A Comparison of Morbidity Between Patients With Pulmonary Atresia With Intact Ventricular Septum and Uni- or Biventricular Circulation

NCT05928234 | Recruiting

• 1 other identifier: PA-IVS, 2023
• Study Type: observational
• Target: 140
• Locations: 1 country
• Sites: 1

Brief Summary

The goal of this observational study is to compare patients with pulmonary atresia with intact ventricular septum (PA-IVS) with univentricular circulation to patients with the same heart defect but that has a biventricular circulation in regards to mortality, quality of life, comorbidity, cardiac function, and work capacity. The main questions the study aims to answer are: • Do mortality, quality of life, comorbidity, cardiac function, and work capacity differ between patients with PA-IVS who have univentricular and biventricular circulation? Participants will be asked to answer a Quality of Life questionnaire. The investigators will also inquire with a sample size of the research subjects (based on place of residence) about their participation in a series of examinations (ergo-spirometry to assess work capacity, transthoracic echocardiogram, magnetic resonance of the heart and blood sampling to assess cardiac function and cardiac health).

Conditions

Pulmonary Atresia With Intact Ventricular Septum

Outcome Measures

Primary Outcomes (1)

• Difference in comorbidity between the two groups

  Difference in myocardial infarction, heart failure, arrhythmia, stroke, malignancy, autoimmune diseases, infections, atopy, certain liver diseases, and protein-losing enteropathy between the two groups

  September 2024

Secondary Outcomes (5)

• Difference in quality of life between the two groups

  September 2024

• Difference in physical capacity between the two groups

  September 2024

• Difference in cardiac capacity between the two groups

  September 2024

• Difference in general health, linked to cardiac health, between the two groups

  September 2024

• Difference in mortality between the groups

  September 2024

Study Arms (2)

Biventricular circulation

Research subjects with PA-IVS who have biventricular circulation

Univentricular circulation

Research subjects with PA-IVS who have univentricular circulation

Eligibility Criteria

• Age: 15 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The study population consists of all individuals with PA-IVS who have been registered in Swedcon (a national register for patients with congenital heart defects) or in the National Patient Register and Cause of Death Register since 1980 and meet other inclusion criteria, including age.

You may qualify if:

• Individuals born with pulmonary atresia with intact ventricular septum (PA-IVS) who are 15 years or older at the start of the study.
• Regarding the mortality variable, research subjects who have died after the age of 15 years old will be included.

You may not qualify if:

• Individuals born with pulmonary atresia with intact ventricular septum who are younger than 15 years old at the start of the study.

Sponsors & Collaborators

• Vastra Gotaland Region: lead

Study Sites (1)

Pediatric Heart Center, Queen Silvia's Children's hospital

Gothenburg, 416 85, Sweden

RECRUITING

MeSH Terms

Conditions

Pulmonary Atresia with Intact Ventricular Septum

Central Study Contacts

stina manhem, MD

CONTACT

+46735080342; stina.manhem@vgregion.se

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER GOV
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: MD

Study Record Dates

• First Submitted: June 4, 2023
• First Posted: July 3, 2023
• Study Start: July 15, 2024
• Primary Completion: December 1, 2025
• Study Completion: January 1, 2026
• Last Updated: March 30, 2025

Record last verified: 2025-03

Locations

SE (1)