Search

Pulmonary embolism (PE) is a life-threatening disease, responsible for up to 300 000 deaths annually in the US, ranking among the leading causes of cardiovascular mortality [1]. Systemic thrombolytic therapy is recommended for patients with high-risk PE [2]. Yet, thrombolysis is contraindicated or has failed in numerous patients. Surgical or percutaneous catheter-directed therapies are alternative treatment options. We present a case of massive pulmonary embolism in a patient with high-risk PE, visualized during pulmonary angiography and extracted en bloc by surgical embolectomy.

Pulmonary hypertension (PH) is a serious hemodynamic condition, characterized by increased pulmonary vascular resistance (PVR), leading to right heart failure (HF) and death when not properly treated. The prognosis of PH depends on etiology, hemodynamic and biochemical parameters, as well as on response to specific treatment. Biomarkers appear to be useful noninvasive tools, providing information about the disease severity, treatment response, and prognosis. However, given the complexity of PH, it is impossible for a single biomarker to be adequate for the broad assessment of patients with different types of PH. The search for novel emerging biomarkers is still ongoing, resulting in a few potential biomarkers mirroring numerous pathophysiological courses. In this review, markers related to HF, myocardial remodeling, inflammation, hypoxia and tissue damage, and endothelial and pulmonary smooth muscle cell dysfunction are discussed in terms of diagnosis and prognosis. Extracellular vesicles and other markers with complex backgrounds are also reviewed. In conclusion, although many promising biomarkers have been identified and studied in recent years, there are still insufficient data on the application of multimarker strategies for monitoring and risk stratification in PH patients.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is a promising therapy for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy. AIMS: The present study aimed to evaluate the safety and efficacy of BPA for CTEPH using the first multicentre registry of a single European country. METHODS: Data were obtained from the Database of Pulmonary Hypertension in the Polish Population (NCT03959748), a prospective, multicentre registry of adult and paediatric pulmonary arterial hypertension (PAH) and CTEPH, for a total of 236 patients with confirmed CTEPH (124 women; mean age 67 years) who underwent 1,056 BPA procedures at eight institutions in Poland. RESULTS: In 156 patients who underwent follow-up assessments after a median of 5.9 (IQR: 3.0-8.0) months after final BPA, the mean pulmonary arterial pressure decreased from 45.1±10.7 to 30.2±10.2 mmHg (p

Background Little attention has been paid to chest high resolution computed tomography (HRCT) findings in idiopathic pulmonary arterial hypertension (IPAH) patients so far, while a couple of small studies suggested that presence of centrilobular ground-glass opacifications (GGO) on lung scans could have a significant negative prognostic value. Therefore, the aims of the present study were: to assess frequency and clinical significance of GGO in IPAH, and to verify if it carries an add-on prognostic value in reference to multidimensional risk assessment tool recommended by the 2015 European pulmonary hypertension guidelines. Methods Chest HRCT scans of 110 IPAH patients were retrospectively analysed. Patients were divided into three groups: with panlobular (p)GGO, centrilobular (c)GGO, and normal lung pattern. Association of different GGO patterns with demographic, functional, haemodynamic, and biochemical parameters was tested. Survival analysis was also performed. Results GGO were found in 46% of the IPAH patients: pGGO in 24% and cGGO in 22%. Independent predictors of pGGO were: positive history of haemoptysis, higher number of low-risk factors, and lower cardiac output. Independent predictors of cGGO were: positive history of haemoptysis, younger age, higher right atrial pressure, and higher mixed venous blood oxygen saturation. CGGO had a negative prognostic value for outcome in a 2-year perspective. This effect was not seen in the longer term, probably due to short survival of cGGO patients. Conclusions Lung HRCT carries a significant independent prognostic information in IPAH, and in patients with cGGO present on the scans an early referral to lung transplantation centres should be considered.

Background: A pulmonary embolism response team (PERT) is a multidisciplinary team established to improve clinical care for patients with pulmonary embolism (PE). However, data regarding detailed institutional experience and clinical outcomes from such teams are sparse. Aims: We aim to assess the frequency of activations, patients’ characteristics, PE severity, applied treatments, and outcomes of PE patients treated by Polish PERTs. Methods: The survey registry was conducted between June 2018 and July 2020. All consecutive PERT activations of four institutionalized PERTs in Poland were analyzed. Patients’ characteristics, therapies applied, and in-hospital outcomes were evaluated. Results: There were 680 unique PERT activations. The majority of activations originated from emergency departments (44.9%), and the remaining originated from internal medicine/cardiology units (31.1%), surgery/orthopedics (9.1 %), oncology (6.3%), intensive care units (6.0%), and others (2.5%). The origin of activation varied significantly between institutions (P

Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success.

