Last updated on January 11, 2022
Content By: Dr. Nikita Toshi BDS, Assistant Manager (Medical Review), Dr. Ritu Budania MBBS, MD (Pharmacology) Head, Medical Affairs
Last updated on January 11, 2022
The heart, one of the most important organs in our body, is responsible for pumping blood around your body, delivering oxygen and nutrients to the cells. It is divided into four chambers The upper chambers which are the left and right atrias receive blood while the lower chambers which are the left and right ventricles pump blood out for the rest of the body. This is generally how a healthy heart functions.
Truncus arteriosus is an uncommon heart disease that is found at birth (congenital). Usually, two blood vessels that emerge from the heart, one from the left ventricle and one from the right ventricle. For people with truncus arteriosus, only one blood vessel comes out of the heart. Because of this, oxygen-poor blood that is supposed to go to the lungs and oxygen-rich blood that is supposed to flow to the rest of the body get mixed with each other and this causes a lot of circulatory problems for the baby.
The heart, one of the most important organs in our body, is responsible for pumping blood around your body, delivering oxygen and nutrients to the cells. It is divided into four chambers The upper chambers which are the left and right atrias receive blood while the lower chambers which are the left and right ventricles pump blood out for the rest of the body. This is generally how a healthy heart functions.
Truncus arteriosus is an uncommon heart disease that is found at birth (congenital). Usually, two blood vessels that emerge from the heart, one from the left ventricle and one from the right ventricle. For people with truncus arteriosus, only one blood vessel comes out of the heart. Because of this, oxygen-poor blood that is supposed to go to the lungs and oxygen-rich blood that is supposed to flow to the rest of the body get mixed with each other and this causes a lot of circulatory problems for the baby.
Written by
BDS, Assistant Manager (Medical Review)
Reviewed by
MBBS, MD (Pharmacology) Head, Medical Affairs
People with truncus arteriosus (TA) have one blood vessel coming out of the left and right ventricle instead of two. It is a rare birth defect of the heart. There are different types of truncus arteriosus and they all have common anomalies (abnormalities) relating to the arteries (aorta and pulmonary artery) and ventricular septal defect, which is a hole between the lower two chambers of the heart.
The diagnosis of truncus arteriosus can be done during pregnancy, or even after birth. Truncus arteriosus, if untreated, can be fatal. Therefore, it is necessary to seek immediate medical attention if it is suspected. Truncus arteriosus can be treated using surgery and medications. There are also lifestyle restrictions that may be placed on people with truncus arteriosus.
Truncus arteriosus is one single vessel that is present in the heart of a developing fetus. Normally, truncus arteriosus divides into two different blood vessels namely- the aorta and pulmonary artery. In some cases, during development, the condition fails to divide into two separate arteries which lead to a mix up of oxygenated and deoxygenated blood. In a baby with normal heart development, both an aortic valve (the valve located at the exit of the left ventricle of the heart where the aorta begins) and a pulmonary valve (the valve that is present between the right ventricle and the pulmonary artery) is present. But in babies with this condition, only a common valve known as the truncal valve is present. Truncus repair is often required to fix the abnormalities of the truncal valve. The truncal valve can become too thick or too narrow, which blocks blood from leaving the heart. The truncal valve can also leak, making the blood leaving the heart flow back to it. To prevent blockage or leakage, truncus repair is required.
TA is a quite rare form of congenital heart disorder occurring in one out of every ten thousand births in India. All over the world, ten out of ten thousand infants may be affected by it. Gender does not play a role in this condition as male and female infants are affected in equal ratios.
More than 80% of infants with truncus arteriosus die within the first year if they don’t get the proper treatment. Therefore, the condition (another term fortruncus arteriosus) is quite rare in adults. Adults with this condition have a very high risk of developing heart failure and pulmonary hypertension.
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Detecting the signs and symptoms of truncus arteriosus at an early stage is crucial to getting the right treatment for this condition. Once you are aware of these symptoms, you can talk to your doctor so can proceed on the right course. Some of these symptoms include.
