What Is Unilateral Absence of a Pulmonary Artery?
Source: Genetic and Rare Diseases (GARD) Information Center
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Unilateral Absence of a Pulmonary Artery
UAPA; Congenital absence of the pulmonary artery
Unilateral absence of the pulmonary artery (UAPA) is a heart defect that is present from birth. The pulmonary artery takes blood from the heart to the lungs. In the absence of a pulmonary artery, other blood vessels compensate by supplying blood to the lungs.
3D printed pulmonary arteries
Image by Jan Witowski/Wikimedia
Chest X-ray showing left pulmonary agenesis with mediastinal shift and right lung hyperinflation
Image by /Wikimedia
Source: Genetic and Rare Diseases (GARD) Information Center
Surgery in operating room
Image by sasint/Pixabay
Source: Genetic and Rare Diseases (GARD) Information Center
Prognosis Icon
Image by mcmurryjulie/Pixabay
There is very limited data regarding long-term outcomes of treated infants with isolated UAPA. Most of the information available on this topic is individual case reports. These individual cases often report successful outcomes. The estimated overall mortality rate across all age groups for people with UAPA and pulmonary hypertension is 7%. Infantswith severe pulmonary hypertension tend to have a poorer outcome, thanthose whose symptoms develop later in life.
Source: Genetic and Rare Diseases (GARD) Information Center
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