Background: Balloon pulmonary angioplasty (BPA) is a form of therapy for chronic thromboembolic pulmonary hypertension (CTEPH). The study objective is to assess the clinical usefulness of resting ECG (PH-ECG score) in monitoring the efficacy of BPA in CTEPH patients. Methods and results: Ninety-four (n = 94) CTEPH patients were included in the analysis. A standard 12-lead-ECG was performed before the first BPA session and after completion of treatment. The whole analysed population (n = 94) was divided into the following two groups: derivation cohort (n = 41) and validation cohort (n = 53). The derivation cohort was divided into the following two subgroups: patients with mean pulmonary artery pressure (mPAP) after the completion of therapy &lt, 25 mmHg (n = 21) and patients with mPAP after the completion of therapy ≥ 25 mmHg (n = 20). In the first subgroup, four (R-wave V1 + S-wave V5/V6 &gt, 10.5 mm, QRS-wave axis &gt, 110 degrees, R-wave V1 &gt, S-wave V1, SIQIII pattern) of the six ECG parameters of overload of the right cardiac chambers showed statistically significant differences (p &lt, 0.005). That was followed by a determination of the sensitivity and specificity, positive (PPV) and negative predictive value (NPV), and ROC curve (AUC 0.9, 95% CI: 0.792–1.000) for the variable that was a sum of the above four ECG parameters (PH-ECG score). The absence of all of the four ECG parameters at rest (PH-ECG score = 0) well reflected patients with mPAP &lt, 25 mmHg (sensitivity, 100%, specificity, 80%, PPV, 84%, NPV, 100%). In the validation cohort with mPAP &lt, 25 mmHg and PH-ECG score = 0, sensitivity, specificity, PPV, and NPV were 86%, 77%, 73%, and 89%, respectively. Conclusions: Resting ECG trace is clinically useful in the monitoring of therapeutical effects of BPA in CTEPH patients.

Background: Stroke related to percutaneous coronary interventions (PCIs) is an infrequent complication, which can be potentially life-threatening and can lead to serious disability. Aims: This study aimed to assess the relationship between the type of coronary procedure and incidence of stroke, as well as its predictors. Methods: This retrospective analysis was performed on prospectively collected data gathered in the Polish National Registry of Percutaneous Coronary Interventions (ORPKI) between January 2014 and December 2019 and included 1 177 161 coronary procedures. Among them, 650 674 patients underwent isolated diagnostic coronary angiography (DCA), and 526 487 PCI. Stroke was diagnosed in 157 patients (0.013%), of which 100 (0.015%) happened during DCA and 57 (0.011%) during PCI. Multivariable logistic regression analysis was performed to separate predictors of stroke in patients undergoing coronary angiography and PCI. Results: The percentage of patients with periprocedural stroke was higher in the group treated with isolated DCA during the analyzed time. Among predictors of stroke in patients undergoing DCA, we confirmed prior stroke (P

Background: Balloon pulmonary angioplasty (BPA) is a novel treatment option for inoperable or persistent chronic thromboembolic pulmonary hypertension (CTEPH). Little is known about renal function in CTEPH patients undergoing BPA. Objectives: The aim of this study was to assess the frequency of contrast-induced acute kidney injury (CI-AKI) in patients with CTEPH undergoing BPA and to evaluate the relationship between hemodynamic and renal function. Methods: A total of 41 CTEPH patients were included and 250 consecutive BPA sessions were analyzed for frequency of CI-AKI. The serum creatinine (SC) concentration was measured and the glomerular filtration rate (GFR) was estimated using the Modification of Diet in Renal Disease equation before and 72 h after each BPA procedure. CI-AKI was defined as an increase of 25% or 0.5 mg/dL in SC from the baseline value within 48–72 h of contrast administration. SC and GFR were assessed before and after 3–6 months of completing the BPA treatment and correlated with hemodynamic parameters. Results: The SC concentration and GFR did not change significantly within 72 h after BPA (+1%, p = 0.921, and +4%, p = 0.112, respectively). CI-AKI was noted in 2 cases (0.8%). Significant improvement was noted in GFR (75.4 ± 21.2 vs. 80.9 ± 22.4 mL/min/1.73 m2; p = 0.012) in addition to improvement in right atrial pressure (RAP; 9.1 ± 4.1 to 5.0 ± 2.2 mm Hg; p < 0.001), mean pulmonary artery pressure (49.1 ± 10.7 to 29.8 ± 8.3 mm Hg; p < 0.001), cardiac index (CI; 2.42 ± 0.6 to 2.70 ± 0.6 L/min/m2; p = 0.004), and pulmonary vascular resistance (9.42 ± 3.6 to 4.4 ± 2.3 Wood units; p < 0.001). In a subpopulation of 12 patients with impaired renal function at baseline, the relative increase in GFR was significantly correlated with relative improvement in CI (r = 0.060; p = 0.037), RAP (r = –0.587; p = 0.044), and mixed venous saturation (r = 0.069; p = 0.012). Conclusions: Hemodynamically effective BPA procedures improve renal function in patients with CTEPH with a minimal risk of CI-AKI in the course of treatment.

Background: Percutaneous pulmonary interventions require extensive and accurate navigation planning and guidance, especially in regard to the three-dimensional (3D) relationships between anatomical structures. In this study, we are demonstrating the feasibility of novel visualization techniques: 3D printing (3DP) and augmented reality (AR) in planning transcatheter pulmonary interventions. Methods: Two patients were qualified for balloon pulmonary angioplasty (BPA) for treatment of chronic thromboembolic pulmonary hypertension (CTEPH) and stent implantation for pulmonary artery stenosis, respectively. Computed tomography images of both patients were processed with segmentation algorithms and subsequently submitted to 3D modelling software. Microsoft HoloLens ® AR headsets with dedicated CarnaLife Holo ® software were utilized to display surface and volume rendering of pulmonary vessels as holograms. Results: Personalized life-sized models of the same structures were additionally 3D-printed for preoperative planning. Holograms were shown to physicians throughout the procedure and were used as a guidance and navigation tool. Operative team was able to manipulate the hologram and multiple users of the AR system could share the same image in real time. Clinicians expressed their satisfaction with the quality of imaging and potential clinical benefits. Conclusions: This study reports the potential value of AR in pulmonary interventions, however, prospective trials need to be conducted to decide on whether novel 3D visualization techniques affect perioperative treatment and outcomes.