Blue colouring of the skin
This is one of the major symptoms. The bluish colouring of the skin indicates that the body is facing trouble getting a sufficient amount of oxygenated blood due to the mixing up of oxygenated and deoxygenated blood.
Excessive sleepiness
Excessive sleepiness indicates that your baby lacks energy due to poor oxygen circulation in the body.
Abdominal swelling
Another sign and symptom of the condition is abdominal swelling. Abdominal swelling might occur due to other health issues. Therefore, this issue is often neglected.
Pounding heart
One of the major symptoms of this condition is a pounding heart or palpitations. The heart in an infant with this condition has to work hard to pump blood to the entire body. Therefore, the heart beats faster than usual and creates a pounding feeling.
Rapid breathing
Infants with this condition often experience rapid breathing. This happens due to the lack of oxygen in the body.
Poor feeding
Infants with the condition can face difficulty in breastfeeding.
Poor growth
The condition is also associated with poor growth and low body weight in infants. If you see constricted growth in your infant, do not hesitate to visit the doctor.
The symptoms of this condition can be noticed in infants right after birth. Since the heart works too hard to pump blood, the symptoms are quite visible. The symptoms create a need for truncus arteriosus repair.
Doctor consultation should be opted for immediately after noticing the symptoms in your infant. Some emergencies in infants in infants with truncus arteriosus include the following:
Loss of consciousness
Worsening of cyanosis (bluish tint on the skin)
Shallow, rapid or laboured breathing
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Truncus arteriosus occurs during the development of the baby in the womb. The exact cause of the condition is unknown, but factors that increase the risk include:
Alcohol
Women who drink alcohol during pregnancy are at higher risk of giving birth to babies with this condition. Especially during the first trimester of pregnancy, alcohol consumption can be quite risky.
Women with obesity are at an increased risk of giving birth to babies with this condition and other congenital diseases.
Smoking during pregnancy
If a woman continues smoking during pregnancy, it increases the risk of giving birth to a baby with heart defects.
Chromosomal disorders
Chromosomal disorders like velocardiofacial syndrome (a genetic disorder involving a cleft palate) or DiGeorge’s syndrome (a condition that results in the poor development of many body parts) increase the risk of the condition in infants.
Uncontrolled diabetes during pregnancy
This can increase the risk in newborns. High blood sugar levels can also cause other heart disorders in newborns.
Certain medications during pregnancy
During pregnancy, a woman should be careful about the medicines they are taking. No medicines should be taken during pregnancy without a doctor’s consultation. Some medications can cause a developmental anomaly in newborns like persistent truncus arteriosus.
Viral illnesses like Rubella during pregnancy
If the mother contracts viral diseases like Rubella during pregnancy, it puts the baby at risk of congenital heart diseases. Persistent truncus arteriosus is one of the heart disorders that can occur due to viral illnesses.
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Diagnosing and treating truncus arteriosis in newborns is key to helping them find the best method in controlling this condition. With this in mind and depending on the health condition of the newborn, there are diagnostic tests that may be recommended by your doctor. A few of those tests are given below.
Diagnosis during pregnancy
The process helps in checking the heart of a developing baby. It is beneficial in detecting heart defects in babies before they are born. The test allows the doctor to see the heart structure of an unborn baby and find out if it’s working effectively. The test is typically done between 18 to 24 weeks which is the second trimester of pregnancy.
External breathing assessment
The breathing rate of infants is examined to check if they are facing any breathing trouble. If their breathing is shallow, rapid or laboured, it might be indicating heart defects.
Assessment for arrhythmias and murmurs
A doctor listens to a baby’s heart to determine if it beats irregularly (arrhythmia). Sometimes an abnormal sound in the heart is also caused due to the turbulent flow of blood. This abnormal sound is known as a heart murmur.
Echocardiogram
An echocardiogram reveals the structure and function of the heart. Here, sound waves are used to produce images of the heart on a monitor. If an infant has the condition, the echocardiogram will show the presence of a single vessel from the lower chambers of the heart. Sometimes there can also be a hole in the wall between the right and left ventricles of the heart. An echocardiogram also shows how much blood flows to the baby’s lungs. It will help in knowing if there’s a high risk of increased blood pressure in the lungs.
