Therapie der chronischen Thromboembolie (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a frequent cause of pulmonary hypertension, however, it still is underdiagnosed. Up to 4% of all individuals that survive pulmonary embolism develop CTEPH. Increasing insights into the pathophysiology of CTEPH indicate a distinct overlap with pulmonary arterial hypertension (PAH). Pulmonary endarterectomy (PEA), which lowers pulmonary resistance and can lead to normalization of hemodynamics, is the treatment of choice for eligible patients, however, only a proportion of patients fulfill the criteria for surgical intervention. Patients who are not eligible for PEA may benefit from specific medical therapy for pulmonary hypertension, as do patients before and after PEA with significant peripheral involvement of the pulmonary vasculature. Direct evidence from clinical trials in CTEPH is promising, but is to date limited. Further studies are necessary to define criteria for specific medical therapy for CTEPH.     

Chronic Thromboembolic Pulmonary Hypertension Medical Management

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.     

The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study.

The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, many patients develop a severe progressive small vessel pulmonary arteriopathy that is inaccessible to surgical intervention and is associated with poor survival. The purpose of the present study was to evaluate the medium-term efficacy and safety of the dual endothelin receptor antagonist, bosentan, in inoperable CTEPH. Forty-seven patients with inoperable CTEPH (distal disease or persistent pulmonary hypertension following PEA) underwent evaluation after 1 yr of bosentan therapy. Outcomes included assessment of 6-min walk test (6MWT), haemodynamics and World Health Organization functional classification. Monitoring of serious adverse effects and changes in therapy was undertaken. Patients showed sustained improvements in 6MWT (49+/-8 m), functional classification, cardiac index (+0.2+/-0.07 L.min(-1).m(-2)) and total pulmonary resistance (-139+/-42 dyn.s.cm(-5)). Those patients with persisting pulmonary hypertension following PEA showed the greatest improvement. One-yr survival was 96%, and bosentan was well tolerated with only one patient developing deranged liver function. Although all patients with chronic thromboembolic pulmonary hypertension should be considered for pulmonary endarterectomy, bosentan provides an alternative medical therapy to improve function and delay the progression of this devastating disease in those in whom surgery is not suitable.     

Pulmonary microvascular disease in chronic thromboembolic pulmonary hypertension

Distal, small-vessel vasculopathy is generally considered a major contributor to the progression of pulmonary hypertension (PH) as chronic thromboembolic pulmonary hypertension (CTEPH) develops over time and is a major determinant of postoperative outcome after pulmonary endarterectomy (PEA). The pathogenesis and natural history of microvascular disease in CTEPH remain uncharacterized. Mechanisms for significant distal disease may involve the following processes: (1) predominant obstructions of "small" subsegmental elastic pulmonary arteries, (2) classical pulmonary arteriopathy of small muscular arteries and arterioles distal to nonobstructed vessels, (3) pulmonary arteriopathy of small muscular arteries and arterioles distal to totally or partially obstructed vessels. Patients in whom obstructed vessels are mainly subsegmental are considered poor surgical candidates. Distal pulmonary vasculopathy in both the occluded and nonoccluded pulmonary vascular bed is characterized by lesions considered typical for idiopathic pulmonary arterial hypertension, including plexiform lesions. The pathogenesis and time course of these vascular lesions remain unclear, but may involve endothelial and/or platelet production and release of mediators and/or altered pulmonary blood flow. The reciprocal contribution of large-vessel (operable) and small-vessel lesions in CTEPH is crucial for the indication and results of PEA. A combination of investigations is used to identify the extent of small-vessel disease, including right-heart catheterization, perfusion lung scan, multidetector spiral computed tomography, pulmonary angiography, and pulmonary arterial occlusion wave-form analysis. Preliminary evidence suggests that medical therapy may provide hemodynamic and clinical benefits for patients in whom PEA cannot be applied, in those who have persistent postoperative PH, or in selected patients with advanced preoperative hemodynamic changes.     

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.     

Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension: How can patients be better selected?

