Heart and Blood Diseases

All Conditions

Respiratory Tract (Lung and Bronchial) Diseases

Symptoms and General Pathology

Blood and Lymph Conditions

Rare Diseases

Hypertension

Hypertension, Pulmonary

Philadelphia Chromosome

Myeloproliferative Disorders

Vascular Diseases

Lung Diseases

Respiratory Tract Diseases

Bone Marrow Diseases

Hematologic Diseases

Chronic Myeloproliferative Disorders

Only a small subsample will require CT and right heart catherisation

Radiology

Mette Brabrand, MD

This is an observational prospective study aiming to clarify the prevalence of pulmonary hypertension in patients with Myeloproliferative Neoplasms and their prognosis.

All patients attending our department with the above mentioned neoplasms will be offered inclusion in this study. All will have an echo performed and patients identified as being at risk of pulmonary hypertension will be offered complete investigation as specified by the European Cardiology Association.

All patients will be followed up for a total of five years to identify prognosis.

All patients will submit a blood sample for later analysis

Pulmonary Hypertension in Patients With Myeloproliferative Neoplasms

Pulmonary Hypertension in Patients With Philadelphia Chromosome Negative Myeloproliferative Neoplasms; an Observational Prospective Study

Odense University Hospital

Staff Specialist

Neoplasms

Immune System Diseases

Lymphangioleiomyomatosis

Lymphangiomyoma

Neoplasms by Histologic Type

Perivascular Epithelioid Cell Neoplasms

Neoplasms, Connective and Soft Tissue

Lymphoproliferative Disorders

Lymphatic Diseases

Immunoproliferative Disorders

Vincent Cottin, MD

This is a descriptive study of patients with Lymphangioleiomyomatosis and precapillary pulmonary hypertension.

Descriptive study of patients with Lymphangioleiomyomatosis and precapillary pulmonary hypertension This will include modality of diagnosis of lLymphangioleiomyomatosis, results of right-sided heart catheterization, and pulmonary function tests

Pulmonary Hypertension in Lymphangioleiomyomatosis

Observational Study of Patients With Lymphangioleiomyomatosis and Pulmonary Hypertension

Hospices Civils de Lyon

Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires

Pr Cottin, Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires

Digestive System Diseases

Diseases and Abnormalities at or Before Birth

Fibrosis

Cystic Fibrosis

Pancreatic Diseases

Gastrointestinal Diseases

Genetic Diseases, Inborn

Infant, Newborn, Diseases

Newly developed waterproof catheter dressing may be used for bathing activities

No specific dressing will be provided to the patient to protect the catheter entry site during bathing activities.

Shower patch will be available for the patient for bathing activities

Shower patch IV catheter protection

No Shower patch will be provided during study period

Conventional IV catheter protection

Godelieve A Goossens, PhD

Venous catheters are necessary for the treatment of many patients. Showering with a venous catheter is often prohibited due to the infection risk when the insertion site becomes wet. Therefore these patients are challenged to keep the catheter insertion site dry and always covered with a dressing. Washing themselves is often impossible without assistance of a nurse or significant other. For patients with a catheter connected to an infusion line, it is even more difficult. Showerpatch is a newly developed dressing that safeguards the insertion site of an IV catheter from water during bathing activities. The purpose of this trial is to evaluate the impact of the availability of Showerpatch by comparing the outcomes in patients regarding the patient's autonomy in bathing activities, the material use and the time needed from caregivers in home care. Additionally qualitative data on patient's bathing activities and the use of Showerpatch will be collected.

Impact of Availability of Showerpatch for Patients With Intravenous Catheters

Comparison of Showerpatch Versus Other Measures to Protect Intravenous Catheter Entry Site During Bathing Activities

Redness or other sign of skin irritation at the place where adhesive of the new protection dressing had contact with the skin on a yes/no basis

Expressed on a 3-point scale from 0 which means correct in place to 2 totally removed

autonomy regarding bathing activities will be scored on a newly developed autonomy scale on a weekly basis

Number and type of bathing activities per week

Expressed on a 3-point scale from 0 which means not wet to 2 totally wet

Expressed on a 3-point scale from 0 which means not loose to 2 totally loose

Description of material which was used for catheter dressing protection and/or for the extra dressing change and/or extra securement of the dressing afterwards.

