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Updated: Aug 24, 2026
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Pulmonary arterial hypertension in children
Erika Berman Rosenzweig1, Allison C Widlitz, Robyn J Barst
1Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York 10027, USA. esb14@columbia.edu
Insights
Pulmonary arterial hypertension (PAH) is a serious condition often diagnosed late. Advances in understanding, diagnosis, and treatment have significantly improved outcomes for children with PAH.
Area of Science:
- Cardiology
- Pediatric Pulmonology
- Medical Research
Background:
- Pulmonary arterial hypertension (PAH) is a progressive condition with poor prognosis if not treated early.
- Nonspecific symptoms and subtle physical findings often lead to late diagnosis of PAH.
- Significant advancements have been made in understanding PAH pathology and pathobiologic mechanisms.
Purpose of the Study:
- To review the current state of the art in pulmonary arterial hypertension (PAH) in 2004.
- To emphasize childhood pulmonary arterial hypertension.
- To provide specific recommendations for current practice and future directions in PAH management.
Main Methods:
- Review of current literature and clinical practice regarding pulmonary arterial hypertension.
- Characterization of risk factors and genetics associated with PAH.
- Assessment of technological advancements in PAH diagnosis and severity evaluation.
Main Results:
- Improved understanding of PAH pathology and pathobiology.
- Identification of risk factors and genetic factors in PAH.
- Development of new therapeutic modalities, including drugs (epoprostenol, treprostinil, bosentan) and surgical options (transplantation, atrial septostomy).
Conclusions:
- Despite the lack of a cure, advances in medical treatments over two decades have significantly improved outcomes for children with PAH.
- Early diagnosis and treatment are crucial for better prognosis in pulmonary arterial hypertension.
- Ongoing research and development are essential for further improving the management and outcomes of PAH.
Abstract:
Pulmonary arterial hypertension is a serious progressive condition with a poor prognosis if not identified and treated early. Because the symptoms are nonspecific and the physical findings can be subtle, the disease is often diagnosed in its later stages. Remarkable progress has been made in the field of pulmonary arterial hypertension over the past several decades. The pathology is now better defined, and significant advances have occurred in understanding the pathobiologic mechanisms. Risk factors have been identified, and the genetics have been characterized. Advances in technology allow earlier diagnosis as well as better assessment of disease severity. Therapeutic modalities such as new drugs, e.g., epoprostenol, treprostinil, and bosentan, and surgical/interventional options, e.g., transplantation and atrial septostomy, which were unavailable several decades ago, have had a significant impact on prognosis and outcome. Thus, despite our inability to cure pulmonary arterial hypertension, advances in medical treatments over the past two decades have resulted in significant improvement in outcomes for children with various forms of pulmonary arterial hypertension. This report is a review the current state of the art for pulmonary arterial hypertension in 2004, with an emphasis on childhood pulmonary arterial hypertension and specific recommendations for current practice and future directions.
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