Chronic thromboembolic pulmonary hypertension (CTEPH) comprises organizing thrombotic obstructions in the pulmonary arteries by nonresolving thromboemboli, formation of fibrosis and remodeling of pulmonary blood vessels. Surgical pulmonary endarterectomy (PEA) is the therapy of choice for patients with surgically accessible CTEPH, which leads to a profound improvement in hemodynamics, functional class and survival. Select- ing the candidates that will benefit from surgery is still a challenging task. Criteria for surgical suitability have been described but the decision-making for or against surgical intervention remains still subjective. The optimal characterization of the reciprocal contribution of large vessel and small vessel disease in the elevation of pulmonary vascular resistance is crucial for the indication and outcome of PEA. Recently, Toshner et al intended to validate the partition resistance into small and large vessels compartments (upstream resistance:Rup) by the occlusion technique in the preoperative assessment of PEA. We discuss the advantages and disadvantages of Rup and compare it with other hemodynamic predictor to evaluate operative risk in CTEPH patients.     

Medical therapies for chronic thromboembolic pulmonary hypertension: an evolving treatment paradigm

Pulmonary endarterectomy (PEA) is recommended as the treatment of choice for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, only a proportion of patients fulfill the criteria for surgical intervention. In addition, operated patients with CTEPH may experience a gradual hemodynamic and symptomatic decline related to a secondary hypertensive arteriopathy in the small precapillary pulmonary vessels. It has also been questioned what can be done to reduce risks from PEA surgery to improve outcome in "high risk" patients with CTEPH with substantial impairment of pulmonary hemodynamics before surgery. Such patients may benefit from preoperative reduction of pulmonary vascular resistance by means of medical therapy. Conventional medical treatments, such as anticoagulation, diuretics, digitalis, and chronic oxygen therapy, show low efficacy in the treatment of CTEPH as they do not affect underlying disease processes. Over the last decade, several novel therapies have been developed for pulmonary arterial hypertension (PAH), including prostacyclin analogs (epoprostenol, beraprost, iloprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase-5 inhibitors (sildenafil). Evidence of efficacy in PAH, coupled with studies showing histopathologic similarities between CTEPH and PAH, provides a rationale to extend the use of some of these medications to the treatment of CTEPH. However, direct evidence from clinical trials in CTEPH is limited to date. This article reviews evidence supporting, and issues surrounding, the possible use of novel PAH medications in CTEPH.     

Fibrin structure in organized thrombotic material removed during pulmonary artery endarterectormy: the effect of vessel calibre

Pulmonary endarterectomy (PEA) is a curative therapeutic approach in patients with chronic thromboembolic pulmonary hypertension (CTEPH). The location-dependent structural differences of thrombotic material found in pulmonary arteries in CTEPH are poorly investigated. We present the case of a 47-year-old woman with antiphospholipid syndrome, diabetes mellitus and abnormal fibrin phenotype, who underwent PEA for CTEPH. Intravascular material removed bilaterally during PEA (from lobar, segmental and sub-segmental arteries) has been studied using light and scanning electron microscopy (SEM). Light microscopy showed tighter fibrous network in the portions of intraluminal thrombotic material facing the vessel wall, which contained collagen and fibrin fibers, and abundant cells. Cells, evaluated by immunostaining, were present in the whole removed material. Tissue factor expression was also observed with the highest values in the portions of intravascular material facing the vessel wall. In the main pulmonary arteries, SEM images revealed thick fibers of fibrous proteins loosly meshed and few erythrocytes and platelets between them (both dysmorphic “wedged” and fresh cells were present). In the fibrotic layers, containing mainly collagen and fibrin, removed from the lobar/segmental pulmonary arteries we found a stepwise increase in fiber density with decreasing vessel calibre, followed by denser fibrous networks composed of thinner fibers. Elastic fibers in the lobar and segmental arteries were aligned along the blood flow vector. These findings demonstrate differences in the structure of endarterectomized PEA material dependent on the vessel calibre and might contribute to understanding of CTEPH pathophysiology.     

