The two types of heart disease in children are "congenital" and "acquired." Congenital heart disease (also known as a congenital heart defect) is present at birth. Some defects in this category are patent ductus arteriosis, atrial septal defects and ventricular septal defects. Acquired heart disease, which develops during childhood, includes Kawasaki disease, rheumatic fever and infective endocarditis.

Congenital Heart Defects in Children Fact Sheet

What is a congenital heart defect?
Congenital heart defects are structural problems with the heart present at birth. They result when a mishap occurs during heart development soon after conception and often before the mother is aware that she is pregnant. Defects range in severity from simple problems, such as "holes" between chambers of the heart, to very severe malformations, such as complete absence of one or more chambers or valves.

Is all heart disease in children congenital?
No, but most is. These defects are usually but not always diagnosed early in life. Rarely, heart disease is not congenital but may occur during childhood such as heart damage due to infection. This type of heart disease is called acquired; examples include Kawasaki disease and rheumatic fever. Children also can be born with or develop heart rate problems such as slow, fast, or irregular heart beats, known as "arrhythmias".

Who is at risk to have a child with a congenital heart defect?
Anyone can have a child with a congenital heart defect. Out of 1000 births, 8 babies will have some form of congenital heart disorder, most of which are mild. If you or other family members have already had a baby with a heart defect, your risk of having a baby with heart disease may be higher.

How many people in the India  have a congenital heart defect?
The incidence of congenital heart disease is 1 out of every 100 new born. That implies that in India about 2,50,000 children with heart problem are born every year. This is a number ten times that of adults with heart problems. Hardly 5% of cases of congenital heart diseases are treated in India and that makes it amongst the top five causes of child mortality in India. The most common heart complication is a hole in the heart.

Pediatric cardiac care requires extremely varied types of surgeries and the complexities can have multiple combinations as against adult surgeries.

But the facilities for treatment of children are few and far between in India.

The treatment of children with congenital heart disease has improved dramatically over the past decade or so.

Why do congenital heart defects occur?
Most of the time we do not know. Although the reason defects occur is presumed to be genetic, only a few genes have been discovered that have been linked to the presence of heart defects. Rarely the ingestion of some drugs and the occurrence of some infections during pregnancy can cause defects.

How can I tell if my baby or child has a congenital heart defect?
Severe heart disease generally becomes evident during the first few months after birth. Some babies are blue or have very low blood pressure shortly after birth. Other defects cause breathing difficulties, feeding problems, or poor weight gain. Minor defects are most often diagnosed on a routine medical check up. Minor defects rarely cause symptoms. While most heart murmurs in children are normal, some may be due to defects.

How serious is the problem?
Congenital heart defects are the most common birth defect and are the number one cause of death from birth defects during the first year of life. Nearly twice as many children die from congenital heart disease.

Are things improving?
Definitely. Overall mortality has significantly declined over the past few decades. For example, in the 1960s and 1970s the risk of dying following congenital heart surgery was about 30% and today it is around 5%.

How well can people with congenital heart defects function?
Virtually all children with simple defects survive into adulthood. Although exercise capacity may be limited, most people lead normal or nearly normal lives. For more complex lesions, limitations are common. Some children with congenital heart disease have developmental delay or other learning difficulties.

What is the social/financial impact of congenital heart defects?
Successful treatment requires highly specialized care. Severe congenital heart disease requires extensive financial resources both in and out of the hospital. Children with developmental delay also require community and school-based resources to achieve optimum functioning.

What is the impact of congenital heart disease on families?
The presence of a serious congenital heart defect often results in an enormous emotional and financial strain on young families at a very vulnerable time. Patient/family education is an important part of successful coping.

Prevalence of Congenital Heart Diseases in India

The incidence of congenital heart disease is 1 out of every 100 new born. That implies that in India about 2,50,000 children with heart problem are born every year. This is a number ten times that of adults with heart problems. Hardly 5% of cases of congenital heart diseases are treated in India and that makes it amongst the top five causes of child mortality in India. We also have a large no of adult patients with CHD, primarily because of lack of health awareness and inadequate health care facilities.

Profile of CHD in India

The profile of CHD varies depending upon the age group. Simple and potentially correctable heart defects, like ventricular septal defect, patent ductus arteriosus and atrial septal defect, are common at all ages.

