Balloon pulmonary angioplasty - efficient therapy of chronic thromboembolic pulmonary hypertension in the patient with advanced sarcoidosis - a case report.

BACKGROUND: Approximately a quarter of patients with advanced sarcoidosis develop pulmonary hypertension (PH), which affects their prognosis. We report unusual case of confirmed chronic thromboembolic pulmonary hypertension (CTEPH) in a patient with stage IV sarcoidosis successfully treated with balloon pulmonary angioplasty (BPA). CASE PRESENTATION: A 65 years old male with a history of colitis ulcerosa, and pulmonary sarcoidosis diagnosed in 10 years before, on long term oral steroids, with a history of deep vein thrombosis and acute pulmonary embolism chronically anticoagulated was referred to our center due to severe dyspnea. On admission he presented WHO functional class IV, mean pulmonary artery pressure (mPAP) in right heart catheterization (RHC) was elevated to 54 mmHg. Diagnosis of CTEPH was definitely confirmed with typical V/Q scan, and with selective pulmonary angiography (PAG) completes by intravascular imagining (intravascular ultrasound, optical coherent tomography). The patient was deemed inoperable by CTEPH team and two sessions of BPA with multimodal approach resulted in significant clinical and haemodynamical improvement to WHO class II and mPAP decrease to 27 mmHg. CONCLUSIONS: Balloon pulmonary angioplasty, rapidly developing method of treatment of inoperable CTEPH patients, is also extremely useful therapeutic tool in complex PH patients.

Improvement in Quality of Life and Hemodynamics in Chronic Thromboembolic Pulmonary Hypertension Treated With Balloon Pulmonary Angioplasty.

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<0.05), mPAP (51.7±10.6 vs. 35.0±9.1 mmHg; P<0.05), CI (2.2±0.5 vs. 2.5±0.4 L/min·m2; P=0.04), PVR (10.4±3.9 vs. 5.5±2.2 Wood units; P<0.05), functional class (96% vs. 20% in WHO class III and IV, P<0.05) and improvement in 6MWT distance (323±135 vs. 410±109 m; P<0.05) was observed. The only significant correlation was between the mental component summary score of QoL after completion of treatment and percentage improvement in the 6MWT (-0.404, P<0.05). CONCLUSIONS: Alongside improvement in functional and hemodynamic parameters, BPA also provides significant improvement in QoL.

Chronic thromboembolic disease among patients undergoing surgical pulmonary embolectomy for acute pulmonary embolism.

BACKGROUND: This study aimed to assess the prevalence of chronic thromboembolic lesions in the pulmonary arteries among patients undergoing pulmonary embolectomy for acute pulmonary embolism and their impact on treatment outcomes. METHODS: We conducted a retrospective, single-center analysis of consecutive patients undergoing emergency pulmonary embolectomy for acute pulmonary embolism between 2013 and August 2021. According to European Society of Cardiology guidelines, the diagnosis was based on clinical presentation, imaging studies and laboratory tests. Surgery was selected as the optimal treatment modality within the Pulmonary Embolism Response Team. Based on the intraoperatively identified chronic lesions patients were divided into two groups: acute only and acute/chronic. The analysis comprised history, laboratory and imaging studies, early and long-term mortality, and postoperative complications. We determined predictive factors for chronic thromboembolic lesions and risk factors for death. RESULTS: The analysis included 33 patients. Intraoperatively, 42% (14) of patients had chronic lesions. Predictive factors for these lesions are the duration of symptoms >1 week (OR=13.75), pulmonary artery dilatation >3.15 cm (OR=39.00) and right ventricle systolic pressure >52 mmHg (OR=29.33). No hospital deaths occurred in the acute only group and two in the acute/chronic group (0% vs. 14.3%; P=0.172). Risk factors for death are the duration of symptoms >3 weeks (HR=7.35) and postoperative use of extracorporeal membrane oxygenation (HR=7.04). CONCLUSIONS: Acute thromboembolic disease overlapping chronic clots is relatively common among patients undergoing pulmonary artery embolectomy. A detailed evaluation of the patient's medical history and imaging studies can identify these patients, as they require special attention when making treatment decisions. Surgical treatment in a center of expertise in pulmonary endarterectomy seems reasonable.

