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. In every patient with suspected PH, CTEPH or chronic thromboembolic disease (CTED, i.e. symptomatic residual vasculopathy without pulmonary hypertension) should be excluded. Primary treatment is surgical pulmonary endarterectomy (PEA) in a multidisciplinary CTEPH centre. Inoperable patients or patients with persistent or recurrent CTEPH after PEA are candidates for targeted drug therapy. There is increasing experience with balloon pulmonary angioplasty (BPA) for inoperable patients; this option, like PEA, is reserved for specialised centres with expertise in this treatment method.

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. The RHF section comprises definition and pathophysiology, diagnosis and monitoring, identification of triggering factors and supportive therapy of RHF, volume management as well as PAH targeting therapy, therapy with inotropic, inodilator and vasopressor drugs, extracorporeal support and transplantation. The second part of this article summarizes preoperative management, perioperative monitoring and choice of anesthesia.

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 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH. METHODS: We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry. The CTEPH incidence was calculated based on German population data, and patient characteristics and treatment patterns were described. RESULTS: A total of 392 patients were newly diagnosed with CTEPH within 2016 in Germany, yielding an incidence of 5.7 new cases per million adults. The (mean ± standard deviation) age was 63.5 ± 15.0 years; males and females were equally affected; 76.3% of the patients had a history of venous thromboembolism. A total of 197 (50.3%) patients underwent pulmonary endarterectomy. Almost all non-operated patients received targeted drug therapy, and 49 patients (25.1% of the non-operated patients) were treated with balloon pulmonary angioplasty. CONCLUSION: The incidence of CTEPH in Germany 2016 was 5.7 per million adults and thus higher than previously reported from other countries. Half of the patients were operated while the remaining patients received medical or interventional therapies. CLINICAL TRIALS REGISTRATION: http://www.clinicaltrials.gov NCT02660463 and NCT01347216.

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. Lung donors were divided into 3 groups according to age (donor aged <60 years, 60-69 years and ≥70 years). We examined the effect of donor age on various short- and long-term outcome parameters. RESULTS: Lungs harvested from very old donors had a lower percentage of smoking history and shorter ventilation time. Survival rates of recipients did not show significant differences between older and younger donor groups. Most of the short- and long-term outcome parameters in recipients of lungs from very old donors did not differ significantly among the 3 age groups, except for post-transplant best forced expiratory volume in 1 s and treated acute rejections, which were lower and higher, respectively, in donors aged ≥70 years. CONCLUSIONS: This dual-centre analysis showed that lung transplantation from donors aged ≥70 years was not associated with worse outcomes compared with the younger donors. This study supports the idea that it might be possible to use an extraordinarily cautious selection of lungs from very old donors to increase the pool of suitable donors, given the shortage of suitable organ donors available for lung transplantation.

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.BPA resulted in improvements in WHO functional class, 6 min walk distance (mean change, +33 m), right ventricular function and haemodynamics, including a decline in mean pulmonary artery pressure by 18% and in pulmonary vascular resistance by 26%. Procedure-related adverse events occurred in 9.4% of the interventions. The most common complications were related to pulmonary vascular injury and consecutive pulmonary bleeding. Most of these events were asymptomatic and self-limiting, but one patient died from pulmonary bleeding, resulting in a mortality rate of 1.8%.BPA resulted in haemodynamic and clinical improvements but was also associated with a considerable number of complications, including one fatal pulmonary bleeding. As the effects of BPA on survival are unknown, randomised controlled outcome trials comparing BPA with approved medical therapies in patients with inoperable CTEPH are required to allow for appropriate risk-benefit assessments.

