1. What is a congenital cardiovascular defect?
Congenital means inborn or existing at birth. Congenital heart defects are the most common birth defects. A congenital cardiovascular defect occurs when the heart or blood vessels near the heart don’t develop normally before birth. A baby’s heart begins to develop at conception, but is completely formed by eight weeks into the pregnancy. Congenital heart defects happen during this crucial first eight weeks of the baby’s development. Specific steps must take place in order for the heart to form correctly. Often, congenital heart defects are a result of one of these crucial steps not happening at the right time
2. What causes congenital cardiovascular defects?
Congenital cardiovascular defects are present in about 1 percent of live births. They’re the most common congenital malformations in newborns. The vast majority of them have no known cause Some heart problems are likely to occur if the mother had a disease while pregnant and was taking medications, such as anti-seizure medicines. Sometimes a viral infection causes serious problems. German measles (rubella) is an example. If a woman contracts German measles while pregnant, it can interfere with how her baby’s heart develops or produce other malformations. Other viral diseases also may cause congenital defects.
Heredity sometimes plays a role in congenital cardiovascular defects. More than one child in a family may have a congenital cardiovascular defect, but this rarely occurs. Certain conditions affecting multiple organs, such as Down’s syndrome, can involve the heart, too. Some prescription drugs and over-the-counter medicines, as well as alcohol and “street” drugs, may increase the risk of having a baby with a heart defect. Researchers are studying other factors.
3.What are the types of congenital defects?
We can classify congenital heart defects into several categories in order to better understand the problems the baby will experience. They include:
- problems that cause too much blood to pass through the lungs
These defects allow oxygen-rich blood that should be traveling to the body to re-circulate through the lungs, causing increased pressure and stress in the lungs. It is also called Left to Right Shunt. In layman terminology, they are called pink babies with CHD.
- problems that cause too little blood to pass through the lungs
These defects allow blood that has not been to the lungs to pick up oxygen (and, therefore, is oxygen-poor) to travel to the body. The body does not receive enough oxygen with these heart problems, and the baby will be cyanotic, or have a blue coloring. It is also called Right to Left Shunt, Blue babies with CHD.
- problems that cause too little blood to travel to the body
These defects are a result of underdeveloped chambers of the heart or blockages in blood vessels that prevent the proper amount of blood from traveling to the body to meet its needs.
Signs and Symptoms
Pink babies with birth defects in the heart have increased pulmonary blood flow. They develop congestive heart failure with repeated chest infection (pneumonia) seeking medical attention. They have fast breathing and get tired easily difficult to suck mother’s milk (interrupted feeds). Because of insufficient feeds, they have problem of gaining weight (failure to thrive). They develop Pulmonary Arterial Hypertension (high lung pressure) and if they don’t seek proper treatment, they develop irreversible Pulmonary Arterial Hypertension (Eisenmenger Syndrome), which will be an inoperable condition.
Blue babies with CHD develop bluish discoloration first on the lips, tongue and finger and toe tips. Long standing cases, they develop clubbing of the fingers and toes. Some of them will have cyanotic spells and they become unconscious, sometime life threatening also. In long and neglected cases, the blood becomes high viscosity (thick) and sluggish to flow in the circulation, leads to blood clotting and absis formation, particularly in the Brain can lead to Neurological complications.
Most of the children are symptomatic and on clinical examination, one can hear heart murmurs. Apart from routine blood tests, X-Ray and ECG; Echocardiogram is the main investigative modality. Cardiac catheterization, CT and MRI Angio cardiogram supplement the diagnosis in difficult cases.
Treatment for these children could be medical, surgical and catheter interventions.
Pink babies with congestive heart failure can be treated with decongestive therapy and respiratory infections managed with antibiotics. Cyanosis and cyanotic spells are medical emergencies, should be treated with sedation, Oxygen and Metabolic correction. However, medical treatment is to stabilize the children and it is not a final treatment. Almost all children require surgery or catheter intervention, unless they have insignificant or most complex problem.
There are two ways of performing surgical corrections
- Open heart surgery
- Closed heart surgery
Open heart surgery
After opening the breast bone, child is put on heart lung machine which takes care of the heart and lung function (Cardiopulmonary Bypass) safely for sometime. The heart is arrested with a specially designed pharmacological solution. Then heart is opened and surgically repair the defects. After completion of the repair, patient is weaned off from heart lung machine.
