sildenafil-citrate and Ductus-Arteriosus--Patent

The difference in underlying pathophysiology in different congenital heart disease (CHD) may have an influence on clinical outcome. It remains unclear whether the effect of sildenafil on pulmonary arterial hypertension (PAH) varies in different types of CHD.. The potential effect of sildenafil on pulmonary arterial hypertension related to CHD may be associated with shunt location.. In this 12-week, prospective, open label, multicenter trial, 55 patients with CHD were divided into the 3 groups: atrial septal defects group (ASD, n = 15), ventricular septal defects group (VSD, n = 24), and patent ductus arteriosus group (PDA, n = 16). Exercise capacity, hemodynamic parameters, and arterial oxygen saturation were assessed at baseline and after sildenafil therapy (25 mg, 3 times daily).. Six-minute walk distance significantly increased from 377.2 ± 68.7 m to 436.0 ± 70.4 m in patients with ASD, from 371.2 ± 66.0 m to 413.7 ± 83.1 m in VSD, and from 384.3 ± 90.2 m to 440.9 ± 71.8 m in PDA (P<0.01, respectively). Moreover, sildenafil also improved the pulmonary vascular resistance and pulmonary blood flow index in the 3 groups, whereas no significant changes in systemic vascular resistance and systemic arterial pressure were observed. However, arterial oxygen saturation was significantly improved in the ASD group only. The incidence of adverse events was similar among the 3 groups.. Sildenafil therapy seems to be effective and safe for PAH secondary to ASD, VSD, and PDA, although some clinical and hemodynamic parameters were changed in a different manner among the 3 groups.

Topics: Adolescent; Adult; Antihypertensive Agents; Chi-Square Distribution; China; Ductus Arteriosus, Patent; Exercise Tolerance; Familial Primary Pulmonary Hypertension; Female; Heart Defects, Congenital; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Hemodynamics; Humans; Hypertension, Pulmonary; Male; Oxygen; Piperazines; Prospective Studies; Purines; Sildenafil Citrate; Sulfones; Time Factors; Treatment Outcome; Vasodilator Agents; Young Adult

The use of pulmonary vasodilator therapy in children born preterm is largely unknown. Our aim was to map prescription patterns in children with bronchopulmonary dysplasia in Sweden.. This was a descriptive national registry-based study of children <7 years who had been prescribed a pulmonary vasodilator during 2007-2017, were born preterm and classified as having bronchopulmonary dysplasia. Information on prescriptions, patient characteristics and comorbidities were retrieved from the Swedish Prescribed Drug Register and linked to other national registers.. The study included 74 children, 54 (73%) born at 22-27 weeks' gestation and 20 (27%) at 28-36 weeks. Single therapy was most common, n = 64 (86.5%), and sildenafil was prescribed most frequently, n = 69 (93%). Bosentan, iloprost, macitentan and/or treprostinil were used mainly for combination therapies, n = 10 (13.5%). Patent ductus arteriosus or atrial septal defect were present in 29 (39%) and 25 (34%) children, respectively, and 20 (69%) versus 3 (12%) underwent closure. Cardiac catheterisation was performed in 19 (26%) patients. Median duration of therapy was 4.6 (1.9-6.8, 95% CI) months. Mortality was 9%.. Preterm children with bronchopulmonary dysplasia were prescribed pulmonary vasodilators, often without prior catheterisation. Sildenafil was most commonly used. Diagnostic tools, effects, and drug safety need further evaluation.

Topics: Bronchopulmonary Dysplasia; Child; Ductus Arteriosus, Patent; Humans; Infant, Newborn; Infant, Premature; Outpatients; Sildenafil Citrate; Vasodilator Agents

Topics: Cyanosis; Ductus Arteriosus, Patent; Female; Humans; Hypertension, Pulmonary; Osteoarthropathy, Secondary Hypertrophic; Sildenafil Citrate; Vasodilator Agents; Young Adult

Topics: Blood Transfusion; Bosentan; Diagnostic Imaging; Dobutamine; Ductus Arteriosus, Patent; Eisenmenger Complex; Epoprostenol; Female; Humans; Hypertension, Pulmonary; Magnetic Resonance Imaging; Menorrhagia; Piperazines; Purines; Sildenafil Citrate; Sulfonamides; Tomography, X-Ray Computed; Vasodilator Agents; Young Adult

Pulmonary hypertension (PHT) is a rare entity that is difficult to treat. Prognosis is poor. Sildenafil, a selective inhibitor of type 5 phosphodiesterase, has been proposed among the many treatments available for primary and secondary pulmonary hypertension. We report our experience with an infant with pulmonary hypertension due to congenital mitral stenosis and persistent ductus arteriosus, who developed congestive cardiac failure with persistent PHT despite surgical correction. Conventional treatment was unsuccessful and the patient was treated with sildenafil. The clinical course was satisfactory, allowing extubation and withdrawal of vasoactive drugs; pulmonary and left atrial pressure decreased and the patient was discharged. She is currently being treated on an outpatient basis with oral sildenafil and shows satisfactory hemodynamic status. We review alternatives to conventional treatments for pulmonary hypertension with special reference to pediatrics.

Topics: Ductus Arteriosus, Patent; Humans; Hypertension, Pulmonary; Infant; Mitral Valve Stenosis; Phosphodiesterase Inhibitors; Piperazines; Purines; Sildenafil Citrate; Sulfones; Vasodilator Agents