(1) Background: Prostacyclin analogues (epoprostenol, treprostinil, and iloprost) induce vasodilation in pulmonary arterial hypertension (PAH) but also inhibit platelet function. (2) Objectives: We assessed platelet function in PAH patients treated with prostacyclin analogues and not receiving prostacyclin analogues. (3) Methods: Venous blood was collected from 42 patients treated with prostacyclin analogues (49.5 ± 15.9 years, 81% female) and 38 patients not receiving prostacyclin analogues (55.5 ± 15.6 years, 74% female). Platelet reactivity was analyzed by impedance aggregometry using arachidonic acid (AA; 0.5 mM), adenosine diphosphate (ADP; 6.5 µM), and thrombin receptor-activating peptide (TRAP; 32 µM) as agonists. In a subset of patients, concentrations of extracellular vesicles (EVs) from all platelets (CD61+), activated platelets (CD61+/CD62P+), leukocytes (CD45+), and endothelial cells (CD146+) were analyzed by flow cytometry. Platelet-rich thrombus formation was measured using a whole blood perfusion system. (4) Results: Compared to controls, PAH patients treated with prostacyclin analogues had lower platelet reactivity in response to AA and ADP (p = 0.01 for both), lower concentrations of platelet and leukocyte EVs (p ≤ 0.04), delayed thrombus formation (p ≤ 0.003), and decreased thrombus size (p = 0.008). Epoprostenol did not affect platelet reactivity but decreased the concentrations of platelet and leukocyte EVs (p ≤ 0.04). Treprostinil decreased platelet reactivity in response to AA and ADP (p ≤ 0.02) but had no effect on the concentrations of EVs. All prostacyclin analogues delayed thrombus formation and decreased thrombus size (p ≤ 0.04). (5) Conclusions: PAH patients treated with prostacyclin analogues had impaired platelet reactivity, EV release, and thrombus formation, compared to patients not receiving prostacyclin analogues.

Background:Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients’ management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies.Methods:We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry.Results:We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants ( n = 301; 58.3%) were preferred over vitamin K antagonists ( n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment.Conclusions:The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH.Clinical Trial Registration:clinicaltrials.gov/ct2/show/NCT03959748

Background: The aim of the study was to assess soluble ST2 (sST2) concentration and its dynamic changes in the periprocedural period in patients with chronic thromboembolic pulmonary hypertension (CTEPH) treated with balloon pulmonary angioplasty (BPA). Methods: We prospectively analyzed 57 procedures of BPA performed in 37 patients with CTEPH. Biomarkers, such as N-terminal pro B-type natriuretic peptide (NT-proBNP), troponin T (TnT), and sST2 were assessed at four time points: Before the BPA procedure, 24 h and 48 h after the procedure, and at the discharge from hospital. Each postprocedural period was assessed for complications. Results: Before the BPA procedure, median sST2 concentration was 26.56 ng/mL (IQR: 16.66&ndash, 40.83 ng/mL). sST2 concentration was significantly higher 24 h and 48 h after the BPA compared to the baseline measurements (33.31 ng/mL (IQR: 20.81&ndash, 62.56), p = 0.000 and 27.45 ng/mL (IQR: 17.66&ndash, 54.45), p = 0.028, respectively). sST2 level 24 h after the BPA procedure was significantly higher in the group with complications compared to the group without complications in the postprocedural period (97.66 ng/mL (IQR: 53.07&ndash, 126.18) vs. 26.86 ng/mL (IQR: 19.10&ndash, 40.12), p = 0.000). Conclusions: sST2 concentration in patients with CTEPH treated with BPA changes significantly in the postprocedural period and is significantly higher in the group with complications in postprocedural period.

The coronavirus pandemic disease 2019 (COVID-19) has changed the face of contemporary medicine. However, each and every medical practitioner must be aware of potential early and late complications of COVID-19, its impact on chronic diseases - especially ones as common as atrial fibrillation (AF) - and the possible interactions between patients' chronic medications and pharmacotherapy of COVID-19. Patients with AF due to comorbidities and, often, elderly age are assumed to be at a higher risk of a severe course of COVID-19. This expert consensus summarizes the current knowledge regarding the pharmacotherapy of AF patients in the setting of the COVID-19 pandemic. In general, anticoagulation principles in quarantined or asymptomatic individuals remain unchanged. Nevertheless, it is advisable to switch from vitamin K antagonists to non-vitamin K antagonist oral anticoagulants (NOACs) whenever possible due to their consistent benefits and safety with fixed dosing and no monitoring. Additionally, in AF patients hospitalized due to mild or moderate COVID-19 pneumonia, we recommend continuing NOAC treatment or to switching to low-molecular-weight heparin (LMWH). On the other hand, in severely ill patients hospitalized in intensive care units, intravenous or subcutaneous dosing is preferable to oral, which is why the treatment of choice is either LMWH or unfractionated heparin. Finally, particularly in critical scenarios, the treatment strategy in COVID-19 patients with AF should be individualized based on possible interactions between anticoagulants, antiarrhythmics, antivirals, and antibiotics. In this consensus, we also discuss how to safely perform COVID-19 vaccination in anticoagulated AF patients.

