Thromboembolic Hypertension Research Articles

Treatment and long term follow-up results in patients with pulmonary vascular thrombosis related to COVID-19.

Pulmonary embolism is a complication of COVID-19 infection. The aim of this study is to assess prognosis and treatment response, including incidences of chronicity, relapse, and mortality among outpatients diagnosed with COVID-19-related pulmonary embolism between 2020 and 2022. A total of 101 patients with pulmonary embolism, started on anticoagulation during or within a month of COVID-19 infection, were included after testing positive by PCR. Data about comorbidities, Pulmonary Embolism Severity Index scores, PE diagnostic modalities, biochemical parameters, and transthoracic echocardiographic findings at diagnosis and at 24-month follow-up were collected. Cardiac catheterization parameters were recorded and compared between groups at diagnosis and at the 24-month follow-up. Groups were comparable with respect to gender, age, body mass index, and comorbidity score. Use of Q-SPECT for diagnosis was found significantly higher in patients with COVID-19-related pulmonary embolism (P < .001). The incidence of deep vein thrombosis was similar. In the study group, 43.6% of patients received anticoagulants for 3 months, with 49.1% using low molecular weight heparin and 50.9% using direct oral anticoagulants. At 24 months, rate of patients continuing treatment was comparable between groups. Specific pulmonary artery blockage value was found to be higher in patients with chronic thromboembolic pulmonary hypertension compared to those who demonstrated a response to pulmonary embolism treatment (P = .009). No adverse effects of anticoagulant therapy were observed during course of treatment. Over 24-month follow-up period, mortality, relapse, chronic thromboembolic hypertension and thromboembolic disease was observed in 2%, 2.2%, 4.9%, and 9.9% of patients, respectively.

Induced Ablation of Prmt1 in Endothelial Cells Causes Endothelial Dysfunction Associated with Pulmonary Thromboembolic Hypertension

Endothelial cells (ECs) play vital roles in regulation of vascular homeostasis, vascular tone, leukocyte traffcking, and barrier function to ensure pulmonary function. Endothelial dysfunction is a key factor for the progression of pulmonary pathogenesis. Protein arginine methyltransferase 1 (Prmt1) catalyzes asymmetric di-methylation of substrates on arginine residues and is implicated in a variety of cellular processes, including cardiovascular function. In the current study, we examined the role of Prmt1 in endothelial function by using tamoxifen-inducible EC-specific Prmt1 ablation (KO) mice. EC-specific Prmt1 ablation in adult mice (KO) for 8 weeks led to lethality, concurrent with pulmonary hypertension and thromboembolism. The lung bulk RNA sequencing and single EC RNA sequencing analyses revealed that Prmt1 ablation increased inflammation, endothelial junction disruption, apoptosis, and fibrosis. Comprehensive analysis through bulk RNA and single cell RNA sequencing revealed that Prmt1 ablation in ECs triggers homeostatic disturbance accompanied by inflammation and endothelial dysfunction, associated with hyperactivation of NFkB and Stat signaling. In conclusion, the current study suggests that Prmt1 plays a key role in pulmonary endothelial function and vascular homeostasis via the suppression of NFkB-induced endothelial dysfunction. The National Research Foundation Grant funded by the Korean Government (MIST) (NRF-2022R1A2B5B02001482). This is the full abstract presented at the American Physiology Summit 2024 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Pulmonary Thromboendarterectomy for Chronic Pulmonary Thromboembolism

Pulmonary embolism in terms of causes of mortality is the third most common cause, when talking about cardiovascular diseases, yet most undiagnosed or misdiagnosed one. Among causes of pulmonary thromboembolism protein C deficiency is a rare one and idiopathic most common entity. In general population the incidence of severe protein C deficiency is about 1 in 500,000-750,000 people. We present a case of chronic pulmonary thromboembolism in young female of 23 years. She presented with history of dyspnea, dry cough and bilateral DVT of legs. On pulmonary CT diagnosis of massive pulmonary thrombus was made and her pulmonary thromboendarterectomy was done in our center. Conclusion: All patients with chronic pulmonary thromboembolic hypertension should be assessed for operability by proper referral to an experienced CTEPH (chronic thromboembolic pulmonary hypertension) team to determine if they are viable candidate for PEA (pulmonary endarterectomy). PEA is standard and recommended operative technique for treatment of CTEPH. Keywords: pulmonary endarterectomy, pulmonary embolism, protein C deviciency

Principles of healthcare management for patients with pulmonary hypertension: focus on continuity

In managing care for patients with rare, rapidly progressive pathologies such as pulmonary hypertension (PH) and chronic thromboembolic hypertension (CTEPH), early diagnosis and speed of routing are of particular importance. Reducing the time spent on patient routing reduces the risk of disease progression, the rate of hospitalizations, and thereby the socioeconomic burden of the disease.In the Republic of Karelia, an algorithm for routing patients with PH and CTEPH has been created, which is an example of a successful healthcare management.

Value of Right and Left Ventricular T1 and T2 Blood Pool Mapping in Patients with Chronic Thromboembolic Hypertension before and after Balloon Pulmonary Angioplasty

Parametric imaging has taken a steep rise in recent years and non-cardiac applications are of increasing interest. Therefore, the aim of our study was to assess right (RV) and left ventricular (LV) blood pool T1 and T2 values in patients with chronic thromboembolic pulmonary hypertension (CTEPH) compared to control subjects and their correlation to pulmonary hemodynamic. 26 patients with CTEPH (mean age 64.8 years ± 12.8 SD; 15 female), who underwent CMR and right heart catheterization (RHC) before and 6-months after balloon pulmonary angioplasty (BPA), were retrospectively included. Ventricular blood pool values were measured, compared to control subjects (mean age 40.5 years ± 12.8 SD; 16 female) and correlated to invasive measures (CI, mPAP, PVR). In both, control subjects and CTEPH patients, RVT1 and RVT2 were significantly reduced compared to LVT1 and LVT2. Compared to control subjects, RVT2 was significantly reduced in CTEPH patients (p = 0.0065) and increased significantly after BPA (p = 0.0048). Moreover, RVT2 was positively correlated with CI and negatively correlated with mPAP and PVR before (r = 0.5155, r = -0.2541, r = -0.4571) and after BPA (r = 0.4769, r = -0.2585, r = -0.4396). Ventricular blood pool T2 mapping might be novel non-invasive CMR imaging marker for assessment of disease severity, prognosis, follow-up and even therapy monitoring in PH.

Abstract No. 273 Balloon pulmonary angioplasty in patients with non-operable or residual chronic thromboembolic hypertension: initial 5-year experience in a national referral center

Balloon pulmonary angioplasty (BPA) has become an emerging and complementary strategy for chronic thromboembolic hypertension (CTEPH) patients who are not suitable for pulmonary endarterectomy (PEA) or present recurrent symptoms after PEA. The purpose of our study was to evaluate the efficacy and safety of BPA in patients with non-operable or residual CTEPH during the initial 5-year experience in a single national referral center.

Hospital costs of Balloon Pulmonary Angioplasty (BPA) procedure and management for CTEPH patients: An observational study based on the French national hospital discharge database (PMSI).