Implementation of a new programme for the surgical treatment of CTEPH in the Czech Republic--Pulmonary endarterectomy

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) in indicated cases can be successfully cured by endartectomy of pulmonary arteries (PEA). Symptomatic nontreated CTEPH has a very poor prognosis; the five-year survival rate in patients with a medium pressure of over 50 mmHg in the main pulmonary artery is as low as 10 %. This kind of operation was previously not available in the Czech Republic. In 2004, a PEA programme was launched at the Cardiocentre of the General Teaching Hospital in Prague in co-operation with the institution of a well known specialist in this field (Prof. Mayer, Johannes Gutenberg University in Mainz, Germany). PATIENTS: Between September 2004 and January 2006, 21 patients (14 males and 7 females; average age 48 years) with CTEPH were operated on, after a complex investigation. The mean pressure in the main pulmonary artery in these patients was 54.8 mmHg; 7 patients suffered from coagulopathy. METHOD: The new surgical technique, modifications of which are used at most facilities, was developed by Jamieson and Daily at the University of California in San Diego: an arrest of circulation in deep hypothermia to protect the brain is vital for the visualisation of distal branches of the pulmonary artery. RESULTS: 21 patients were operated on with a mortality of 4.76 % (1 patient died). Other surgeries performed were suture of a defect of the atrial septum (three times), aortocoronary bypass (three times), and cryoablation of the right atrium for flutter (once). The average circulatory arrest time was 42 minutes, the average total pumping time was 331 minutes, and the average total duration of an operation was 450 minutes; the average duration of mechanical ventilation was 58 hours. Within one month there was a considerable improvement or normalisation of haemodynamic parameters and an increase in the average walking distance on the six-minute walking test by 132 metres. CONCLUSIONS: PEA is a curative method for patients with CTEPH with a surgically accessible obstruction of the pulmonary artery. Centralisation of the care of these patients is a rational necessity, as this enables the centre to gain a maximum of experience with this complicated diagnosis and treatment. Multidisciplinary co-operation is a sine qua non for success in these programmes.     

Assessment of operability in chronic thromboembolic pulmonary hypertension

Pulmonary endarterectomy surgery (PEA) offers the possibility of a cure for patients suffering from chronic thromboembolic pulmonary hypertension (CTEPH). Despite growing experience worldwide, the approach and criteria for patient selection remain variable and center- or expert-dependent. A significant proportion of patients with CTEPH may be turned down for PEA for a number of reasons, most frequently over concerns of distal or inaccessible disease. Although traditional preoperative testing and currently available tools are adequate in identifying the presence of proximal disease in CTEPH, they provide only limited information on the status of the microvasculature. Because persistent pulmonary hypertension is the most important determinant of post-PEA outcome, the preoperative identification of patients with CTEPH with concomitant small-vessel disease and/or microvascular disease is crucial. Pulmonary vascular resistance (PVR) is a useful parameter for assessing potential concomitant small-vessel disease. By assessing the relative contribution of small vessels to the PVR, the pulmonary artery occlusion technique represents a promising tool for determining the risk of surgery in patients with high PVR. More information is required regarding the potential value or risk of preoperative medical therapies. Although traditional surgical classification of CTEPH takes place intraoperatively, there is a need for a preoperative classification system and consensus on operability. A preliminary preoperative classification system has been proposed.     

Hemodynamic and clinical correlates of endothelin-1 in chronic thromboembolic pulmonary hypertension.

