Efficacy of sildenafil as a rescue therapy for patients with severe pulmonary arterial hypertension and given long-term treatment with prostanoids: 2-year experience.
BACKGROUND
Both prostanoids and sildenafil are effective treatments for severe pulmonary arterial hypertension (PAH). The combined use of these drugs to maximize the clinical benefit is an emerging treatment option. This study describes a 2-year experience with adjunct sildenafil as a rescue therapy for patients with severe PAH treated long term with prostanoids and who showed clinical deterioration or onset of heart failure.


METHODS
Twenty patients (11 men, 9 women; mean age 42 +/- 11 years) with severe PAH, who showed clinical or functional worsening despite ongoing treatment with prostanoids (8 subcutaneous, 7 intravenous, 5 inhaled), were started on adjunct oral sildenafil. New York Heart Association (NYHA) functional class, 6-minute walking test, signs of right ventricular failure and echocardiography were assessed before and after 1 and 2 years of combined therapy.


RESULTS
There was a significant improvement of NYHA functional class and signs of right heart failure after 1- and 2-year follow-up. Patients showed a mean increase in 6-minute walking distance of 79 m and 105 m after 1 and 2 years of adjunct sildenafil, respectively. Two patients died during follow-up. The echocardiographic parameters showed a significant reduction of right ventricular end-diastolic diameter and left ventricular diastolic eccentricity index. No serious side effects related to sildenafil were observed.


CONCLUSIONS
Adjunct sildenafil to long-term prostacyclin therapy in patients with severe PAH provided sustained clinical stabilization and an improved clinical situation, exercise capacity and echocardiographic parameters of right ventricular function. The beneficial effects were strong and lasted >24 months.
Ruiz
Maria Jose
MJ
Department of Cardiology, Hospital 12 de Octubre, Madrid, Spain. majorcardio@yahoo.es <majorcardio@yahoo.es>
Escribano
Pilar
P
Delgado
Juan F
JF
Jiménez
Carmen
C
Tello
Rocío
R
Gómez
M Angel
MA
de la Calzada
C Sáenz
CS
D000328:Adult; D001794:Blood Pressure / Q000187:drug effects / Q000502:physiology; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D004452:Echocardiography; D017079:Exercise Tolerance / Q000187:drug effects / Q000502:physiology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000473:pathology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009518:New York; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use*; D011453:Prostaglandins / Q000494:pharmacology / Q000627:therapeutic use*; D011687:Purines; D000068677:Sildenafil Citrate; D013318:Stroke Volume / Q000187:drug effects / Q000502:physiology; D013450:Sulfones; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use*; D018497:Ventricular Dysfunction, Right / Q000473:pathology / Q000503:physiopathology
D010879:Piperazines; D011453:Prostaglandins; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate