Survival Differences in Pediatric Pulmonary Arterial Hypertension

Abstract

Survival rates of pediatric PAH patients differed between 3 major referral centers. This could be explained by differences between the center cohorts in patients' diagnoses and measures of disease severity, which were identified as important predictors of outcome. In this study, treatment with PAH-targeted combination therapy during the study period was independently associated with improved survival.

Pulmonary Hypertension in Children

“…2,98,99 It is unclear at this time whether the criteria for acute vasoreactivity are the same in adults as in children. 2,3,100,101 There was no difference in the number of vasoreactivity responders in children with IPAH using the Barst or Sitbon criteria in a Netherlands study (Figure 12). 100 …”

“…106 Prostacyclin administered over the long term, utilizing intravenous epoprostenol, has shown to improve survival and quality of life in adults and children with idiopathic pulmonary arterial hypertension. 2,3,103,107–109 …”

“…Treatment with PAH-targeted combination therapy during the study period was independently and strongly associated with improved survival compared to monotherapy (Figure 17). 3 …”

“…2 Recent advances in the understanding of the pathobiology of idiopathic pulmonary arterial hypertension and new treatment therapies have resulted in marked improvement in the prognosis for children with PAH (Figure 1). 3,4 Similarities and differences persist in comparison of children and adults with PAH. 5 In both groups, disease progression is rapid, perhaps more rapid in children than in adults, and left untreated elevation of pulmonary arterial pressure and resistance leads to right ventricular failure, clinical deterioration and death.…”

Almanac 2014: Congenital Heart Disease.

“…50, 51 Kako bi se iscrpnije istražile razlike u preživljenju, prediktorima rizika i optimalnim metodama liječenja potrebna su daljnja istraživanja.…”