Redness, tenderness, warmth, swelling or pus leakage recorded on a yes/no basis

Collected at the end of the study by retrospective analysis of the patient file in case of hospital admission

BEDAL

Remedus

Universitaire Ziekenhuizen KU Leuven

Leukemia

Leukemia, Myeloid

Leukemia, Myelogenous, Chronic, BCR-ABL Positive

Chronic Disease

Disease Attributes

Chronic Graft Versus Host Disease

Myeloid Leukemia

Chronic Myeloid Leukemia

All Drugs and Chemicals

Antineoplastic Agents

Nilotinib

Dasatinib

echo, exercise echo, and if indicated, right heart catheter

routine echocardiography and special measurements of the right heart are performed at rest and during exercise

Echo, exercise echo, and if indicated, right heart catheter

Horst Olschewski, MD

Chronic myelogenous leukemia (CML) is a chronic myeloproliferative disorder characterized by a translocation between chromosome 9 and 22, leading to a pathogenic tyrosine kinase signal transduction protein. CML can be treated with tyrosine kinase inhibitors (TKIs), which inhibit BCR/ABL kinase, such as imatinib. In about 20% of CML patients who are treated by imatinib, a complete cytogenetic response cannot be achieved. The other two novel TKIs (dasatinib and nilotinib), achieve higher rates of complete cytogenetic response and they are proposed as second-line therapy for imatinib-resistant patients or for those who do not tolerate imatinib. Dasatinib inhibits BCR/ABL kinase in about \>300 times in vitro in more than imatinib and also inhibits several other kinases, including the Src family. Src tyrosine kinase is crucial for potassium channel function in human pulmonary arteries. Imatinib and nilotinib do not inhibit the Src.

Incident cases of precapillary PH have been reported in patients who have CML treated with the dasatinib. Improvements were usually observed after withdrawal of dasatinib.

This study is designed to identify incident cases of dasatinib-associated PH and describe pulmonary vascular changes induced by dasatinib. As comparison population will be patients who receive another second-line TKI (nilotinib).

Doppler echocardiography at rest will be performed in each patient. Patients without exercise capacity limitation an exercise test (Doppler echocardiography with spiroergometry) will be performed. Patients who show elevated SPAP at rest or during exercise (in this study SPAP ≥ 40 mmHg) or with reduced exercise capacity (peak VO2 \< 75%) a right heart catheterization (RHC) will be suggested. Additionally for the evaluation of exercise capacity a 6 MWD will be performed. This work- up of patients allows clinical and hemodynamic evaluation.

Samples with DNA will be retained for later examinations at the Biobank, in case that the patient agrees (extra patient information). The blood samples are taken only during routine tests.

Pulmonary Vasculopathy Under Second-line Therapy of Chronic Myeloid Leukemia

Pulmonary Vascular Changes in Patients With Chronic Myeloid Leukemia With Second-line Therapy Dasatinib vs. Nilotinib

In patients who undergo stressechocardiography: systolic pulmonary arterial pressure (SPAP) at 50W will be measured and the comparison between patients under dasatinib and nilotinib therapy will be performed.

Mean PAP at rest, mPAP at 50W, peak VO2, 6 minute walk distance (6MWD), N terminal pro brain natriuretic peptide (NT-proBNP) at "dasatinib" vs."nilotinib" patients.

Changes of SPAP at 50 W, pulmonary vascular resistance (PVR) at rest, changes of mPAP at rest and at 50W, peak VO2, 6 MWD, NT-pro BNP- in patients with dasatinib and nilotinib between the baseline and 6 months after.

Changes of SPAP at 50 W, pulmonary vascular resistance (PVR) at rest, changes of mPAP at rest and at 50W, peak VO2, 6 MWD, NT-pro BNP- in patients with dasatinib and nilotinib between the baseline and 6 months after.

In patients who undergo a RHC: pulmonary vascular resistance (PVR) at rest will be measured and the comparison of patients with dasatinib and nilotinib therapy will be performed.

