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Pulmonary atresia with ventricular septal defect

L'atrèsia pulmonar amb comunicació intraventricular a Vall d'Hebron

Pulmonary atresia with ventricular septal defect is a rare heart condition characterised by a lack of connection between the right ventricle and the pulmonary arteries. 

 

Description

This is a rare congenital heart defect characterised by no connection between the right ventricle and the pulmonary arteries. It is an extreme type of Tetralogy of Fallot in which blood enters the lungs to be oxygenated by bypassing the heart.

Blood can reach the lungs via the pulmonary arteries themselves, which are not connected to the heart, or via the collateral arteries, which originate from the thoracic aorta and directly supply the lung. There are significant anatomical differences between vessels which must be studied in each individual child.

This condition is very heterogeneous, which creates the variability seen in the pulmonary arteries. Two groups can be distinguished:

  • Good sized pulmonary arteries with pulmonary flow connected to the ductus arteriosus.
  • Hypoplastic pulmonary arteries with collateral arteries, in which pulmonary circulation is mixed.
  • Absence of pulmonary arteries with pulmonary circulation exclusively through the collateral arteries.

 The prognosis of this disease depends on the growth of the pulmonary arteries to be able to surgically repair the condition.

 

Who is affected by pulmonary atresia with ventricular septal defect?

It is a rare congenital heart condition which makes up 1-2% of all congenital heart defects.

 

Diagnosis

In most cases, diagnosis is via foetal echocardiogram. This ultrasound will show the lack of connection between the heart and the pulmonary arteries, as well as the presence of VSD. Through this test the size and position of the pulmonary arteries can also be measured.

When a child is born, it has a certain quantity of oxygen, known as “saturation”, in its blood which is around 80-90% of the normal level, although this is enough for the child to develop normally. 

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