Long-Term Follow-Up Comparing Health and Quality of Life in Patients With Pulmonary Atresia With Intact Ventricular Septum Having Uni- or Biventricular Circulation

What this Trial Is About

Researchers are comparing long-term outcomes in patients born with pulmonary atresia with intact ventricular septum PA-IVS who have either univentricular or biventricular circulation. The study aims to understand differences in mortality, quality of life, comorbidities, cardiac function, and work capacity between these two groups. This observational study is sponsored by Vastra Gotaland Region and seeks to provide insight into how the heart condition and type of circulation affect patients over time. Participants fall into two groups those with biventricular circulation and those with univentricular circulation. Participants will be invited to complete a Quality of Life questionnaire. A sample of participants, selected based on their place of residence, will be asked to undergo additional tests including ergo-spirometry to measure work capacity, transthoracic echocardiogram, cardiac magnetic resonance imaging, and blood sampling to assess heart function and health. During the study, researchers will review data on comorbidities, quality of life, physical and cardiac capacity, general health related to heart function, and mortality. The main outcome of interest is the difference in comorbidity between the two groups as of September 2024. The study began in July 2024 and will continue through January 2026, with participants contributing information through questionnaires and clinical evaluations during this period.

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