Chest x-ray
A chest x-ray will help in producing still images of the heart and lungs with radiation. The size of the heart and any abnormalities in the heart and lungs can be revealed in a chest x-ray. The presence of excessive fluid in the lungs can also be determined with the help of a chest x-ray.
A mother needs to be aware of both her own and the child’s father’s medical history. Some heart problems are genetic, so knowing the family medical history can help a great deal. You can prepare a list of questions that might be helpful to ask the doctor. It is also necessary to ask what kind of diagnostic tests your child needs. The doctor might ask you a few questions related to the symptoms of truncus arteriosus. The doctor might ask if your baby appears blue and for how many hours your baby sleeps. It is a good idea to write down the symptoms you notice in your baby.
Your doctor will advise on the best course to start the treatment. Following the detection of symptoms and the procedure, thorough follow-ups will be conducted. With that being said, there are quite a few approaches to treating truncus arteriosus, which are listed below.
Surgery
The surgery is usually done within the first few weeks of birth. The surgical procedure primarily depends on the health condition of the baby. Some common steps included in the surgery are as follows:
After having corrective surgery, a child needs to be under lifelong follow-up with a cardiologist. Some physical activities might be restricted after the corrective surgery. Sometimes a child needs multiple corrective surgeries.
A child with corrective surgeries will have to be on antibiotic dosage before dental surgeries or other surgical procedures. Antibiotics will help in preventing infections. As the artificial valve will not grow as your child grows, follow-up surgeries will need to be conducted.
Cardiac catheterization
Treatment is also possible with minimally invasive procedures. These procedures using a cardiac catheter are good alternatives for traditional open-heart surgeries. The catheter (a thin tube) is implanted into a blood vessel and threaded up to the heart. Sometimes cardiac catheterisation with a balloon tip is done to reopen a narrowed or blocked artery.
Medications
Treatment in infants is done by a pediatric cardiologist. In adults, the treatment is done by a cardio-thoracic cardiac surgeon. These specialists will prescribe certain medications based on the patient’s age, the severity of the symptoms and their underlying conditions. These medications can help manage the condition better and improve the outlook.
Nutrition
Babies with the condition become tired while being fed. When they don’t eat adequately, their growth is hindered. Most babies with the condition are underfed and underweight. Therefore, a high-calorie formula might be prescribed to promote weight gain in infants. Infants who become too tired while eating are required to be fed using a feeding tube. Food is given to them through these small and flexible tubes that pass through the nose, down the oesophagus and into the stomach.
If the condition is not treated properly using the appropriate medications or corrective surgeries, it can increase the risk of developing other health conditions. Here are a few of the complications that truncus arteriosus can lead to.
Progressive pulmonary hypertension
If the condition is left untreated, it leads to progressive pulmonary hypertension. It is characterised by high blood pressure in the lung arteries without any reason.
Irregular heart rhythm
The condition also leads to irregular heart rhythm in patients. The medical term for irregular heartbeats is arrhythmia. Arrhythmia feels like the heart skipped or added a beat or is fluttering. The heart might beat too fast or too slow due to arrhythmia.
Problems related to the prior operation
TA also causes issues related to other surgeries. It can increase the risk of infections from prior surgeries. Patients with TA need to be on antibiotics before undergoing any kind of surgery.
Leaky heart valves
TA is also associated with leakage in the heart valves. Due to the leakage, the blood flowing out of the heart can flow back to the heart.
Death
Children with the condition have a high chance of mortality in the first year of life.
The hospitals in India have the best facilities for conducting the surgery. The medical practitioners involved with this surgery are well-equipped with all the necessary skills and qualifications. Moreover, the cost of surgery for this condition in India is quite low. Therefore, you will be able to save a considerable amount without compromising on the quality of the operation.
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With this condition comes certain lifestyle changes that will help you maintain this condition and live a healthier life. However, there are certain things that you can control to help you prevent the condition. These are listed below.