BACKGROUND: In non-thromboembolic pulmonary hypertension, endothelin (ET)-1 levels are increased and correlate with the hemodynamic severity of the disease. Whether such correlations exist in chronic thromboembolic pulmonary hypertension (CTEPH) is unknown, nor whether ET-1 levels correlate with hemodynamic outcome after pulmonary endarterectomy (PEA). METHODS AND RESULTS: ET-1 levels were determined by ELISA. ET-levels were increased in 35 CTEPH patients (1.62+/-0.21 pg/ml) compared with healthy controls (n=11: 0.75+/-0.06 pg/ml, p<0.02). ET-1 levels correlated (all p<0.0001) with mean pulmonary artery pressure (mPAP) (r=0.70), cardiac index (r=-0.76), total pulmonary resistance (r=0.72), mixed venous oxygen saturation (r=-0.87), and the distance walked in the 6-min walk test (r=-0.59; p<0.005; n=23). Three months after PEA, ET-1 levels had decreased (p<0.002), and were similar between patients with and without residual pulmonary hypertension (p=0.4). Preoperative ET-1 levels, however, were higher in patients with bad postoperative outcome; that is, patients who either died because of persistent pulmonary hypertension or had residual pulmonary hypertension after PEA (2.68+/-0.48 pg/ml, and 1.13+/-0.15 pg/ml, respectively; p<0.002). The levels also correlated with hemodynamic outcome after PEA (mPAP: r=0.67, p<0.0001). By receiver-operator characteristic curve analysis, ET-1>1.77 pg/ml detected a bad postoperative outcome with a sensitivity and specificity of 79% and 85%, respectively, and a likelihood ratio of 5.2. CONCLUSION: ET-1 levels in CTEPH closely correlated with the hemodynamic and clinical severity of disease in a large cohort of patients. Preoperative ET-1 levels may be useful for better identification of patients at risk for persistent pulmonary hypertension after PEA.     

Riociguat: A Novel Therapeutic Option for Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are serious and often fatal diseases. The pathophysiology of both diseases is complex and in part is attributed to alterations in the nitric oxide (NO)-soluble guanylate cyclase (sGC)-cyclic guanosine monophosphate pathway. Riociguat, a novel sGC stimulator, acts on this pathway through a dual mechanism of action by directly stimulating sGC in a NO-independent manner and also increasing the sensitivity of sGC to NO. Based on benefits from clinical trials for both PAH and CTEPH, riociguat was approved by Health Canada and the US Food and Drug Administration as the first medical therapy for patients with CTEPH who are deemed inoperable or have residual/recurrent PH after pulmonary endarterectomy (PEA), and as a novel treatment option for patients with PAH. This article reviews the key findings from the phase III trials for riociguat that led to these approvals and the implications this has for the treatment of patients with PAH and CTEPH.     

Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease that results from obstruction of the major pulmonary arteries by incompletely resolved or organized pulmonary emboli that have become incorporated into the pulmonary artery wall, eventually causing an increase in pulmonary vascular resistance. From 0.1 to 4.0% of patients recovering from acute pulmonary embolism develop CTEPH. Without intervention, CTEPH is a progressive and lethal disease for which there is no effective medical therapy. Pulmonary endarterectomy (PEA) is the treatment of choice. Careful pre- and postoperative management is essential for a successful outcome after PEA. Lung transplantation is indicated only in few cases when PEA is not feasible. In 1994, we started a program (in Pavia, Italy) in which members of a multidisciplinary team work closely with the aim of increasing experience in the challenging problems these patients present in the evaluative, surgical, and postoperative phases of their care. To date, 134 PEAs have been performed. Preoperatively, New York Heart Association (NYHA) class distribution was three class II, 56 class III, and 75 class IV patients, respectively; mean pulmonary artery pressure and pulmonary vascular resistance values were 47 +/- 13 mm Hg and 1149 +/- 535 dyn/s/cm (-5), respectively. The overall operative mortality has been 9.7% (4.5% in 2004). Survival at 3-month, 1-year, and 3-year follow-up was 89.5 +/- 2.6%, 87.8 +/- 2.9%, and 83.3 +/- 3.5%, respectively; this last rate was unchanged up to 10 years. After PEA, mean pulmonary artery pressure and pulmonary vascular resistance values were 25 +/- 9 mm Hg and 322 +/- 229 dyn/s/cm (-5), respectively, and these results were stable over time. At the 3-year follow-up, 94% of patients were in NYHA class I or II and were being treated with oral anticoagulants only.     

