BackgroundChronic thromboembolic pulmonary hypertension is surgically curable by pulmonary endarterectomy (PEA). It is unclear whether PEA impacts primarily steady state right ventricular afterload (ie, pulmonary vascular resistance (PVR)) or pulsatile right ventricular afterload (ie, pulmonary arterial compliance (CPA)). Our objectives were to (1) quantify PEA specimens and measure the impact of PEA on PVR and CPA in a structure/function study and (2) analyse the effects of haemodynamic changes on long-term survival/freedom of lung transplantation in an outcome study.MethodsThrombi were laid out, weighed, photographed and measured. PVR, CPA and resistance times compliance (RC-time) were assessed at baseline, within 4 days after PEA (‘immediately postoperative’) and 1 year after PEA, in 110 consecutive patients who were followed for 34.5 (11.9; 78.3) months.ResultsLengths and numbers of PEA specimen tails were inversely correlated with immediate postoperative PVR (p<0.0001, r=−0.566; p<0.0001, r=−0.580). PVR and CPA normalised immediately postoperatively while RC-time remained unchanged. Immediate postoperative PVR was the only predictor of long-term survival/freedom of lung transplantation (p<0.0001). Patients with immediate postoperative PVR<590 dynes.s.cm−5 had better long-term outcomes than patients with PVR≥590 dynes.s.cm−5 (p<0.0001, respectively).ConclusionsPEA immediately decreased PVR and increased CPA under a constant RC-time. However, immediate postoperative PVR was the only predictor of long-term survival/freedom of lung transplantation. Our study confirms the importance of a complete, bilateral surgical endarterectomy. Low PVR measured immediately postoperative predicts excellent long-term outcome.

BackgroundPulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy, and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH registry identifies clinical characteristics of patients, diagnostic algorithms, and treatment decisions in a global context.Methods1010 newly diagnosed consecutive patients were included into the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography, and/or invasive pulmonary angiography after at least 3 months on anticoagulation.ResultsOverall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability: technical inaccessibility (n=235), co-morbidities (n=63), and patient refusal (n=44). In Europe, America and other countries (AAO), 72% of patients were deemed suitable for PEA whereas in Japan, 70% of patients were offered BPA as first choice. Gender was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates, and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of PH-related causes (3.5% after PEA and 1.8% after BPA).ConclusionsThe registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and gender) and therapeutic approaches in Japan compared with Europe and AAO.

No abstract

Pulmonary hypertension is defined as an increase in mean pulmonary arterial pressure ≥25 mm Hg at rest and occurs in a majority of patients with heart failure. Diagnostic imaging targets the right ventricle and the pulmonary vasculature. Although echocardiography is cost-effective for screening and follow-up, right heart catheterization is still mandatory to differentiate pre- from post-capillary disease and to directly measure pressure and flow. An important goal is to rule out chronic thromboembolic pulmonary hypertension. This diagnostic step can be achieved by perfusion scintigraphy, whereas computed tomography and cardiac magnetic resonance have become indispensable ancillary methods for the diagnostic allocation to other World Health Organization subtypes of pulmonary hypertension.