Medical University of Graz

Infections

Nervous System Diseases

Ear, Nose, and Throat Diseases

Occupational Diseases

Behaviors and Mental Disorders

Wounds and Injuries

Communicable Diseases

Lung Diseases, Obstructive

Pulmonary Disease, Chronic Obstructive

Asthma

Lung Neoplasms

Hypersensitivity

Apnea

Sleep Apnea Syndromes

Sleep Apnea, Obstructive

Pulmonary Embolism

Embolism

Pulmonary Fibrosis

Pulmonary Emphysema

Emphysema

Respiratory Tract Infections

Lung Diseases, Interstitial

Mesothelioma

Mesothelioma, Malignant

Virus Diseases

Bronchiectasis

Rhinitis, Allergic, Seasonal

Asbestosis

Syndrome

Respiration Disorders

Parasomnias

Sleep Disorders, Intrinsic

Dyssomnias

Sleep Wake Disorders

Thrombosis

Embolism and Thrombosis

Adenoma

Neoplasms, Glandular and Epithelial

Neoplasms, Mesothelial

Bronchial Diseases

Pneumoconiosis

Lung Injury

Malignant Mesothelioma

Coal Worker's Pneumoconiosis

Central Nervous System Depressants

Xenon

Participants will inhale investigational gases (HP 3He, HP 129Xe, PFP and SF6) according to the procedure for that intervention. All participants can potentially inhale all the gases. Magnetic resonance imaging will be performed during breath-holds or continuous breathing (gas-dependent) with the appropriate investigational human lung coil (3He Human Lung Coil, 129Xe Small and Large Human Lung Coil, or PFP and SF6 Human Lung Coil), using the 3T Research MRI at the Thunder Bay Regional Health Sciences Centre.

3He will be hyperpolarized using a custom-built polarizer to enhance its MRI signal. Hyperpolarization does not change the chemical or physical properties of helium gas.

HP 3He

129Xe will be hyperpolarized using a Xemed polarizer to enhance its MRI signal. Hyperpolarization does not change the chemical or physical properties of xenon gas.

HP 129Xe

Perfluoropropane (PFP) will be premixed and delivered to the participants with no further processing.

Sulfur hexafluoride (SF6) will be premixed and delivered to the participant with no further processing.

The investigational 129Xe small and large quadrature lung coils are sensitive to 129Xe resonant frequencies and therefore will be paired with the investigational drug 129Xe. The size of the coil used will depend on the participants' body size.

129Xe Small and Large Human Lung Coil

The investigational 3He quadrature lung coil is sensitive to 3He resonant frequencies and therefore will be paired with the investigational drug 3He.

3He Human Lung Coil

The investigational PFP and SF6 quadrature lung coil is sensitive to inert fluorinated gas resonant frequencies and therefore will be paired with both PFP and SF6.

PFP and SF6 Human Lung Coil

Mitchell S Albert, PhD

Yurii Shepelytskyi, MSc

Birubi Biman, MD

Karl Granberg, MD

Yurii Shepelytskyi, PhD

The goal of this research is to optimize the MRI system to obtain ideal lung images using Hyperpolarized (HP) Noble and Inert Fluorinated Gases as contrast agents. Lung coils tuned to the frequencies of these gases will be used. This study will take place at TBRHSC in the Cardiorespiratory Department and in the Research MRI facility.

MR images of the lung obtained using HP 3He, HP 129Xe, and inert fluorinated gases essentially show the distribution of the inhaled gas inside the lungs. Measurements can be performed on these images to provide additional information that can be related to pulmonary function.

Participation will involve at least two study visits. During the first visit, participants will provide written, informed consent, and undergo eligibility screening. Study drug administration, use of the investigational devices, and MR imaging for this study will be performed at the 3T Research MRI at the Thunder Bay Regional Health Sciences Centre (TBRHSC). Pulmonary Function Tests (PFTs) will be performed at TBRHSC by a Registered Respiratory Therapist (RRT) and an MR Tech will be present for the duration of participant scans.

Participants will be placed in the MRI with the appropriate chest coil wrapped around them like a vest. Several inhalation procedures will be carried out during scanning. Participants safety will be closely monitored throughout the entire study.

Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders

Developing Optimal Parameters for Hyperpolarized Noble Gas (3He and 129Xe) and Inert Fluorinated Gas Magnetic Resonance Imaging of Lung Disorders

The primary objective of this study is to determine an optimized set of scan parameters that will produce clear, anatomically and clinically relevant images of the lungs for healthy participants and various types of lung disorder participants, and using one or more gas type (3He, 129Xe, PFP, SF6).

The secondary objective of this study is to correlate the results with PFTs, including spirometry and plethysmography.

Thunder Bay Regional Health Sciences Centre

St. Joseph's Care Group

Lakehead University

Thunder Bay Regional Health Research Institute

Carcinoma, Non-Small-Cell Lung

Esophageal Cancer

CT Muijs, MD PhD

P van Luijk, DR

Karin Haustermans, Prof. Dr.

J Bussink, Prof, Dr

Gill McColl, Msc

Radiotherapy improves locoregional control and survival of thoracic tumour patients. However, the associated exposure of normal tissues, often leads to side effects and possibly even reduces survival. Indeed, there is growing evidence that overall survival after radiotherapy for lung and oesophageal cancer is related to the radiation dose to heart and lungs. This suggests that thoracic radiotherapy causes mortality, which is currently not recognized as radiation-induced toxicity. So the question arises how to explain this treatment-related mortality.