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 (VO2 ) 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. RESULTS: One-hundred and fifty-one patients (82 female (54.3%), mean age 61 ± 12.4 years) presented with a mean pulmonary arterial pressure of 40.2 ± 14.2 mmHg and pulmonary vascular resistance (PVR) of 641.9 ± 374.8 dyne∗s/cm5 . The peak VO2 (mean 13.1 ± 4.5 mL∗kg-1 ∗min-1 ) was measured at initial referral. Over a follow-up of up to 10 years (mean 4.41 ± 2.57 years), 31 patients had died. Patients with a baseline peak VO2 ≥ 10.7 mL∗kg-1 ∗min-1 [area under the receiver-operating characteristic curve (AUC) = 0.728, P = 0.001] had better survival than those with a peak VO2 ≤ 10.7 mL∗kg-1 ∗min-1 using Kaplan-Meier analysis (88.8% vs 60.1%; log rank P = 0.001). Adjusting for age, gender and PVR, multivariate analysis identified peak VO2 as a predictor of mortality [hazard ratio (HR): 2.78, 95% CI 1.01-7.63, P = 0.047]. In addition, peak VO2 failed as an independent prognostic factor in a stepwise multivariate model including all variables significant in the univariate analysis. CONCLUSIONS: In patients with inoperable CTEPH the peak VO2 is a significant predictor of survival, when adjusting for age, gender and PVR. However, peak VO2 failed as an independent prognostic factor when correcting for all significant baseline variables, which is limiting the clinical usability.

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. Novel alternative treatment options for inoperable CTEPH patients will be discussed, i.e. targeted medical therapy and balloon pulmonary angioplasty. For the future the major task will be to define a clear selection process for the optimal treatment of the individual CTEPH patient.

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 56.7 ± 16, 28 female) underwent PC-MRI before and after PEA as part of their peri-operative routine workup. Pulmonary artery (PA) maximum flow velocity (maxV), acceleration time/ejection time (AT/ET), distensibility [(PA maximum area - PA minimum area)/PA minimum area], mid-systolic flow deceleration (notch), and the timing of deceleration (notch ratio) were recorded. Mean PA pressure was obtained from standard right heart catheter procedures. maxV and AT/ET were decreased before PEA and significantly improved afterwards (60.8 ± 16 vs. 73.8 ± 19 cm/s, p = 0.007; 0.32 ± 0.06 vs. 0.36 ± 0.09, p = 0.0015). Surprisingly, distensibility did not change significantly (30 ± 19 vs. 26 ± 12%, p = 0.11). Forty-five patients (78%) had a systolic notch before PEA that persisted in only 10 (18%; p = 0.00001). Among patients with a persisting notch, the notch ratio did not significantly increase (1.3 ± 0.2 vs. 1.6 ± 1.5, p = 0.32). Our data show early PA reverse remodeling after PEA. Flow velocities increase while PA flow wave reflections represented by mid-systolic flow deceleration are abolished. In some patients a mid-systolic notch persists, suggesting increased downstream resistance as a consequence of small vessel arteriopathy.

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. By use of cardiovascular magnetic resonance (CMR) combined with hemodynamic measurements pulmonary arterial elastance (Ea-pulm_i), end-systolic right ventricular elastance (Ees-RV_i) and ventriculo-arterial coupling (Ea-pulm_i/Ees-RV_i) can be studied before and after PEA. METHODS: Sixty-five patients (mean age 41±12 years, 28 female) underwent CMR pre- and post-PEA. Ejection fraction (EF), end-diastolic (EDVi), end-systolic (ESVi), and stroke (SVi) volumes were indexed for body surface area. Ea-pulm_i was calculated as pulmonary artery mean pressure (mPAP)/SVi, and Ees-RV_i as mPAP/ESVi. RESULTS: mPAP decreased from 47±12 to 25±9 mmHg, p=0.0001. Ea-pulm_i was increased before PEA and normalized afterwards (2.8±2.1 vs. 0.85±0.4 mmHg/ml/m2, p=0.0001). Ees-RV_i was depressed before and after PEA (0.72±0.27 vs. 0.66±0.3 mmHg/ml/m2, p=0.13). EF improved from 25±12% to 46±10%, p=0.0001, because ventriculo-arterial coupling was restored (4.2±3 vs. 1.4±0.6, p=0.0001). EDVi and ESVi mproved significantly (EDVi 92±32 to 72±23 ml, p=0.0001; ESVi 69±31 to 41±18 ml, p=0.0001). CONCLUSION: RV function is largely determined by afterload and returns to normal once afterload is normalized. This is paralleled by a significant improvement of CMR indices of right ventricular remodelling.

