It is a cyanotic congenital heart disease (CHD).

Normally, there are four pulmonary veins that carry pure blood from both lungs that finally drains into left atrium (LA).

When instead of connection or drainage of pulmonary veins into left atrium, it connects elsewhere, it is known as Total Anomalous Pulmonary Venous Connection (TAPVC) or Total Anomalous Pulmonary Venous Drainage (TAPVD).

In TAPVC, pulmonary veins generally forms a common chamber behind LA in which all pulmonary veins drain and that chamber can connect at several sites and that site connects with right sided cardiac chambers (these chambers carry impure blood to lungs for blood purification)..

So, pure blood does not enter the body and so survival will not be possible without any hole/communication in heart for mixing of impure and pure blood.

Normally, there is connection in the form of Patent formen ovale (PFO) or atrial septal defect (ASD) between two upper chambers of heart. At this level, mixing of blood occurs for survival of the child at the cost of mild bluish discolouration of lips and fingers (cyanosis).

Cause of TAPVC

Cause of TAPVC occurrence is not known in most of the children.

Types of TAPVC

There are 4 types of TAPVC/TAPVD:

  1. Supracardiac TAPVC: When pulmonary veins drain above the heart into superior vena cava {SVC} and then into right side of heart.
  2. Cardiac TAPVC:When pulmonary veins drain into the right heart directly through the coronary sinus or into right atrium directly.
  3. Infracardiac TAPVC: When pulmonary veins drain below the heart into portal or hepatic veins, and finally drain into right sided heart. Majority of this variety is obstructive in nature so patient becomes very symptomatic early after birth.
  4. Mixed TAPVC:Any of combination mentioned above.

TAPVC can be obstructive or non-obstructive.

If obstructed TAPVC, patient will be symptomatic early. Obstruction can occur at PFO level or in vertical channel that drains into SVC or in downward channel that drains into portal or hepatic veins.

Clinical presentation of TAPVC

Babies with TAPVD can have the following problems:

  • Rapid breathing
  • Cyanosis
  • Difficulty in feeding
  • TAPVC Murmur on auscultation

Symptoms of TAPVD/TAPVC depend on the size of connection for mixing in heart (at PFO level).

If PFO is small, then child will be having difficulty in breathing with early cyanosis (bluishness).

If size of PFO is adequate then patient will be asymptomatic in early part of the life.

In case of TAPVC, lungs receive double amount of blood supply so lung pressures (Pulmonary Hypertension) can be little high even without obstruction. But in case of obstruction, pulmonary pressures will be high so patient needs to treat early.

Diagnosis of TAPVC in children

TAPVC can be diagnosed antenatally by doing “Fetal Echocardiogram Test” but sometimes it can be missed by antenatal scan as well.

After birth of the baby, it can be easily diagnosed by doing “Paediatric Echocardiogram Test’.

Chest X ray and ECG may help in suspicion of TAPVC problem.

Sometimes, we need to do cardiac catheterization for assessing operability if patient presents in later part of the life.

Treatment of TAPVC

Only option is to correct TAPVC/TAPVD by open heart surgery.

By surgery, we will connect the common chamber with left atrium along with closure of hole if any with tied off all abnormal vessels that r draining at various sites.

Timing of surgery depends on the type of total anomalous pulmonary venous return, condition of the child and whether obstruction is present or not.

Early surgical repair is done in cases of obstructed TAPVC. It is mainly in infracardiac TAPVC and occasionally in few cases of cardiac and supracardiac TAPVC.

Children with unobstructed total anomalous pulmonary venous return or drainage or return (TAPVC/TAPVD/TAPVR) needs surgical repair in first 2-3 months of age as there is not of much benefit by waiting more than two to three months.

Sometimes, we need to do balloon dilatation of atrial septum for increasing size of PFO (restricted PFO) in a sick neonate, known as “Balloon Atrial Septostomy (BAS) procedure”. It is carried out by angiographic technique in cath lab (by without surgery).

Occasionally we need to open this channel by putting a stent (vertical vein stenting) in it if it is severely obstructed and child is sick and can not be taken up for surgery. By this procedure, child can be stabilised in the mean time and later on can be taken up for surgery. This is done by non-surgical technique in cath lab by angiography.

What activities can my child do

Children with repaired TAPVC can be advised to do his or her activities if there is no associated cardiac issue like pulmonary hypertension, some form of heart beat or heart rhythm disturbance (arrhythmia) etc.

Follow-up after TAPVC repair

There is very good long term outlook if TAPVC is operated in early infancy.

However, child shall be in regular follow-up with a paediatric cardiologist lifelong. Follow-up is required for assessing whether any heart beat disturbances is there or not, residual narrowing in pulmonary veins etc.

Follow-up tests that can be required are Echocardiogram, ECG, Holter test etc. Sometimes CT or MR angio may be needed

Pregnancy with Total Anomalous Pulmonary Venous Connection

If TAPVC is repaired in early infancy with no residual problems then pregnancy can be planned with close follow-up.

Re-surgery or intervention for TAPVC is required?

Sometimes, surgeon is leaving vertical channel during surgery that needs to be blocked later on. This can be done by non-surgical technique angiographically by putting a device in the channel itself (Vertical Vein device closure).

Coronary sinus TAPVC

Supracardiac TAPVC

Infracardiac TAPVC

Infracardiac TAPVC