Zürich Pulmonary Hypertension Outcome Assessment Cohort

Study Overview
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Brief Summary
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PH is a serious disease with a dismal prognosis and impaired quality of life when left untreated. Reliable patient centered and prognostically relevant outcome measures are highly warranted in the field of PH. With this prospective cohort study the investigators intend to gain important information on the course of the disease and knowledge on the value of different new and already established outcome parameters.

Detailed Description
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PH is a serious disease with a dismal prognosis and impaired quality of life when left untreated. Advances in medical therapy have improved survival according to recent registries, but are associated with high healthcare costs. Most treatment trials and clinical follow-up assessments rely on the 6 minute walk distance as main outcome parameter although submaximal exercise performance reflects only one disease aspect. Reliable patient centered and prognostically relevant outcome measures are highly warranted in the field of PH. With this prospective cohort study the investigator intend to gain important information on the course of the disease and knowledge on the value of different new and already established outcome parameters. New outcomes to be assessed will be home based activity monitoring, novel physiological measures, namely cerebral and muscle tissue oxygenation at rest and during exercise testing, stair ascent on mechanograph, sit to stand test and several serum markers and micro-RNA. All these assessments will be compared with hard endpoints events (death, lung-transplantation, hospitalization for PH), and with traditional outcome measures such as NYHA functional class, 6 minute walk distance, generic quality of life, cardiopulmonary exercise tests, sleep studies, health preference (utility) and cognitive function.

Official Title
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Zürich Pulmonary Hypertension Outcome Assessment Cohort

Conditions
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Pulmonary Hypertension Outcome Assessment

Intervention / Treatment
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* Other: PH target therapy according to physician


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with precapillary pulmonary hypertension Adult male and female patients of all ages referred to our center for evaluation/treatment of PH Written informed consent by the subject after information about the research project Exclusion Criteria: Patients with severe concomitant left heart disease (left ventricular ejection fraction <40%). Patients with restrictive lung disease (FVC<60% predicted); obstructive lung disease (forced expiratory volume (FEV) <60% predicted, with FEV 1/FVC<70%). Patients with any other disease limiting their ability to move (skeletal disease, neurological disease, etc.)

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| PH target therapy<br>Patients receiving PH target therapy | Other: PH target therapy according to physician<br>* Patients receiving PH target therapy<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Survival | Transplant free survival will be registered | 10 years | 
| Change in the 6 minute walk distance | The change in 6 minute walk distance will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| New York Heart association (NYHA) functional class | The change in NYHA class will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Score in health related Quality of Life (HRQoL) questionnaires | The change in HRQoL class will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Hemodynamic parameters by echocardiography | The change in echo parameters will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Hospital days | Days spent in the hospital | 10 years | 
| red- and white blood cell counts | The change in blood cell count will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Partial pressures of oxygen and carbon dioxide, pH, bicarbonate, electrolytes | The change in arterial blood gas parameters taken from radial artery will be registered at 3, 6, 12 months and yearly thereafter, (ABL '90Flex'-blood gas analyzer, Radiometer, Switzerland) | 10 years | 
| Score on Short-Form-6 D, Euro-Qol 5 D (EQ-5 D), visual analogue scale, time trade-off, standard gamble test | The change in utility will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Overnight sleep study parameters | The change Overnight sleep study parameters will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Score in cognitive function tests | The change cognitive function will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Cardiopulmonary exercise test parameters | The change cardiopulmonary exercise test parameters will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| ECG parameters | The change in ECG parameters will be registered at 3, 6, 12 months and yearly thereafter | 10 years | 
| Motion, step counts, metabolic equivalent | The change in Activity assessment parameters will be registered at 3, 6, 12 months and yearly thereafter with actigraphy | 10 years | 
| Level of tissue oxygenation and blood volume index of prefrontal and quadriceps muscle tissue | Changes in cerebral and muscle tissue oxygenation will be assessed after 3, 6, 12 months and yearly thereafter by Near-infrared-Spectroscopy | 10 years | 
| Time, maximal power in stair-ascent test | Changes in performance of stair ascent test will be assessed after 3, 6, 12 months and yearly thereafter | 10 years | 
| Performance in Sit to stand test | Changes in sit- to stand test will be assessed after 3, 6, 12 months and yearly thereafter | 10 years | 
| Level of C-reactive protein (CRP) | The change in CRP will be registered at 3, 6, 12 months and yearly thereafter, taken from venous blood sample, measured by immunoassay ('Immulite 2000'; 'DPC'; Los Angeles; USA) | 10 years | 
| Level of N-terminal brain natriuretic peptide (BNP) | The change in BNP will be registered at 3, 6, 12 months and yearly thereafter, taken from venous blood sample, measured by Roche Modular Systems (Roche; Switzerland) | 10 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary Hypertension, Chronic thromboembolic pulmonary hypertension, Pulmonary arterial hypertension, Outcome