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).
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.
None
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.
Cohort
Cross-sectional
Not listed
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
Difference in quality of life between the two groups
A validated quality of life questionnaire (PROMIS Scale v1.2-Global Health) will be sent to all the research subjects and the answers will be compared between the two groups. The questionnaire consists of a ten-item patient reported measure of physical, mental and social health. Each statement is answered on a scale from 1-5, higher scores mean better outcome.
Difference in physical capacity between the two groups
Ergospirometry, outcome variables: exercise test (exercise capacity measured in watt and over time, counted in minutes), oxygen saturation (measured in percent), blood pressure (measured in millimetre of mercury)
Difference in cardiac capacity between the two groups
Transthoracic echocardiogram
Difference in general health, linked to cardiac health, between the two groups
The following blood tests will be analysed: N-terminal pro-brain natriuretic peptide (a marker for heart failure), Alanine aminotransferase (a liver enzyme), Aspartate aminotransferase (a liver enzyme), Creatinine (a waste product from muscle metabolism, used to assess kidney function), Sodium (an electrolyte), Potassium (an electrolyte), Hemoglobin (a protein in red blood cells that carries oxygen), White blood cell count (Leukocyte count), Platelet count (Thrombocyte count), Albumin: A protein produced by the liver that helps maintain fluid balance in the body, TREC (T-receptor excision circles): A marker used to assess immune function, specifically the production of new T cells, Expanded lymphocyte typing (a comprehensive examination of lymphocyte subtypes)
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