catheterization findings. The patients were recieved Bosentan, Sildenafil, Iloprost treatment in accordance with guidelines recommendations. Clinical worsening in patients was defined as death, requirement of hospitalization for PAH, a 15% decline in the 6 minute walking test (6 MWT) distance, deterioration in functional capacity and symptoms and findings of right heart failure.
Methods
PAH was diagnosed according to clinical, echocardiographic and right heart catheterization findings. The patients were recieved Bosentan, Sildenafil, Iloprost treatment in accordance with guidelines recommendations. Clinical worsening in patients was defined as death, requirement of hospitalization for PAH, a 15% decline in the 6 minute walking test (6 MWT) distance, deterioration in functional capacity and symptoms and findings of right heart failure.
Results
At the end of the follow-up period clinical, echocardiographic findings, BNP levels and oxygen saturation were similar between patients who completed the study with monotherapy and with combination therapy. Only follow-up period was significantly longer in patients who required combination treatment. Mortality was observed in 2 patients (6,9%). 4 patients (13,8%) due to recurrent symptoms and findings of right heart failure were hospitalized. At the end of follow-up, 10 patients (34,5%) completed the study with a single drug, 15 patients (51,7% ) with two drugs and 4 patients (13,8%) with three drugs.
Conclusion
In our study, combination therapy was given to patients as ‘sequential add-on therapy’ approach. At the end of the follow-up period monotherapy was sufficient in 34,5 % patients of the study group and in 8 patients sildenafil or prostaglandin analogues are added and total 15 patients (48,4 %) completed the study under dual therapy. 4 patients (12.9%) received combination therapy with triple drugs.
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