Pulmonary artery coming from the aorta

Overview

Type of disease: Rare conditions

Pulmonary artery coming from the aorta (PACA) is a heart malformation characterized by uncommon origin of the lung arteries coming from the aorta (the main artery in the human body) and the outflow tract where air comes out. PACA is found in the newborns where it usually results in respiratory distress, heart failure and failure to survive within the first days/months of life. Infants may present with chest pain with exertion. Two forms may be distinguished: anomalous origin of the left pulmonary artery (AOLPA; about 25% of cases) and anomalous origin of the right pulmonary artery from the aorta (AORPA; about 75% of cases).

Patients treated early in life have good short and long-term outcome. If left untreated, the disease may lead to cardiac failure and pulmonary hypertension, with 30% of infants dying within 3 months.

Pulmonary artery coming from the aorta incidence is estimated at 0.1% of all heart problems present at birth and around 200 cases have been described in the world literature.

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