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. Any patient with unexplained PH should be evaluated for the presence of CTEPH, and a ventilation/perfusion (V/Q) lung scan is recommended as screening method of choice. If the V/Q scan or CT angiography reveals signs of CTEPH, the patient should be referred to a specialized center with expertise in the medical and surgical management of this disease. Every case has to be reviewed by an experienced PEA surgeon for the assessment of operability. In this updated recommendation, important contents of the European guidelines were commented, and more recent information regarding diagnosis and treatment was added.

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. It is not known whether such patients display chronic immune activation as well. METHODS AND RESULTS: We studied 49 patients with CTEPH (50±2 years, right ventricular ejection fraction [RVEF] 29±2%, left ventricular ejection fraction [LVEF] 51±3%, mean±SEM) and compared their results with 17 patients with CHF (71±2 years, LVEF 23±1%) and 34 age-matched control subjects (age 57±2 years). We studied serum levels of tumor necrosis factor-α (TNFα), its soluble receptors 1 and 2 (sTNFR1 and 2), interleukin-10 (IL-10) and plasma N-terminal-pro-B-type natriuretic peptide (NT-proBNP). Serum TNFα was not different in CTEPH compared with CHF patients (p=0.67) but both their levels were significantly higher than in controls (both p<0.001). Similar results were obtained for sTNFR1, sTNFR2, and IL-10. Levels of NT-proBNP were not different in patients with CTEPH or CHF (p=0.54), but significantly higher than in control subjects (both p<0.001). There were significant correlations between RVEF as assessed by magnetic resonance imaging and sTNFR1, sTNFR2, IL-6, high sensitivity C-reactive protein, and NT-proBNP (all p<0.05) in patients with CTEPH. CONCLUSION: Similar levels of immune activation as reflected by high levels of pro-inflammatory cytokines are present in patients with isolated right ventricular dysfunction due to CTEPH and patients with CHF and left ventricular dysfunction.

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. METHODS: Heart-lung blocks of 25 New Zealand white rabbits were used. After measurement of baseline values (haemodynamics and gas exchange) the lungs were exposed to 120 minutes of hypoxic warm ischaemia followed by repeated measurements during reperfusion. Group A was immediately reperfused using a flow of 100 ml/min whereas groups B, C and D were initially reperfused with a maximum pressure of 5 mmHg for 5, 15 or 30 minutes, respectively. The control group had no period of ischaemia or PCR. RESULTS: Uncontrolled reperfusion (group A) caused a significant pulmonary injury with increased pulmonary artery pressures (PAP) and pulmonary vascular resistance and a decrease in oxygen partial pressure (PO2), tidal volume and in lung compliance. All groups with PCR had a significantly higher PO2 for 5 to 90 min after start of reperfusion. At 120 min there was also a significant difference between group B (264 +/- 91 mmHg) compared to groups C and D (436 +/- 87 mmHg; 562 +/- 20 mmHg, p < 0.01). All PCR groups showed a significant decrease of PAP compared to group A. CONCLUSION: Uncontrolled reperfusion results in a severe lung injury with rapid oedema formation. PCR preserves pulmonary haemodynamics and gas exchange after ischaemia and might allows for recovery of the impaired endothelial function. 30 minutes of PCR provide superior results compared to 5 or 15 minutes of PCR.

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. We report the results of a double-blinded randomized trial with the aerosolized prostacyclin analogue iloprost in patients with residual pulmonary hypertension after PEA. METHODS: Twenty-two patients (age, 55+/-13 years; 8 females; propofol- and sufentanil-based anesthesia; pressure-controlled mechanical ventilation) were randomized to receive either a single dose of 25 microg aerosolized iloprost (iloprost group; n=11) or normal saline (placebo group; n=11) immediately after postoperative ICU admission. Primary endpoints were changes in gas exchange, pulmonary and systemic hemodynamics, and clinical outcome. RESULTS: Iloprost significantly enhanced cardiac index (CI) and reduced mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance [PVR (dynscm(-5))] in contrast to placebo. Placebo: pre-inhalation 413+/-195 versus post-inhalation 404+/-196 at 30 min (p=0.051), 415+/-189 at 90 min (p=0.929). Iloprost: pre-inhalation 503+/-238 versus post-inhalation 328+/-215 at 30 min (p=0.001), 353+/-156 at 90 min (p=0.003). Blood oxygenation remained unchanged. CONCLUSION: In addition to the effect of PEA, iloprost reduces residual postoperative pulmonary hypertension, decreases right ventricular afterload and may facilitate the early postoperative management after PEA.

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). Secondary end points included 6-min walk test, peak oxygen uptake (V(O2)), New York Heart Association functional class, serum levels of N-terminal-pro brain natriuretic peptide (NT-pro-BNP), and various other hemodynamic parameters. RESULTS: After 3 months of treatment with bosentan, PVR decreased from 914 +/- 329 to 611 +/- 220 dyne.s.cm(-5) (p < 0.001). Functional class and peak V(O2) remained unchanged, but 6-min walk distance increased from 340 +/- 102 to 413 +/- 130 m (p = 0.009), and serum NT-pro BNP levels improved from 2,895 +/- 2,620 to 2,179 +/- 2,301 (p = 0.027). One patient died, presumably from influenza A infection, and another patient experienced progressive fluid retention despite reduction of PVR. Other than that, treatment was well tolerated by all patients. CONCLUSIONS: This open-label pilot trial suggests that bosentan may offer a therapeutic option for patients with inoperable CTEPH. Randomized controlled trials are warranted to confirm these findings.

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. The recordings were evaluated offline and independently by both raters for visualization of morphological and functional features of the tricuspid valve according to a subjective 3-point scale. Statistical analyses were performed for interrater agreement and for comparison of imaging quality between the two study groups. In addition, we present an illustrative case report. RESULTS: Visualization of the spatial relationship between the tricuspid valve and vicinal structures, of the commissures, the orifice, and entirety of valve depiction were better in the ventricular failure group as compared to the control group. Annular dimensions were equally assessable in both groups, leaflet thickness and mobility were not significantly different. Interrater agreement on assessability was slight for leaflet thickness, fair for leaflet mobility and orifice area, and good for the remaining features. The 3D-TTE exam including offline evaluation took 6.5 minutes on average and maximally 14 minutes. CONCLUSION: 3D-TTE of the tricuspid valve can be performed in addition to routine 2D echocardiography within a reasonable time and with high assessability of important features in patients with right ventricular failure. Interrater agreement was fair to good overall. Thus, its feasibility may encourage prospective studies on its potential for more detailed noninvasive diagnosis and preoperative planning.

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. Contrast-enhanced MR angiography was compared with selective digital subtraction angiography (DSA) for depiction of central thromboembolic material and visualization of the pulmonary arterial tree. Functional analysis included calculation of left and right ventricular ejection fractions and peak velocities, net forward volumes per heartbeat, and blood volume per minute in the left and right pulmonary arteries and ascending aorta. Flow measurements were compared with invasively measured mean pulmonary arterial pressure (MPAP) and pulmonary vascular resistance (PVR) measurements. Nonparametric Wilcoxon and sign tests were used for statistical analysis. RESULTS: MR angiography revealed typical findings of CTEPH (intraluminal webs and bands, vessel cutoffs, and organized central thromboemboli) in all patients. It depicted pulmonary vessels up to the segmental level in all cases. For subsegmental arteries, DSA revealed significantly more patent vessel segments than did MR angiography (733 versus 681 segments, P <.001). MR angiography revealed technical success of surgery in 33 of 34 patients. Patients had reduced right ventricular ejection fractions and pulmonary peak velocities that significantly increased after PTE (P <.001 for both). Right ventricular ejection fraction had good correlation with PVR (r = 0.6) and MPAP (r = 0.7). The postoperative decrease in MPAP correlated well with the increase in right ventricular ejection fraction (r = 0.8). Postoperatively, there was complete reduction of a preoperatively existing bronchosystemic shunt volume in 33 of 34 patients. CONCLUSION: Breath-hold MR imaging techniques enable morphologic and semiquantitative functional assessment of patients with CTEPH.

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). Effects on pulmonary and systemic hemodynamics and gas exchange were recorded and compared with preinhalation baseline values. RESULTS: Preoperatively, inhaled iloprost did not significantly change mean pulmonary artery pressure (mPAP), cardiac index (CI), or pulmonary vascular resistance (PVR). Postoperatively, inhaled iloprost induced a significant reduction of mPAP and PVR and a significant increase of CI at T2 and T3. Preinhalation versus postinhalation PVR was as follows: at T1, 847 versus 729 dynes. s. cm(-5), p = 0.45; at T2, 502 versus 316 dynes. s. cm(-5), p = 0.008; and at T3, 299 versus 227 dynes. s. cm(-5), p = 0.004. CONCLUSIONS: In patients with CTEPH, inhalation of iloprost elicits no significant pulmonary vasodilation before surgery, and may have detrimental effects on systemic hemodynamics. Postoperatively, it significantly reduces mPAP and PVR, and enhances CI. Following PTE, inhalation of iloprost is useful to improve early postoperative hemodynamics.

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. Data sets were assessed for number of patent segmental, subsegmental arteries, and number of vascular segments with thrombotic wall thickening, intraluminal webs, and abnormal proximal to distal tapering. Image quality for MRA/CTA was scored excellent in 16 of 16, good in 11 of 14, moderate in 2 of 5, and poor in no examinations. The MRA/CTA showed 357 of 366 patent segmental and 627 of 834 patent subsegmental arteries. CTA was superior to MRA in visualization of thrombotic wall thickening (339 vs 164) and of intraluminal webs (257 vs 162). Abnormal proximal to distal tapering was better assessed by MRA than CTA (189 vs 16). In joint assessment of direct and indirect signs, MRA and CTA were equally effective (353 vs 355). MRA and CTA are equally effective in the detection of segmental occlusions of the pulmonary arteries in CTEPH. CTA is superior for the depiction of patent subsegmental arteries, of intraluminal webs, and for the direct demonstration of thrombotic wall thickening.

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. Geometry of the tricuspid valve as well as right ventricular size and function were determined with echocardiography. Mean pulmonary arterial pressure was determined invasively. RESULTS: After PTE, mean pulmonary arterial pressure was significantly lower (48+/-10 versus 25+/-7 mm Hg, p < 0.05). Most of the patients had a distinct reduction of TR, and the improvement trend showed on the severity scale: number of patients with 4+TR (23 --> 4), 3+TR (12 --> 12), 2+TR (2 --> 13), and 1+TR (2 --> 10). Examination after PTE revealed profound reduction of right ventricular size and annulus diameter, with a normalization of the valvular geometry. However, none of the study variables were useful as indicators of the postoperative outcome. CONCLUSIONS: After PTE without additional valve repair most patients show significantly reduced severity of TR soon afterward; the very few cases in which TR does not improve remain unidentifiable before operation. Our recommendation is consequently to refrain from additional tricuspid repair in patients undergoing PTE.