Interestingly, Ghobadi et al demonstrated in rats that thoracic irradiation can lead to pulmonary hypertension (PH). Histopathological analysis showed that radiation-induced PH closely resembles the pulmonary arterial hypertension (PAH) subtype. Moreover, in a clinical pilot study we confirmed early signs of PH including dose-dependent reductions in blood flow towards the lungs in radiotherapy patients.

In general PH significantly affects survival. Moreover, the PAH subtype is the most-rapidly progressive and lethal subtype. However, medical treatment can significantly slow down PAH progression, providing opportunities for secondary prevention. Yet, hard evidence that radiation-induced PH is a clinically relevant phenomenon in patients treated for thoracic tumours, is lacking.

In the present study, the incidence and time course of treatment-related changes in cardio-pulmonary physiology will be assessed using standard diagnostic tools such as echocardiography, cardiac MRI (CMR) and serum biomarkers and relate them to the radiation dose distribution. Such insight in the characteristics of this possible radiation-induced PH and contributing risk factors is essential to develop primary (radiation dose optimization) prevention strategies.

The general objective of this study is to test the hypothesis that pulmonary hypertension (PH) is a clinically relevant radiation-induced side effect of thoracic irradiation. If confirmed this allows us to take appropriate measures in patient care to improve quality of life in thoracic cancer patients.

To investigate this hypothesis, the following specific aims have been defined:

* To assess the incidence and time course of PH in a prospective cohort study in patients treated with radiotherapy for lung or oesophageal cancer.
* To characterize other changes in myocardial function and pulmonary arteries, and their function using cardiac MR.
* To determine treatment-related risk factors, in particular radiation dose factors to the lungs and heart that could be used for future optimization strategies to minimize the risk of inducing PH in these patients.
* To determine the clinical impact by correlating PH to patient-rated outcome measure (PROMs) and survival. Taken together this study will determine if radiation-induced pulmonary hypertension is a clinically relevant toxicity and will provide information required for future studies on its prevention and treatment. In addition, more insight will be obtained on other forms of cardiovascular damage and complications that may occur in these patients.

Cardiopulmonary Toxicity of Thoracic Radiotherapy

High-risk pulmonary hypertension according to ESC/ERS classification

Change in Troponine T concentration, between baseline and at 1 year

Change in NTproBNP concentration, between baseline and at 1 year

Intermediate risk of pulmonary hypertension according to ESC/ERS classification

Cumulative incidence of other late cardiopulmonary toxicity, as classified by CTCAE4.0

PROMs (EORTC QoL C30), including five functional scales (physical, role, cognitive, emotional, and social), three symptom scales (fatigue, pain, and nausea and vomiting),

PROMs (EORTC QoL LC13), including lung cancer-associated symptoms (cough, haemoptysis, dyspnoea and site specific pain), treatment-related side effects (sore mouth, dysphagia, peripheral neuropathy and alopecia) and pain medication.

University Medical Center Groningen

Natriuretic Agents

Natriuretic Peptide, Brain

Nesiritide 0.01 mcg/kg/min by vein over 30 minutes during right heart catheterization procedure.

Given initially as a bolus (2 mcg/kg) and than infused for 30 minutes (0.01 mcg/kg/min) during right heart catheterization.

Nesiritide (Natrecor)

Small tubes placed in neck vein to heart as means of measuring pressure in heart and lungs; treatment with nesiritide delivered during procedure.

Right Heart Catheterization

Daniel J. Lenihan, MD

The goal of this clinical research study is to learn if the drug nesiritide (Natrecor) is effective in lowering the pressure in your lungs.

The primary objective of this study is to establish that Nesiritide (Natrecor) is effective in reducing pulmonary hypertension (PHTN) acutely as measured by a 20% reduction in the mean pulmonary arterial (PA) pressure. The secondary objectives will include: improvement in pulmonary vascular resistance (PVR), patient symptoms, exercise tolerance, frequency of toxicity, and surgeon's willingness to proceed with operation.

Patients with high pressure in their lungs often have many symptoms such as, shortness of breath, low energy, and fatigue. Decreasing the pressure in the lungs may help these patients feel better. The drug nesiritide was designed to help treat heart failure, however, it may also help to decrease the pressure in the lungs.

Before treatment, you will be asked questions about your medical history and about any medications you are currently taking. You will have a focused physical exam. You will have an electrocardiogram (ECG - a test that measures the electrical activity of the heart). You will have an ultrasound on your heart called an echocardiogram to measure the pressures in your lungs and your ability to breathe will be evaluated. With the aid of the research nurse, you will have a 6-minute walk test if you are able. You may stop or sit down at any time during the test. This test is being done to evaluate your energy level. You will also be asked to complete a questionnaire about your symptoms. This questionnaire will take no longer than 10 minutes to complete.

