Clinical trial NCT05928234: A Comparison of Morbidity Between Patients With Pulmonary Atresia With Intact Ventricular Septum and Uni- or Biventricular Circulation
Official title: A Longterm Follow-up and Comparison of Morbidity and Heart Health Between Patients With Pulmonary Atresia With Intact Ventricular Septum and Uni- or Biventricular Circulation
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Pulmonary Atresia With Intact Ventricular Septum
Lead sponsor: Vastra Gotaland Region
Sponsor class: OTHER_GOV
First posted: 2023-07-03
Enrollment: 140
Sex: ALL
Minimum age (years): 15
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: CROSS_SECTIONAL
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.

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).


Eligibility criteria: Inclusion Criteria:

* 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.

Exclusion Criteria:

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

Primary outcomes:
measure: Difference in comorbidity between the two groups
timeFrame: September 2024
Secondary outcomes:
measure: Difference in quality of life between the two groups
timeFrame: September 2024
measure: Difference in physical capacity between the two groups
timeFrame: September 2024
measure: Difference in cardiac capacity between the two groups
timeFrame: September 2024
measure: Difference in general health, linked to cardiac health, between the two groups
timeFrame: September 2024
measure: Difference in mortality between the groups
timeFrame: September 2024
Other outcomes:
Locations:
city: Gothenburg
country: Sweden
facility: Pediatric Heart Center, Queen Silvia's Children's hospital
Study officials:
References: