Chronic Thromboembolic Pulmonary Hypertension Patients Research Articles

Chronic thromboembolic pulmonary hypertension in cancer patients with pulmonary embolism

Introduction: According to European guidelines, diagnostic evaluation for chronic thromboembolic pulmonary hypertension (CTEPH) is recommended in patients with persistent dyspnea after pulmonary embolism (PE), because of its severity and the existence of effective therapies. Cancer is associated with CTEPH in patients with pulmonary hypertension. CTEPH incidence after PE is not well known (between 0.1-9.1%) in the general setting, but unknown in cancer patients with pulmonary embolism.

Balloon pulmonary angioplasty relieves haemodynamic stress towards untreated-side pulmonary vasculature and improves its resistance in patients with chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by organised thrombotic obliteration of major vessels and small-vessel arteriopathy in the non-thrombosed vessels. The aim of this study was to investigate the impact of balloon pulmonary angioplasty (BPA) on the non-BPA-side pulmonary vasculature in patients with CTEPH. This study explored the outcomes of 20 unilateral BPA sessions in 13 CTEPH patients. We measured the pulmonary vascular resistance (PVR), pulmonary artery (PA) flow in the BPA-side and non-BPA-side lungs, respectively, using phase contrast MRI and cardiac catheterisation. The interval from BPA to the follow-up evaluation was 92.8±52.0 days. A single session of BPA decreased mean PA pressure from 37.4±6.2 to 30.9±6.5 mmHg (p<0.001). In the BPA side, BPA increased the PA flow from 1.58±0.65 to 1.95±0.62 L/min (p=0.001) and decreased the PVR from 27.3±27.4 to 14.4±9.0 Wood units (p=0.004). In contrast, it decreased both the non-BPA-side PA flow from 2.25±0.64 to 1.90±0.23 L/min (p=0.008) and the non-BPA-side PVR from 14.8±6.6 to 12.8±3.9 Wood units (p=0.01). BPA could relieve haemodynamic stress towards the non-BPA-side vasculature and decrease its PVR in patients with CTEPH, suggesting that it can suppress or regress the progression of the small-vessel arteriopathy in non-BPA-side vasculature, presumably due to haemodynamic unloading.

Metabolomics Analysis in Chronic Thromboembolic Pulmonary Hypertension

The pathobiology of chronic thromboembolic pulmonary hypertension (CTEPH) is poorly understood. Metabolic dysregulation is prominent in idiopathic pulmonary arterial hypertension (IPAH). Using an “omics” approach, we sought to determine the metabolic fingerprint of CTEPH patients compared to IPAH and healthy controls.

Double Lungtransplantation In Patients with End-Stage Pulmonary Arterial Hypertension or End-Stage Inoperable Chronic Thromboembolic Pulmonary Hypertension

In the last two decades survival is improved in patients with pulmonary arterial hypertension (PAH) due to targeted therapy but the long-term outcomes remain uncertain. Favorable results are obtained in patients with chronic thromboembolic pulmonary hypertension (CTEPH) due to surgical and percutaneous treatments except for inoperable patients. Lung transplantation (lung-tx) is an important option for patients with inadequate clinical response despite maximal medical or interventional therapy. The aim of the study is to compare survival of PAH and CTEPH patients listed for double lung-tx who underwent transplantation and patients listed who didn't.

Angioplastia pulmonar con balón en la hipertensión pulmonar tromboembólica crónica no operable. Estudio observacional en una unidad de referencia

Introduction and objectivesBalloon pulmonary angioplasty (BPA) for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) is becoming widely accepted. Procedural refinement has reduced complications. Our primary objective was to analyze the results and complications of the first national BPA program. MethodsObservational, prospective series that included all consecutive BPA procedures in inoperable CTEPH patients between May 2013 and February 2017 performed at a single institution. We analyzed clinical and hemodynamic improvement, reperfusion pulmonary edema, and mortality. ResultsWe performed 156 BPA sessions in 46 patients. Pulmonary vascular resistance was reduced by 44% (10.1 ± 4.9 vs 5.6 ± 2.2 WU; P < .001) and mean pulmonary arterial pressure by 23.6% (49.5 ± 12 vs 37.8 ± 9mmHg; P < .001); cardiac index rose by 17.1% (2.3 vs 2.7 L/min/m2; P = .002), N-terminal pro-B-type natriuretic peptide levels were reduced by 79.2% (1233 ± 1327 vs 255.5 ± 318 pg/dL; P < .001) and the 6-minute walk test distance improved by 74 meters (394 vs 468 m; P = .001). Reperfusion pulmonary edema developed after 9 interventions (5.8%) and 1 patient died (mortality 2.1%). ConclusionsDue to its current refinement, BPA has become a safe and effective treatment for inoperable CTEPH that improves hemodynamics, functional status, and biomarkers with a low rate of severe periprocedural complications and mortality.Full English text available from: www.revespcardiol.org/en