Since 2014, Balloon Pulmonary Angioplasty (BPA) has become an emerging and complementary strategy for chronic thromboembolic hypertension (CTEPH) patients who are not suitable for pulmonary endarterectomy (PEA) or who have recurrent symptoms after the PEA procedure. To assess the hospital cost of BPA sessions and management in CTEPH patients. An observational retrospective cohort study of CTEPH-adults hospitalized for a BPA between January 1st, 2014 and June 30th, 2016 was conducted in the 2 centres performing BPA in France (Paris Sud and Grenoble) using the French national hospital discharge database (PMSI-MCO). Patients were followed until 6 months or death, whichever occurred first. Follow-up stays were classified as stays with BPA sessions, for BPA management or for CTEPH management based on a pre-defined algorithm and a medical review using type of diagnosis (ICD-10), delay from last BPA procedure stay and length of stay. Hospital costs (including medical transports) were estimated from National Health Insurance perspective using published official French tariffs from 2014 to 2016 and expressed in 2017 Euros. A total of 191 patients were analysed; mainly male (53%), with a mean age of 64,3 years. The first BPA session was performed 1.1 years in median (IQR 0.3-2.92) after the first PH hospitalisation. A mean of 3 stays with BPA sessions per patient were reported with a mean length of stay of 8 days for the first stay and 6 days for successive stays. The total hospital cost attributable to BPA was € 4,057,825 corresponding to €8,764±3,435 per stay and €21,245±12,843 per patient. Results were sensitive to age classes, density of commune of residence and some comorbidities. The study generated robust real-world data to assess the hospital cost of BPA sessions and management in CTEPH patients within its first years of implementation in France.

Age- and Sex-Specific Rates of Heart Failure and other Adverse Cardiovascular Outcomes in Systemic Sclerosis

Abstract Background Age at disease onset and sex appear to modify the disease course in patients with systemic sclerosis (SSc). Although patients with SSc have a higher risk of adverse cardiovascular outcomes than people without SSc, there are few data on age- and sex-specific risks of heart failure (HF) and other adverse cardiovascular outcomes in patients with SSc. Objectives To investigate the long-term rates of HF and other adverse cardiovascular outcomes (including arrhythmias, myocardial infarction, ischemic stroke, venous thromboembolism, and pulmonary hypertension) in a nationwide cohort of patients with SSc compared with the background population according to age and sex, separately. Methods Using Danish nationwide registries, all patients &amp;gt;18 years with newly diagnosed SSc (1996–2018) were identified. SSc patients were matched at a 1:4 ratio by age, sex, and comorbidities with controls from the background population without SSc. Rates of outcomes according to age (above/below median age) and sex were compared between cases and controls using Cox regression. Results Of the 2,019 patients diagnosed with SSc, 1,569 patients were matched with 6,276 controls from the background population (median age 55 years, 80.4% women). SSc was associated with a higher rate of HF in both women (HR 2.99 [95% CI, 2.18–4.09]) and men (HR 3.01 [1.83–4.95]) (Pfor interaction=0.88), with similar findings for other cardiovascular outcomes.For age interaction, SSc was associated with an increased rate of HF in patients &amp;lt;55 years (HR 4.14 [2.54–6.74]) and ≥55 years (HR 2.74 [1.98–3.78]), with similar effect of younger and older groups on HF (P for interaction=0.21), and other cardiovascular outcomes. Conclusions SSc was associated with an increased long-term rate of cardiovascular outcomes compared with a matched background population, with similar extent in different gender and age groups. Funding Acknowledgement Type of funding sources: None. Adjusted hazard ratios according to sexAdjusted hazard ratios according to age

Complications of balloon pulmonary angioplasty for chronic thromboembolic pulmonary disease according to the classification proposed by the 6th world symposium on pulmonary hypertension

