Infusion of Allogeneic Mesenchymal Stem Cells in Patients With Diffuse Cutaneous Systemic Sclerosis...
Systemic Sclerosis PulmonaryPulmonary Hypertension1 moreProgressive SSc is an entity with limited therapeutic alternatives and with asurvival rate of less than 45% in the first 3 to 5 years. The disease causessevere limitation in quality of life ranging from functional limitation to depression. Up to 20% of patients will be refractory to conventional treatment with diseasemodifying anti-rheumatic drugs (DMARDs) and cyclophosphamide therapy.This favors the progression to visceral involvement including gastrointestinal,lung and pulmonary hypertension. The latter being a poor prognostic factor,increases mortality in this group of patients and drastically affects their qualityof life. For this reason, different therapeutic options have been considered including cell transplantation and Stem Cell use. Among the options that have been studied so far are stromal mesenchymal cells from Wharton ́s jelly. These have been used in intravenous infusion or direct application in different disease scenarios ranging from vascular involvement to interstitial lung involvement and cases of pulmonary hypertension, with promising results in terms of clinical progression,improvement in quality of life and prognostic indices. This therapy has proven to have a significant margin of safety at the time of administration and a low rate of adverse events, a self-limiting fever as the most frequent event. Based on the above and considering the possibility of offering patients without therapeutic alternatives to their disease in addition to palliative options, an intravenous infusion of stromal mesenchymal stem cells from Wharton ́s jellyis proposed for three patients with progressive SSc refractory to conventional therapy with pulmonary involvement due to pulmonary hypertension. Under this premise the question posed in our work is; What are the effects of the infusion of allogeneic mesenchymal stromal cells from Wharton ́s jellyin patients with systemic sclerosis refractory to conventional treatment with Methotrexate or Cyclophosphamide in a population of three patients with severe pulmonary involvement due to pulmonary hypertension.
New International CTEPH Database
Chronic Thromboembolic Pulmonary HypertensionCTEPHThe New International CTEPH Database is a prospective, observational multi-center disease registry run by the International CTEPH Association (ICA), which will collect data in chronic thromboembolic pulmonary hypertension (CTEPH) patients worldwide. The registry will run for approximately 5 years. Its objective is to provide an overview on epidemiology of CTEPH, mode of diagnosis and treatment approaches worldwide as well as determinants of long-term outcomes as measured by New York Heart Association (NYHA) functional class and survival. The data collected will improve the knowledge and understanding of this condition, support the further development of diagnosis and treatment guidelines for patients with CTEPH, and contribute to improving patient care in the long-term.
Early Detection of Pulmonary- and Pulmonary Vascular Disease in Sjögren Syndrome
Sjögren SyndromePulmonary HypertensionAccording to the literature available pulmonary hypertension is present in 12 to 23% of patients suffering from Sjögren Syndrome. However epidemiological data are based on non-invasive measurements using echocardiography. Furthermore, no data are available regarding exercise hemodynamics in those patients. This study investigates pulmonary hemodynamics at rest and during exercise in patients suffering from primary and secondary Sjögren Syndrome. Patients under suspicion for pulmonary hypertension (PH) will be offered further investigations including right heart catheterization.
Qualitative Research to Explore Treatment Preference in Pulmonary Arterial Hypertension (PAH) Subjects...
HypertensionPulmonaryGlaxoSmithKline (GSK) is embarking on a clinical program to assess the treatment of PAH with an Angiotensin converting enzyme 2 (ACE2). This new treatment may require subcutaneous administration, in comparison to current treatments which are taken orally. Hence, GSK would like to conduct this qualitative interview study with PAH subjects to explore subject's perspective and preferences for various modes of treatment administration (daily or weekly subcutaneous injection versus current treatment options). This will be a qualitative study comprising the conduct of semi-structured telephone concept elicitation interviews with 8 to 10 PAH subjects (each approximately 30 minutes in duration) in the United States (US).
Perioperative Portal Vein Pulsatility as a Postoperative Prognostic Indicator in Pulmonary Endarterectomy...