Multicentre observational screening survey for the detection of CTEPH following pulmonary embolism.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe complication of pulmonary embolism. Its incidence following pulmonary embolism is debated. Active screening for CTEPH in patients with acute pulmonary embolism is yet to be recommended.This prospective, multicentre, observational study (Multicentre Observational Screening Survey for the Detection of Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Following Pulmonary Embolism (INPUT on PE); ISRCTN61417303) included patients with acute pulmonary embolism from 11 centres in Switzerland from March 2009 to November 2016. Screening for possible CTEPH was performed at 6, 12 and 24 months using a stepwise algorithm that included a dyspnoea phone-based survey, transthoracic echocardiography, right heart catheterisation and radiological confirmation of CTEPH.Out of 1699 patients with pulmonary embolism, 508 patients were assessed for CTEPH screening over 2 years. CTEPH incidence following pulmonary embolism was 3.7 per 1000 patient-years, with a 2-year cumulative incidence of 0.79%. The Swiss pulmonary hypertension registry consulted in December 2016 did not report additional CTEPH cases in these patients. The survey yielded 100% sensitivity and 81.6% specificity. The second step echocardiography in newly dyspnoeic patients showed a negative predictive value of 100%.CTEPH is a rare but treatable disease. A simple and sensitive way for CTEPH screening in patients with acute pulmonary embolism is recommended.

The Use of Biophysical Flow Models in the Surgical Management of Patients Affected by Chronic Thromboembolic Pulmonary Hypertension.

Introduction: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) results from progressive thrombotic occlusion of the pulmonary arteries. It is treated by surgical removal of the occlusion, with success rates depending on the degree of microvascular remodeling. Surgical eligibility is influenced by the contributions of both the thrombus occlusion and microvasculature remodeling to the overall vascular resistance. Assessing this is challenging due to the high inter-individual variability in arterial morphology and physiology. We investigated the potential of patient-specific computational flow modeling to quantify pressure gradients in the pulmonary arteries of CTEPH patients to assist the decision-making process for surgical eligibility.Methods: Detailed segmentations of the pulmonary arteries were created from postoperative chest Computed Tomography scans of three CTEPH patients. A focal stenosis was included in the original geometry to compare the pre- and post-surgical hemodynamics. Three-dimensional flow simulations were performed on each morphology to quantify velocity-dependent pressure changes using a finite element solver coupled to terminal 2-element Windkessel models. In addition to transient flow simulations, a parametric modeling approach based on constant flow simulations is also proposed as faster technique to estimate relative pressure drops through the proximal pulmonary vasculature.Results: An asymmetrical flow split between left and right pulmonary arteries was observed in the stenosed models. Removing the proximal obstruction resulted in a reduction of the right-left pressure imbalance of up to 18%. Changes were also observed in the wall shear stresses and flow topology, where vortices developed in the stenosed model while the non-stenosed retained a helical flow. The predicted pressure gradients from constant flow simulations were consistent with the ones measured in the transient flow simulations.Conclusion: This study provides a proof of concept that patient-specific computational modeling can be used as a noninvasive tool for assisting surgical decisions in CTEPH based on hemodynamics metrics. Our technique enables determination of the proximal relative pressure, which could subsequently be compared to the total pressure drop to determine the degree of distal and proximal vascular resistance. In the longer term this approach has the potential to form the basis for a more quantitative classification system of CTEPH types.

Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.