Abstract Introduction Balloon pulmonary angioplasty (BPA) is a complex procedure and not risk free. In an attempt to standardize reports of BPA complications in the several centers, a classification of complications was proposed by the task force on chronic thromboembolic hypertension (CTEPH) in the 6th World Symposium on Pulmonary Hypertension (WSPH). Purpose To determine the prevalence of BPA complications according to the classification of 6th WSPH and to identify its predictors. Methods Detailed procedural and technical aspects were collected for consecutive patients (pts) with inoperable, residual/recurrent chronic thromboembolic disease, undergoing BPA at a single institution from December/2017 to December/2020. Per procedure logistic regression analysis was used to evaluate the predictive variables for complications. Results A total of 76 BPA sessions in 15 pts were performed (mean age 63.2±14.0 years; 60.0% women; 86.7% CTEPH). Mean pulmonary artery pressure and pulmonary vascular resistance before the first BPA session were 33.1±13.3 mmHg and 4.8±3.2 woods unit, respectively (73.3% of pts under vasodilator therapy). Femoral access was used for all pts. Mean vessels treated per procedure were 4.3±1.9 (324 vessels in total). Webs, subtotal occlusions, ring-like stenosis and total occlusions were noted in 215 (66.4%), 58 (17.9%), 31 (9.6%) and 20 (6.2%) treated vessels, respectively. We performed 21 pressure-wire-guided sessions (27.6%). Intravascular imaging was used in 6 procedures (7.9%). Average time of fluoroscopy was 60.3±14.0 minutes and volume of contrast 273.0±73.0 mL per session. Procedure-related adverse events occurred in 25.0% of the interventions (27.6% in the first two years vs 16.7% in the last two). Pulmonary artery vascular injuries were noted in 6 BPA vessels (7.9% per procedure and 1.9% per treated vessel): haemoptysis in all, but perforation was only detected angiographically in 3 of them (balloon inflation was performed for 2 distal perforations, and 1 perforation sealed without any intervention). Vascular dissection in distal lesions occurred in 4 cases (5.3%) with no need of transcatheter or surgical procedures. We had 3 lung injuries, all grade 2. None of the pts required oral intubation or mechanical ventilation. Extra-pulmonary complications were illustrated in table. Importantly, there was no peri-procedural death. The occurrence of vascular or lung injuries was 0% in pressure-wire-guided BPA versus 14.5% in non-guided (p=0.098). Multivariate analysis revealed that age (OR 1.05; CI 1.01–1.10; p=0.030) was the only independent predictor of complications. Conclusions In our experience, BPA can be safely performed in inoperable, residual or recurrent CTEPH or CTED pts, with 25% minor procedural-related complications but no major adverse event. Age was the strongest factor related to the occurrence of complications. Funding Acknowledgement Type of funding sources: None.

Use of direct oral anticoagulants in chronic thromboembolic pulmonary hypertension: a systematic review.

Direct oral anticoagulants (DOACs) are being increasingly used in patients with chronic thromboembolic hypertension (CTEPH), however, the data on their safety and efficacy are scarce and contradictory. We systematically searched MEDLINE and Google Scholar databases from January 2010 to January 2021 for studies of DOACs in CTEPH. Three observational studies, 2 abstracts and one case series met our inclusion criteria. While these studies reported similar or even less rates of major bleeding in patients receiving DOACs compared with vitamin K antagonists, there were concerns about the possibility of increased risk of venous thromboembolism recurrence with DOAC therapy. Further studies are warranted to better define the role of DOACs in CTEPH.

Luspatercept: A Review in Transfusion-Dependent Anaemia due to Myelodysplastic Syndromes or β-Thalassaemia

Luspatercept (Reblozyl®), a first-in-class erythroid maturation agent, is approved in several countries worldwide for the treatment of adults with transfusion-dependent anaemia due to myelodysplastic syndromes (MDS), who have failed prior erythropoiesis-stimulating therapy, or β-thalassaemia. In pivotal, placebo-controlled, phase III trials, subcutaneous luspatercept significantly reduced red blood cell (RBC) transfusion requirements in patients with MDS or β-thalassaemia. Luspatercept had a generally manageable tolerability profile in clinical trials. Adverse events of special interest include thromboembolic events, hypertension and bone pain. Thus, luspatercept is an emerging treatment option in adults with transfusion-dependent anaemia due to MDS or β-thalassaemia.

Motion Reduction for C-Arm Computed Tomography of the Pulmonary Arteries: Image Quality of a Motion Correction Algorithm in Patients with Chronic Thromboembolic Hypertension During Balloon Pulmonary Angioplasty.

