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

Vastra Gotaland Region

Status

Enrolling

Conditions

Pulmonary Atresia With Intact Ventricular Septum

Study type

Observational

Funder types

Other

Identifiers

NCT05928234

PA-IVS, 2023

Details and patient eligibility

About

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).

Enrollment

140 estimated patients

Sex

All

Ages

15+ years old

Volunteers

No Healthy Volunteers

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.