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??Abstract??The pulmonary thromboembolism is divided into acute pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH).Antithrombotic treatment of acute pulmonary embolism is divided into the initial treatment and long-term treatment.Initial treatment includes parenteral anticoagulation??systemic thrombolysis??catheter thrombectomy??surgical thrombectomy and vena cava filter implantation.Long-term treatment needs comprehensive consideration in patients with predisposing factors for VTE and bleeding risk factors.It is divided into (1)treatment of a shorter period??(2)treatment with anticoagulation for 3 months??(3)treatment of a longer time-limited period (eg??6 or 12 months)??(4)extended therapy.In selected patients with CTEPH??such as those with central disease under the care of an experienced thromboendarterectomy team??we suggest pulmonary thromboendarterectomy (PEA).In those patients not suitable for the PEA surgery??it is recommended that the use of the pulmonary targeting antihypertensive drugs and long-term anticoagulation.     

Long-term bosentan in chronic thromboembolic pulmonary hypertension

BACKGROUND: There is no approved pharmacological treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not suitable for pulmonary endarterectomy (PEA). OBJECTIVE: The study investigates the effect of the dual endothelin receptor antagonist bosentan on exercise tolerance (6-min walking distance, 6MWD) and right ventricular function (Tei index) in patients with CTEPH over 24 months. METHODS: Twelve consecutive patients (5 males and 7 females) with CTEPH not eligible for PEA or following partial or complete failure of PEA were included in a non-randomized, open-label prospective study. All patients were WHO class III. They were included, if progressive pulmonary hypertension was diagnosed despite best supportive treatment. Bosentan was started at 62.5 mg b.i.d. and increased to the final dose of 125 mg b.i.d. RESULTS: 6MWD and the Tei index were assessed every 6 months. We observed a significant increase in 6MWD from 319 +/- 85.0 m at baseline to 391 +/- 76.9 m at 6 months and a significant decrease in the Tei index from 0.39 +/- 0.10 at baseline to 0.34 +/- 0.08 at 6 months. This improvement was maintained over 24 months (6 MWD: 381 +/- 101 m; Tei index: 0.31 +/- 0.03). Six patients exhibited an improvement in WHO class at 6, 12 and 18 months, 5 demonstrated improvement at the 24-month follow-up. The remainder were stable throughout the study period. CONCLUSION: This is the first study demonstrating a long-term beneficial effect of bosentan on exercise tolerance (6MWD) and right heart function (Tei index) in CTEPH.     

Balloon pulmonary angioplasty vs. pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis

Heart Failure Reviews - Although balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) are effective in chronic thromboembolic pulmonary hypertension (CTEPH), the comparison of...     

A novel echocardiographic predictor of in-hospital mortality and mid-term haemodynamic improvement after pulmonary endarterectomy for chronic thrombo-embolic pulmonary hypertension.

AIMS: To study whether pre-operative assessment, using echocardiography, of the timing of a particular feature in the pulmonary flow (pulmonary flow systolic notch) may predict in-hospital mortality and mid-term haemodynamic improvement after pulmonary endarterectomy (PEA) for chronic thrombo-embolic pulmonary hypertension (CTEPH). METHODS AND RESULTS: Fifty-eight of 61 consecutive CTEPH patients (aged 53 +/- 14 years; 36 women) who underwent PEA between June 2002 and June 2005 were studied. Clinical, haemodynamic, and echocardiographic variables were assessed pre-operatively and at 3 months post-PEA. Timing of the notch was expressed as notch ratio (NR). Pre-operatively, seven patients had no notch, 33 had NR < 1.0, and 18 had NR > 1.0. NR was associated with in-hospital mortality (P < 0.01). Moreover, multivariable analysis revealed that among pre-operative variables, NR was an independent predictor of residual-increased pulmonary artery systolic pressure (>40 mmHg) at 3 months post-PEA (P = 0.01). Receiver operator characteristic analysis established NR = 1.0 as optimal cutoff to distinguish patients at risk of such unfavourable outcomes, with NR > 1.0 conferring higher risk. CONCLUSION: NR is related with in-hospital mortality and residual pulmonary hypertension after PEA. NR > 1.0 is associated with a higher risk of such unfavourable outcomes. NR may be considered a determinant of eligibility for PEA.     