To evaluate the feasibility and image quality of a motion correction algorithm for supra-selective C-arm computed tomography (CACT) of the pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing balloon pulmonary angioplasty (BPA). CACT raw data acquired during 30consecutive BPAs were used for image reconstruction using either standard (CACTorg) or a motion correction algorithm (CACTmc), using 400 iterations. Two readers independently evaluated 188 segmental and 564 sub-segmental contrast-enhanced pulmonary arteries in each reconstruction. The following categories were assessed: Sharpness of the vessel, motion artifacts, delineation of bronchial structures, vessel geometry, and visibility of treatable lesions. The mentioned criteria were rated from grade 1 to grade 3: grade 1: excellent quality; grade 2: good quality; grade 3: poor/seriously impaired quality. Inter-observer agreement was calculated using Cohen's Kappa. Due to an excellent agreement, the ratings of both readers were merged. Differences in the assessed image quality criteria were evaluated using pairwise Wilcoxon signed-rank test. Inter-observer agreement was excellent for all evaluated image quality criteria (κ > 0.81). For all assessed image quality criteria, the ratings on CACTorg were good but improved significantly for CACTmc to excellent for the whole vascular tree (p < 0.01). When considering segmental and sub-segmental levels individually, all image quality criteria improved significantly for CACTmc on both levels (p < 0.01). While ratings of CACTmc were constant for both levels (segmental and sub-segmental) for all criteria, the ratings of CACTorg were slightly impaired for the sub-segmental arteries. Motion correction for supra-selective contrast-enhanced CACT of the pulmonary arteries is feasible and improves the overall image quality. · Motion artifacts can severely impair the diagnostic accuracy of CACT.. · A motion correction algorithm can significantly improve image quality in CACT of the pulmonary arteries.. · Especially the overall image quality of sub-segmental branches is significantly improved.. · Maschke S, Werncke T, Becker LS et al. Motion Reduction for C-Arm Computed Tomography of the Pulmonary Arteries: Image Quality of a Motion Correction Algorithm in Patients with Chronic Thromboembolic Hypertension During Balloon Pulmonary Angioplasty. Fortschr Röntgenstr 2021; 193: 1074 - 1080.

Experience of chronic thromboembolic pulmonary hypertension (CTEPH) in two cases with scleroderma and immunopathogenesis overview: Case report

Systemic Sclerosis (SSc) is a multi-systemic connective tissue disease of unknown etiology. Although many pathological processes play a role in the basis of pulmonary hypertension (PHT) that develops secondary to SSc, vasculopathy has an important place. Chronic thromboembolic hypertension (CTEPH) is in the group 4 PHT class. CTEPH distinguishes it from other causes of PHT by having both surgical and medical treatment options. CTEPH is a pathology that develops chronically and can be overlooked due to its nonspecific symptoms. Early diagnosis and treatment can reduce morbidity and mortality. The physiopathology of vasculopathy secondary to CTEPH and vasculopathy of SSc made us suspect that similar processes operate in both diseases. The processes that cause and follow endothelial damage are similar in both diseases. If this pathophysiological mechanism can be clarified, possible new treatment options can be discovered. We diagnosed CTEPH with the examinations we performed in two of our patients with SSc and interstitial lung disease, both of which developed PHT. We aimed to discuss the immunopathogenesis with two case reports.

PCV25 Hospital Costs of Balloon Pulmonary Angioplasty (BPA) Procedure and Management for Chronic Thromboembolic Hypertension Patients: An Observational Study Based on the French National Hospital Discharge Database (PMSI)

