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  1. Matthias Gorenflo1,
  2. David Pittrow2,
  3. Dörte Huscher3,
  4. Victoria Ziesenitz4,
  5. Joseph Pattathu4,
  6. Alexander Praus5,
  7. Peter Ewert5,
  8. Harald Kaemmerer1,5,
  9. COMPERA-Kids Steering Committee Study Group
  1. 1COMPERA-Kids Steering Committee and University Hospital Heidelberg
  2. 2Technical University Dresden
  3. 3German Rheumatism Research Centre & Charité, Berlin
  4. 4University Hospital Heidelberg
  5. 5German Heart Centre Munich


Background Pulmonary hypertension (PH/PAH) can have many possible causes in childhood. The aim of the COMPERA registry is the characterization of patients of all ages with PH/PAH and their treatment patterns.

Methods Since June 2013, paediatric patients can be included in the COMPERA registry ( NCT01347216) which has originally been established for adult patients with pulmonary hypertension in 2007.

Results Until 2015, 78 patients <18 years (47 Pat <6 years) with pulmonary hypertension were enrolled, of whom 65.4% had PAH due to congenital heart disease (PAH-CHD), 25.6% had idiopathic PAH (iPAH), 3 had persistent PH of the newborn, 2 had PH associated with interstitial lung disease, and two had other PH. The patients were 6.1±6.0 years old, 52.6% girls; NYHA functional class I/II in 55.0%, and III in 42.3%. Mean disease duration after diagnosis was 37.7±55.8 months. Right heart catheterization data were available for 82.1% of the patients. Mean pulmonary artery pressure was 42.8±19.4 mmHg, right atrial pressure was 8.0±8.8 mmHg, cardiac index was 3.6±1.2 l/min/m2. Monotherapy was received by 65.4% of the patients whereas 33.4% of the patients had combination therapy. Phosphodiesterase-5 inhibitors (PDE5I) were administered to 76.9% of the patients, 35.9% of the patients received endothelin receptor antagonists and 3.8% received prostacyclins. About 24% of patients received anticoagulation therapy.

Conclusion The most common form of PH in this study cohort is PAH-CHD, followed by iPAH. Treatment options for children primarily comprise PDE5I. Only a small number of paediatric patients receive anticoagulation therapy.

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