Zürich Pulmonary Hypertension Outcome Assessment Cohort (ZHPHCohort)

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.

Study Overview

• Status: Recruiting
• Conditions: Pulmonary Hypertension Outcome Assessment
• Intervention / Treatment: Other: PH target therapy according to physician

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.

• Study Type: Observational
• Enrollment (Anticipated): 100

Contacts and Locations

• Study Contact: Name: Silvia Ulrich, MD; Phone Number: ++41442552220; Email: pulmonalehypertonie@usz.ch
• Study Contact Backup: Name: Isabel Schmied; Phone Number: ++41442552220; Email: pulmonalehypertonie@usz.ch

Study Locations

• Switzerland
  • Zurich, Switzerland, 8091
    • Recruiting
    • University Hospital Zurich
    • Contact: Silvia Ulrich, PD MD; Phone Number: 0041 44 255 22 20; Email: silvia.ulrich@usz.ch
    • Principal Investigator: Silvia Ulrich, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 90 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients with precapillary pulmonary hypertension classified according to WHO

Description

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.)

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
PH target therapy; Patients receiving PH target therapy	Other: PH target therapy according to physician; Patients receiving PH target therapy

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: University of Zurich
• Investigators: Principal Investigator: Silvia Ulrich, MD, University Hospital Zurich, Department of Pulmonology

Publications and helpful links

General Publications

• 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.

Study record dates

Study Major Dates

• Study Start: September 1, 2014
• Primary Completion (Anticipated): September 1, 2024
• Study Completion (Anticipated): September 1, 2024

Study Registration Dates

• First Submitted: September 16, 2014
• First Submitted That Met QC Criteria: September 25, 2014
• First Posted (Estimate): September 26, 2014

Study Record Updates

• Last Update Posted (Actual): November 2, 2022
• Last Update Submitted That Met QC Criteria: November 1, 2022
• Last Verified: November 1, 2022

More Information

Terms related to this study

• Keywords: Pulmonary Hypertension; Outcome; Pulmonary arterial hypertension; Chronic thromboembolic pulmonary hypertension
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension; Hypertension, Pulmonary
• Other Study ID Numbers: 2014-0214_ZH PH Cohort