Rechtsherzversagen bei chronischer pulmonaler Hypertonie und akuter Lungenembolie

Right-sided heart failure is a severe and often life-threatening complication of chronic pulmonary hypertension. The detection of trigger factors that induce right heart failure in previously stable patients is important to initiate a causal therapeutic strategy. Pulmonary embolism (PE) is a frequent cause of acute right heart failure and therapeutic strategies for PE are well documented in the current guidelines. Treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is surgical pulmonary endarterectomy (PEA) and patients with possible CTEPH should be referred to an experienced PEA surgeon without delay. Intensive care management for overt right heart failure is complex and includes the use of pulmonary vasodilators, individual adjustment of diuretic or volume therapy, augmentation of myocardial contractility and left ventricular afterload. Therapeutic regimens aim at optimized filling of the right ventricle, improvement of myocardial perfusion by avoiding tachycardia, elevating systemic pressure and reducing right ventricular afterload. Early communication with a specialized center for pulmonary hypertension is recommended.     

Histopathological multiple recanalized lesion is critical element of outcome after pulmonary thromboendarterectomy

Pulmonary thromboendarterectomy (PEA) is a curative therapy for chronic thromboembolic pulmonary hypertension (CTEPH), but the postoperative mortality remains unsatisfactory (4-10%). Residual pulmonary hypertension is the most common cause of perioperative death. Although PEA specimens seem to contain lesions responsible for hemodynamic improvement, relevant histopathological findings have still to be identified.The aim of this study was to identify histopathological findings that predict postoperative residual pulmonary hypertension after PEA.PEA specimens obtained from 51 consecutive patients with CTEPH were histopathologically assessed. The patient characteristics and disease location were reviewed by medical records. The associations with residual pulmonary hypertension were analyzed.The mean values of preoperative and postoperative vascular resistance (PVR) were 1142 ± 454 and 496 ± 368 dynes?sec/cm(-5), respectively. Twenty of 51 patients (39%), including 2 patients who died, continued to have residual pulmonary hypertension (PVR ≥ 500 dynes?sec/cm(-5)). Statistical tests indicated that male, proximal disease type and the presence of histopathological multiple recanalized thrombus were associated with good surgical outcome (PVR < 500 dynes?sec/cm(-5)). The positive and negative predictive values for surgical outcome estimated by the presence of multiple recanalized lesions were higher than the values estimated by proximal disease type (85% and 88% versus 73% and 71%, respectively). Moreover, the number of multiple recanalize lesions was significantly correlated to the reduction in PVR (P = 0.03).The presence of histopathological multiple recanalized lesions was significantly associated with a decrease in PVR after PEA. Histopathological study may be a potent diagnostic strategy for accurately predicting surgical outcome in the early perioperative period.     

Balloon Pulmonary Angioplasty in Patients With Thromboembolic Pulmonary Hypertension

Purpose of review

Chronic thromboembolic pulmonary hypertension (CTEPH) is the only potentially curable form of precapillary pulmonary hypertension. Although pulmonary endarterectomy (PEA) is the preferred management strategy, a significant number of CTEPH patients will have an inoperable disease. As drug therapy is not expected to offer relief from the mechanical component of the disease, the novel technique of balloon pulmonary angioplasty (BPA) has provided a new therapeutic option for patients with inoperable CTEPH. This review will discuss the contemporary use of BPA technique in inoperable CTEPH patients highlighting the effectiveness and safety of this therapeutic option.

Recent findings

Data supporting the role of BPA in inoperable CTEPH are limited to observational studies. However, these observational studies report consistent findings that BPA results in marked improvements in pulmonary hemodynamics and exercise capacity indicating its efficacy and safety as a treatment strategy in inoperable CTEPH patients.

Summary

Summarizing, BPA is an emerging treatment option providing marked improvements in parameters affecting the outcome of CTEPH patients, but multicenter studies are needed to confirm the safety and the long-term efficacy of the procedure, before BPA can be recommended as an established treatment for CTEPH.