To assess the hospital cost of BPA sessions and management in patients with CTEPH in France. An observational retrospective cohort study was conducted using the French national hospital discharge database (PMSI-MCO). All adult patients hospitalized for a BPA between January 1st, 2014 and June 30th, 2016 in the 2 centres performing BPA in France (Paris Sud and Grenoble University hospitals) were included. Patients were followed until 6 months or death, whichever occurred first. Follow-up stays were classified as stays with BPA sessions, for BPA management or for CTEPH management based on a pre-defined algorithm and a medical review using type of diagnosis (ICD-10), delay from the last BPA procedure stay and length of stay. Costs were determined from National Health Insurance perspective. Hospital costs (including medical transports) were estimated using published official French tariffs from 2014 to 2016 and expressed in 2017 Euros. Over the study period, a total of 191 patients were analysed; mainly male (53%), with a mean age of 64.3 years and without previous surgery (3%) when going back to 2006. The first BPA session was performed 1.1 years (median; min-max: 0.0-10.0) after the first PH hospitalisation. A mean of 3 stays with BPA sessions per patient were reported with a mean length of stay of 7.8±4.5 days for the first stay and 5.8±2.3 days for successive stays. The hospital cost attributable to BPA sessions + management was €8,792±3,414 per stay, €21,221±12,819 per patient and represented a total of €4,053,303 over the study period. Results were sensitive to age classes, density of city of residence and some comorbidities. The study allows assessing in real-life the hospital cost of BPA sessions and management in CTEPH patients, especially relevant at a time when the position of BPA in the therapeutic algorithm is regularly re-assessed.

The assessment of lung function and residual hypoxemia after balloon pulmonary angioplasty for patients with chronic thromboembolic hypertension

Abstract Background It has been reported that balloon pulmonary angioplasty (BPA) could dramatically improve hemodynamics in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients have shown poor response in oxygenation and lung function even after BPA. The aim of our study was to evaluate the efficacy of BPA using clinical indicators and to investigate lung function and residual hypoxemia. Method Consecutive 103 patients who underwent BPA from September 2011 to August 2018 were enrolled. We evaluated hemodynamics with right heart catheterization, respiratory function test, arterial blood gas examination, and exercise capacity with 6 minute-walk-distance (6MWD). Result At the median 12-month follow-up after the final BPA session, following examination findings were significantly improved. Mean pulmonary artery pressure (39.0±8.2mmHg, 23.2±8.4mmHg, p&amp;lt;0.001), Pulmonary vascular resistance (782±378 dynes / sec / cm-5, 331±187 dynes / sec / cm-5, p&amp;lt;0.001), 6 MWD (310±94 m, 363±119 m, p&amp;lt;0.001), PaO2 (63.1±15.8 mmHg, 68.5±13.6 mmHg, p=0.044),%VC (88.5±17.1, 91±16.3, p=0.036). However, the following examination findings did not improve after BPA. Oxygen desaturation during 6MWD test (−11.9±6.3, 12±7.9, p=0.65), %DLCO (64.2 17.1, 61.3 16.8, p=0.147). Conclusion BPA could dramatically improve hemodynamics and exercise tolerance. However, arterial oxygenation was not normalized after BPA. Moreover, oxygen desaturation in exercise, and %DLCO were almost unchanged. These observations might suggest the remaining arteriopathy in capillary level of pulmonary artery, but further research is needed. Funding Acknowledgement Type of funding source: None

Physical activity in incident patients with pulmonary arterial and chronic thromboembolic hypertension.

The cardinal symptom of pulmonary hypertension (PH) is dyspnea on exertion, leading to decreased activity in daily living. The aim of this study was to analyze daily physical activity in incident patients with arterial or chronic thromboembolic PH (PAH/CTEPH) and to investigate its correlation with pulmonary hemodynamics, symptoms, exercise capacity, and other outcomes. Incident patients with PAH/CTEPH had a 1-week activity assessment by the arm-worn accelerometer SenseWear within - 3months/+ 2weeks of the diagnostic right heart catheterization (RHC) and baseline assessments including 6-minute walking distance (6MWD). Activity was correlated to RHC data at rest and exercise and to other outcomes. Thirty-nine PH-patients (24 PAH, 15 CTEPH, 23 females, 65(54;73) years, mean pulmonary artery pressure (mPAP) 38(30;46) mmHg, cardiac output (CO) 5.2(4.6;6.3)l/min, 6MWD 458(300;593) m) were included. 64% had a sedentary lifestyle ( < 5000 steps/day), 26% were moderately active (5000-9999 steps/day), and 10% were active. In a multivariate stepwise regression analysis including age, gender, 6MWD and hemodynamics at rest and during exercise (heart rate, mPAP, stroke volume), the 6MWD was the only independent predictor of steps/day (B = 16.8 (95% CI 11.6-22.0), p < 0.001). Daily physical activity as steps/day assessed in incident patients with PAH/CTEPH did not well correlate with invasive hemodynamics at rest or during exercise, but very well with the 6MWD. Whether daily activity assessments provide additional information to simple walk distance on risk factor profiles during follow-up in patients with PAH/CTEPH remains to be clarified.

Assessment of usability and viability in clinical practice of the Nabelia mHealthCare Platform to support integrated care management of patients with pulmonary hypertension

Introduction : Pulmonary hypertension (PH) is a rare, life-threating disease that requires complex long term management, often involving coordination of actions across different agencies and jurisdictions. This is a challenging scenario to the effective delivery of care. Adoption of an integrated care (IC) approach with a sensible use of enabling technology can be a way of successfully dealing with this complexity. Aims : a) To assess the usability and potential feasibility in clinical practice of the Nabelia mHealthCare Platform, in the IC management of patients with Pulmonary Hypertension and; b) to identify areas in need of improvement in collaboration with patients and professionals Material and method : This qualitative research study involved two distinct groups chosen for convenience: 1) Patients’ group, patients with a pulmonary arterial hypertension (HAP) diagnose and/or chronic thromboembolic hypertension (HPTEC); and, 2) Health professionals’ group. In both groups, we carried out : 1) user tests followed by administration of a questionnaire (adaptation of the SUMI questionnaire items); 2) Focus groups sessions (1 patients, 1 professionals). A total of 23 people participated in this study (12 patients, 7 practitioners and 4 nurses). Informed consent was obtained from all participants. The study was completed by a heuristic assessment of the platform that was undertaken by a specially trained researcher. Results : Professionals highlighted that the Platform facilitated: 1) the sharing of information (notably in the case of treatment prescribed), 2) coordination of actions (remote monitoring data) and 3) communication with patients. Patients’ appraisals of the platform differed. Globally, it was viewed as an interesting tool and patients especially valued the accessibility to the information about their condition and the easiness to share this information with the different health professionals. Perceptions on the benefits of using the mobile application to manage everyday life depended on patients’ age and treatments’ complexity. Thus, younger patients were more favorable towards its use while elders were more reluctant. Heuristic evaluation. The aspects that were best ranked where those related with the clarity of the interface (adequate management of spaces and text, visual structure of the information, easily recognizable links), consistency in its design (elements, actions, language, navigation), and architecture (organizational system, elements and terms used in the navigation menu). Usability elements in need of attention were those related with system status, information flow, aid in its actions, clarity of information, control by user, readability and configuration. The application is easy to learn and uses standard tags and a language close to professionals with helpful graphics though in some parts readability could be improved. Control of the application by the user was acceptable at the current status of development. Conclusions : At its current stage of development the Nabelia mHealthCare Platform has a good usability and can be potentially applied in the management of patients with pulmonary hypertension. Professionals and patients think that the application facilitates the management of the disease, allows closer follow-up and monitoring and improves coordination across levels of health care. Attention to acceptance and usability issues in the group of older patients is, however, required.