Chronic thromboembolic pulmonary hypertension (CTEPH), classified as World Health Organization (WHO) group 4 pulmonary hypertension (PH), is an interesting and rare pulmonary vascular disorder secondary to mechanical obstruction of the pulmonary vasculature from thromboembolism resulting in PH. The pathophysiology is complex, beginning with mechanical obstruction of the pulmonary arteries, which eventually leads to arteriopathic changes and vascular remodeling in the nonoccluded arteries and in the distal segments of the occluded arteries mediated by thrombus nonresolution, abnormal angiogenesis, endothelial dysfunction, and various local growth factors. Based on available data, CTEPH is a rare disease entity occurring in a small proportion (0.5-3%) of patients after acute pulmonary embolism with an annual incidence ranging anywhere between 1 and 7 cases per million population. It is often underdiagnosed or misdiagnosed as idiopathic pulmonary arterial hypertension due to a lack of clinical suspicion or the under-utilization of radionuclide ventilation/perfusion scan. Although the current standard remains planar ventilation/perfusion scintigraphy as the initial imaging study to screen for CTEPH, and invasive pulmonary angiography with right heart catheterization as confirmatory modalities, they are likely to be replaced by modalities that can provide both anatomic and functional data while minimizing radiation exposure. Surgery is the gold standard treatment and offers better improvements in clinical and hemodynamic parameters compared with medical therapy. The management of CTEPH requires a multidisciplinary team, operability assessment, experienced surgical center, and the consideration of medical PH-directed therapies in patients who have inoperable disease, in addition to supportive therapies. Although, balloon pulmonary angioplasty is gaining interest to improve pulmonary hemodynamics and symptoms in CTEPH patients not amenable to surgery, further investigative randomized studies are needed to validate its use. It is very important for the present-day physician to be familiar with the disease entity and its appropriate evaluation to facilitate early diagnosis and appropriate management.

Bosentan therapy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: A systemic review and meta-analysis.

Bosentan therapy has been recommended for pulmonary arterial hypertension (PAH) and might be beneficial for chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to evaluate the specific effects of bosentan for PAH and CTEPH. We performed a systemic review and meta-analysis of randomized controlled trials (RCTs), comparing efficacy and safety of bosentan treatment for PAH and CTEPH through major biomedical database. A total of 10 RCTs including 1185 patients were enrolled. For PAH patients, bosentan prolonged 6-minute walk distance with a weighted mean difference of 35.7m, reduced mean pulmonary arterial pressure by 5.7mm Hg, increased cardiac index by 0.4L/min/m2 , reduced pulmonary vascular resistance by 305.1dyn·s/cm5 , prevented functional class from deterioration and reduced clinical worsening as compared with placebo. For CTEPH patients, bosentan improved cardiac index by 0.3L/min/m2 and reduced pulmonary vascular resistance by 176.0dyn·s/cm5 . Other efficacy outcomes regarding CTEPH did not attain statistical difference. For both PAH and CTEPH, there was no significant difference in mortality or adverse event between bosentan and placebo group. However, bosentan raised the risk of abnormal liver function in both PAH and CTEPH patients. Bosentan is effective in treating PAH, whereas it improves only certain hemodynamic parameters of CTEPH. Incidence of liver function abnormality is higher in bosentan treatment.

Adherence and medication belief in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A nationwide population-based cohort survey.

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare diseases with a gradual decline in physical health. Adherence to treatment is crucial in these very symptomatic and life threatening diseases. To describe PAH and CTEPH patients experience of their self-reported medication adherence, beliefs about medicines and information about treatment. A quantitative, descriptive, national cohort survey that included adult patients from all PAH-centres in Sweden. All patients received questionnaires by mail: The Morisky Medication Adherence Scale (MMAS-8) assesses treatment-related attitudes and behaviour problems, the Beliefs about Medicines Questionnaire-Specific scale (BMQ-S) assesses the patient's perception of drug intake and the QLQ-INFO25 multi-item scale about medical treatment information. The response rate was 74% (n = 325), mean age 66 ± 14 years, 58% were female and 69% were diagnosed with PAH and 31% with CTEPH. Time from diagnosis was 4.7 ± 4.2 years. More than half of the patients (57%) reported a high level of adherence. There was no difference in the patients' beliefs of the necessity of the medications to control their illness when comparing those with high, medium or low adherence. Despite high satisfaction with the information, concerns about potential adverse effects of taking the medication were significantly related to adherence. Treatment adherence is relatively high but still needs improvement. The multi-disciplinary PAH team should, together with the patient, seek strategies to improve adherence and prevent concern.

Balloon Pulmonary Angioplasty in Patients With Thromboembolic Pulmonary Hypertension.

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. 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. 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.

