Sildenafil in pulmonary hypertension secondary to unilateral agenesis of pulmonary artery.
Unilateral agenesis of the pulmonary artery (PA) is a rare anomaly that can lead to pulmonary hypertension (PHTN). We report the case of a 10-month-old female with agenesis of the left PA, ventricular septal defect, and patent ductus arteriosus who developed PHTN precluding surgical repair. The use of sildenafil decreased PA pressure and normalized pulmonary vascular resistance, after which surgery was successfully performed.
Hamdan
M A
MA
Section of Pediatric Cardiology, King Abdulaziz Cardiac Center, King Fahad National Guard Hospital, Riyadh, Saudi Arabia. mhamdan@tawam-hosp.gov.ae
Abu-Sulaiman
R M
RM
Najm
H K
HK
D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D007223:Infant; D010726:Phosphodiesterase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use*; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use*; D011651:Pulmonary Artery / Q000002:abnormalities*; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D014655:Vascular Resistance / Q000187:drug effects
D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate