Clinical trial NCT02249806: Zürich Pulmonary Hypertension Outcome Assessment Cohort
Official title: Zürich Pulmonary Hypertension Outcome Assessment Cohort
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Pulmonary Hypertension Outcome Assessment
Keywords: Pulmonary Hypertension
Chronic thromboembolic pulmonary hypertension
Pulmonary arterial hypertension
Outcome
Lead sponsor: University of Zurich
Sponsor class: OTHER
First posted: 2014-09-26
Enrollment: 100
Sex: ALL
Minimum age (years): 18
Maximum age (years): 90
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patients with precapillary pulmonary hypertension classified according to WHO

Brief summary: 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: 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.

Eligibility criteria: 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.)

Primary outcomes:
measure: Survival
timeFrame: 10 years
measure: Change in the 6 minute walk distance
timeFrame: 10 years
Secondary outcomes:
measure: New York Heart association (NYHA) functional class
timeFrame: 10 years
measure: Score in health related Quality of Life (HRQoL) questionnaires
timeFrame: 10 years
measure: Hemodynamic parameters by echocardiography
timeFrame: 10 years
measure: Hospital days
timeFrame: 10 years
measure: red- and white blood cell counts
timeFrame: 10 years
measure: Partial pressures of oxygen and carbon dioxide, pH, bicarbonate, electrolytes
timeFrame: 10 years
measure: Score on Short-Form-6 D, Euro-Qol 5 D (EQ-5 D), visual analogue scale, time trade-off, standard gamble test
timeFrame: 10 years
measure: Overnight sleep study parameters
timeFrame: 10 years
measure: Score in cognitive function tests
timeFrame: 10 years
measure: Cardiopulmonary exercise test parameters
timeFrame: 10 years
measure: ECG parameters
timeFrame: 10 years
measure: Motion, step counts, metabolic equivalent
timeFrame: 10 years
measure: Level of tissue oxygenation and blood volume index of prefrontal and quadriceps muscle tissue
timeFrame: 10 years
measure: Time, maximal power in stair-ascent test
timeFrame: 10 years
measure: Performance in Sit to stand test
timeFrame: 10 years
measure: Level of C-reactive protein (CRP)
timeFrame: 10 years
measure: Level of N-terminal brain natriuretic peptide (BNP)
timeFrame: 10 years
Other outcomes:
Locations:
city: Zurich
country: Switzerland
facility: University Hospital Zürich
Study officials:
affiliation: University Hospital Zurich, Department of Pulmonology
name: Silvia Ulrich, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Saxer S, Lichtblau M, Berlier C, Hasler ED, Schwarz EI, Ulrich S. Physical activity in incident patients with pulmonary arterial and chronic thromboembolic hypertension. Lung. 2019 Oct;197(5):617-625. doi: 10.1007/s00408-019-00248-x. Epub 2019 Jul 1.