Closed heart surgery
Surgery is performed on a beating heart and it is mainly extra cardiac and small intra cardiac repairs also.
Nature of surgery would be corrective (complete repair), palliative (to relieve the symptoms) and also it could be single stage primary correction and some complex cases require staged procedure to achieve final correction.
Catheter Interventions (Non surgical closures of defects)
Device closures can be done in some of the birth defects in heart like ASD, VSD, PDA; coil closure of MAPCAs, PDA and AV fistulas; neonatal balloon valvuloplasty for critical aortic, pulmonary, mitral and tricuspid valve stenosis; Balloon Angioplasty for Coarctation of Aorta, Pulmonary stenosis (for both proximal and distal); Balloon Atrial septostomy and PDA stentings etc.
About Individual Diseases
Problems that cause too much blood to pass through the lungs
Patent ductus arteriosus (PDA)
This defect allows blood to mix between the pulmonary artery and the aorta. Before birth an open passageway (the ductus arteriosus) exists between these two blood vessels. Normally this closes within a few hours of birth. When this doesn’t happen, some blood that should flow through the aorta and on to nourish the body returns to the lungs. A ductus that doesn’t close is quite common in premature infants but rather rare in full-term babies.
If the ductus arteriosus is large, a child may tire quickly, grow slowly, catch pneumonia easily and breathe rapidly. In some children symptoms may not occur until after the first weeks or months of life. If the ductus arteriosus is small, the child seems well. If surgery is needed, the surgeon can close the ductus arteriosus by tying it, without opening the heart. If there’s no other defect, this restores the circulation to normal.
Some congenital cardiovascular defects let blood flow between the heart’s right and left chambers. This happens when a baby is born with an opening between the wall (septum) that separates the right and left sides of the heart. This defect is sometimes called “a hole in the heart.”
The two most common types of this defect are atrial septal defect and ventricular septal defect. Two variations are Eisenmenger’s complex and atrioventricular canal defect.
- Atrial septal defect (ASD) — An opening exists between the heart’s two upper chambers. This lets some blood from the left atrium (blood that’s already been to the lungs) return via the hole to the right atrium instead of flowing through the left ventricle, out the aorta and to the body. Many children with ASD have few, if any, symptoms. Closing the atrial defect by open-heart surgery in childhood can prevent serious problems later in life.
- Ventricular septal defect (VSD) — An opening exists between the heart’s two lower chambers. Some blood that’s returned from the lungs and been pumped into the left ventricle flows to the right ventricle through the hole instead of being pumped into the aorta. Because the heart has to pump extra blood and is overworked, it may enlarge.
If the opening is small, it doesn’t strain the heart. In that case, the only abnormal finding is a loud murmur. But if the opening is large, open-heart surgery is recommended to close the hole and prevent serious problems. Some babies with a large ventricular septal defect don’t grow normally and may become undernourished. Babies with VSD may develop severe symptoms or high blood pressure in their lungs. Repairing a ventricular septal defect with surgery usually restores normal blood circulation. The long-term outlook is good, but long-term follow-up is required.
People with unrepaired ventricular septal defects are at risk for getting an infection of the heart’s walls or valves (endocarditis). After a VSD has been successfully fixed with surgery, antibiotics should no longer be needed.
Eisenmenger’s complex- Eisenmenger’s syndrome is a condition that includes a collection of symptoms: cyanosis (pale blue or grayish skin due to decreased oxygen in the blood), pulmonary hypertension (high blood pressure in the blood vessels of the lungs), and erythrocytosis (increased number of red blood cells in the blood due to decreased oxygen in the blood).
Eisenmenger’s syndrome primarily affects adolescents and adults with certain congenital heart defects that are repaired late (after the first year of life) or that are never repaired. Eisenmenger’s syndrome progresses over time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs because of congenital heart defects that cause blood flow from the left side of the heart to the right side of the heart (left-to-right shunt).
People with Eisenmenger’s complex, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis). Some individuals with Eisenmenger’s syndrome who have no other health complications may live into middle adulthood, and a few may survive into the fifth or sixth decade of life.
