Download Total anomalous pulmonary venous return

Great Ormond Street Hospital for Children NHS Foundation Trust: Information for Families
Total anomalous pulmonary
venous return
This information sheet from Great Ormond Street Hospital
explains the causes, symptoms and treatment of total
anomalous pulmonary venous return and where to get help.
Total anomalous pulmonary venous
return is a congenital heart defect - that
is, it was present when a child is born.
Around eight in every 1000 babies born
have a congenital heart defect.
The normal heart
The heart consists of four chambers
– two upper filling chambers (atria)
and two lower pumping chambers
(ventricles). In between each atrium
and ventricle is a valve that stops blood
flowing backwards. There is also a valve
where the pulmonary artery and aorta
join the heart. The right and left sides of
the heart are divided by a thick wall of
heart muscle called the septum.
Aorta
Pulmonary
artery
Left
atrium
Mitral
valve
Right
atrium
Tricuspid
valve
Sheet 1 of 3
Left
ventricle
Ref: 2013F1124
The function of the heart is to pump
blood around the body. Blood comes into
the right atrium from the body, through
the tricuspid valve and into the right
ventricle. From here, it is pumped up the
pulmonary artery to the lungs to pick up
oxygen. Oxygen-rich blood comes back to
the heart through the pulmonary veins
into the left atrium. It flows through the
mitral valve into the left ventricle. This
pumps the blood into the aorta and from
there around the body.
What is total
anomalous pulmonary
venous return?
Normally the blood is pumped from
the right lower pumping chamber
(right ventricle) through the pulmonary
artery to the lungs to pick up oxygen. It
then returns to the heart through the
pulmonary vein into the left atrium from
where it reaches the left ventricle for
pumping around the body.
In total anomalous pulmonary venous
return the veins carrying red blood
draining from the lungs reaches the
‘wrong’ atrium by some abnormal route..
This causes problems for the lungs which
© GOSH NHS Foundation Trust October 2013
have to deal with high blood flows. There
is a hole between the two collecting
chambers of the heart (atrial septal
defect) which is helpful as it allows blood
to reach the left ventricle for pumping
around the body.
Pulmonary veins
connecting
Opening
between atria
What are the signs
and symptoms of total
anomalous pulmonary
venous return?
Almost all babies with total anomalous
pulmonary venous return become sick
soon after birth. Breathlessness is the
most common symptom and on checking,
the baby’s skin and lips are also a little
blue. Sometimes the abnormal veins are
blocked as well as having an abnormal
route and these babies can get very
sick or collapse. Other babies without
obstruction may be a little older before
breathlessness interferes with feeding and
the baby fails to thrive.
Right atrium
How is total anomalous
pulmonary venous return
diagnosed?
What causes total
anomalous pulmonary
venous return?
The heart is formed very early in
pregnancy. We do not fully understand
why some children’s hearts do not
develop properly so have a congenital
heart defect. We know that the chance
increases if one or both parents had a
congenital heart defect. Congenital heart
defects can also be more common in
children with other congenital conditions.
Sheet 2 of 3
Ref: 2013F1124
Doctors will use chest X-rays,
electrocardiograms (ECG) and
echocardiograms (Echo) to diagnose total
anomalous pulmonary venous return.
An ECG measures the electric current
passing through the heart. An Echo is an
ultrasound of the heart and shows not
only the structure of the heart but the
blood flow through it.
Children may also have a cardiac magnetic
resonance imaging (MRI) scan, which uses
a strong magnetic field, radio waves and
a computer to form detailed pictures of
the heart.
© GOSH NHS Foundation Trust October 2013
How is total anomalous
pulmonary venous
return treated?
Total anomalous pulmonary venous return
needs to be corrected in an open heart
operation. The surgeons will connect the
pulmonary veins to the ‘correct’ left filling
chamber (atrium) and close the atrial
septal defect.
What happens next?
All children with congenital heart defects
(even when corrected) will need regular
check ups in hospital for a long time,
usually continuing into adulthood. These
will usually involve repeat Echo and ECG
scans and sometimes cardiac magnetic
resonance imaging (MRI) scans. The
aim of these check ups is to monitor
the child’s heart function so that any
future heart problems are diagnosed and
treated quickly.
Notes
Compiled by the Web team in collaboration with the Child and Family Information Group
Great Ormond Street Hospital for Children NHS Foundation Trust
Great Ormond Street
London WC1N 3JH
www.gosh.nhs.uk
Sheet 3 of 3
Ref: 2013F1124
© GOSH NHS Foundation Trust October 2013