P28 Chronic thromboembolic pulmonary hypertension: long term outcomes in surgical and non-surgical patients

Abstract

IntroductionChronic thromboembolic pulmonary hypertension (CTEPH) is commonly associated with a history of venous thromboembolism. Pulmonary endarterectomy (PEA) offers a potential cure in surgically accessible disease. However, a significant proportion of patients with CTEPH may not undergo surgery due to various reasons including disease distribution, comorbidities and patient choice This group of patients have previously been considered to have a poor outcome although an international registry has recently reported on improved medium term outcomes in this patient population.Aims and objectivesTo compare long term survival of patients with CTEPH undergoing pulmonary endarterectomy (CTEPH-surgical-operated), surgically accessible disease not undergoing pulmonary endarterectomy (CTEPH-surgical-not-operated), surgically inaccessible disease (CTEPH-non-surgical).MethodsData was retrieved from hospital records and departmental database for consecutive, treatment-naïve patients with CTEPH diagnosed between 1st January 2001 and 30th November 2014 and followed up till 30th November 2015 at the Sheffield Pulmonary Vascular Disease Unit and collected in the ASPIRE registry. Patients with suspected CTEPH undergo systematic evaluation but formal pulmonary angiography is only performed when other imaging modalities such as CTPA, MR imaging and nuclear medicine imaging are non-diagnostic.Results592 patients, mean age (± standard deviation), 65 ± 22 years, mean pulmonary arterial pressure 48 ± 13 mmHg and median pulmonary vascular resistance 480 ± 463 dynes/sec/cm-5 were identified and followed for 4.3 ± 3.2 years. 5 year survival was significantly (p < 0.001) better in CTEPH-surgical-operated (n = 279) at 82.9 ± 3.1% compared to CTEPH-surgical-not-operated (n = 206) at 44.4 ± 5% (66.7 ± 9.1% patient choice, 39.4 ± 6% comorbidities) and 53.4 ± 5.8% in CTEPH-non-surgical (n = 107). Only 4% of the patients in our study were investigated with conventional pulmonary angiography.The median time to PEA surgery from diagnosis was 10.2 months and did not affect long term survival (p = 0.52).ConclusionsFor operable patients with CTEPH pulmonary endarterectomy is associated with an excellent long term outcome, the long-term survival of patients with surgical disease who decline surgery is significantly better than historically reported and that a non-invasive multimodality imaging approach can be used to assess patients with suspected CTEPH. Furthermore there is no time from diagnosis to surgery which predicts outcome.Abstract P28 Figure 1Kaplan-Meier estimates survival from date of diagnosis in CTEPH, surgical, operated, CTEPH, surgical, not-operated and CTEPH, non-surgical patients,log-rank test, p < 0.001

The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension

“…The aim of the current study was to provide data to help inform patient choice by identifying outcomes and prognostic factors in a large cohort of consecutive patients with CTEPH. Some of the results have been reported previously in abstract form [28, 29].…”

Lėtinė Tromboembolinė Plautinė Hipertenzija: Diagnostika Ir Gydymo Metodai

“…Išgyvenamumas po 3 metų po šios procedūros siekia 90 procentų [3]. Po 5 metų išgyvenamumas pacientų su proksimalinių segmentų liga, kurie atsisako šio gydymo, yra 53 proc., o pacientų, kuriems atliekamas operacinis gydymas, išgyvenamumas siekia 83 procentus [5]. Vis dėlto, apie 25 proc.…”

Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension

“…[2][3][4] However, 30-40% of patients with CTEPH are considered inoperable. [4][5][6] Furthermore, after PEA, up to a third of the patients have persistent or recurrent PH and may benefit from disease-targeted drug therapy. [1][2][3][4][5][6] Currently, only one drug specifically targeting CTEPH is approved and available in Sweden, 7,8 but based on clinical experience and in symptomatic patients, off-label use of pulmonary arterial hypertension specific therapies is common.…”

“…[4][5][6] Furthermore, after PEA, up to a third of the patients have persistent or recurrent PH and may benefit from disease-targeted drug therapy. [1][2][3][4][5][6] Currently, only one drug specifically targeting CTEPH is approved and available in Sweden, 7,8 but based on clinical experience and in symptomatic patients, off-label use of pulmonary arterial hypertension specific therapies is common. [9][10][11] CTEPH, with or without PEA, requires lifelong treatment with anticoagulants and, in cases of heart failure or hypoxaemia, diuretics, and oxygen therapy can be indicated.…”