The goals of treatment for Eisenmenger’s syndrome are aimed at decreasing the pulmonary artery pressure, improving oxygenation, and decreasing degree of cyanosis and erythrocytosis.
Atrioventricular (A-V) canal defect (also called endocardial cushion defect or atrioventricular septal defect) —
Atrial and ventricular septal defects occur when the partitioning process does not occur completely, leaving openings in the atrial and ventricular septum. Also, the tricuspid and mitral valves that normally separate the heart’s upper and lower chambers aren’t formed as individual valves. Instead, a single large valve forms that crosses the defect. The large opening in the center of the heart lets oxygen-rich (red) blood from the heart’s left side – blood that’s just gone through the lungs – pass into the heart’s right side. There, the oxygen-rich blood, along with venous (bluish) blood from the body, is sent back to the lungs. The heart must pump an extra amount of blood and may enlarge. Most babies with an atrioventricular canal don’t grow normally and may become undernourished. Because of the large amount of blood flowing to the lungs, high blood pressure may occur there and damage the blood vessels.
In some babies the common valve between the upper and lower chambers doesn’t close properly. This lets blood leak backward from the heart’s lower chambers to the upper ones. This leak, called regurgitation or insufficiency, can occur on the right side, left side or both sides of the heart. With a valve leak, the heart pumps an extra amount of blood, becomes overworked and enlarges.
In babies with severe symptoms or high blood pressure in the lungs, surgery usually must be done in infancy. Surgical repair consists of closing the large hole with one or two patches and dividing the single valve between the heart’s upper and lower chambers to make two separate valves. Surgical repair of an atrioventricular canal usually restores the blood circulation to normal. However, the reconstructed valve may not work normally.
Rarely, the defect may be too complex to repair in infancy. In this case, the surgeon may do a procedure called pulmonary artery banding to reduce the blood flow and high pressure in the lungs. When a child is older, the band is removed and corrective surgery is done. More medical or surgical treatment is sometimes needed.
Another type of heart defect is congenital cyanotic heart defects. In these defects, blood pumped to the body contains less oxygen than normal. This causes a condition called cyanosis, a blue discoloration of the skin. Infants with cyanosis are often called “blue babies.”
Examples of cyanotic defects are tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, pulmonary atresia, truncus arteriosus and total anomalous pulmonary venous connection.
- Tetralogy of Fallot has four components. The two major ones are a large hole, or ventricular septal defect, that lets blood pass from the right to the left ventricle without going through the lungs; and a narrowing (stenosis) at or just beneath the pulmonary valve. This narrowing partially blocks the blood flow from the heart’s right side to the lungs. The other two components are: the right ventricle is more muscular than normal; and the aorta lies directly over the ventricular septal defect.
This results in cyanosis (blueness), which may appear soon after birth, in infancy or later in childhood. These “blue babies” may have sudden episodes of severe cyanosis with rapid breathing. They may even become unconscious. During exercise, older children may become short of breath and faint. These symptoms occur because not enough blood flows to the lungs to supply the child’s body with oxygen.
Some infants with severe tetralogy of Fallot may need an operation to give temporary relief by increasing blood flow to the lungs with a shunt. This is done by making a connection between the systemic artery and the pulmonary artery. Then some blood from the aorta flows into the lungs to get more oxygen. This reduces the cyanosis and allows the child to grow and develop until the problem can be fixed when they are older.
Most children with tetralogy of Fallot have open-heart surgery before school age. The operation involves closing the ventricular septal defect and removing the obstructing muscle with or without transannular patch. After surgery the long-term outlook varies, depending largely on how severe the defects were before surgery.
- Transposition of the great arteries —
The positions of the pulmonary artery and the aorta are reversed. The aorta is connected to the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs. The pulmonary artery is connected to the left ventricle, so most of the blood returning from the lungs goes back to the lungs again.
Infants born with transposition survive only if they have one or more connections that let oxygen-rich blood reach the body. One such connection may be a hole between the two atria, called atrial septal defect, or between the two ventricles, called ventricular septal defect. Another may be a vessel connecting the pulmonary artery with the aorta, called patent ductus arteriosus. Most babies with transposition of the great arteries are extremely blue (cyanotic) soon after birth because these connections are inadequate.
To improve the body’s oxygen supply, a special procedure called balloon atrial septostomy is used in some patients. Two general types of surgery may be used to help fix the transposition. One is a atrial switch or intra-atrial baffle procedure that creates a tunnel inside the atria. Another is an arterial switch. After surgery, the long-term outlook varies quite a bit. It depends largely on how severe the defects were before surgery. Lifelong follow-up is needed.
- Tricuspid atresia —
In this condition, there’s no tricuspid valve. That means no blood can flow from the right atrium to the right ventricle. As a result, the right ventricle is small and not fully developed. The child’s survival depends an atrial septal defect and a ventricular septal defect. Because the circulation is abnormal, the blood can’t get enough oxygen, and the child looks blue (cyanotic).
Often a surgical shunting procedure is needed to increase blood flow to the lungs. This reduces the cyanosis. Some children with tricuspid atresia have too much blood flowing to the lungs. They may need a procedure (pulmonary artery banding) to reduce blood flow to the lungs. Bidirectional Glenn operation is required later .Other children with tricuspid atresia may have a more functional repair (Fontan procedure). Children with tricuspid atresia require lifelong follow-up.
- Pulmonary atresia—
No pulmonary valve exists, so blood can’t flow from the right ventricle into the pulmonary artery and on to the lungs. The right ventricle acts as a blind pouch that may stay small and not well developed. The tricuspid valve is often poorly developed, too.
An opening in the atrial septum lets blood exit the right atrium, so venous (bluish) blood mixes with the oxygen-rich (red) blood in the left atrium. The left ventricle pumps this mixture of oxygen-poor blood into the aorta and out to the body. The baby appears blue (cyanotic) because there’s less oxygen in the blood circulating through the arteries. The only source of lung blood flow is the patent ductus arteriosus (PDA), an open passageway between the pulmonary artery and the aorta. If the PDA narrows or closes, the lung blood flow is reduced to critically low levels. This can cause very severe cyanosis.
Early treatment often includes using a drug to keep the PDA from closing. A surgeon can create a shunt between the syatemic and the pulmonary artery to help increase blood flow to the lungs. A more complete repair depends on the size of the pulmonary artery and right ventricle. If they’re very small, it may not be possible to correct the defect with surgery. In cases where the pulmonary artery and right ventricle are a more normal size, open-heart surgery may produce a good improvement in how the heart works.
If the right ventricle stays too small to be a good pumping chamber,Fontan procedure can be done. Children with tricuspid atresia require lifelong follow-up by a cardiologist.
- Truncus arteriosus—
This is a complex malformation where only one artery arises from the heart and forms the aorta and pulmonary artery. Surgery for this condition usually is required early in life. It includes closing a large ventricular septal defect within the heart, detaching the pulmonary arteries from the large common artery, and connecting the pulmonary arteries to the right ventricle with a tube graft. Children with truncus arteriosus need lifelong follow-up to see how well the heart is functioning.
- Total anomalous pulmonary venous (P-V) connection —
The pulmonary veins that bring oxygen-rich (red) blood from the lungs back to the heart aren’t connected to the left atrium. Instead, the pulmonary veins drain through abnormal connections to the right atrium.
In the right atrium, oxygen-rich (red) blood from the pulmonary veins mixes with venous (bluish) blood from the body. Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium. From there it goes into the left ventricle, to the aorta and out to the body. The rest of the poorly oxygenated mixture flows through the right ventricle, into the pulmonary artery and on to the lungs. The blood passing through the aorta to the body doesn’t have enough oxygen, which causes the child to look blue (cyanotic).
This defect must be surgically repaired in early infancy. The pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. When surgical repair is done in early infancy, the long-term outlook is very good.
An obstruction is a narrowing that partly or completely blocks the flow of blood. Obstructions called stenoses can occur in heart valves, arteries or veins.
The three most common forms are pulmonary stenosis, aortic stenosis and coarctation of the aorta. Related but less common forms include bicuspid aortic valve, sub aortic stenosis and Epstein’s anomaly.
- Pulmonary stenosis (PS) — A defective pulmonary valve that doesn’t open properly is called stenotic. This forces the right ventricle to pump harder than normal to overcome the obstruction.
If the stenosis is severe, especially in babies, some cyanosis (blueness) may occur. Older children usually have no symptoms. Treatment is needed when the pressure in the right ventricle is higher than normal. In most children the obstruction can be relieved by a procedure called balloon valvuloplasty. Others may need open-heart surgery. Surgery usually opens the valve satisfactorily. The outlook after balloon valvuloplasty or surgery is favorable, but follow-up is required to determine if heart function returns to normal.
- Aortic stenosis (AS) — The aortic valve is narrowed. The heart has difficulty pumping blood to the body. Aortic stenosis occurs when the aortic valve didn’t form properly. A normal valve has three leaflets (cusps) but a stenotic valve may have only one cusp (unicuspid) or two cusps (bicuspid), which are thick and stiff.
Sometimes stenosis is severe and symptoms occur in infancy. Otherwise, most children with aortic stenosis have no symptoms. Some children may have chest pain, unusual tiring, dizziness or fainting. The need for surgery depends on how bad the stenosis is. In children, a surgeon may be able to enlarge the valve opening. Surgery may improve the stenosis, but the valve remains deformed. Eventually the valve may need to be replaced with an artificial one.
Balloon valvuloplasty has been used in some children with aortic stenosis. The long-term results of this procedure are still being studied. Children with aortic stenosis need lifelong medical follow-up. Even mild stenosis may worsen over time, and surgical relief of a blockage is sometimes incomplete .
- Coarctation of the aorta (“Coarct”) — The aorta is pinched or constricted. This obstructs blood flow to the lower body and increases blood pressure above the constriction. Usually there are no symptoms at birth, but they can develop as early as a baby’s first week. A baby may develop congestive heart failure or high blood pressure that requires early surgery. Otherwise, surgery usually can be delayed. A child with a severe coarctation should have surgery in early childhood. This prevents problems such as developing high blood pressure as an adult.
The outlook after surgery is favorable, but long-term follow-up is required. Rarely, coarctation of the aorta may recur. Some of these cases can be treated by balloon angioplasty. The long-term results are still being studied. Also, blood pressure may stay high even when the aorta’s narrowing has been repaired.
- Bicuspid aortic valve —
The normal aortic valve has three flaps (cusps) that open and close. A bicuspid valve has only two flaps. There may be no symptoms in childhood, but by adulthood (often middle age or older), the valve can become stenotic (narrowed), making it harder for blood to pass through it, or regurgitant (allowing blood to leak backward through it). Treatment depends on how well the valve works.
- Subaortic stenosis—
Stenosis means constriction or narrowing. Subaortic means below the aorta. Subaortic stenosis refers to a narrowing of the left ventricle just below the aortic valve, which blood passes through to go into the aorta. This stenosis limits the flow of blood out of the left ventricle. This condition may be congenital or may be due to a particular form of cardiomyopathy known as “idiopathic hypertrophic subaortic stenosis” (IHSS). Treatment depends on the cause and the severity of the narrowing. It can include drugs or surgery.
- Ebstein’s anomaly is a congenital downward displacement of the tricuspid valve (located between the heart’s upper and lower chambers on the right side) into the heart’s right bottom chamber (right ventricle). It’s usually associated with an atrial septal defect (see below).
Hypoplastic left heart syndrome
In hypoplastic left heart syndrome, the left side of the heart is underdeveloped – including the aorta, aortic valve, left ventricle and mitral valve. Blood returning from the lungs must flow through an opening in the wall between the atria, called an atrial septal defect. The right ventricle pumps the blood into the pulmonary artery, and blood reaches the aorta through a patent ductus arteriosus.
The baby often seems normal at birth, but will come to medical attention within a few days as the ductus closes. Babies with this syndrome have rapid and difficult breathing and have difficulty feeding. This heart defect is usually fatal within the first days or months of life without treatment.
This defect isn’t correctable, but some babies can be treated with a series of operations or with a heart transplant. Until an operation is performed, the ductus is kept open by intravenous (IV) medication. Surgery is done in several stages. The first stage, called the Norwood procedure or Sano procedure, allows the right ventricle to pump blood to both the lungs and the body. It must be performed soon after birth. The final stage operation includes bi-directional Glenn, Fontan operation and lateral tunnel. These operations create a connection between the veins returning blue blood to the heart and the pulmonary artery. The overall goal is to allow the right ventricle to pump only oxygenated blood to the body and to prevent or reduce mixing of the red and blue blood. Some infants require several intermediate operations to achieve the final goal.
Some surgeons recommend a heart transplant to treat this problem. Although it provides the infant with a heart that has normal structure, the infant will require lifelong medications to prevent rejection. Many other problems related to transplants can develop. Children with hypoplastic left heart syndrome require lifelong follow-up by a cardiologist for repeated checks of how their heart is functioning. Virtually all the children will require heart medicines.
Management of conduction disturbance
The rate and rhythm disturbances can occur with birth defects or following the surgical correction. Some of the rate and rhythm disturbances can be managed medically. The slower heart rate is also called complete heart block, can be treated with permanent pace maker implantation which need to be changed once in 5-10 years.
The American Heart Association has recently changed its guidelines for prevention of endocarditis. Some children who used to take antibiotics before going to the dentist no longer have to do so.
The AHA now recommends these routine antibiotics before dental visits for patients who are at the highest risk for adverse outcomes if they develop endocarditis. This includes:
- People with a prosthetic cardiac valve
- People who have previously had endocarditis
- People with certain types of congenital heart defects, including
- Unrepaired cyanotic congenital heart defects, including palliative shunts and conduits
- Completely repaired congenital heart defects with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first six months after the procedure (prophylaxis is recommended for first six months because endothelialization of prosthetic material occurs within six months after the procedure)
- Repaired congenital heart defect with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization)
- Cardiac transplantation recipients who develop heart valve dysfunction
Except for the conditions listed above, antibiotic prophylaxis is no longer recommended for any other form of congenital heart disease.
Routine antibiotics before gastrointestinal or genitourinary tract procedures solely to prevent endocarditis are no longer recommended for any patients.
Timing of Surgery
Neonatal (<1 month) surgeries to be done in more severe form of heart conditions like
– Duct dependent pulm. Circulation
– Pulmonary stenosis/atresia ± VSD
– Duct dependent syst. Circulation
– Coarctation/ interrupted aortic arch
– Obstructed TAPVC
Infant (<1 year) corrections are to be done in more severe form of heart conditions like
- AV canal defects
- Truncus arteriosus
- AP window
Ideally they should be operated around 3 months of age before they develop pulmonary arterial hypertension
Pre School (<3 year) corrections are to be done in less severe form of heart diseases like
- Moderate sized VSD
- PDA . CoA
- Single ventricle, PS
- Rastelli (conduit) procedure
- Presence of CHD – indication for surgery
- Almost all cases should be corrected by preschool
- Young age is not a risk factor
After surgical correction, these children require regular follow up for atleast once in 1-2 years after initial follow up after surgery. This is like a preventive check up for these children for early detection and treatment.
Life after surgery
Many children who have undergone complete correction live like normal children without any need for cardiac medication. There life expectancy and quality of life are near normal to normal population. They can get married and can have children. Children who had palliative surgery and surgery for complex congenital heart disease may have some long term associated problems requiring medication and surgical or catheter intervention. They need to use bacterial endocardiatic prophylaxis before any surgical intervention.
Advantages of early correction
By performing early correction, we can avoid repeated hospitalization, prevent pulmonary arterial hypertension and cyanosis and their complications. They can have early normalization of cardiovascular physiology and their life expectancy would be normal or near normal. They will have good physical development and prevent emotional instability.
Sick child increases the family burden in terms of familiar economic and social aspects. It can create adverse impact on healthy siblings. Early correction brings near normal children and they could be useful citizens, can take care of themselves and their families.
Myths about spontaneous closure of heart defects
Very few percentage of small heart defects like VSD and ASD will have spontaneous closure but not all. The chances of spontaneous closure will decrease as the child grows older. The defects rarely close after one year of age. Early correction is required if the child has intractable congestive heart failure, failure to thrive and develop severe pulmonary arterial hypertension. Some of the defects like large perimembranous VSD and supracrystal VSDs do not have spontaneous closure.
