Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension

• Register: ClinicalTrials.gov
• Last refreshed on: 24 April 2023
• Main ID: NCT02610660
• Date of registration: 10/11/2015
• Prospective Registration: No
• Primary sponsor: Association for Pediatric Pulmonary Hypertension
• Public title: Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension TOPP-2
• Scientific title: Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension
• Date of first enrolment: August 2015
• Target sample size: 388
• Recruitment status: Completed
• URL: https://clinicaltrials.gov/show/NCT02610660
• Study type: Observational [Patient Registry]

Countries of recruitment

Australia, Brazil, Canada, China, Colombia, France, Germany, Hungary, Israel, Italy, Japan, Mexico, Netherlands, Poland, Saudi Arabia, Sweden, Switzerland, United States

Contacts

• Name: Dunbar Ivy, Prof
• Affiliation: Association for Pediatric Pulmonary Hypertension

Key inclusion & exclusion criteria

Inclusion Criteria:

- Patient must be an incident patient, i.e.newly diagnosed with PH

- Age at time of diagnosis is at least 3 months and less than 18 years

- Patients must present with PH belonging to one of the following categories

- Group 1 according to updated Nice clinical classification

- Group 3 according to updated Nice clinical classification

- Group 4 according to updated Nice clinical classification

- Group 5 according to updated Nice clinical classification

- PH confirmed by heart catheterisation (HC)

- At HC, the patient must present with mean pulmonary arterial pressure (PAP) of at
least 25 mmHg at rest, a pulmonary vascular resistance index (PVRi) equal to or below
3 Wood units*m^2 and mean pulmonary arterial wedge pressure (PAWP) below or equal to
15 mmHg

- In case of congenital heart disease (CHD) patients who had undergone palliative
procedure or repair to close systemic to pulmonary shunt, the diagnosis of PH must
have been confirmed by HC at least 6 months after surgery/palliative procedure took
place

- For patients with PAH-CHD open shunt, only those considered not operable due to
advanced pulmonary vascular disease are eligible

- Patients to be included into the registry, and/or their legal guardians, must give
informed consent. Where applicable patients will be asked for their written assent

- Participating sites must agree to adhere to the recommendations of the WSPH 2015 Nice,
Pediatric Taskforce.

Exclusion Criteria:

- Patients belonging to Group 2 according to updated Nice clinical classification



Age minimum: 3 Months
Age maximum: 18 Years
Gender: All

Health Condition(s) or Problem(s) studied

Hypertension, Pulmonary

Primary Outcome(s)

Transplantation [Time Frame: Over registry run-time (5.5 years)]

Death [Time Frame: Over registry run-time (5.5 years)]

Adverse events [Time Frame: Over registry run-time (5.5 years)]

Secondary Outcome(s)

Decline in 6-minute walk test (6MWT) [Time Frame: Over registry run-time (5.5 years)]

Reasons for treatment change [Time Frame: Over registry run-time (5.5 years)]

Increase in Brain Natriuretic Peptide (BNP) [Time Frame: Over registry run-time (5.5 years)]

Time to clinical worsening [Time Frame: Over registry run-time (5.5 years)]

Worsening of echocardiographic parameters (ECHO) [Time Frame: Over registry run-time (5.5 years)]

Atrial septostomy [Time Frame: Over registry run-time (5.5 years)]

Potts shunt [Time Frame: Over registry run-time (5.5 years)]

Switch in treatment [Time Frame: Over registry run-time (5.5 years)]

Decline in WHO functional class [Time Frame: Over registry run-time (5.5 years)]

Hospitalisation related to pulmonary arterial hypertension (PAH) [Time Frame: Over registry run-time (5.5 years)]

Type of treatment [Time Frame: Over registry run-time (5.5 years)]

Use/initiation of i.v./s.c. prostanoids [Time Frame: Over registry run-time (5.5 years)]

Escalation of treatment [Time Frame: Over registry run-time (5.5 years)]

Decline in Panama functional class [Time Frame: Over registry run-time (5.5 years)]

Increase in N-terminal-proBNP (NT-proBNP) [Time Frame: Over registry run-time (5.5 years)]

Secondary ID(s)

TOPP-2

Source(s) of Monetary Support

Please refer to primary and secondary sponsors