Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference 2018.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a subgroup of pulmonary hypertension that differs from all other forms of PH in terms of its pathophysiology, patient characteristics and treatment. For implementation of the European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension in Germany, the Cologne Consensus Conference 2016 was held and last updated in spring of 2018. One of the working groups was dedicated to CTEPH, practical and controversial issues were commented and updated.

Decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension: Updated recommendations from the Cologne Consensus Conference 2018.

In June 2016, members of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) met for a Consensus Conference in Cologne, Germany. Aim of this Conference was to compile consensus based practice recommendations based on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. This article summarizes the results and updated recommendations 2018 of the working group on decompensated right heart failure (RHF), intensive care and perioperative management in patients with pulmonary hypertension.

Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany.

Background: The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.

Use of very old donors for lung transplantation: a dual-centre retrospective analysis.

Objectives: To reduce the shortage of organs for transplantation by expanding organ selection criteria as a means to increase the pool of potential lung donors. In this study, we sought to investigate the impact of using lungs from very old donors aged >70 years on outcomes after lung transplantation.

Methods: Between January 2010 and November 2016, 96 patients with end-stage lung disease underwent lung transplantation in our centres.

Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: the initial German experience.

Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).We report on a prospective series of 56 consecutive patients who underwent 266 BPA interventions (median, five per patient) at two German institutions. All patients underwent a comprehensive diagnostic work-up including right heart catheterisation at baseline and 24 weeks after their last intervention.

Pulmonary endarterectomy for the treatment of chronic thromboembolic pulmonary hypertension.

Pulmonary endarterectomy is a curative treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH). There is a growing body of evidence suggesting that not only patients with CTEPH but also patients with pulmonary arterial obstructions and mean pulmonary artery pressures < 25 mmHg should be offered surgery. In this review, the recent literature regarding pathophysiology, diagnostic methods, decision making by an expert CTEPH team, and surgical techniques will be summarized.

The prognostic relevance of oxygen uptake in inoperable chronic thromboembolic pulmonary hypertension.

Background: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) present with a decreased oxygen uptake, however, the prognostic relevance of oxygen uptake (VO ) in inoperable CTEPH is unknown.

Methods: Patients with inoperable CTEPH were retrospectively analyzed. All patients were assessed by means of right heart catheterisation and cardio pulmonary exercise testing in semisupine position with a 30 Watt increment step-protocol.

Pulmonary vascular remodeling before and after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: a cardiac magnetic resonance study.

Phase-contrast magnetic resonance imaging (PC-MRI) offers a range of surrogate markers to quantify the hemodynamic changes associated with chronic thromboembolic pulmonary hypertension (CTEPH). Our aim was to noninvasively monitor effects of pulmonary vascular remodeling before and after endarterectomy (PEA) in patients with CTEPH by using PC-MRI. Fifty-seven consecutive patients (mean age 56.

Right ventricular adaptation to pulmonary pressure load in patients with chronic thromboembolic pulmonary hypertension before and after successful pulmonary endarterectomy--a cardiovascular magnetic resonance study.

Background: The aim of the study was to characterize RV adaptation to varying loading conditions in patients with chronic thromboembolic hypertension (CTEPH) before and after pulmonary endarterectomy (PEA). Nearly 4% of patients with pulmonary embolism develop CTEPH. PEA offers a cure with excellent outcome.

Chronic thromboembolic pulmonary hypertension (CTEPH): updated Recommendations of the Cologne Consensus Conference 2011.

In the 2009 European Guidelines on the diagnosis and treatment of pulmonary hypertension (PH), one section covers aspects of pathophysiology, diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The practical implementation of the guidelines for this disease is of crucial importance, because CTEPH is a subset of PH which can potentially be cured by pulmonary endarterectomy (PEA). Nowadays, CTEPH is commonly underdiagnosed and not properly managed.

Inflammation in right ventricular dysfunction due to thromboembolic pulmonary hypertension.

Objectives And Background: Activation of the immune system is well established in patients with chronic heart failure (CHF) and impaired left ventricular function. High levels of pro-inflammatory cytokines are associated with a poor prognosis. Chronic thromboembolic pulmonary hypertension (CTEPH) frequently leads to impaired right ventricular function.

Length of pressure-controlled reperfusion is critical for reducing ischaemia-reperfusion injury in an isolated rabbit lung model.