Children after prosthetic valve or conduit replacements need to take anti coagulants (blood thinning tablets) based on the INR status. These children can have bleeding problems for over dosage and clotting problems with under use of medication. Palliative / staged procedure patients require medical supervision for the final correction. If they have significant residual problems, may require catheter or surgical interventions.
Embryology and Fetal medicine
When the embryo is two weeks old in the mother’s womb, two heart tubes are formed in the region of the chest. In the third week, both the tubes unite to form a single tube. In the fourth week of gestation, the heart tubes forms five divisions by four constructions which lead to aortic arch, ventricles, sinus venosus, bulbus cardis and atrium. By 16 weeks of gestation, the heart is fully formed.
Antenatal diagnosis and Intervention
With advanced technology, antenatal diagnosis of birth defects in the heart can be diagnosed by echocardiogram by specialists like pediatric cardiologists and sonologists. This should be done after 18 weeks of gestation. High risk pregnancy mothers like elderly primies, families of congenital heart defects, diabetics and any other genetic and metabolic abnormality mothers. This helps counseling of the parents and proper planning of delivery and management of the child after birth.
Apart from the routine surgical corrections, we have great interest and World class experience in corrections of complex congenital heart defects such as Single Stage Unifocalization for VSD, Pulmonary Atreisa and Major Aorto Pulmonary Collateral Arteries (MAPCAs), Single Stage Correction for intra cardiac anomalies associated with arch obstruction, AV Canal repair, TAPVC corrections and neonatal corrections like Arterial Switch Operation etc.
Rheumatic Heart Diseases
In children, after Congenital Heart Disease, second most leading causes of heart disease. Even though, incidence of rheumatic heart disease has markedly reduced in developing countries, Worldwide, especially in developing countries it remains a major health problem. Chronic rheumatic heart disease is estimated to occur 5-30 million children and young adults and about 90 thousand individuals die each year.
Rheumatic fever develops following pharyngitis with group A beta hemolytic streptococcus. They develop joint pains, heart infection, neurological and skin involvements. About 40% of patients with acute rheumatic fever may develop varying degrees of heart disease. With chronic Rheumatic Heart Disease, patients develop valvular stenosis regurgitation, arrhythmias and heart failure. It licks the joints and bites the heart. Diagnosis can be made by blood tests like ESR, C reactive protein, ASO titre and anti-DNAse B. Echocardiogram is the main stay for diagnosis valvular heart disease.
Acute rheumatic fever is treated with penicillin, ampiciline, amoxicillin etc. Acute rheumatic cardiatis is treated with Acetylsalicylic acid (Aspirin) and Steroids. Long acting penicillin or oral penicillin are used for rheumatic prophylaxis which need to be taken for 40-45 years of age to prevent progress of Rheumatic Valvular Heart Disease. Valvular Heart Disease is treated medically with decongestive therapy and surgically by valve replacement or repair. Stenotic valves can be dilated non surgically by balloon dilatation.
Idiopathic Heart Disease
In this spectrum, the exact cause of heart disease is not known and hence treatment options are very limited and symptomatic oriented. They include dilated cardiomyopathy, Hypertrophic cardiomyopathy, Arrhythmogenic right ventricular cardiomyopathy, restrictive cardiomyopathy, Noncompaction Cardiomyopathy and post viral cardiomyopathy etc. These children present with congestive heart failure requiring decongestive therapy and sometimes inotropic support. End stage disease requires heart transplantation.
Primary pulmonary arterial hypertension children are managed with pulmonary vaso dilators. They present with right heart failure. Severe form of disease requires Lung or Heart and Lung transplant.
44. Dedicated pediatric cardiac centre: How different and how good ?
Children’s are not miniature adults. Their physiology and disease process are entirely different from adults. Surgeries on heart and lungs among children are the most intricate of all surgeries done on the human body. This is so particularly because of small size, tissue immaturity, high energy and metabolic requirement and immunity. They need technologically advanced miniature equipment for the diagnosis and treatment. Specially trained medical professionals and para medical staff are required to take care of the tiny tots. Because of small in nature, safety margin is less in these children, require high surgical skills. By having a dedicated pediatric cardiac team, they can perform more surgeries and can achieve excellent results.
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