MRI-derived Regional Biventricular Function in Patients with Chronic Thromboembolic Pulmonary Hypertension Before and After Pulmonary Endarterectomy

The aim of this study was to assess regional myocardial function in patients with chronic thromboembolic hypertension (CTEPH) before and after successful pulmonary endarterectomy (PEA) using magnetic resonance imaging. Twenty-two patients with CTEPH underwent cardiac magnetic resonance imaging before and 12 (11, 17) days after PEA. Mean pulmonary artery pressure was evaluated preoperatively by right heart catheterization and during post-PEA intensive care unit-stay using a Swan-Ganz catheter. Biventricular peak systolic longitudinal, radial, circumferential strain and time-to-peak strain were obtained by tissue-tracking analysis. Mean pulmonary artery pressure decreased (46 mmHg (34.5, 55) to 24 mmHg (16, 27); P < .0001) and stroke volume increased (P < .0001) after PEA. In the right ventricle (RV) peak radial strain increased in the anterior (P = .04) and in the inferior wall (P = .0012) and slightly missed statistical significance in the lateral wall (P = .051) and septum (P = .07). Circumferential strain increased in the lateral (P = .0002) and inferior wall of the RV (P = .03) and in the lateral as well as in the inferior wall of the left ventricle (P = .01; P = .03). Radial, longitudinal, and circumferential time-to-peak strain shortened (P < .0001) with resynchronization of the ventricles 12 days after PEA. While biventricular resynchronization and recovery of global predominantly RV function was observed, regional circumferential function mainly improved in the lateral and inferior walls of both ventricles and regional radial function in the RV wall and septum 12 days after PEA, suggesting fibers primarily affected by myocardial stress in patients with CTEPH possibly need a relatively longer recovery time.

Consensus Statement on Chronic Thromboembolic Disease, Prophylaxis and Special Situations / Abridged Version

Chronic thromboembolic disease refers to a set of conditions that manifest over time after acute pulmonary embolism (PE). In most cases, this episode is associated with acute deep vein thrombosis (DVT). These conditions include chronic pulmonary thromboembolic hypertension (CTEPH), post-thrombotic syndrome (PTS) and chronic venous insufficiency (CVI). (1) A timely diagnosis and treatment may modify the prognosis of this entity during the course of its free progression. (2) Damage caused by DVT may lead to valve dysfunction in the deep venous system. (3) Both CTEPH, PTS and CVI are associated with gradual impairment in the quality of life of those affected. Hence, it is important to postulate prophylaxis strategies in a patient at risk of suffering from a DVT-PE event.

Feasibility of quantitative regional ventilation and perfusion mapping with phase-resolved functional lung (PREFUL) MRI in healthy volunteers and COPD, CTEPH, and CF patients.

In this feasibility study, a phase-resolved functional lung imaging postprocessing method for extraction of dynamic perfusion (Q) and ventilation (V) parameters using a conventional 1H lung MRI Fourier decomposition acquisition is introduced. Time series of coronal gradient-echo MR images with a temporal resolution of 288 to 324 ms of two healthy volunteers, one patient with chronic thromboembolic hypertension, one patient with cystic fibrosis, and one patient with chronic obstructive pulmonary disease were acquired at 1.5 T. Using a sine model to estimate cardiac and respiratory phases of each image, all images were sorted to reconstruct full cardiac and respiratory cycles. Time to peak (TTP), V/Q maps, and fractional ventilation flow-volume loops were calculated. For the volunteers, homogenous ventilation and perfusion TTP maps (V-TTP, Q-TTP) were obtained. The chronic thromboembolic hypertension patient showed increased perfusion TTP in hypoperfused regions in visual agreement with dynamic contrast-enhanced MRI, which improved postpulmonary endaterectomy surgery. Cystic fibrosis and chronic obstructive pulmonary disease patients showed a pattern of increased V-TTP and Q-TTP in regions of hypoventilation and decreased perfusion. Fractional ventilation flow-volume loops of the chronic obstructive pulmonary disease patient were smaller in comparison with the healthy volunteer, and showed regional differences in visual agreement with functional small airways disease and emphysema on CT. This study shows the feasibility of phase-resolved functional lung imaging to gain quantitative information regarding regional lung perfusion and ventilation without the need for ultrafast imaging, which will be advantageous for future clinical translation. Magn Reson Med 79:2306-2314, 2018. © 2017 International Society for Magnetic Resonance in Medicine.