title: [Chronic thromboembolic pulmonary hypertension: Time delay from onset of symtoms to diagnosis and clinical condition at diagnosis].
abstract: OBJECTIVE
  Chronic-thromboembolic pulmonary hypertension (CTEPH) is a serious complication of acute pulmonary embolism (PE). In untreated patients prognosis is poor. It depends on WHO-functional class. A delay from onset of symptoms and diagnosis can lead to a further worsening of prognosis. A pulmonary endarterectomy is the treatment of choice. We aimed to evaluate the time delay from onset of symptoms to diagnosis and the WHO-functional class at primary diagnosis in patients with CTEPH.
  
  
  METHODS
  Retrospective analysis of data from 70 monocentrically registered patients (48 women, 22 men, mean age 66,2 years  ±  13,8 years) with confirmed CTEPH from the pulmonary hypertension expert center Missionsärztliche Klinik. Diagnostic work-up was performed according to the current guidelines.
  
  
  RESULTS
  Mean delay from onset of symptoms to diagnosis of CTEPH was 18 ± 26 months. Time delay was only slightly shorter in patients with a history of PE (n = 56; 81 %) than in patients without a history of PE (n = 13; 19 %): 16,9  ±  23,8 vs. 23,5  ±  36,9 months. Time delay was higher in patients who received vasoactive medication before the first contact with a PH expert center and in patients who were classified as technically not suitable for a thrombendarterectomy. 38 patients with a history of acute PE did not have a period without symptoms. In 18 patients symptoms had transiently gone after PE. More than 70 % presented in WHO functional class III or IV.
  
  
  CONCLUSIONS
  Time delay between onset of symptoms and diagnosis of CTEPH and referral to a PH expert center is long and the majority of patients presented in WHO-functional class III or IV. Prognosis is poor in untreated patients and getting worse with a higher WHO-functional class. For this reason, and because CTEPH can be cured by a pulmonary endarterectomy, each patient with suspected PH should be referred to a PH expert center to exclude CTEPH.
authors.affiliation: Abteilung Innere Medizin, Missionsärztliche Klinik, Akademisches Lehrkrankenhaus der Julius-Maximilians-Universität Würzburg.
authors.forename: M
authors.initials: M
authors.lastname: Held
authors.affiliation: Abteilung Innere Medizin, Missionsärztliche Klinik, Akademisches Lehrkrankenhaus der Julius-Maximilians-Universität Würzburg.
authors.forename: M
authors.initials: M
authors.lastname: Grün
authors.affiliation: Abteilung Innere Medizin, Missionsärztliche Klinik, Akademisches Lehrkrankenhaus der Julius-Maximilians-Universität Würzburg.
authors.forename: R
authors.initials: R
authors.lastname: Holl
authors.affiliation: Abteilung Innere Medizin, Missionsärztliche Klinik, Akademisches Lehrkrankenhaus der Julius-Maximilians-Universität Würzburg.
authors.forename: F
authors.initials: F
authors.lastname: Walter
authors.affiliation: Klinik für Thorax- und Herz-Gefäßchirurgie, Universitätsklinikum des Saarlandes, Homburg/Saar.
authors.forename: H-J
authors.initials: HJ
authors.lastname: Schäfers
authors.affiliation: Klinik für Thorax- und Herz-Gefäßchirurgie, Universitätsklinikum des Saarlandes, Homburg/Saar.
authors.forename: T
authors.initials: T
authors.lastname: Graeter
authors.affiliation: Klinik für Innere Medizin V, Pneumologie, Beatmungs- und Umweltmedizin, Universitätsklinikum des Saarlandes, Homburg/Saar.
authors.forename: H
authors.initials: H
authors.lastname: Wilkens
authors.affiliation: Abteilung Innere Medizin, Missionsärztliche Klinik, Akademisches Lehrkrankenhaus der Julius-Maximilians-Universität Würzburg.
authors.forename: B
authors.initials: B
authors.lastname: Jany
mesh_terms: D000368:Aged; D000465:Algorithms; D002908:Chronic Disease; D003299:Cooperative Behavior; D057210:Delayed Diagnosis; D017128:Embolectomy; D004691:Endarterectomy; D005260:Female; D005858:Germany; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D033183:Interdisciplinary Communication; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011651:Pulmonary Artery / Q000601:surgery; D011655:Pulmonary Embolism / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D012017:Referral and Consultation; D012189:Retrospective Studies; D015996:Survival Rate; D017131:Thrombectomy