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

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

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).

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.