Surgical pulmonary embolectomy: state of the art.

Acute pulmonary embolism is a significant cause of morbidity and mortality. Patients in life-threatening conditions require timely and effective interventions to improve pulmonary perfusion. The indications for surgical embolectomy in the thrombolysis era have been limited. This article discusses surgical techniques and outlines the position of surgical embolectomy concerning other treatment modalities.

Acute pulmonary embolism with coexisting right heart thrombi in transit-surgical treatment of 20 consecutive patients.

OBJECTIVES: The presence of right heart thrombi in transit (RHTiT) in the setting of acute pulmonary embolism (PE) is associated with high mortality. The optimal management in such cases is inconclusive. We present the results of surgical treatment of 20 consecutive patients diagnosed with high- or intermediate-high-risk PE with coexisting RHTiT. METHODS: A retrospective analysis was performed of all consecutive patients undergoing surgical treatment in the Medicover Hospital between 2013 and 2021 for acute PE with coexisting thrombi in-transit in right heart cavities. The diagnosis was based on echocardiography, computed tomography pulmonary angiography and laboratory tests. Eligibility criteria for surgical treatment were acute PE with RHTiT, right ventricular overload on imaging studies and significantly elevated levels of cardiac troponin and NTproBNP. All patients were operated on with extracorporeal circulation using deep hypothermia and total circulatory arrest. The primary end point was hospital all-cause mortality; secondary end points were perioperative complications and long-term mortality. RESULTS: The analysis included 20 patients. There was no in-hospital death. Nearly one-third of patients required temporal hemofiltration for postoperative renal failure, but this did not involve the need for dialysis at discharge. No neurological complications occurred in any patient. The mean follow-up was 46 months (range 13-98). There was 1 death in the long-term follow-up, not related to PE. CONCLUSIONS: Surgical treatment of patients with acute PE and coexisting RHTiT can provide favourable results.

An unusual case of CTEPH treated by BPA in a patient with a single lung after cancer surgery.

A 46-year-old man 1 year after left-sided pneumonectomy for squamous cell lung cancer presented with severely limited exercise tolerance and dyspnea corresponding to World Health Organization functional class IV (WHO Class IV). After right heart catheterization (RHC), mean pulmonary artery pressure (mPAP) was 43 mmHg and pulmonary vascular resistance (PVR) was 10.2 Wood units (WU). Arteriography revealed organized clots located at the proximal level of the right pulmonary artery, leading to a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). The CTEPH team disqualified the patient from surgical treatment due to high perioperative risk and referred him for balloon pulmonary angioplasty (BPA) together with pulmonary hypertension-specific pharmacotherapy (sildenafil). The patient underwent a cycle of nine BPA sessions and completed treatment without complications. Follow-up showed sustained hemodynamic improvement in RHC (mPAP 23 mmHg, PVR 2.6 WU), improved physical capacity (WHO Class II), and relief of dyspnea symptoms.

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for chronic thromboembolic pulmonary hypertension patients after acute pulmonary embolism: Update.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism (APE). Both pharmacological and invasive treatments for CTEPH are available in Poland, and awareness of the disease among physicians is growing. It has been suggested that the COVID-19 pandemic may increase the incidence of CTEPH and facilitate disease detection during more advanced stages of the illness. Thus, the Polish Cardiac Society's Working Group on Pulmonary Circulation, in cooperation with independent experts in this field, launched the updated statement on the algorithm to guide a CTEPH diagnosis in patients with previous APE. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months of effective anticoagulation, particularly when specific risk factors are present. Echocardiography is the main screening tool for CTEPH. A diagnostic workup of patients with significant clinical suspicion of CTEPH and right ventricular overload evident on echocardiography should be performed in reference centers. Pulmonary scintigraphy is a safe and highly sensitive screening test for CTEPH. Computed tomography pulmonary angiography with precise detection of thromboembolic residues in the pulmonary circulation is important for the planning of a pulmonary thromboendarterectomy. Right heart catheterization definitively confirms the presence of pulmonary hypertension and direct pulmonary angiography allows for the identification of lesions suitable for thromboendarterectomy or balloon pulmonary angioplasty. In this document, we propose a diagnostic algorithm for patients with suspected CTEPH. With an individualized and sequential diagnostic strategy, each patient can be provided with suitable and tailored therapy provided by a dedicated CTEPH Heart Team.

Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: a multicentre registry.

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<0.001) and pulmonary vascular resistance from 642±341 to 324±183 dynes (p<0.001), and the six-minute walking test (6MWT) improved from 341±129 to 423±136 m (p<0.001). Pulmonary injury related to the BPA procedure occurred in 6.4% of all sessions. Eighteen patients (7.6%) died during follow-up, including 4 (1.7%) who died within 30 days after BPA. Overall survival was 92.4% (95% confidence interval [CI]: 87.6%-94.9%) three years after the initial BPA procedure. CONCLUSIONS: This multicentre registry confirmed significant improvement of haemodynamic, functional, and biochemical parameters after BPA. Complication rates were low and overall survival comparable to the results of another registry. Therefore, BPA may be an important therapeutic option in patients with CTEPH in Poland.

Balloon Pulmonary Angioplasty in Technically Operable and Technically Inoperable Chronic Thromboembolic Pulmonary Hypertension.

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 < 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.

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL).

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.

Renal carcinoma infiltrating inferior vena cava and combined valvular heart disease--one-stage uro-cardiological procedure: a case report.

Standard treatment of patients with coexisting cardiac and non-cardiac diseases includes two separate operations. We report a case of 55-year-old man with combined valvular heart disease and renal carcinoma infiltrating inferior caval vein, who underwent one-stage cardio-urologic procedure. In the first step, mitral and tricuspid valvuloplasty were performed by cardiac surgeons. Then, urologists performed radical nephrectomy and thrombectomy. The postoperative course was uneventful. In twelve months follow-up the patient shows no signs of recurrence and he had no symptoms of cardiac disease. To the best of our knowledge such a case has never been reported before in the literature.

Usefulness of 6-minute walk test, plasma neurohumoral and cytokine activation in the assessment of symptomatic patients with left ventricle dysfunction caused by chronic severe mitral valve regurgitation.

OBJECTIVE: The aim of our study was to select the most relevant markers of impaired left ventricle (LV) function in patients with heart failure (HF) symptoms due to severe chronic mitral regurgitation (MR). METHODS AND RESULTS: Thirty-six patients with decompensated HF due to severe MR underwent echocardiography, 6-minute walk test (6MWT) and measurements of plasma renin activity, angiotensin II, aldosterone, noradrenaline (NA), brain natriuretic peptide (BNP), tumour necrosis factor alpha (TNFalpha) with its receptors, and interleukine-6. Patients presented with significant neurohumoral/cytokine activation. By stepwise multiple regression analysis the strongest prediction model for 6MWT included LVEDVI (R2 = 0.95, P = 0.024), BNP (R2 = 0.67, P = 0.0006), IL-6 (R2 = 0.90, P = 0.044); for BNP: 6MWT (R2 = 0.36, P = 0.003), LA (R2 = 0.56, P = 0.0077), LVESVI (R2 = 0.83, P = 0.0072); for NA: EF (R2 = 0.4 1, P = 0.036), and for TNFalpha: LVESVI (R2 = 0.65, P = 0.003). CONCLUSIONS: 6MWT and neurohumoral markers (mainly BNP, but also NA and TNFalpha) are good predictors of the degree of LV remodelling, showing an independent correlation with the level of LV dilatation/dysfunction in chronic severe MR.These assessments may supplement standard echocardiography in LV decompensation due to severe MR.