Power of resting echocardiographic measurements to classify pulmonary hypertension patients according to European society of cardiology exercise testing risk stratification cut-offs

BackgroundRight ventricular function is the major determinant of morbidity and mortality in pulmonary arterial hypertension (PAH). The ESC risk assessment strategy for PAH is based on clinical status, exercise testing, NTproBNP, imaging and haemodynamics but does not include right ventricular function. Our aims were to test the power of resting echocardiographic measurements to classify PAH patients according to ESC exercise testing risk stratification cut-offs and to determine if the classification power of echocardiographic parameters varied in chronic thrombo-embolic pulmonary hypertension (CTEPH). MethodsWe prospectively and consecutively recruited 46 PAH patients and 42 CTEPH patients referred for cardio-pulmonary exercise testing and comprehensive transthoracic echocardiography. Exercise testing parameters analyzed were peak oxygen consumption, percentage of predicted maximal oxygen consumption and the slope of ventilation against carbon dioxide production. Receiver operator characteristic curves were used to determine the optimal diagnostic cut-off values of echocardiographic parameters for classifying the patients in intermediate or high risk category according to exercise testing. ResultsMeasurements of right ventricular systolic function were the best for classifying in PAH (area under the curve 0.815 to 0.935). Measurements of right ventricular pressure overload (0.810 to 0.909) were optimal for classifying according to exercise testing in CTEPH. Measurements of left ventricular function were of no use in either group. ConclusionsMeasurements of right ventricular systolic function can classify according to exercise testing risk stratification cut-offs in PAH. However, this is not the case in CTEPH where pressure overload, rather than right ventricular function seems to be linked to exercise performance.

Use of direct oral anticoagulants for chronic thromboembolic pulmonary hypertension

OBJECTIVES:Chronic thromboembolic pulmonary hypertension is one of the most prevalent forms of pulmonary hypertension and is a major complication of acute pulmonary embolism. One mainstay of chronic thromboembolic pulmonary hypertension treatment is lifelong anticoagulation. The recent advent of direct oral anticoagulants for acute pulmonary embolism treatment has provided a viable and effective alternative for treating this condition. However, little is known about the efficacy of this new class of drugs for treating chronic thromboembolic pulmonary hypertension. We aimed to evaluate the safety and efficacy of direct oral anticoagulants in the treatment of chronic thromboembolic pulmonary hypertension.METHODS:A cohort of chronic thromboembolic pulmonary hypertension patients who initiated treatment with direct oral anticoagulants between June 2015 and November 2016 were enrolled in this study.RESULTS:Sixteen patients used rivaroxaban, three used dabigatran and one used apixaban for a mean follow-up of 20.9 months. The mean age was 51 years, and eighteen patients were classified as functional class II/III. Eight patients underwent a pulmonary endarterectomy and exhibited clinical, hemodynamic and functional improvement and currently continue to use direct oral anticoagulants. No episode of venous thromboembolism recurrence was identified during the follow-up period, but there was one episode of major bleeding after a traumatic fall.CONCLUSIONS:Although direct oral anticoagulants appear to be a safe and effective alternative for treating chronic thromboembolic pulmonary hypertension, larger studies are needed to support their routine use.

Impact of Balloon Pulmonary Angioplasty on Hemodynamics and Clinical Outcomes in Patients with Chronic Thromboembolic Pulmonary Hypertension: the Initial Korean Experience.

BackgroundThe treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, not all patients are eligible for PEA, and some patients experience recurrence of pulmonary hypertension even after PEA.MethodsPatients who underwent balloon pulmonary angioplasty (BPA) between December 2015 and April 2017 were enrolled from the Samsung Medical Center CTEPH registry. Enrolled patients underwent right heart catheterization, echocardiography, and 6-minute walk distance (6MWD) at baseline, 4 and 24 weeks after their first BPA session. We compared clinical and hemodynamic parameters at the baseline and last BPA session.ResultsFifty-two BPA sessions were performed in 15 patients, six of whom had a history of PEA. BPA resulted in improvements in World Health Organization (WHO) functional class (2.9 ± 0.8 to 1.7 ± 0.6, P = 0.002), 6MWD (387.0 ± 86.4 to 453.4 ± 64.8 m, P = 0.01), tricuspid annular plane systolic excursion (14.1 ± 3.6 to 15.6 ± 4.3 mm, P = 0.03) and hemodynamics, including a decline in mean pulmonary artery pressure (41.1 ± 13.1 to 32.1 ± 9.5 mmHg, P < 0.001) and in pulmonary vascular resistance (607.4 ± 452.3 to 406.7 ± 265.4 dyne.sec.cm−5, P = 0.01) but not in cardiac index (2.94 ± 0.79 to 2.96 ± 0.93 L/min/m2, P = 0.92). Six cases of complications were recorded, including two cases of reperfusion injury.ConclusionBPA might be a safe and effective treatment strategy for both inoperable CTEPH patients and patients with residual pulmonary hypertension after PEA.

Does pulmonary endarterectomy have arrhythmia prevention effect?

Background: The aim of the present study was therefore to evaluate the evolution of electrocardiography (ECG) markers indicator of morbidity and mortality after pulmonary endarterectomy (PEA). It may be a good predictor of mortality and morbidity in chronic thromboembolic pulmonary hypertension (CTEPH) with patients who underwent PEA. PEA may be reduced risk of arrhythmia in patients with CTEPH. However, this claim must to be supported with long-term results. Materials and Methods: We collected demographic, ECG, and echocardiographic parameters data (baseline and after the operation) in patients undergoing PEA for CTEPH at our institution from 2009 to 2013.We assessed 62 CTEPH patients who underwent PEA. Results: P wave amplitude in DII, PR interval, P and QT dispersion changed significantly at 3 months after surgery. The P dispersion (17.66 ± 6.2, P < 0.04) and QT dispersion (23.75 ± 11.37, P < 0.015) were longer in before operation than in after operation. Conclusions: In our study, we found in ECG analyses of CTEPH with patients who are undergoing PEA that P dispersion, QT dispersion were changed when compared with before operation. For this reason, we think that PEA reduces the risk of atrial fibrillation and malignant arrhythmia.

CARDIOPULMONARY EXCERSISE TESTING IN RISK STRATIFICATION IN PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION AND CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION

In recent years the information about relevance of cardiopulmonary exercise testing (CPET) in complex assessment of pulmonary arterial hypertension (PAH) patients and chronic thromboembolic pulmonary hypertension (CTEPH) patients have been accumulating. Parameters of CPET, such as peak oxygen consumption (Vo2 peak) and ventilation equivalents (VE/Vco2) are very important in risk stratification in patients with PAH, also as distance in 6-minute walking test, the echocardiography and right heart catheterization results, N-terminal prohormone of natriuretic peptide according to the European Society of Cardiology and European Respiratory Society guidelines on diagnosis and treatment of pulmonary hypertension. However, there are no parameters, which can be used in clinical practice to reflect the risk of mortality during 1 year in patients with CTEPH. The aim of our study was to assess the role of CPET in risk stratification, achieving therapy goals and making decision about therapy escalation in patients with PAH and CTEPH. Results of our pilot study demonstrated, that CPET parameters are the reliable markers to reflect the PAH and CTEPH progression. The CPET is necessary for pathogenic therapy efficacy assessment and for making decision of therapy escalation in patients with PAH, also as in CTEPH patients. The critical role of CPET in risk stratification in PAH and CTEPH patients should be emphasized.

The clinical value of assessing right ventricular diastolic function after balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) has a poor prognosis because of the associated progressive right heart failure. Accurate evaluation of right ventricular (RV) function would thus be useful to predict prognosis. However, the significance of RV diastolic function remains unclear. We aimed to identify which echocardiographic measures are most accurate, and potentially useful, in assessing RV diastolic function in patients with CTEPH, and to study the effects of balloon pulmonary angioplasty (BPA) on them. We enrolled 53 CTEPH patients who underwent BPA. Echocardiographic parameters, including two-dimensional speckle-tracking echocardiography, were compared to the hemodynamic parameters measured by right heart catheterization before and after BPA. RV strain rate during early diastole (SR_E), tricuspid e' and right atrial area (RAA) were ameliorated after BPA, concomitant with a decrease in the time constant of the RV pressure curve during diastole (tau), indicating the improvement of RV diastolic function. Among them, SR_E had the strongest correlation with tau (r = - 0.39, p < 0.001). Furthermore, the receiver operating characteristic analyses revealed that E/SR_E (AUC 0.704) and inferior vena cava diameter (AUC 0.726) had a stronger association with higher mean right atrial pressure than RAA (AUC 0.632). In contrast, RAA had a stronger correlation with 6min-walk distances than SR_E (r = - 0.39, p < 0.001 vs. r = 0.30, p = 0.005). Taken together, echocardiographic assessment of RV diastolic function might be associated with hemodynamics as well as exercise tolerance in patients with CTEPH, indicating its benefits in evaluating the therapeutic effects of BPA.

The changing landscape of chronic thromboembolic pulmonary hypertension management.

