In order to mitigate the lack of consensual diagnosis and treatment approaches regarding pediatric pulmonary hypertension, clinicians and scientists from the American Heart Association and American Thoracic Society have created a set of clinical care guidelines and recommendations, based on extensive literature review. The full report, entitled “Pediatric Pulmonary Hypertension – Guidelines from the American Heart Association and American Thoracic Society” was recently published in Circulation.

The new guidelines focus on pulmonary hypertension (PH) in children resulting from pulmonary vascular disease (PVD), disease related to cardiac, lung and systemic disorders, and idiopathic pulmonary artery hypertension (IPAH), where disease causes remain unknown. PVD and IPAH cause significant morbidity and mortality in children with diverse diseases, and despite advancements in medicine research, the long-term outcomes remain poor. Although current classification links the conditions together, adult and pediatric PH present important differences. The main distinction between adult and children PH is the fact that pediatric PH is related to deficient lung development and growth, and is often related to the poor adaptation of pulmonary circulation from fetal to postnatal environment which disrupts the proper vascular and lung development and maturation. Because more conditions are associated with PH in children than adults, the classification system and treatments used for adults has raised doubts in clinical children management. Several factors have contributed to the gaps in knowledge of appropriate classification and therapeutic strategies and limit the ability to perform relevant clinical trials, such as lack of suitable biomarkers for risk prediction, disease severity and progression.

Despite the lack of clinical trials and research in children, the committee of excerpts generated a set of practical guidelines that aim to assist healthcare practitioners in diagnosis and clinical treatment decision making. The quality of the recommendations is defined through a scoring system based on a risk-benefit approach, where the team of excerpts provides information on the strength and certainty of the evidence that led to a specific treatment, diagnosis or monitoring recommendation.

The document aims to be a foundation for the much needed future research work and to present specific recommendations regarding specific populations and clinical scenarios. However, the committee considers that the clinician must attend to the circumstances of each specific situation, stating “there will likely be clinical settings in which decisions that differ from these guidelines might be appropriate. Decisions should also involve consideration of the expertise at the specific center where care is provided. When these guidelines are used as the basis for regulatory or payer decisions, the goal should be improvement in quality of care.”