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.
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.
Study Type
OBSERVATIONAL
Enrollment
100
Patients receiving PH target therapy
University Hospital Zürich
Zurich, Switzerland
RECRUITINGSurvival
Transplant free survival will be registered
Time frame: 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
Time frame: 10 years
New York Heart association (NYHA) functional class
The change in NYHA class will be registered at 3, 6, 12 months and yearly thereafter
Time frame: 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
Time frame: 10 years
Hemodynamic parameters by echocardiography
The change in echo parameters will be registered at 3, 6, 12 months and yearly thereafter
Time frame: 10 years
Hospital days
Days spent in the hospital
Time frame: 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
Time frame: 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)
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Time frame: 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
Time frame: 10 years
Overnight sleep study parameters
The change Overnight sleep study parameters will be registered at 3, 6, 12 months and yearly thereafter
Time frame: 10 years
Score in cognitive function tests
The change cognitive function will be registered at 3, 6, 12 months and yearly thereafter
Time frame: 10 years
Cardiopulmonary exercise test parameters
The change cardiopulmonary exercise test parameters will be registered at 3, 6, 12 months and yearly thereafter
Time frame: 10 years
ECG parameters
The change in ECG parameters will be registered at 3, 6, 12 months and yearly thereafter
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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)
Time frame: 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)
Time frame: 10 years