The definition of heart failure with preserved ejection fraction (HFpEF) has evolved from a clinically based “diagnosis of exclusion” to definitions focused on objective evidence of diastolic dysfunction and/or elevated left ventricular filling pressures. Despite advances in our understanding of HFpEF pathophysiology and the development of more sophisticated imaging modalities, the diagnosis of HFpEF remains challenging, especially in the chronic setting, given that symptoms are provoked by exertion and diagnostic evaluation is largely conducted at rest. Invasive hemodynamic study, and in particular — invasive exercise testing, is considered the reference method for HFpEF diagnosis. However, its use is limited as opposed to the high number of patients with suspected HFpEF. Thus, diagnostic criteria for HFpEF should be principally based on non-invasive measurements. As no single non-invasive variable can adequately corroborate or refute the diagnosis, different combinations of clinical, echocardiographic, and/or biochemical parameters have been introduced. Recent years have brought an abundance of HFpEF definitions. Here, we present and compare four of them: 1) the 2016 European Society of Cardiology criteria for HFpEF; 2) the 2016 echocardiographic algorithm for diagnosing diastolic dysfunction; 3) the 2018 evidence-based H(2)FPEF score; and 4) the most recent, 2019 Heart Failure Association HFA-PEFF algorithm. These definitions vary in their approach to diagnosis, as well as sensitivity and specificity. Further studies to validate and compare the diagnostic accuracy of HFpEF definitions are warranted. Nevertheless, it seems that the best HFpEF definition would originate from a randomized clinical trial showing a favorable effect of an intervention on prognosis in HFpEF.

IntroductionNon-invasive tests that may improve clinical evaluation of pulmonary hypertension (PH) are needed. The purpose of this study was to assess the role of soluble ST2 (sST2) in patients with PH.Material and methodsA total of 57 patients with chronic thromboembolic PH and 43 patients with idiopathic arterial PH were enrolled in this study. All patients were evaluated for World Health Organization (WHO) functional class (FC), N-terminal prohormone B-type natriuretic peptide (NT-proBNP), troponin T (TnT), and hemodynamics. Plasma sST2 was assessed by an immu­nofluorescent in vitro diagnostic assay. All patients were followed up from the date of blood sampling. The endpoint was all-cause death.ResultsThe median sST2 concentration was 32.8 ng/ml (IQR: 21.6–48.5 ng/ml) in the whole study population. Significant differences were found between median sST2 in successive WHO FCs (FC II vs. FC III, p = 0.002; FC III vs. FC IV, p = 0.12; FC II vs. FC IV, p = 0.008). Significant correlations were found between sST2 and hemodynamic parameters: mean right atrial pressure (r = 0.56; p ConclusionssST2 seems to be a marker of poor clinical prognosis in patients with PH.

Background The remodeling of the right heart in patients with chronic pulmonary hypertension (cPH) is associated with the appearance of electrocardiographic (ECG) abnormalities. We investigated the resolution of ECG markers of right ventricular hypertrophy (RVH) caused by acute and long‐term hemodynamic improvement. Methods Twenty‐nine (29) patients with chronic thromboembolic pulmonary hypertension (CTEPH) and seven patients with pulmonary arterial hypertension (PAH) were included in the analysis. Patients with CTEPH achieved a significant long‐term hemodynamic improvement following the treatment with balloon pulmonary angioplasty (BPA); all the patients with PAH reported significant acute hemodynamic relief after a single inhalation of iloprost, fulfilling the criteria of responder. Standard 12‐lead ECG was performed before and after intervention. Results The interval between baseline and control ECG in CTEPH and PAH groups was 28 (IQR: 17–36) months and 15 min (IQR: 11–17), respectively. Despite similar hemodynamic improvement in both groups, only the CTEPH group presented significant changes in most analyzed ECG parameters: T‐wave axis (p = .002), QRS‐wave axis (p = .012), P‐wave amplitude (p

Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all Polish PAH centers. The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females &ge, 65 years old. The most frequent type of PAH was idiopathic (n = 444, 46%) followed by PAH associated with congenital heart diseases (CHD-PAH, n = 356, 36.7%), and PAH associated with connective tissue disease (CTD-PAH, n = 132, 13.6%). At enrollment, most incident cases (71.9%) were at intermediate mortality risk and the prevalent cases had most of their risk factors in the intermediate or high risk range. The use of triple combination therapy was rare (4.7%). A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. In contrast, we found: a female predominance across all age groups, a high proportion of patients with CHD-PAH as compared to patients with CTD-PAH and a low use of triple combination therapy.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapy (MT). Assessment in a multidisciplinary team of experts (CTEPH team) is currently recommended for treatment decision making. The aim of the present study was to report the effects of such an interdisciplinary concept. Methods and results: A total of 160 patients were consulted by the CTEPH team between December 2015 and September 2018. Patient baseline characteristics, CTEPH team decisions and implementation rates of diagnostic and therapeutic procedures were analysed. Change in World Health Organization (WHO) functional class and survival rates were evaluated by treatment strategy. A total of 51 (32%) patients were assessed as operable and 109 (68%) were deemed inoperable. Thirty-one (61% of operable patients) underwent PEA. Patients treated with PEA, BPA(+MT) and MT alone were 50.9 ± 14.7, 62.9 ± 15.1 and 68.9 ± 12.7 years old, respectively. At the follow-up, PEA patients had the highest WHO functional class improvement. Patients treated with BPA(+MT) had significantly better survival than PEA ( p = 0.04) and MT patients ( p = 0.04; 2-year survival of 92%, 79% and 79%, respectively). Conclusions: The CTEPH team ensures that necessary diagnostic procedures are performed. A relatively low proportion of patients was assessed by the CTEPH team as operable and underwent surgery, which in survivors resulted in the best functional improvement. Although patients undergoing BPA(+MT) were older than patients treated with PEA, their survival was better than patients subjected to PEA or MT alone. The reviews of this paper are available via the supplemental material section.

