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Systematic Review of the Application of Pulmonary Hypertension Treatments in Ventricular Septal Defect, Pulmonary Atresia, and Major Aortopulmonary Collateral Arteries - PubMed

8 days ago
  • #pulmonary hypertension
  • #congenital heart disease
  • #pulmonary atresia
  • Systematic review on pulmonary hypertension (PH) treatments in patients with pulmonary atresia (PA), ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs).
  • PH medications like sildenafil and bosentan showed clinical improvements in pulmonary vascular resistance, oxygen saturation, and symptoms in some patients.
  • Adverse effects were noted in five patients, leading to treatment discontinuation in two cases.
  • Limited sample size (n=22) and heterogeneity in patient anatomy, age, and treatment regimens restrict interpretability and clinical applicability.
  • PH medications should be used cautiously and restricted to specialized centers with expertise in congenital heart disease (CHD) and PH management.