Chronic thromboembolic pulmonary hypertension: Time delay from onset of symtoms to diagnosis and clinical condition at diagnosis

Abstract

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. 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. 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. 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.