Background and aim: To assess the safety and efficacy of a refined balloon pulmonary angioplasty (BPA) strategy in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: There were 157 BPA sessions performed in 56 CTEPH patients (47 non-operable, nine after pulmonary endarterectomy; aged 58.6 ± 17.9 years; 28 females) with severely impaired pulmonary haemodynamics (mean pulmonary artery pressure [mPAP]: 51.3 ± 12.2 mm Hg, pulmonary vascular resistance [PVR]: 10.1 ± 3.9 Wood Units). The first 50 sessions aimed to recanalise chronic occlusions and prevent reocclusion with aggressive anticoagulation. The next 107 sessions aimed to relieve “web” and “ring” lesions using reduced tip load guidewires and less intensive anticoagulation. Results: There was significant reduction in haemoptysis (22% vs. 7%, p = 0.01), vessel injury (30% vs. 13%, p = 0.01), and reperfusion pulmonary injuries (22% vs. 4%, p = 0.01) after changing the BPA strategy. Mortality at 14 days was also reduced (6% vs. 0%; p = 0.05). The cumulative survival rate was 94.6% at 24 months after the first BPA, which was more favourable than medically treated historic controls. In the 31 patients with > 3 BPA sessions, there was significant reduction of PVR (10.3 ± 3.7 vs. 5.9 ± 2.8 Wood Units; p = 0.01), mPAP (50.7 ± 10.8 vs. 35.6 ± 9.3 mm Hg; p = 0.01) and improvement in World Health Organisation functional class (3.19 ± 0.48 vs. 1.97 ± 0.80; p < 0.001). Conclusions: Balloon pulmonary angioplasty improves haemodynamics and outcome but requires refined strategy to limit early complication rate.

Pulmonary Embolism Response Team (PERT) is a multidisciplinary team established to stratify risk and choose optimal treatment in patients with acute pulmonary embolism (PE). Established for the first time at Massachusetts General Hospital in 2013, PERT is based on a concept combining a Rapid Response Team and a Heart Team. The growing role of PERTs in making individual therapeutic decisions is identified, especially in hemodynamically unstable patients with contraindications to thrombolysis or with co-morbidities, as well as in patients with intermediate-high risk in whom a therapeutic decision may be difficult. The purpose of this document is to define the standards of PERT under Polish conditions, based on the experience of teams already operating in Poland, which formed an agreement called the Polish PERT Initiative. The goals of Polish PERT Initiative are: improving the treatment of patients with PE at local, regional and national levels, gathering, assessing and sharing data on the effectiveness of PE treatment (including various types of catheter-directed therapy), education on optimal treatment of PE, creating expert documents and supporting scientific research, as well as cooperation with other communities and scientific societies.

Background: In this study, we aimed to assess the efficacy and safety of balloon pulmonary angioplasty (BPA) in patients with technically inoperable distal-type chronic thromboembolic pulmonary hypertension (d-CTEPH) and technically operable proximal-type disease (p-CTEPH) by analyzing the results of BPA treatment in two collaborating CTEPH referral centers. Methods and results: We assessed hemodynamic results, functional efficacy, complication and survival rate after BPA treatment in 70 CTEPH patients (median age 64 years, (interquartile range (IQR): 52–73 years)), of whom 16 (median age 73 years, (QR 62–82 years)) were in the p-CTEPH subgroup. Altogether, 377 BPA procedures were performed, resulting in significant (p &lt, 0.001) improvement in mean pulmonary artery pressure (mPAP 48.6 ± 10 vs. 31.3 ± 8.6 mmHg), pulmonary vascular resistance (694 ± 296 vs. 333 ± 162 dynes*s*cm−5), six-minute walk test (365 ± 142 vs. 433 ± 120 metres) and N-terminal pro B-type natriuretic peptide (1307 (510–3294) vs. 206 (83–531) pg/mL). The rate of improvement did not differ between the sub-groups. Lung injury episodes and severe hemoptysis were similarly infrequent in d-CTEPH and p-CTEPH (6.4% vs. 5%, p = 0.55 and 1.0% vs. 2.5, p = 0.24, respectively). There was no significant difference between the sub-groups regarding survival (p = 0.53 by log-rank test). Conclusion: BPA may be beneficial in patients with p-CTEPH who cannot undergo pulmonary endarterectomy (PEA). Larger long-term studies are needed to better define the efficacy, safety, and optimal BPA procedural standards in this population.