In order to measure the pressure in your lungs, you will have a procedure called a "right heart catheterization". This is a procedure that may have been done as part of your standard of care if you were not enrolled in this study. For this procedure, a small tube will be placed in a vein in your neck. A longer tube will be inserted into the first tube and fed through the vein and into your heart. This tube will be used to measure the pressure in your heart and lungs. If the pressure in your lungs is above a certain level, you will receive treatment with nesiritide during the right heart catheterization procedure. Nesiritide will be given by vein over 30 minutes. After treatment, the pressure in your heart and lungs will be measured again. You will be awake during this procedure and lying flat on your back. An anesthetic will be used to numb the area of your neck where the tube is placed. The entire procedure (including treatment) should take no longer than 2 hours. When complete, the tubes will be removed.

Within 15 minutes of the end of the procedure, the 6-minute walk test (if you are able) and the questionnaire about your symptoms will be repeated and your breathing will be reassessed.

You will also have around 2 teaspoons of blood collected for special lab tests. These tests are being done to check for certain molecules in your blood. The blood that is leftover after these tests may be stored in a freezer.

Around 30 days after the procedure, you will have a follow-up visit scheduled or contacted by phone. At this visit, you will have a complete physical exam done by the doctor, and possibly have blood work done if required by the doctor as part of your routine care. There will be no additional blood work done for study purposes.

This is an investigational study. Nesiritide is FDA approved and is commercially available only for the treatment of heart failure. However, the use of nesiritide in this study is experimental. Around 20 patients will be enrolled at M. D. Anderson.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Pulmonary Hypertension in Patients With Myeloproliferative Neoplasms	NCT02580903	Myeloproliferat... Pulmonary Hyper...	Radiology	18 Years -	Odense University Hospital
Pulmonary Hypertension in Lymphangioleiomyomatosis	NCT00960895	Lymphangioleiom... Pulmonary Hyper...		18 Years -	Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires
Pulmonary Hypertension in Patients With Myeloproliferative Neoplasms	NCT02580903	Myeloproliferat... Pulmonary Hyper...	Radiology	18 Years -	Odense University Hospital
Impact of Availability of Showerpatch for Patients With Intravenous Catheters	NCT02324868	Cystic Fibrosis Pulmonary Hyper... Neoplasms	Shower patch IV... Conventional IV...	18 Years -	Universitaire Ziekenhuizen KU Leuven
Pulmonary Vasculopathy Under Second-line Therapy of Chronic Myeloid Leukemia	NCT01805843	Chronic Myeloid...	Echo, exercise ...	18 Years - 95 Years	Medical University of Graz
Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders	NCT02748798	Lung Transplant Lung Resection Lung Cancer Asthma Cystic Fibrosis Chronic Obstruc... Emphysema Mesothelioma Asbestosis Pulmonary Embol... Interstitial Lu... Pulmonary Fibro... Bronchiectasis Seasonal Allerg... Cold Virus Lung Infection Pulmonary Hyper... Pulmonary Dyspl... Obstructive Sle...	HP 3He HP 129Xe PFP SF6 129Xe Small and... 3He Human Lung ... PFP and SF6 Hum...	18 Years -	Thunder Bay Regional Health Research Institute
Pulmonary Hypertension in Lymphangioleiomyomatosis	NCT00960895	Lymphangioleiom... Pulmonary Hyper...		18 Years -	Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires
Cardiopulmonary Toxicity of Thoracic Radiotherapy	NCT03978377	Oesophageal Can... Non Small Cell ...		18 Years -	University Medical Center Groningen
Natrecor in Pulmonary Hypertension	NCT00075179	Pulmonary Hyper... Cancer Lung Disease Cardiothoracic ...	Nesiritide (Nat... Right Heart Cat...	18 Years - 85 Years	M.D. Anderson Cancer Center
Pulmonary Vasculopathy Under Second-line Therapy of Chronic Myeloid Leukemia	NCT01805843	Chronic Myeloid...	Echo, exercise ...	18 Years - 95 Years	Medical University of Graz
Natrecor in Pulmonary Hypertension	NCT00075179	Pulmonary Hyper... Cancer Lung Disease Cardiothoracic ...	Nesiritide (Nat... Right Heart Cat...	18 Years - 85 Years	M.D. Anderson Cancer Center

Spots Global Cancer Trial Database for pulmonary hypertension