Related Experiment Video
Updated: Aug 7, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
[Sildenafil in the treatment of pulmonary hypertension]
E García Martínez1, I Ibarra de la Rosa, J L Pérez Navero
1Servicio de Críticos y Urgencias de Pediatría. Departamento de Pediatría. Hospital Universitario Reina Sofia. Cordoba. Spain.
Insights
Sildenafil effectively treated pulmonary hypertension in an infant with congenital heart defects, improving hemodynamics and allowing discharge. This offers a promising alternative for pediatric pulmonary hypertension cases.
Area of Science:
- Pediatric Cardiology
- Pharmacology
- Critical Care Medicine
Background:
- Pulmonary hypertension (PHT) is a severe condition with limited treatment options, particularly in pediatric cases.
- Congenital heart defects like mitral stenosis and persistent ductus arteriosus can lead to complex PHT.
- Conventional therapies often prove insufficient for severe, persistent pediatric PHT.
Observation:
- An infant with PHT secondary to congenital mitral stenosis and PDA experienced persistent symptoms despite surgical correction.
- The patient developed congestive cardiac failure and refractory PHT, unresponsive to standard treatments.
- Sildenafil, a phosphodiesterase type 5 inhibitor, was administered as a salvage therapy.
Findings:
- Sildenafil treatment led to a satisfactory clinical course, enabling extubation and discontinuation of vasoactive drugs.
- Hemodynamic improvements were observed, including decreased pulmonary and left atrial pressures.
- The infant was discharged on oral sildenafil and maintained a stable condition during outpatient follow-up.
Implications:
- Sildenafil represents a viable therapeutic option for refractory pediatric pulmonary hypertension, especially when associated with congenital heart disease.
- This case highlights the potential of targeted pharmacotherapy in managing complex pediatric cardiovascular conditions.
- Further research into PDE5 inhibitors for pediatric PHT is warranted to establish optimal protocols and long-term efficacy.
Abstract:
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.
Related Concept Videos
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Angina IV: Management