We present a case of a 67-year-old female with proximal chronic thromboembolic pulmonary hypertension (CTEPH), disqualified from pulmonary endarterectomy due to multiple comorbidities and high risk-to-benefit ratio as assessed by multidisciplinary CTEPH team. She was referred for balloon pulmonary angioplasty (BPA) and underwent three sessions with balloon catheters up to 8 mm diameter. During the second procedure, the elastic recoil phenomenon was observed in the treated post-thrombotic lesion of the right lower lobe artery, which made the balloon angioplasty ineffective. It was decided to implant a self-expanding stent for the prevention of restenosis. The procedure resulted in significant improvement of regional perfusion, as confirmed by control angiography. We feel that it contributed to the significant improvement of hemodynamic parameters and exercise capacity, as assessed three months after the last BPA procedure. In conclusion, pulmonary artery stenting may be an option in proximal CTEPH when elastic recoil phenomenon makes balloon angioplasty of a large vessel ineffective.

Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). However, persistent pulmonary hypertension continues in 5-35% of patients after PEA. Recently, balloon pulmonary angioplasty (BPA) showed promise as a strategy for patients with non-operable CTEPH. Therefore, we investigated the usefulness of BPA for residual pulmonary hypertension after PEA.Fifteen patients with residual pulmonary hypertension after PEA received 71 BPA sessions (4.7 ± 1.4 sessions/patient). The mean time between the PEA and the first BPA session was 28.1 ± 25.8 months. All patients underwent a comprehensive diagnostic work-up, including right heart catheterization, functional and laboratory tests, before, and 6-4 weeks after the BPA sessions.After BPA, the mean pulmonary arterial pressure decreased from 44.7 ± 6.4 to 30.8 ± 7.5 mm Hg (31% decline; p 0.001). Pulmonary vascular resistance decreased from 551.9 ± 185.2 to 343.8 ± 123.8 dyn∗s/cmBPA could be a promising therapeutic strategy for persistent pulmonary hypertension after PEA in patients with CTEPH.

This study evaluated the incremental effect of riociguat on pulmonary hemodynamics in patients with inoperative or persistent chronic thromboembolic pulmonary hypertension (CTEPH) treated previously with sildenafil.The retrospective study included 28 patients diagnosed with CTEPH who were ineligible for surgical treatment due to distal thrombi location or who suffered from persistent CTEPH after pulmonary endarterectomy and who were treated with sildenafil at a dose of 25 mg TID for a minimum of 3 months. Sildenafil was subsequently discontinued, and riociguat therapy was started with gradually increasing doses. Right heart catheterization was performed and WHO functional class (FC) was assessed in each patient at three time points: before starting sildenafil therapy (baseline), before the transition to riociguat, and after 3 to 6 months of therapy with riociguat.Compared to baseline, the use of sildenafil and riociguat significantly decreased pulmonary vascular resistance (PVR) (10.47 ± 3.56 vs. 7.81 ± 3.58 Wood units, p 0.001) and mean pulmonary arterial pressure (PAP) (54.1 ± 11.6 vs. 46.1 ± 13.2 mm Hg; p 0.001) while increasing cardiac output (CO) (4.31 ± 0.88 vs. 4.85 ± 0.87 L/min; p = 0.007). Switching from sildenafil to riociguat reduced PVR by 14% (p = 0.005) and the mean PAP by 6% (p = 0.03) while increasing CO by 11% (p = 0,002). The number of patients with WHO FC III and IV symptoms decreased from 71,4% to 57,1% (p = 0,02) after the change from sildenafil to riociguat.Replacing sildenafil with riociguat in patients with inoperable or persistent CTEPH may improve pulmonary hemodynamics and FC.