Pulmonary HypertensionThromboembolism4 moreThe investigators aim to evaluate the utility of portal vein pulsatility as a predictor of the composite outcome of persistent organ dysfunction plus death in patients undergoing elective or urgent pulmonary endarterectomy for thromboembolic pulmonary hypertension. The investigators' hypothesis is that the portal vein pulsatility fraction, measured using transesophageal echocardiography immediately after weaning of cardiopulmonary bypass, is proportional to the risk of developing subsequent end-organ dysfunction in the postoperative setting.
Evaluation of Radiation Induced Pulmonary Hypertension Using MRI in Stage III NSCLC Patients Treated...
Pulmonary HypertensionIn the radiotherapeutic treatment of lung cancer, the dose that can be safely applied to the tumour is limited by the risk of radiation induced lung damage. This damage is characterized by parenchymal damage and vascular damage. In rats, we have found that radiation-induced vascular damage results in increased pulmonary artery pressure. Interestingly, the consequent loss of pulmonary function could be fully explained by this increase in pulmonary artery pressure. We hypothesize that also in patients a radiation induced increase in pulmonary artery pressure can be observed after radiotherapy, which may contribute to the development of radiation pneumonitis. The objective is to test the hypothesis that radiotherapy for lung cancer induces an increase in pulmonary artery pressure.
The Regulation of Pulmonary Vascular Resistance in Patients With Heart Failure
Diastolic Heart FailurePulmonary HypertensionPeripheral blood and blood following a pulmonary wedge pressure will be obtained from patients undergoing right heart catheterizations.
The FVC/DLCO Ratio is a Useful Predictor of Pulmonary Hypertension in Patients With Interstitial...
Interstitial Lung DiseasePulmonary HypertensionPulmonary hypertension (PH) is a common disorder among patients with interstitial lung disease (ILD). The symptoms are usually nonspecific and overlooked. Thus, a noninvasive screening modality is recommended for early detection of PH because of its potentially significant impact on treatment strategy and clinical outcomes. Objectives: To evaluate the usefulness of assessing forced vital capacity (FVC%), diffusion capacity of the lung for carbon monoxide (DLCO%), and FVC%/DLCO% ratio to predict pulmonary hypertension among patients with ILDs.
Thoracic Bio-reactance Measurement of Cardiac Output in Pulmonary Hypertension
Pulmonary HypertensionPulmonary Hypertension (PH) is a rare disease characterized by an increase in pulmonary vascular resistance, leading to a progressive decline in cardiac output (CO).Since cardiac output is correlated with the prognosis of the disease, the conduct of a right heart catheterization (thermodilution measurement, reference technique) during the annual follow-up visit of patients in competences centres is recommended. In practice, it is not systematically performed because of it is invasive, with potential risk for the patient, and there is limited logistical capacity for its implementation. The estimation of cardiac output is thus often based on cardiac ultrasound but its measurement is potentially biased. Starling ™ SV is a non-invasive cardiac output monitor based on thoracic bio-reactance measurement. Several clinical studies have shown that cardiac output measured by bio-reactance is strongly correlated with the measurement obtained by catheterization in different indications. Rich et al. (2013) assessed bio-reactance specifically in 50 patients with Pulmonary Hypertension compared with thermodilution. The results showed that the measurement of cardiac output by bio-reactance was feasible in these patients, had a better accuracy than by catheterization, and was reliable for detecting changes in cardiac output after a vasodilatation test. The Starling ™ SV monitor could thus have a place in the follow-up of patients who are being treated for Pulmonary Hypertension. Since it is a rapid, non-invasive examination, it could be performed on an outpatient basis, especially as a first-line test to check the stability of the CO, thus avoiding the systematic use of right heart catheterization, which would then be reserved only to patients for whom the measurement by bio-reactance would show a decrease of cardiac output compared to the previous value. Before being able to propose this use in current practice, it is necessary to carry out a pilot study which will make it possible to explore the interest of bio-reactance in this situation.
Evaluation of Echocardiographic Indices and Blood Biomarkers in Group 1 Pulmonary Hypertension
Pulmonary HypertensionTo evaluate different echocardiographic indices in diagnosis and follow up of group 1 pulmonary hypertension. To evaluate blood biomarkers (troponin, uric acid and micro RNA) in naïve group 1 pulmonary hypertension.