In-person or hybrid surveillance, which includes medical history, physical examination, and diagnostic testing, is still essential for the best management of children with pulmonary arterial hypertension (PAH), according to a new study published in Pulmonary Circulation

“These results support current recommendations for evaluation and testing in children with PAH,” the authors of the study wrote. 

During the coronavirus disease 2019 (COVID-19) pandemic, there have been changes in care models involving virtual and hybrid systems. To better characterize which data critically altered treatment recommendations in children with PAH, a team of researchers led by Mary P. Mullen, PhD, from Boston Children’s Hospital in Massachusetts conducted a retrospective review. The ultimate aim was to identify the best visit and diagnostic strategies.

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The team analyzed 398 visits made by 48 patients. Among these, 88 visits by 38 patients led to a change in management. The most common change was involving the patient’s medication.

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In 15% of cases, changes were driven by symptoms alone, while in 47% of cases, they were based on diagnostic testing alone. In only 2% of cases, they were driven by physical examination alone, and in another 2% of cases, they were based on symptoms and physical examination. A combination of diagnostic testing and either symptoms or a physical exam led to changes in management in 25% of cases. In 9% of cases, changes were driven by other reasons.

The data also showed that patients with more severe disease (World Health Organization [WHO] functional class III or IV) were more likely to undergo a change in management than those with less severe disease. However, among those with the least severe disease (WHO class I), 18% of visits led to changes in management due to test results.

The researchers said that multiple factors affected the management of children with PAH who were still able to walk but that symptoms or physical examination were not enough to identify those who needed a clinical change in management. They added that testing accounted for most changes.


Critser PJ, Collins SL, Elia EG, et al. Factors determining change in treatment for ambulatory children with pulmonary arterial hypertension: implications for monitoring. Pulm Circ. 2022;12(3):e12121. doi:10.1002/pul2.12121