For patients with chronic thromboembolic pulmonary hypertension (CTEPH), the current standard of care involves surgical removal of fibro-thrombotic obstructions by pulmonary endarterectomy. While this approach has excellent outcomes, significant proportions of patients are not eligible for surgery or suffer from persistent/recurrent pulmonary hypertension after the procedure. The availability of balloon pulmonary angioplasty and the approval of the first medical therapy for use in CTEPH have significantly improved the outlook for patients ineligible for pulmonary endarterectomy. In this comprehensive review, we discuss the latest developments in the rapidly evolving field of CTEPH. These include improvements in imaging modalities and advances in surgical and interventional techniques, which have broadened the range of patients who may benefit from such procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed, particularly the encouraging data from the recent MERIT-1 trial, which demonstrated the beneficial impact of using macitentan to treat patients with inoperable CTEPH, including those on background therapy. As the treatment options for CTEPH improve, hybrid management involving more than one intervention in the same patient may become a viable option in the near future.

N-terminal pro–B-type natriuretic peptide for monitoring after balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

Balloon pulmonary angioplasty (BPA) is an emerging interventional treatment option for chronic thromboembolic pulmonary hypertension (CTEPH). The non-invasive monitoring of CTEPH patients is a clinical challenge. In this study we examined changes in N-terminal pro-B-type natriuretic peptide (NT-proBNP) in patients undergoing BPA for inoperable CTEPH and related them to peri-procedural success. In this study we analyzed a total of 51 consecutive patients who underwent BPA treatment and completed a 6-month follow-up (6-MFU) between March 2014 and March 2017. Serum samples for NT-proBNP measurement were collected before every BPA and at 6-MFU. The 51 patients underwent 265 interventions involving angioplasty of a total of 410 vessels. The 6-month survival rate was 96.1%. The baseline (BL) mean pulmonary artery pressure (PAP) was 39.5 ± 12.1 mm Hg, pulmonary vascular resistance (PVR) was 515.8 ± 219.2 dynes/s/cm5 and the median NT-proBNP level was 820 (153 to 1,871.5) ng/liter. At BL, World Health Organization functional class (FC) was ≥III in 96.1% of the patients, whereas, at 6-MFU, 11.8% were in WHO FC ≥III. At 6-MFU, mean PAP (32.6 ± 12.6 mm Hg; p < 0.001), PVR (396.9 ± 182.6 dynes/s/cm5; p < 0.001) and NT-proBNP (159.3 [84.4 to 464.3] ng/liter; p < 0.001) levels were reduced. The decrease in NT-proBNP levels correlated with the decrease in mean PAP (rrs = 0.43, p = 0.002) and PVR (rrs = 0.50, p = 0.001). A reduction in the NT-proBNP level of 46% indicated a decrease in mean PAP of ≥25% (area under the curve [AUC] = 0.71) and a reduction of 61% indicated a decrease in PVR of ≥35% (AUC 0.77). Our results demonstrate that NT-proBNP levels decrease after BPA, providing valuable evidence of procedural success. NT-proBNP measurement allows identification of patients who are BPA non-responders and may thus be a valuable adjunct in therapy monitoring.

Hipertensión pulmonar tromboembólica crónica: caracterización, endarterectomía pulmonar y nuevas opciones terapéuticas

Chronic thromboembolic pulmonary hypertension is considered the only type of pulmonary hypertension with a potentially curative surgical treatment. Even today it is under-diagnosed or often diagnosed late with a worsening of the prognosis. Chronic thromboembolic pulmonary hypertension presents with nonspecific symptoms and general practitioners may not be aware of the condition or potential for treatment. Current medical treatment is, at best, palliative. Pulmonary endarterectomy offers the only possibility of symptomatic and prognostic improvement, being curative in most cases in the short and long term. Not only the identification of the pathology can be difficult and delayed, moreover after the diagnosis has been established, the estimation of operability can be challenging. The operability is based on the preoperative estimation of postoperative surgical classification and probable pulmonary vascular resistance, which determine the risk of intervention and the probable outcome. This complex procedure that includes, characterization of the pathology, the surgical intervention going through the whole decision process requires a multidisciplinary collaboration of experts in pulmonary hypertension, with a dedicated surgical team, and with very precise protocols. At our center, we have built a team of dedicated specialists including radiologists, cardiologists, cardiac surgeons, anesthesiologists and physiotherapists. Together, not only we have been able to obtain surgical results comparable to higher European centers, but also, to develop and implement other therapeutic options such as pulmonary angioplasty with balloon, dedicated to patients at high risk and discarded for surgery.In the present paper, we present a review of the pathology and, as an example, our single center experience with a multidisciplinary and dedicated management of chronic thromboembolic pulmonary hypertension patients, specifically diagnosis, pulmonary endarterectomy indication, protocols and results.