Embracing the challenges of neonatal and paediatric pulmonary hypertension

Dunbar Ivy1, Erika B Rosenzweig2, Steven H Abman3

  • 1Pediatric Cardiology, University of Colorado School of Medicine, and Children's Hospital Colorado, Aurora, CO, USA.

PubMed

Insights

Paediatric pulmonary arterial hypertension (PAH) requires unique approaches due to associated disorders. This review updates diagnosis, risk stratification, and treatment for paediatric PH, highlighting knowledge gaps and challenges in clinical trials.

Area of Science:

  • Pediatric Cardiology
  • Pulmonology
  • Critical Care Medicine

Background:

  • Paediatric pulmonary arterial hypertension (PAH) shares traits with adult PAH but presents unique challenges.
  • Associated disorders and specific patient populations necessitate distinct management strategies.

Purpose of the Study:

  • To review recent advances, ongoing challenges, and distinct approaches in paediatric pulmonary hypertension (PH).
  • To provide updates on diagnosis, classification, risk stratification, and treatment for paediatric PH.
  • To identify critical knowledge gaps and propose solutions for paediatric PH management.

Main Methods:

  • Consensus review by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension.
  • Inclusion of updated risk stratification tools and treatment algorithms.
  • Discussion of novel strategies for PAH associated with congenital heart disease and neonatal PH.

Main Results:

  • An updated risk stratification tool and treatment algorithm for paediatric PH, including strategies for associated cardiopulmonary conditions.
  • Highlighting advances in diagnosing neonatal PH using echocardiography and emphasizing a team approach.
  • Discussion of challenges in paediatric drug approval and clinical trial design.

Conclusions:

  • Paediatric PH management requires tailored approaches, addressing specific patient populations and associated conditions.
  • Despite advances, treatment remains hindered by a lack of robust clinical trials and challenges in drug development.
  • A multimodal and team-based approach is crucial for optimizing outcomes in infants and children with PH.

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...
158
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,...
187
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...
142
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...
160
Pulmonary Cycle: Exhalation01:17

Pulmonary Cycle: Exhalation

In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...
1.5K
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...
147