Right Atrial Contractile Function in Pediatric Pulmonary Hypertension
A Novel Marker for Disease Severity?
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Predicting outcomes in children with pulmonary arterial hypertension (PAH) remains challenging. This is related to the rarity of the disease, the variable etiologies, the different pathophysiology in nonidiopathic pediatric PAH and the constantly changing treatments. Data from adult PAH studies cannot be readily extrapolated to pediatric cohorts as disease progression and specific pediatric studies are necessary. Recent guidelines summarize the current knowledge on pediatric PAH and its clinical management.1
See Article by Jone et al
Apart from clinical parameters and exercise capacity (6-minute walk test), the role of imaging parameters has become well established in outcome prediction of both adult and pediatric PAH. Echocardiography, in particular, plays an important role in diagnosis and follow-up. In recent years, different echocardiographic parameters have been identified to be useful in identifying high-risk patients who are likely to develop adverse clinical outcomes. In particular, a progressive increase in right ventricular (RV) size and deterioration in RV functional parameters results in increased risk for adverse outcomes. How the RV adjusts and copes with the increased ventricular afterload resulting from the pulmonary vascular changes, importantly influences patients’ clinical status and long-term clinical outcomes. Recent data demonstrated that parameters reflecting RV size (RV dimensions and volumes) and RV systolic function (RV ejection fraction, fractional area change, and RV longitudinal strain values) can serve as outcome predictors also in pediatric PAH.2–5
The most recent studies have focused on the assessment of …