Diagnosis and treatment of pulmonary hypertension in infancy

Robin H Steinhorn1

  • 1Department of Pediatrics, University of California Davis Children's Hospital, Sacramento, CA, United States.

Early Human Development
|October 3, 2013
PubMed

Insights

Infant pulmonary hypertension arises from impaired lung development, affecting growth and function. Effective treatments target pulmonary vascular resistance (PVR) by understanding altered signaling pathways in remodeled lung vasculature.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Physiology
  • Vascular Biology

Background:

  • Normal pulmonary vascular development in infancy is crucial for lung function and growth.
  • Adverse influences on the developing lung can lead to pulmonary hypertension in infants.
  • Pulmonary hypertension in neonates is associated with conditions like PPHN, CDH, and BPD.

Purpose of the Study:

  • To review the factors affecting normal pulmonary vascular development in infants.
  • To discuss the classification of pulmonary hypertension in neonates.
  • To highlight current pharmacotherapeutic strategies for infant pulmonary hypertension.

Main Methods:

  • Literature review of pulmonary vascular development and infant pulmonary hypertension.
  • Analysis of current classifications for pediatric pulmonary hypertension.
  • Examination of pharmacotherapeutic targets for pulmonary hypertension in infants.

Main Results:

  • Pulmonary hypertension in infants results from disrupted lung adaptation and development.
  • New classifications are emerging to categorize diverse pulmonary hypertension phenotypes in neonates.
  • Targeting pulmonary vascular resistance (PVR) is key for treating PPHN.

Conclusions:

  • Understanding altered signaling pathways in remodeled vasculature is essential for effective treatment.
  • Pharmacotherapeutic strategies for infant PPHN focus on selective PVR reduction.
  • Continued research into the mechanisms of infant pulmonary hypertension is vital.

Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
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...
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue, improving...
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care

Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
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: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as 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...