Background: Pericardial effusion is associated with high mortality in oncology. The etiology of infectious pericarditis and iatrogenic effects of previous radio-/chemotherapy may be always suspected, especially when a subsequent episode is observed. Patients and Methods: The study included 17 hemodynamically-unstable patients with cancer due to recurrent pericardial bloody effusion after previous pericardiocentesis and analyzed survival determinants after intrapericardial chemotherapy with cisplatin. Results: The mortality rate was not significantly associated with the level of N-terminal pro-B type natriuretic peptide, low hemoglobin (104/μl), large volume (>1500 ml) and long duration (>8 days) of pericardial drainage, cardiac arrhythmias, positive culture test results nor fever occurring during cisplatin administration. Subsequent systemic anticancer therapy was the strongest factor determining a longer survival (hazard ratio(HR)=0.31, 95% confidence interval(CI)=0.11-0.9; p=0.03). Conclusion: Efficacy of rescue intrapericardial chemotherapy with cisplatin is independent of parameters of hemodynamic instability and levels of inflammatory markers in recurrent pericardial effusion.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially life-threatening disease of the pulmonary circulation [1]. The pathogenesis of CTEPH is not entirely clear. The most accepted scenario is that of aborted recanalisation of pulmonary arteries after a thromboembolic episode. While some post-embolic residua may persist in up to 50% of survivors of acute pulmonary embolism (aPE), only 0.5% to 2% will progress to CTEPH [2, 3]. This is believed to occur in the presence of significant redistribution of flow to remaining unoccluded pulmonary bed with resulting elevation of intravascular pressure and shear stress. Remodelling of initially patent pulmonary arterioles leads to an increase in pulmonary vascular resistance similar to that observed in left-to-right shunting in congenital heart disease. Progressive uncoupling of pulmonary and right ventricular elastance results in a fall of pulmonary flow, left ventricular preload, systemic blood pressure, and right ventricular (RV) coronary perfusion leading to right heart failure with severe functional disability and eventually to death. Management of CTEPH requires precise differential diagnosis and qualification for surgical treatment by an experienced multidisciplinary team. Indeed, in operable patients pulmonary endarterectomy (PEA) is highly effective in restoring functional status and improving life expectancy. A surgical technique has been optimised and implemented worldwide by a group from San Diego – University of California [4]. Nevertheless, PEA performed in deep hypothermia and intermittent total cardiac arrest remains one of the most demanding cardiovascular interventions and is performed only in a limited number of highly dedicated centres. As an example, Papworth Hospital is the only centre performing PEA in the UK, while Marie-Lannelongue Hospital in Paris remains a referral centre for France for this type of surgery. Usually, individual cardiac surgeons are responsible for PEA in their centres, as the learning curve for this intervention has been well documented [5]. With growing experience of clinicians, radiologists, surgeons, and anaesthesiologists, an increasing proportion of patients with CTEPH may benefit from PEA despite distal, less accessible intravascular residua and/or advanced age and comorbidities. This is of paramount importance since the outcome of non-operated patients is drastically worse (Figure 1), despite identical baseline haemodynamic characteristicsand significant perioperative mortality of 2–10% in patients submitted to PEA [6]. Nevertheless, even in the leading CTEPH referral centres almost 50% patients remain on medical treatment alone, with grim perspectives regarding life quality and expectancy. Based on a large randomised trial and promising long-term effects on exercise tolerance [7, 8] direct guanylyl cyclase stimulator (riociguat) has been approved for treatment of inoperable CTEPH. Riociguat may protect patent pulmonary arterioles from progressive remodelling [9] but is unlikely to affect the culprit post-embolic residua. Recently, balloon pulmonary angioplasty (BPA) has emerged as a promising new interventional option in non-operable CTEPH. In 2001, Feinstein et al. from Harvard Medical School described a group of 18 CTEPH patients treated with BPA [10]. Of these, 16 were excluded from PEA due to distal lesions, and two due to the presence of comorbidities increasing the risk associated with surgical treatment. In total, 47 procedures were performed, thus dilating or restoring the patency of 107 arteries. In the periprocedural period, 1 patient died of reperfusion pulmonary oedema and right ventricular failure. In total, reperfusion oedema occurred in 23% of cases, and its presence correlated with the value of pulmonary artery pressure (PAP) prior to the procedure. Long-term follow-up (mean, 34 months) showed an increase in physical capacity, manifesting itself as an improvement in NYHA class from a mean value of 3.3 prior to the procedure to a mean value of 1.8 following the procedure. Figure 1 Survival curves for patients with CTEPH treated with PEA (shadow line) and who were treated by pharmacotherapy only (solid line) – reprinted from Wieteska et al. [6] The succeeding years saw the development of the BPA technique mainly in Japanese centres. Japanese researchers have refined the BPA technique by reducing the number of segments treated during one session [11], by using smaller balloons [12], and by wider use of intravascular imaging [13, 14]. The experience of the Japanese centres shows that a series of BPA procedures performed in experienced centres has lead to regression of right ventricular dysfunction [15, 16] and are associated with an annual mortality below 5% – also in elderly patients [17]. However, it should be stressed that the population of Japanese patients with CTEPH differs from that observed in the European-American registry [18]. In a registry comprising 519 Japanese patients with CTEPH, lower median pretreatment mPAP (38 mm Hg vs. 47 mm Hg), more frequent use of PAH-like therapy (52% vs. 38%), and a significantly lower rate of cardiac surgical treatment used (14% vs. 57%) was observed [19]. The only larger group of patients in Europe who underwent BPA is that described by Andreassen et al. [20]. The Norwegian team treated 20 CTEPH patients, including 16 with distal lesions, 3 with proximal lesions who had refused PEA, and 1 patient with persistent pulmonary hypertension following surgical treatment. Prior to treatment, 85% of the patients presented with NYHA class III and IV symptoms. During recruitment to a percutaneous treatment program in the same centre, 50 PEAs were performed. In total, 73 BPA procedures were carried out (mean, 3.7 BPAs/patient; range: 2–9), thus performing angioplasty of 371 vessels – 118 segmental arteries and 253 subsegmental arteries. In the periprocedural period, two patients died, and treatment-requiring reperfusion oedema occurred in 7 cases. During 3-month follow-up, an improvement in the functional class (75% of patients in NYHA class I–II), in VO2max in cardiopulmonary exercise test (13.6 ±5.6 vs. 17.0 ±6.5, p < 0.001), in mean pulmonary artery pressure (45 ±11 mm Hg vs. 33 ±10 mm Hg, p < 0.001), in pulmonary vascular resistance (8.8 ±4.0 Wood Units vs. 5.9 ±3.6 Wood Units, p < 0.001), and in NT-pro-BNP levels (194 ±182 ng/ml vs. 90 ±119 ng/ml, p = 0.007) was achieved. Follow-up angiography revealed no restenosis. In Poland, the first BPA procedure was performed in 2013 [21]. Until now, the experience of our team includes 37 BPA procedures, which consisted of angioplasty of 105 vessels in 20 patients with CTEPH. Seventeen patients were excluded from surgical treatment by an experienced PEA cardiac surgery team, and in 3 patients persistent pulmonary hypertension persisted after PEA. Eighty-two percent of patients received PAH-like therapy – most frequently sildenafil. In the periprocedural period, two patients died of severe reperfusion oedema and severe hypoxaemia unresponsive to oxygen therapy (including mechanical ventilation). Those 2 patients were disqualified from PEA due to the presence of extensive lung cavities related to previous mycobacterial infection and due to significant comorbidities and advanced age, respectively – but not because of distal localisation of thrombi. All patients who underwent BPA because of distal lesion localisation survived. In technical terms, BPA does not significantly differ from balloon angioplasty performed in other vessels (Figure 2). Nevertheless, the complicated anatomy of the pulmonary tree, the necessity to advance the instruments through enlarged right heart chambers, and the fact that pulmonary vessels can be easily damaged with the guide wire or balloon catheter requires specific experience. It is not recommended that BPA procedures be performed by cardiologists or interventional radiologists who have experience in other vascular regions but no experience in interventions within the pulmonary circulation. During one procedure, no more than two segmental arteries or their subsegmental equivalents should be dilated due to the risk of reperfusion oedema. Reperfusion oedema results from redistribution of blood flow to areas supplied by dilated vessels, in which vascular resistance has abruptly decreased. This may cause blood cells to migrate into the alveoli, excluding them from gas exchange. One way to prevent reperfusion oedema is to undersize the balloon catheter being used, on the basis of angiography, or by means of intravascular ultrasound (IVUS) or optical coherence tomography (OCT). Also, pressure distal to a residual lesion and a gradient across the lesion can be measured by means of an fractional flow reserve (FFR) probe [22]. As there is no tendency towards restenosis, it is unnecessary to use stents. The results achieved in the group of patients who have completed a series of BPA procedures are very encouraging. A reduction in mean PAP from baseline 58 ±6 mm Hg to 41 ±9 mm Hg and in PVR from 11.7 ±4.3 Wood units to 6.6 ±2.2 Wood units was achieved in our series. The haemodynamic improvement corresponds with an improvement in exercise tolerance. Prior to BPA procedures, 95% of patients were in NYHA class III and IV, and the rate of patients in class III and IV decreased to 35% after treatment. A significant issue limiting growth in the number of such procedures performed in Poland is that BPA is not reimbursed by the National Health Fund. Figure 2 Balloon pulmonary angioplasty in a 67-year-old patient with persistent form of CTEPH. Left panel (A) presents the angiogram of occluded segmental pulmonary artery of left lower lobe. The BPA results in reperfusion of the vessel – right panel ( ...

BACKGROUND The effect of balloon pulmonary angioplasty (BPA) on improvement in functional and hemodynamic parameters in chronic thromboembolic pulmonary hypertension (CTEPH) is known, but the quality of life (QoL) of patients treated with BPA has never been studied before.Methods and Results:Twenty-five patients with inoperable or persistent CTEPH were enrolled in the study and filled out the 36-item Short Form (SF-36v2) questionnaire twice: prior to commencement of BPA treatment and after ≥3 BPA sessions. In addition WHO functional class, distance on the 6-min walk test (6MWT) and hemodynamic parameters such as right atrial pressure (RAP), mean pulmonary artery pressure (mPAP), cardiac index (CI) and pulmonary vascular resistance (PVR) were assessed. QoL improved significantly in all domains, except for physical pain. Improvement in RAP (10.5±3.4 vs. 6.2±2.2 mmHg; P

The purpose of present study was to determine safety and hemodynamic impact of refined balloon pulmonary angioplasty (BPA) procedure. Study population comprised of patients with established diagnosis of CTEPH, who were disqualified from pulmonary endarterectomy due to distal localization of

A 43-year-old female had been diagnosed as having chronic thromboembolic pulmonary hypertension (CTEPH) two years previously with pulmonary artery (PA) thrombus detected by computed tomography (CT) (Fig. 1) and precapillary pulmonary hypertension (mean PA pressure [mPAP] 56 mm Hg, cardiac ouput [CO] 6.03 L/min, PA wedge pressure 8 mm Hg, pulmonary vascular resistance [PVR] 7.96 Wood units) by right heart catheterisation (RHC). She was referred to the cardiac surgeon and was not qualified for pulmonary endarterectomy. The patient was treated with sildenafil (off-label) for one year with no improvement. She was admitted to our institution in July 2013 presenting symptoms of class III NYHA heart failure. Echocardiography revealed the progression of right ventricular failure (Fig. 2). Selective pulmonary arteriography confirmed distal localisation of thrombi in segmental arteries of left lower lobe (Fig. 3). Balloon pulmonary angioplasty (BPA) of two segmental arteries was performed, resulting in an improvement in control angiography (Fig. 4). The next BPA of another two segmental arteries was performed after three months. There were no complications during or after the procedures. Subsequent RHC confirmed an improvement (mPAP 33 mm Hg , CO 4.88 L/min, PVR 4.50 Wood units). The patient presented clinical recovery and symptoms of class II NYHA. Pulmonary endarterectomy is the only potentially curative treatment for CTEPH. The prognosis of CTEPH has been reported to be poor when mPAP is > 30 mm Hg. BPA seems to be a promising strategy for inoperable CTEPH. Reperfusion pulmonary injury is the major complication after BPA. To reduce the size of the area of this complication, it is recommended not to dilate > two vessels at the initial BPA and to perform it in a staged fashion over several separate procedures.