Predictors of the atrial fibrillation occurrence in patients with Wolff-Parkinson-White syndrome.

BACKGROUND: Atrial fibrillation (AF) in WPW syndrome occurs earlier and is more common than in the general population. AIM: To evaluate the predisposing factors for the first episode of AF in patients with WPW. METHODS: We analysed data on 930 patients (510 males, 420 females) with WPW treated in our centre during 1988-2007. AF was diagnosed in 236 patients (25% - 161 males, 75 females, aged 36 +/- 15 years). The AF group was divided into two subgroups - patients with AF and atrio-ventricular reentrant tachycardia (AVRT), and patients with AF only. The analysis included subjects' age and gender, the presence of AVRT, the number and properties of accessory pathways, left ventricular ejection fraction (LVEF) and concomitant cardiovascular diseases. RESULTS: The groups did not differ in terms of concomitant diseases and LVEF. In the whole group of patients with AF, arrhythmia occurred earlier in men than in women (34 +/- 14 vs. 40 +/- 15 years of age, p = 0.013). In the subgroup with AF and AVRT, AF was documented earlier compared to patients with AF only (34 +/- 15 vs. 41 +/- 15 years of age, p = 0.0072). AVRT was more common in patients with AF compared to those without AF (69 vs. 53%, p < 0.001). In the whole group of 930 patients, AF was observed more often in patients with overt pre-excitation compared to concealed WPW (29 vs. 12%, p < 0.001). CONCLUSIONS: In patients with WPW syndrome, AF occurs earlier in patients with AVRT compared to patients with AF and without documented AVRT, earlier in men compared to women, and is more common in patients with overt WPW.

Early echocardiographic results of transapical off-pump mitral valve repair with the NeoChord DS1000 device in patients with severe mitral regurgitation due to posterior leaflet prolapse: first experiences in Poland.

INTRODUCTION: Minimally invasive techniques of mitral valve (MV) repair have been increasingly used in recent years. Transapical implantation of artificial chordae on a beating heart under 2D/3D transesophageal echocardiographic guidance with the NeoChord DS1000 device is a new surgical treatment of degenerative mitral regurgitation (MR). AIM: To evaluate early results of MV repair with the NeoChord DS1000 device in the first group of consecutive patients operated on in Poland. MATERIAL AND METHODS: Twenty-one patients with severe MR due to posterior leaflet prolapse (81% male; mean age: 60.7 ±12.7 years) underwent MV repair with the NeoChord DS1000 system. There were 12 (57.1%) patients with type A (an isolated central prolapse/flail), 8 (38.1%) patients with type B (multisegment disease/flail) and 1 (4.8%) patient with type C (posterior/paracommisural area) MV prolapse. A flail leaflet was present in 12 (57.1%) patients. The median number of neochords was 3 (2-6). We assessed by echocardiography left-sided heart morphology and evaluated MR degree before and 6 months after chords implantation. RESULTS: Early procedural success was achieved in 100% of patients. At the 6-month follow-up nonsignificant MR (trace and mild) was detected in 17 (81.0%) patients, while moderate MR was detected in 4 (19.0%) patients; mean values of left-sided heart dimensions and volumes, mitral E and E' velocity of lateral MV annulus significantly decreased. CONCLUSIONS: A novel procedure with the NeoChord DS1000 device is feasible in properly selected patients, and results in a significant reduction of MR degree and left ventricle and left atrium reverse remodeling at the 6-month follow-up.

Augmented reality and three-dimensional printing in percutaneous interventions on pulmonary arteries.

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.

Transapical beating heart mitral valve repair with the NeoChord system: early outcomes of a single-center experience.

INTRODUCTION: Trans-apical beating heart off-pump mitral valve (MV) repair is a novel surgical technique for treating mitral regurgitation (MR) caused by degenerative flail/prolapse (DLP). AIM: To present early outcomes of a single-center experience with transapical beating heart mitral valve repair with the NeoChord system. MATERIAL AND METHODS: Thirty-seven patients with severe symptomatic MR were treated with the NeoChord technique between September 2015 and December 2018 (78% men; mean age: 62.3 ±13.4 years). We evaluated standard cardiac surgery perioperative complications as well as those related to the NeoChord technique as well as early surgical success as defined by the reduction of MR to less than moderate by implantation of at least 2 neochordae. RESULTS: During this series we had no hemodynamic instability due to bleeding or arrhythmia. There were no transapical technique-related adverse events such as a leaflet perforation or tear, a major native chord rupture, which would require implantation of a new chord, ventricular apex rupture, or left atrial perforation. There were no major adverse events including death, stroke or acute myocardial infarction. Nine (24%) patients developed an episode of perioperative atrial fibrillation. We were able to conclude the operation in 98% of our patients with less than moderate MR. One (2%) patient had moderate MR at the conclusion of the operation. CONCLUSIONS: Trans-apical off-pump MV repair with the NeoChord system is a safe, minimally invasive procedure, with few minor complications. In well-selected candidates it provides successful treatment of degenerative MR. Results are anatomy dependent, so preoperative patient selection is crucial.

Balloon pulmonary angioplasty for the treatment of residual or recurrent pulmonary hypertension after pulmonary endarterectomy.

BACKGROUND: 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. METHODS: Fifteen patients with residual pulmonary hypertension after PEA received 71 BPA sessions (4.7 ±â€¯1.4 sessions/patient). The mean time between the PEA and the first BPA session was 28.1 ±â€¯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. RESULTS: After BPA, the mean pulmonary arterial pressure decreased from 44.7 ±â€¯6.4 to 30.8 ±â€¯7.5 mm Hg (31% decline; p < 0.001). Pulmonary vascular resistance decreased from 551.9 ±â€¯185.2 to 343.8 ±â€¯123.8 dyn∗s/cm-5 (38% decline; p < 0.001). The 6-min walking distance increased from 383 ±â€¯104 to 476 ±â€¯107 m (mean change +93 m; p < 0.001). In two sessions (2.8%), serious periprocedural complications occurred. During a mean follow-up of 18 ±â€¯14.3 months, one patient died two months after the last BPA session. Fourteen patients survived. CONCLUSIONS: BPA could be a promising therapeutic strategy for persistent pulmonary hypertension after PEA in patients with CTEPH.

Expert opinion on the creating and operating of the regional Pulmonary Embolism Response Teams (PERT). Polish PERT Initiative.

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.

Acute and chronic dissection of pulmonary artery: new challenges in pulmonary arterial hypertension?

Right ventricular failure is a leading cause of mortality in patients with pulmonary arterial hypertension (PAH). However, up to 25% of such patients die unexpectedly, without warning signs of hemodynamical decompensation. We previously documented that pulmonary artery (PA) dilatation significantly increases the risk of those deaths. Some of them may be due to dissection of PA resulting in cardiac tamponade. However, direct confirmation of this mechanism is difficult as most of such deaths occur outside hospitals. We present 4 patients with severe PAH and PA dilatation in whom PA dissection has been confirmed. Three patients had IPAH, one had PAH associated with congenital heart disease. All patients had mean pulmonary artery pressure (PAP) > 50 mmHg at diagnosis and dissection occurred late in the course of apparently well controlled disease (6 to 14 years). Several clinical elements were common to our patients - high systolic PAP, long lasting PH, progressive dilatation of PA to more than 50 mm with chest pain prior to dissection. However, clinical course followed three different patterns: sudden death due to cardiac tamponade, hemopericarditis caused by blood leaking from dissected aneurysm with imminent but not immediate cardiac tamponade, or chronic asymptomatic PA dissection. Indeed, two of our patients are alive and on lung transplantation waiting list for more than 2 years now. Further research is needed to suggest optimal management strategies for patients with stable PAH but significantly dilated proximal pulmonary arteries or confirmed PA dissection depending on the clinical presentation and expected outcome.