Initial experiences with the treatment of pulmonary arterial hypertension in congenital heart disease in Slovenia

Background: Pulmonary arterial hypertension with Eisenmenger syndrome as its most advanced form is an important complication of congenital heart disease. In the recent years, advanced therapy for pulmonary arterial hypertension has been introduced. Efficacy and safety of the advanced therapy in our patients with pulmonary arterial hypertension associated with congenital heart disease were analyzed. Methods: We have analyzed the results of advanced therapy for pulmonary arterial hypertension in patients treated between November 2007 and December 2011. Clinical status, systemic oxygen saturation measured by systemic pulse oximetry, six-minute walking distance and laboratory parameters were assessed. Results at 3, 6, 12 and 24 months of treatment were compared to baseline parameters. Results: In the observed period, 23 patients were treated with advanced therapy for pulmonary arterial hypertension. As a first-line drug bosentan was used in 19 and sildenafil in 4 patients. Due to clinical worsening, a second- and a third-line drug had to be added during the study period in 4 and 1 patient, respectively. Eighteen patients (78.3 %) reported improvement in functional capacity. Two patients (8.6 %) died. The mean six-minute walking distance significantly increased over time from 334.7 +/- 87.7 m at base-line to 348.5 +/- 89.1 m at 3 months (p=0.002), 373.2 +/- 74.4 m at 6 months (p=0.005), 383.2 +/- 62.3 m at 12 months (p=0.017) and 396.3 +/- 92.8 m at 24 months of treatment. No significant adverse events were reported. Conclusions: Advanced therapy for pulmonary arterial hypertension is beneficial in patients with congenital heart disease. Significant improvement in exercise capacity is observed. The therapy is safe and no significant adverse events were reported.