AHS/ATA Develop New Pediatric Pulmonary Hypertension Guidelines

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by Charles Moore |

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The American Heart Association (AHA) and American Thoracic Society (ATS) have issued new guidelines pertaining to Pediatric Pulmonary Hypertension. The revisions come as a result of collaboration between the agencies and were developed in a joint effort by an interdisciplinary team of 27 experienced clinicians and clinician-scientists in a review of the current literature in which they analyzed data from more than 600 studies and made recommendations on diagnosis, evaluation, and treatment of pediatric pulmonary hypertension.

The research team, chaired by Steven H. Abman, MD of Children’s Hospital, Colorado, with co-chairs Georg Hansmann, MD, PhD, FAHA of the Department of Pediatric Cardiology and Critical Care at Hannover Medical School, Germany, and Stephen L. Archer, MD, FAHA, Head of Medicine at Queen’s University, Kingston Ontario, Canada notes that pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, they observe that current approaches to caring for pediatric pulmonary hypertension patients have been limited by lack of consensus guidelines from experts in the field. Accordingly, the new guidelines incorporate the results of the research team’s extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.ons for the care of children with pulmonary hypertension.

The Guidelines From the American Heart Association and American Thoracic Society on Pediatric Pulmonary Hypertension (doi: 10.1161/CIR.0000000000000329) were published online before print in the journal Circulation on November 3.

In a 2013 paper published in the Journal of the American College of Cardiology entitled Updates in Pulmonary Hypertension (Journal of the American College of Cardiology 24 December 2013, Vol.62(25):D117D126, doi:10.1016/j.jacc.2013.10.028), an international team of 12 investigators from institutions in the U.S., the Netherlands, France, the U.K., and Switzerland, observe that pulmonary hypertension (PH), while rare among newborns, infants, and children, is associated with significant morbidity and mortality. They note that in the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease, and that management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.

The investigators say that while pulmonary hypertension can present at any age from infancy to adulthood, distribution of etiologies in children is quite different from that of adults, with a predominance of idiopathic pulmonary arterial hypertension (IPAH) and PAH being associated with congenital heart disease (APAH-CHD), and that in pediatric populations, IPAH is usually diagnosed in its later stages due to nonspecific symptoms.

They note that without appropriate treatments, median survival rate after diagnosis of children with IPAH appears worse when compared with that of adults, and that therapeutic strategies for adult PAH have not been sufficiently studied in children, especially regarding potential toxicities, formulation, or optimal dosing, and appropriate treatment targets for goal-oriented therapy in children are lacking. Nevertheless, children with PAH are currently treated with targeted PAH drugs and may benefit from these new therapies.

The AHA/ATS statement also notes that PH and related PVD cause significant morbidity and mortality in diverse childhood diseases, and that despite the availability of new drug therapies, long-term outcomes for children with severe PAH remain poor. They observe that as in adult PAH, IPAH in pediatric patients can be devastating and often contributes to poor outcomes. Unfortunately, whereas the adult PAH literature is a robust with several treatment guidelines, few studies specifically address the safety and efficacy of therapies in children, and there are no treatment guidelines. Indeed, they observe that most studies of potential PAH therapeutics have focused on adults and, because of the nature of adult PAH, have generally been conducted in patients with a limited range of associated conditions. Thus, pediatric PH has been understudied, and little is understood about the natural history, fundamental mechanisms, and treatment of childhood PH.

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The AHA/ATS Pediatric Pulmonary Hypertension guidelines statement concludes that despite many advances in understanding of the pathobiology and treatment of PHVD, care guidelines have not previously been developed for specific use in children, and that the purpose of their document is to provide a general overview of PVD in neonates, infants, and children and recommendations for the assessment and treatment of children with PH. They caution that many gaps still remain in knowledge of the developing lung circulation and its response to injury — unique features of PH in children — and of optimal clinical evaluations and treatment strategies. They affirm that the document is intended to provide a foundation for future work directed toward additional discoveries of basic and clinical aspects of disease that will ultimately improve the quality of life and long-term outcomes of children with PVD in diverse settings.

Timothy B. Cotts, M.D., F.A.C.C. of the University of Michigan Cardiovascular Center in Ann Arbor has highlighted 10 key points to remember from the new AHS/ATA guidelines on pediatric pulmonary hypertension in an artile published in The Journal of the American College of Cardiology.

The AHA/ATS statement document and summary of the new Pediatric Pulmonary Hypertension guidelines can be accessed and downloaded (PDF) at: