Paediatric and Adult Congenital Heart Surgery

Babies born with critical cardiac defects will need life saving surgeries either immediately after birth or within the first month of life. We at Amrita Hospital provides following surgeries for these critically sick neonates.

• Arterial switch operation
• Total anomalous pulmonary venous return (TAPVC) surgery
• Systemic-pulmonary artery shunt to increase pulmonary blood flow
• Pulmonary Artery  band to restrict pulmonary blood flow
• Neonatal arch repairs
• Truncus arteriosus repair
• Neonatal Coarctation repair

We at Amrita hospital provide complex root translocation surgeries to patients. These procedures can convert single ventricle pathways to biventricular pathways with potentially superior longterm survival.

• Pulmonary root translocation
• Aortic root translocation
• Double root translocation

A sizeable  number of children born with congenital heart defects will reach adulthood even without surgery. A even larger group of children who underwent surgery/intervention for CHD in childhood will need re-operations in their adolescence or adulthood for recurrent, residual or acquired heart defects. Our team of adult and congenital heart defects is fully equipped to take care of this ever-growing group of patients.

We need two ventricles for a sustaining life and longevity. However, a large number of children are born with either anatomical or functional single ventricle. Most of these children will need surgery in three stages to improve the quality and quantum of their lives. At Amrita Hospital we provide the following operations for babies born with single ventricles

• Norwood operation
• BT shunt
• Pulmonary artery Banding (PA band)
• Bidirectional Glenn (BDG) operation
• Fontan operation

It is ideal to repair rather than replace valves in children due to lack of growth potential  implanted valves. Our team specialises in the following valve repair surgeries

• Aortic valve repair for acquired or congenital stenosis and regurgitation
• Mitral valve repair for congenital stenosis and regurgitation
• Tricuspid valve repair
• Ozaki repairs

A normal rate and rhythm of  the heart is essential to provide adequate cardiac output to sustain life. Ant disturbance in the rate and rhythm are called arrhythmia. The arrhythmia can either be congenital or acquired. At Amrita Hospital we provide the following services for arrhythmia.

• Permanent Pacemaker implantation (PPI)
• Loop recorder implantation for diagnosis of arrhythmia
• Maze surgery
• Cervico-thoracic Symphectomy for refractory long QTc syndrome 

Vascular rings are birth defects where the airways are abnormally surrounded and/or compressed by the blood vessels. The child may have symptoms of wheezing, noisy breathing (stridor), intractable coughing, breathlessness, difficulty in feeding or swallowing, and respiratory infections. We at Amrita hospital offer following services for the diagnosis and treatment of these lesions.

• Bronchoscopy
• Division of double aortic arch
• Relocation of pulmonary artery for pulmonary artery slings
• Relocation of Innominate artery
• Translocation of circumflex aortic arch
• Pulmonary arteriopexy
• Aortopexy
• Tracheo-bronchiopexy
• Resection of Kommeral diverticulum & relocation of aberrant subclavian artery 

Persistent pulmonary hypertension is debilitating and life limiting disease with very little treatment options. We offer a palliative reverse Pott’s shunt to selective group of patients with PPHN. A communication is created between the pulmonary artery and aorta to decompress an eminently failing right ventricle. This procedure improves the quality of life and potentially increase the lifespan without lung transplant.

Dilated cardiomyopathy is a progressive and debilitating disease which either leads to early demise or cardiac transplant. A specific group of these children are offered Pulmonary artery band to remodel the heart. This procedure leads to improvement in cardiac function and avoid the need of heart transplant is a large group of these patients.

An abnormal tissue growth in the chambers of heart, over valve leaflets or within the blood vessels can not only compromise with the heart function but also can be life threatening. We at Amrita hospital, offer surgery for Fibroma, Myxoma, Hemangioma, Intra-pericardial teratoma, lipoma and metastatic tumours which have spread to heart from other parts of the body.

Any abnormality in the heart present at the time of birth is called as congenital heart defect. One child in 100 will be born with some form of congenital heart defect. These can involve any of the four chambers of the heart, any of the four valves of the heart or any of the tubes connected to the heart. Children may have more than one defect in their heart.

The definitive cause of congenital heart defect is known only in a small number of children. An interaction between genes and environment is responsible for birth defects. Some of the causes for congenital heart defects are air pollution, maternal diabetes, maternal smoking, alcohol consumption by mother, older age of mother. Nutritional deficiency and viral infections like rubella, influenza ect during pregnancy can also cause congenital defects.

The incidence of congenital heart defect in normal population is approximately 1%. This means the one child out of 100 will have a heart defect at birth. But if your first child has a heart defect, the chances that your second child will have a defect too, will increase by 5%.

Yes, upto 90% of the birth defects in the heart can be diagnosed by an ultrasound examination as early as 18 to 20 weeks of pregnancy. Antenatal defects can be detected during early months of pregnancy and decision to terminate very complex defects can be made.

Birth defects can not be always be prevented with certainty. But one can avoid marriage with relatives, avoid pregnancy when “very young” and “old”, avoid smoking and alcohol consumption, avoid harmful drugs in first trimester, Vaccinate against Rubella and take Good nutrition during puberty and pregnancy to reduce the risk of congenital heart defects.

Most congenital heart defects will have certain signs and symptoms which will help in diagnosis. Some of these are blue nails and lips, fast breathing, poor feeding, poor weight gain, excessive sweating, raised chest, and repeated cough, cold or pneumonia. Older children may complain of breathlessness, tiredness, swelling of feet, light-headedness, chest pain and even loss of consciousness.

Not every child with heart defect will need surgery. In fact, a large number of children can be treated medically and some of these can be treated in the catheter lab without surgery. However, most complex heart defects will need surgery.

Many of children with congenital heart defects will need only one surgery. However, a large number of children will need more than one surgery. Most of these children will need regular follow-up.

The outcomes of surgery for most congenital heart defects are excellent and 98% of children undergoing heart surgery will survive.

Once the child has made recovery, he/she is discharged from the hospital. Detailed care instructions are given to the family at the time of discharge.

Their Child’s peri-operative period is perhaps the most stressful period in a parent’s life. This guide has been designed to help parents prepare for their child’s discharge from the hospital after cardiac surgery. Many families find that learning and talking about what to expect before their child goes home can help decrease anxiety and promote speedy recovery.

Incision Care:

• The sutures and dressings are generally removed by the seventh postoperative day. Sponging with lukewarm water and a clean towel is done daily during the first seven days after surgery.
• The child is given bath with mild soap and water daily after discharge from the hospital. The incision site should also be cleaned gently.
• Please do not rub or spray water directly over the incision. The incision is gently moped dry with a soft, clean towel after bathing. Please do not use any lotion or powder on the incision.
• The child should be covered with clean soft cloths without zippers, sequins etc which may hurt the skin.
• The incision should be inspected daily for signs of infection such as redness, swelling or discharge. You must call your doctor immediately if you see any signs of infection.
• The child may occasionally complain of itching over the incision. It is usually self limiting but discourage your child from scratching. Keep fingernails short and clean.
• Do not expose the incision to direct sunlight till one year after surgery.

When to call the doctor?

Your child is discharged from the hospital only after the medical team is satisfied with the child’s condition and is confident that child does not need hospital care. Nevertheless, your child may get unwell after discharge and may need medical attention. Please watch out for following signs and symptoms which may indicate that the child is unwell:

• Fever over 101 F (38.3 C). It is not necessary to check for fever unless your child seems sick or feels warm to touch.
• Poor feeding, Frequent vomiting or diarrhoea
• Decreased urine or dark coloured urine
• Excessive sleepiness (lethargy), tiredness, loss of energy
• Shortness of breath. fast breathing, noisy breathing or difficulty in breathing
• Persistent cough
• Colour change (pale, bluish)
• Fast or slow heart beat
• Increased pain over the incision, Signs of infection along the incision (redness, swelling and/or discharge)
• Excessive sweating
• Swelling of the feet, hands, face or around the eyes
• Irritable, crying for long periods of time

Feeding and Nutrition

Your child’s appetite may be poor when he/she first returns home, but it will improve with time. Please offer small frequent meals.

Nutritional advice for Infants and toddlers:

• Mother’s milk is the best feed for the child and should be the only feed till the age of six months. It meets the psychological as well as the nutritional needs of the child.
• Breast feeds should be supplemented by top-up feeds after six months of age. These top-up feeds should provide one-third of the nutritional needs of the child till the age of one year.
• The staple cereal of the family should be used to make the first feed for your child. It can be Semolina (Suji), half broken Wheat (Dalia), coarse Rice powder, Ragi (Nachani), or Pearl Millet (Bajara). You can prepare porridge using milk or water. The nutritional value can be enhanced by adding sugar/jaggary and oil/ghee.
• The porridge should be thin initially and as the child grows it can be made thicker.
• Fruits like Banana, Mango, Papaya, Chikoo can be mashed and introduced at this stage. Green leafy vegetables like spinach (palak) and Methi can be boiled and added to the feed.
• Breast feeds should be continued till the age of two years or beyond if feasible.

Home made instant formula feeds: This is a viable and cheaper alternative to commercially available formula feeds. It is easy to prepare. Please take 600gm of cereal/millet (Wheat/Rice/Ragi/Bajara/Jawar), 300 gram of pulses (Moong/Chana/Arhar) and 100 gram of Groundnut or white sesame seeds (Til) to prepare 1000gm of formula feed. These are roasted and ground separately and then mixed and stored in airtight container. Instant feed can be prepared by mixing two spoons of this mixture with boiled water or milk. Ghee and jaggary/sugar is added to improve the caloric value. Green leafy vegetables like spinach (Palak), Methi, Coriander can also be added.

Nutritional advice for older children:

• Please give a well balanced diet including fruits, nuts, vegetables, eggs, milk products, and cereals.
• Eat whole grain rather than refined grain preparations.
• Use vegetable oils instead of butter or animal fat products.
• Foods rich in salt like potato chips, French fries, processed meat, fritters, pickles ect should not be given. Please do not add extra salt to food.
• Do not give aerated soft drinks or packaged fruit juices. Their sugar content is very high and these drinks have very poor nutritional value. In addition, soft drinks and juices are bad for dental hygiene. Instead, your child should be encouraged to take whole fruits sand vegetables.
• Chocolates and biscuits should be allowed in moderation.
• Limit eating out and encourage eating at home.

Any special diets will be discussed with you before discharge. Occasionally, you may be asked to restrict your child’d daily fluid intake or you may be asked to give only fat-free diet to your child.

 Medications:

Your child will invariably be prescribed medications at the time of discharge. Please ensure that you have understood the dosages correctly.

• If your child throws up or spits out a medication, DO NOT attempt to re-give it (either partially or whole) unless directed by your doctor.
• Please ensure that you know the names, doses and timing of these medications.
• Check all labels including the date of expiry before giving any medication.
• Give the medications exactly as directed.
• Remember to refill prescriptions several days before you run out.
• Please take all medications in their original containers to your doctor’s visits including vitamins and over-the-counter supplements.
• Be aware of potential side effects and report side effects.
• Do not skip medications.
• Store away from heat, light and humidity and out of child’s reach.
• Consult your Doctor if child is unable to take their medications for more than 24 hours

Activity Instructions for Children:                  
Your child may be tired for a couple of weeks after the operation and may need to rest during the day. However, your child should want to get out of bed for play every day. Notify the doctor if your child is lethargic and does not want to get out of bed.

How should I hold my child?

• Scoop your child up under the bottom rather than lifting him/her under the arms. Although the skin may appear healed, the chest bone takes 6 weeks to heal and lifting the child from under the arms will be painful.
• It is safe to hold your baby chest-to-chest with his head supported on your shoulder. It is also safe to burp or pat your baby in this position. It is safe for your baby to roll from front to back or to crawl. He will not do this on his own if it is painful for him. Baby can lie on his side while awake if tolerated. If baby seems to have pain while in this position, stop side-lying time for 2-3 days, and then try again. All babies less than one-year old should be placed on their backs to sleep. This decreases the risk of sudden infant death syndrome.
• Most operations on the heart are performed after cutting the bone in the front of the chest. This is a controlled fracture of the bone which like any other bone fracture will need six weeks to heal. Any direct injury to the front of the chest may cause severe pain, delayed healing or even injury to the heart. Therefore following activities should be avoided for six weeks after the operation.
• Contact sports like football, basketball, hockey, wrestling etc
• Riding a bicycle or skating
• Swinging, jumping, rough play, pillow fights
• Pulling or swinging from arms
• Heavy weight lifting, Wearing backpacks including school bags

When to resume School/Work ?

• The surgeon will tell you when it is safe for your child to return to school. It is usually after the follow-up visit.
• Talk to the child’d school teacher and the school nurse (if available) about your child’s heart surgery and any restrictions because of it.

Coughing and Deep Breathing:

• Encourage your child to cough and deep breathe several times a day for one week after the operation. He/she may have fun deep breathing by blowing bubbles through a straw in a glass of water.

Emotional Behavior:

As a result of this hospitalisation and surgery, your child may act like he/she did when younger. This can be normal and can be due to stress.

• Toilet habits: Your child may wet the bed or soil his/her pants. Please do not punish or scold your child for this. It should get better within several weeks. If it continues, call your paediatrician for guidance.
• Nightmares: Your child may awaken during nap-time/at night crying or upset. Hold and reassure your child that he/she is home and safe.
• Demanding: Give your child love and affection, but continue to set limits.

Immunisations:

• Your child should receive all vaccines as recommended by the ministry of health.
• Immunisations should not be given for 6 weeks after surgery if your child was on the heart-lung machine (also called “bypass” machine) during his/her operation.
• If your child had received blood product transfusions, the following vaccines should not be given for 7 months after surgery:
  • Measles/Mumps/Rubella (MMR)
  • Varicella (chicken pox)

Your child is at an increased risk of developing infective endocarditis (Infection of the inner lining of the heart chambers)

• Your child may need to take antibiotics to prevent infection of structures of the heart (SBE prophylaxis) before he/she has certain medical, surgical or dental procedures. This antibiotic protection may be needed for 6 months or longer after surgery.
• See that your child’s teeth are kept in the best possible condition by daily brushing/flossing and regular checkups with your dentist.
• Inform your child’s dentist and any doctors involved with your child’s health care that your child had a heart problem and surgery to repair it.
• The dentist or doctor will prescribe the appropriate antibiotic for your child to take prior to and possibly after any dental procedure to prevent SBE.
• Children who have had heart surgery should continue to follow these rules throughout their lives.

Follow-up Appointments:

• Cardiology follow-up: 1 month after discharge
• Cardiothoracic surgery follow up: 1 month after discharge
• Pediatrician follow-up: 6 weeks after discharge for routine care and immunizations

Your child is vulnerable and at high risk of developing infections in the post-operative period. It is of utmost importance that basic hygiene is maintained and a clean environment at home is provided.

• You must wash your hands with soap and water and dry them with clean cloth before preparation of feed and giving medications to your child.
• The child’s cloths and bed linen should be clean and washed daily.
• The room where the child is recovering should be clean and well ventilated.
• Please avoid extremes of temperature in the room.
• The visitors particularly those who are harbouring signs of cough, cold or any illness should not come in contact with you or your child.
• Avoid exposing your child to crowded spaces like markets, theatres for 6 weeks.
• Avoid taking your child to festivals, weddings or other family functions for 6 weeks.

Oral hygiene and dental care

Good oral hygiene is of paramount importance as children with poor dental hygiene are at higher risk of developing infective endocarditis (Infection of the inner lining of Heart chambers)

• Sugar containing foods like juices, aerated drinks, sweets and chocolates should be avoided.
• Mouth should be rinsed after each feed.
• Clean your infant’s teeth with a clean cloth wrapped around your finger.
• Brush teeth at least twice daily after one year of age.
• No dental procedure should be performed for at least six weeks after surgery.
• Your child should begin visiting a dentist after two years of age and thereafter once a year.

Frequent loose motions for more than 48 hours invariably cause diaper rash. It can be very painful leading to excessive crying and irritability.

• The area should be kept dry and preferably exposed to air. It should be cleaned with a soft cloth or cotton wool soaked in warm water.
• Commercially available wipes, soap should be avoided. Talcum powder or any medicated powder should not be used.
• A thick layer of 15% Zinc oxide ointment should be applied over the rash. It should not be removed after each motion as it will disrupt the healing skin under the barrier. Instead any visible feacal matter should be removed and the ointment reapplied where necessary.
• If the diaper rash is not showing signs of healing after 72 hours, consult your doctor.

Excessive crying/irritability

Crying is a way of communication for infants. Most infants will cry for one to three hours daily. However, if the child is crying excessively beyond this period, a cause has to be found and treated. Generally, the surgical site is not painful after 5 to 7 days of surgery. An inconsolable child can be very distressing to the parents. The common caused of excessive crying are the following:

• Fever, pain
• Hunger, thirst, loneliness
• Soiled diaper or diaper rash
• Abdominal colic. Ensure burping the child after each feed.

Check your child’s temperature and diaper for soakage. Hold the child close to your chest; cuddle him/her and initiate feeds. Invariably the child will stop crying with these measures. If the child is still inconsolable beyond 24 hours please visit your doctor.

• Childhood diarrhoea is generally due to poor hygiene. You must wash your hands with soap and water and dry them with clean cloth before preparation of feed and giving medications to your child.
• The utensils should be washed clean and the water used for food preparation should be boiled and cooled.
• Any change in the consistency or frequency of stools should not be ignored. Children and particularly infants can rapidly develop dehydration. Most children are on diuretics (medicines which increase urine output) and are at increased risk of developing dehydration.
• The signs of dehydration are sunken eyes, dry mouth, lack of tears on crying, fall in urine output, irritability, fast shallow breathing and eventually drowsiness. This can be prevented by replacing fluids orally.
• Usually Diarrhoea gets better by 3 to 4 days. But if it persists and is associated with fever, vomiting and signs of dehydration, Child should be seen by the doctor.
• Breast feeds should be continued during diarrhoea. The diuretics (medicines which increase urine output) should be discontinued temporarily after consultation with your doctor. The buttocks should be kept dry to prevent diaper rash.
• Infection due to Rota virus is commonest cause of diarrhoea in children. Do vaccinate your child against Rota virus.

Home made oral rehydration solution (ORS)

• Add six spoons of sugar and half spoon of salt to one litre of boiled and cooled water. Store in a clean utensil and continue giving sips of this solution to prevent dehydration.

Your child may have been prescribed certain medications like warfarin/Acitrom which will increase the tendency to bleed. If your child is taking warfarin or Acitrom following precautions must be followed:-

• Your child must take the medication in the prescribed dose at same time everyday.
• A blood test called INR should be done regularly at a frequency prescribed by your doctor.
• The laboratory where the INR is done should be reliable. You must do the test at the same laboratory each time.
• The value of INR should be within the range as prescribed by your doctor.
• If the INR value is beyond this range, contact the doctor and he will adjust the dosage.
• The child should not indulge in contact sports or any activity which can increase the risk of injury.
• If you notice bruising, gum bleeding, blood in urine or stools, it's likely that the blood has become too thin. You must stop the “blood thinner” medication and visit the doctor immediately.
• If a dose of blood thinner is missed inadvertently, wait for the next dose at regular time.

If your child has undergone surgeries which will culminate into Fontan physiology, your child will have reduced exercise capacity. It is also absolutely necessary that their lung health is optimal. Following measures are advised to increase exercise capacity and maintain good lung-health.

• Maintain optimal weight. A well balanced diet and regular exercise will prevent obesity.
• Breathing exercises including Yoga should become part of your child’s routine. Breath should not be held after inspiration beyond a few seconds as this will compromise the Fontan flow.
• It is proven that training of breathing muscles and aerobic exercises improve the heart and lung function and quality of life in patients who have undergone Fontan operation.
• Children with Fontan should be prophylactically vaccinated against respiratory illnesses like influenza.
• Any sign or symptom of respiratory illness should be promptly treated.
• It is advisable to wear a mask when exposed to polluted, crowded spaces.

Your child may have one of the syndromes associated with heart defects which will reduce the ability to fight infections. Heterotaxy syndrome and DiGeorge syndrome are two such examples.

• Your child will need special care and additional prophylaxis against infections. Any sign or symptom of infection should be promptly evaluated and treated.
• Your child will need vaccinations against Influenza, meningococcal and pneumococcal infections. These vaccines are in addition to regular immunisation schedule.
• If your child has DiGeoge syndrome, he or she may need additional supplements of calcium and vitamin D.
• It is advisable to wear a mask when exposed to polluted, crowded spaces.

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Pacemaker is a device which sends and receives electrical signals to the heart and thereby control your child’d rate and rhythm. If your child has a pacemaker implanted following

 Precautions should be followed:

• The mobile phone should be at least six inches away from the pacemaker site.
• Any toy which has an electric battery or a large headphone with magnets should be at least six inches away from the pacemaker site.
• Your child can not undergo a diagnostic test called MRI.
• Your child will set-off the alarm while passing through the metal detectors at the mall/airports. You may be asked to show the medical report/certificate as proof of pacemaker implantation.
• If your child has to undergo a surgical procedure, use of electric cautery will interfere with the functioning of the pacemaker and the surgical team has to be informed of the same.
• Any activity or sport like kick-boxing/karate which is likely to cause repeated injury to the pacemaker site should be avoided.

Heart is one of the first organs to form in the foetus. An abnormal formation of the heart in the womb can lead to congenital heart defect. In most cases the factors causing this are not known. However, multiple genetic and environmental factors have been attributed. Some of these factors are as follows

• Mother has Diabetes
• Mother is taking medications for seizure disorder
• Mother is on Lithium for depression
• Mother has Phenylketonuria (PKU) and is not compliant with PKU diet
• Mother has Systemic Lupus Erythrometosis (SLE)
• Mother has been smoking (active or passive) or consuming Alcohol
• Mother had Rubelle (a viral infection) during first trimester. It imperative to vaccinate against Rubella before planning pregnancy.

*The incidence of a heart defect in subsequent offsprings is higher than normal population. This can be 3 to 80 times higher depending on the type of defect. Therefore it is imperative that fetal echocardiogram is performed in the second trimester between 18 to 24 weeks during subsequent pregnancy.

*The incidence of heart defect is higher if both parents are blood-relations. It is advisable to avoid marriages within the family.

*The incidence of heart defects is higher in the offsprings of parents with heart defects.

*The incidence of heart defects is higher in pregnancy from assisted reproductive technology.