Background: Ischaemia-reperfusion injury is still a major problem after lung transplantation. Several reports describe the benefits of controlled graft reperfusion. In this study the role of length of the initial pressure-controlled reperfusion (PCR) was evaluated in a model of isolated, buffer-perfused rabbit lungs.

Inhaled iloprost to control residual pulmonary hypertension following pulmonary endarterectomy.

Objective: Pulmonary endarterectomy (PEA) is the standard therapy for patients with chronic thromboembolic pulmonary hypertension (CTEPH). In the immediate postoperative period, persistent pulmonary hypertension increases the risk of acute respiratory or right heart failure. In pulmonary arterial hypertension, prostanoid inhalation has been found to improve pulmonary hemodynamics, right ventricular function, gas exchange, and clinical outcome.

Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension.

Study Objectives: We performed an open-label multicenter study to evaluate the safety and efficacy of the dual endothelin receptor antagonist bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).

Patients: Nineteen patients with inoperable CTEPH were enrolled.

Measurements: The primary end point was a change in pulmonary vascular resistance (PVR).

Assessment of the tricuspid valve morphology by transthoracic real-time-3D-echocardiography.

Aim: To demonstrate the feasibility of transthoracic three-dimensional real-time echocardiography (3D-TTE) supplemental to routine assessments of the tricuspid valve and to analyze interrater agreement.

Methods: Twenty healthy subjects and 74 patients with right ventricular failure were examined with conventional 2D and additionally 3D-TTE (SONOS 7500, Philips, Netherlands). The 3D exams were performed and recorded by one of two raters.

Chronic thromboembolic pulmonary hypertension: pre- and postoperative assessment with breath-hold MR imaging techniques.

Purpose: To evaluate the potential of breath-hold magnetic resonance (MR) imaging techniques in morphologic and functional assessment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) before and after surgery.

Materials And Methods: Thirty-four patients with CTEPH were examined before and after pulmonary thromboendarterectomy (PTE). For morphologic assessment, contrast material-enhanced MR angiography was used; for assessment of hemodynamics, velocity-encoded gradient-echo sequences and cine gradient-echo sequences along the short axis of the heart were performed.

Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: effects before and after pulmonary thromboendarterectomy.

Background: In primary pulmonary hypertension, aerosolized prostanoids selectively reduce pulmonary vascular resistance and improve right ventricular function. In this study, hemodynamic effects of inhaled iloprost, a stable prostacyclin analogue, were evaluated in patients with chronic thromboembolic pulmonary hypertension (CTEPH) before and early after pulmonary thromboendarterctomy (PTE).

Methods: Ten patients (mean age 49 years old [32 to 70 years old], New York Heart Association functional class III and IV) received a dose of 33 micro g aerosolized iloprost immediately before surgery (T1), after intensive care unit admission (T2), and 12-hours postoperatively (T3).

Value of contrast-enhanced MR angiography and helical CT angiography in chronic thromboembolic pulmonary hypertension.

The aim of this study was to evaluate the diagnostic value of contrast-enhanced MR angiography (ce MRA) and helical CT angiography (CTA) of the pulmonary arteries in the preoperative workup of patients with chronic thromboembolic pulmonary hypertension (CTEPH). The ce MRA and CTA studies of 32 patients were included in this retrospective evaluation. Image quality was scored by two independent blinded observers.

Assessment of cardiac performance using Tei indices in patients undergoing pulmonary thromboendarterectomy.

Background: This study was designed to evaluate left and right ventricular performance using Tei indices in patients with severe chronic thromboembolic pulmonary hypertension undergoing pulmonary thromboendarterectomy (PTE). The Doppler-derived indices are easily measurable indicators of ventricular function based on nongeometric assessment, which helps overcome some of the difficulties entailed in the geometric assessment of left ventricular (LV) and right ventricular (RV) function in pulmonary hypertension.

Methods: The indices were derived for 24 patients (aged 54+/-14 years) before and after PTE.

Improvement of tricuspid regurgitation after pulmonary thromboendarterectomy.

Background: For patients with chronic thromboembolic pulmonary hypertension who undergo pulmonary thromboendarterectomy (PTE) it has not yet been systematically investigated how operation affects the severity of tricuspid regurgitation (TR). This study sought (1) to evaluate the extent of TR reversibility after operation, (2) to identify potential predictors of the reversibility of TR, and (3) to investigate the influence of geometric and hemodynamic alterations on the extent of TR severity.

Methods: Thirty-nine patients (55+/-12 years) undergoing PTE without tricuspid valve repair were investigated before and 13+/-8 days after operation by Doppler color flow mapping.