Aortopulmonary septal defect is a rare

congenital heart disorder A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular ...

accounting for only 0.1-0.3% of congenital heart defects worldwide. It is characterized by a communication between the aortic and pulmonary arteries, with preservation of two normal semilunar valves. It is the result of an incomplete separation of the aorticopulmonary trunk that normally occurs in early

fetal development Prenatal development () includes the development of the embryo and of the fetus during a viviparous animal's gestation. Prenatal development starts with fertilization, in the germinal stage of embryonic development, and continues in fetal devel ...

with formation of the spiral septum.Burakovsky, V. I., Falkovsky, G. E., & Ivanitsky, A. V. (1984). Surgical repair of truncus arteriosus. ''Pediatric cardiology'', ''5''(2), 111-114. Aortopulmonary septal defects occur in isolation in about half of cases, the remainder are associated with more complex heart abnormalities.

Causes

Diagnosis

Subtypes

There are numerous types, differentiated by the extent of the defect. These types are: * Type I: simple defects leading to communication between the ascending aorta and pulmonic trunk * Type II: defects that extend to the origin of the right pulmonary artery * Type III: anomalous origin of the right pulmonary artery from the ascending aorta It is also classified as simple or complex. Simple defects are those that do not require surgical repair, occur with no other defects, or those that require minor stright-forward repair (

ductus arteriosus The ''ductus arteriosus'', also called the ''ductus Botalli'', named after the Italian physiologist Leonardo Botallo, is a blood vessel in the developing fetus connecting the trunk of the pulmonary artery to the proximal descending aorta. It allo ...

atrial septal defect Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this d ...

). Complex defects are those that occur with other anatomical anomalies or require non-standard repair.

Management

References

External links

Congenital heart defects Rare diseases {{circulatory-stub