Controlling diabetes: A woman with diabetes should consult the doctor before getting pregnant. Diabetes needs to be managed during pregnancy to avoid heart-related complications in the baby.
Getting vaccinated before getting pregnant: Viral infections like German measles can be deadly during pregnancy. Therefore, it is important to check if you have the proper immunisations before getting pregnant. If you have not been vaccinated with necessary immunisations, take them before planning for pregnancy.
Avoiding dangerous medications: Some medications can have harmful effects on pregnant women. You should always consult your doctor before taking any medication during pregnancy or when you are planning for pregnancy.
A daily dose of folic acid: Taking a daily dose of folic acid can be quite helpful during pregnancy. It can prevent birth defects, including heart problems and spinal cord disorders. However, it is recommended to consult your doctor before taking this.
Avoiding alcohol/smoking: Consumption of alcohol or smoking during pregnancy can be too dangerous for the baby. It can cause a wide range of problems in the baby, including heart issues.
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The support and coping mechanisms parents need to receive will help you form a better frame of mind when dealing with this condition. It is always important to find an outlet and find your sources for the most accurate information.
If you have an infant with truncus arteriosus, it can be quite difficult to take care of them on your own. Do not hesitate to ask for support from friends and relatives. You can ask someone you trust to accompany you to the doctor appointments.
The caregiver must have adequate information about the health condition. You need to learn about the symptoms and causes. You should know which symptoms need you to seek immediate medical attention. It is also important to know about the behaviours and activities that can aggravate the condition. Read relevant articles, watch videos, ask people with medical knowledge to become more aware of the condition.
If your baby has this condition, you should make it a habit to note down their symptoms. The frequency and intensity of the symptoms might change from it. Recording or journaling will ensure that you don’t miss out on any points during the medical appointments. It will also be beneficial when your child moves from pediatric to adult healthcare.
Sometimes dealing with an infant with this condition can become very overwhelming. Joining a support group for parents will help you realise that there are other parents like you going through the same problems. You can vent out your feelings and raise your concerns in those support groups. It will be quite helpful in relieving your mental stress. Remember that you need to think about your mental and physical well-being while taking care of a child with this health condition.
You can ask your doctor any questions you have. Seeking answers to questions will help you realise if you are taking enough care of your child. You should be ready to follow all the instructions given by your doctor. If necessary, you can note down the instructions and refer to them later. Also, prepare a list of questions that you would like to ask the doctor before every appointment. A sample of questions that you might want to ask are as follows:
What food items should I avoid feeding my child?
If I think about another pregnancy in future, what are the chances of this condition happening again?
What are the possible complications of the treatment my child is undergoing?
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Most infants with the condition have to undergo corrective surgeries. They can survive through the surgery, but they might have to undergo more surgeries as they grow up. For instance, since the artificial tube will not grow, it will have to be replaced as the child grows. The condition’s life expectancy ranges up to ten to twenty years in infants who undergo complete surgery.
There are four variations of truncus arteriosus.
Type 1: In this condition, the main pulmonary artery branches into the left and right pulmonary arteries.
Type 2: In this condition, the left and right branches develop adjacently.
Type 3: The left and right branches have separate origins from the left and right lateral segments present in the common trunk.
Type 4: In this condition, the left and right branches do not originate from the common trunk.
A person diagnosed with truncus arteriosus cannot survive through infancy without proper treatment. Surgical repair has to be done to make an infant with the condition live into adulthood. Some infants have to undergo more than one corrective surgery. The condition’s life expectancy varies greatly from person to person. Instead of surgery, a less invasive method like cardiac catheterisation is also used to help some patients. Medications along with some lifestyle changes can also help in the prevention of this type of congenital heart disease.
Without proper treatment, the condition can be life-threatening. Infants with this condition have a high risk of mortality in the first year of their lives.
The condition needs to be treated with surgery. Usually, before the surgery, there are check-ups to detect the state of the baby’s condition. Post this, the surgery is done. It is generally successful especially if it occurs before the baby is 1 month. Medications are also usually prescribed by the doctor to manage the condition.
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