What is Total Anomalous Pulmonary Venous Return (TAPVR)?
Total Anomalous Pulmonary Venous Return (TAPVR) is a life-threatening heart defect in newborns. Babies with this heart condition are blue since birth and have fast breathing, difficulty in feeding and poor weight gain.
Normally- After oxygen in the blood has been used up by the body- oxygen poor blood turns blue. This blue blood from the body returns to the Right upper chamber of the heart (Right Atrium) with the help of two veins (SVC and IVC).
From the right atrium (right upper chamber of the heart that collects blood) blood will go to the Right Pumping chamber (Right Ventricle). The Right ventricle then pumps this blue- oxygen poor blood to the lungs (through a tube-like Great Artery-Pulmonary artery).
The Lungs will put Oxygen in this blue blood, making it Oxygen rich and red again. This Red blood returns (with the help of four small tube-like pulmonary veins) to the left upper (blood collecting) chamber of the heart- Left Atrium.
From the left atrium this red blood will go to the Left Pumping chamber (Left Ventricle). The Left ventricle then pumps this red- oxygen rich blood to the body (through a tube-like Great Artery- AORTA).
So- as you would understand- Normally- two pulmonary veins bring red blood from each lung to the left atrium (left upper chamber of the heart)
In Total Anomalous Pulmonary Venous Return- these pulmonary veins ‘WRONGLY CONNECT TO THE RIGHT SIDE OF THE HEART’. And so- Blue blood and Red Blood will mix on the right side of the heart- and will go partly to the left side through a hole in the heart (ASD or/and VSD) and mostly to the right ventricle.
If the hole in the heart is small- then very little blood will reach the left side of the heart, and so, very little will reach the body- This is a VERY dangerous and life-threatening situation.
If these babies are not treated by surgery in time then-only 50% survive to 3 months and only 20% are alive at one year of age Treatment of this heart condition is an open-heart surgery- Surgical Repair in which these Pulmonary Veins are connected to the Left Atrium
Why do the surgery?
TAPVR is a life-threatening heart condition and untreated – most babies with this birth defect will not survive. On the other hand- most children that undergo Surgical Repair will go on to live a ‘Near Normal’ life.
The Surgical Procedure
Surgical repair is recommended early in life- or as soon as the diagnosis of TAPVR is made. Since these children usually do not become inoperable, they should undergo surgical repair even if the heart condition is identified late.
The surgeon makes an incision down the front of the chest (called a median sternotomy) and divides the breastbone (sternum) in half to get access to the heart. The heart is placed on cardiopulmonary bypass, meaning that a machine takes over the heart’s work of pumping of the blood so the surgeon can enter the heart to repair it.
The repair will consist of the following steps:
- Cutting open the pulmonary veins and/or their communicating chamber, cutting open the Left Atrium and connecting them together using sutures.
- Incorrect connection of the Pulmonary veins with the right side of the heart is closed.
- ASD- The hole between the right and left atrium is usually closed with a patch or sutures.
When the breastbone is put back together, stainless steel wires are usually wrapped around the bone to fix it. The surgery will take an average of six to seven hours from start to finish.
Risks and benefits of the surgery
Risks of any heart surgery include bleeding, infection, stroke, organ damage, requirement for a temporary or permanent pacemaker, or possibly death. However, the risk of these occurring is about 2 to 5%.
The benefits surgery for the child (once recovered from the surgery) is that he/she will have a near normal life expectancy and quality of life. This would mean that the child will not be blue anymore and will not have heart failure symptoms and his/her growth and development will be better. In addition, the risk of death and heart failure are also avoided.
What to expect during the hospital stay
The length of the hospital stay usually varies from 7 to 9 days, depending on how quickly the heart recovers. For a few days following surgery, we will take care of your child in the ICU. In the Intensive Care Unit, we will keep a watch on the child’s Heart Rate, Blood Pressure, Respiration and other organ functions. To avoid frequent interruptions in the care of your child, there is a restriction to visitors, however, parents can visit the child.
The child may need to be in the hospital for longer if they experience any of the following:
- A fast heartbeat (supraventricular tachycardia or SVT), requiring medications to slow down the heart
- Low heart function
- Fluid in the chest (pleural effusions), requiring a chest tube that is put in place at the end of surgery to stay in longer or additional chest tubes to be added
- Needing longer than usual help from a machine (ventilator) to breathe.
These challenges are part of the normal recovery after surgery in a child with Transposition of Great Arteries.
For a few days after surgery, the child will be on heart medications to improve heart function and will be on in sedation and on a ventilator- to help the heart get stronger.
After the ventilator is removed and the child is breathing on its own- he/she will be sleepy while recovering from and will be under the influence of strong pain medications. It is normal for a child to be confused, thirsty, and irritable.
Going home
The hospital will supply specific discharge instructions, and all instructions from the hospital, cardiac surgeon, and cardiologist should be strictly followed. The child should get back to his or her normal routine upon arrival at home. For the first week, the child may wish to take more frequent naps or wake up occasionally at night. The child should be seen by his or her cardiac surgeon/ cardiologist within the first 3-5 days after discharge from the hospital. The incision should be kept clean and dry until it is healed. It should also be protected from the sun for a full year. The child should not be picked up under the arms for four weeks following surgery.
Any of the following symptoms should be reported immediately to the cardiologist:- fever greater than 101,
- redness, puffiness, discharge from the incision, or
- unusual nausea or flu-like symptoms.
In only about 5-8% children, with time there can be narrowing at the connection between the pulmonary veins and the left atrium or in the pulmonary veins. And so, in the initial phase of follow up we see the child frequently, but after a few months- the follow up is usually limited to once a year to check the pulmonary veins.
