title: Noninvasive Screening for Pulmonary Hypertension by Exercise Testing in Congenital Heart Disease.
abstract: BACKGROUND
  Patients with congenital heart disease and native or palliated conditions are at risk to develop pulmonary hypertension (PH) in later life. Screening for PH is currently performed by regular echocardiographic follow-up, which appears to be difficult in several congenital conditions. This study evaluated the screening for PH in congenital heart disease by cardiopulmonary exercise testing (CPET).
  
  
  METHODS
  We analyzed our database including all patients with congenital heart disease referred for CPET in our institution from June 2001 to September 2013 and identified 683 patients who had an accompanied heart catheterization less than 6 month after CPET. Those 130 patients with proven PH were compared with the other 563 patients with congenital heart disease but without PH.
  
  
  RESULTS
  Peak oxygen uptake was the most discriminative variable, showing two thresholds at 16.3 mL/min per kg and 25.2 mL/min per kg. The highest specificity of 95% for PH was found in patients with a peak oxygen uptake of 16.3 mL/min per kg or less and a breathing reserve of 37.4% or less. In patients with a peak oxygen uptake exceeding 16.3 mL/min per kg, there was a high specificity of 86.3% but a low sensitivity of 53.1%. With 25.2 mL/min per kg as the threshold, the sensitivity for PH was only 10.0%.
  
  
  CONCLUSIONS
  Detection of PH in patients with congenital heart disease by CPET is difficult because of many falsely positive tests. However, a peak oxygen uptake higher than 25.2 mL/min per kg makes the diagnosis of PH unlikely.
authors.affiliation: Institute of Preventive Pediatrics, Technische Universität München, München, Germany. Electronic address: j.mueller@tum.de.
authors.forename: Jan
authors.initials: J
authors.lastname: Müller
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Technische Universität München, München, Germany.
authors.forename: Pinar Bambul
authors.initials: PB
authors.lastname: Heck
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Technische Universität München, München, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Ewert
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Technische Universität München, München, Germany.
authors.forename: Alfred
authors.initials: A
authors.lastname: Hager
mesh_terms: D000328:Adult; D005080:Exercise Test / Q000379:methods; D005189:False Positive Reactions; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000378:metabolism; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D010100:Oxygen / Q000378:metabolism*
chemical_list: D010100:Oxygen