Nearly half of children with confirmed disease have chromosomal abnormalities, mainly trisomy 21
WEDNESDAY, Jan. 11 (HealthDay News) -- An analysis of data from the Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry has identified clinical features specific to the care of children with pulmonary hypertension, according to research published online Jan. 11 in The Lancet.
Rolf M.F. Berger, M.D., from the University of Groningen in the Netherlands, and colleagues assessed medical records from 456 consecutive patients (aged ≤18 years at diagnosis) enrolled in the TOPP registry. The patients (seen in 31 centers in 19 countries) had a diagnosis of pulmonary hypertension and increased pulmonary vascular resistance. Patient and disease characteristics were recorded.
The researchers found that 79 percent of consecutive patients had confirmed pulmonary hypertension; of these patients, 88 percent had pulmonary arterial hypertension (PAH) and 12 percent had pulmonary hypertension associated with respiratory disease or hypoxemia. In 47 patients (13 percent) with confirmed disease, chromosomal anomalies were reported, mainly trisomy 21. Diagnosis was made at a median age of 7 years. The most frequent symptoms were dyspnea and fatigue; syncope occurred in 18 percent of patients with repaired congenital heart disease and in 31 percent with idiopathic or familial PAH, and was not seen in children with unrepaired systemic-to-pulmonary shunts. In 64 percent of patients, the functional class was I or II, consistent with preserved right-heart function.
"TOPP identifies important clinical features specific to the care of pediatric pulmonary hypertension, which draw attention to the need for pediatric data rather than extrapolation from adult studies," the authors write.
Several authors disclosed financial relationships with pharmaceutical companies, including Actelion Pharmaceuticals, which funded the study.
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