Reasons for Poor Cardiac Care

1. Lack of awareness. Only a very small fraction of CHD cases are detected at birth and during infancy. This is probably the most important reason for the dismal state of affairs concerning children with CHD in our country. Firstly, most births occur without supervision of a pediatrician. Secondly, the ability of most pediatricians to detect heart disease is very limited, because of inadequate exposure to pediatric cardiology during their postgraduate training program. This is especially true for newborns where the abnormality on clinical examination may be very subtle. Additionally, there are no compulsory update programs for practicing pediatricians to keep them abreast of the upcoming developments. Several pediatricians still believe that a newborn baby with CHD has a near 100% mortality, even if operated.

2. Due to the limited knowledge of natural history of CHD, there is considerable time lag between diagnosis and referral to a pediatric cardiac center for intervention. In addition, delay may also result from inaccurate diagnosis. Often there is total lack of awareness about what facilities are available with in the country and about recent developments in the specialty. Some of the pediatricians specially in rural and semi urban areas, still believe that a child with ventricular septal defect can only be operated after he or she attains a weight of 10 kg. This delay may result in complications like hypoxic brain damage, Eisenmenger’s syndrome etc. These complications not only compromise on the results of intervention, but also result in sub optimal utilization of limited resources available for the pediatric cardiac care in India.

3. There are no pediatric cardiac care programs in several states in India as highlighted earlier. Families have to travel hundreds of kilometers to reach a centre, which is equipped with necessary facilities.

Besides expenses involved in traveling and staying in an alien city, there is considerable income loss due to lost work for number of days. This double disincentive often results in further delay to the point of sometimes making it too late for treatment.

4.  Heart disease in children is not identified as a health priority by the government in our country. There are no government policies for cardiac care in children. This is perhaps related to the prevailing notion that CHD is uncommon, often fatal and is therefore not worth expanding national resources.

5. Many families in rural and semi urban parts of India seek advice from unqualified, self-proclaimed “doctors” and quacks because of common beliefs and myths. This further adds to the delay in diagnosis and proper management of the child with CHD.

Suggested Remedial Measures

1. Financial assistance for providing care to the affected child. This can be given by: (a) Government agencies through various policies/welfare funds (b) Medical insurance of expecting mothers to insure the child (c) Nongovernmental and voluntary organizations

2. Involvement of voluntary organizations and industry to support and develop pediatric cardiac care programs in various existing centers, which have good adult cardiac care facilities. These centers have infrastructure available, which can be utilized. Training in pediatric cardiac care can be initiated by bringing in staff from well-developed centres; either in India or abroad, the local staff can be trained in a gradual manner.

3. Optimal utilization of technology. Unlike in the West, for India, it may be more economical to share infrastructure with adult cardiology and cardiac surgery, which is much better established in different parts of India. The feasibility and logistics can be sorted out if there is willingness to share. Similarly the ancillary staff and junior fellows can be shared with adult cardiology and surgical units which can help to run both the programs with increased buffer available in case there is shortage of personnel. This scheme sometimes has the disadvantage of preferential adult care over pediatric care.

4. Creation of a national database for epidemiology, cardiac surgery, cardiac intervention etc. This database will help us to recognize and understand the problems specific to India, differences from western data and help devise solutions. The response from government is likely to be better, if a plan is formulated based on the experience from our own country. It is difficult to make national policies for the future if accurate statistics and demographic data are not available.

5. Prioritization of care: Since we have limited resources, some people have argued for prioritization of care according to complexity of CHD. For example, parents of a newborn with single ventricle of right ventricular morphology with situs ambiguous may be discouraged to enter the surgical program, which may go on in three stages with impaired quality of life even at the end of complete palliation. This baby, if operated may remain in intensive care unit for several weeks. On the other hand a child with a simple ventricular septal defect, where quality of life is likely to be normal after correction should be given priority. Several others oppose this scheme, as ideally every child should be given equal opportunity to get best treatment available. A consensus needs to evolve on this issue.

6. A more active role is required from the state and federal governments in formulation of policies for betterment of care for children with CHD. For achieving this the pediatric cardiac care centers should provide data to show that the current mortality and morbidity of cardiac surgery for large majority of children is very low. This may change the prevailing beliefs and bring in active participation of the policy makers in the government. Establishment of more government sponsored centers, which can provide excellent care at affordable cost should be encouraged.

Acquired Heart Diseases

Rheumatic Heart Diseases
Children between the age group of five and 15 years are the most possible preys of Rheumatic Heart Diseases. In India, this is one of the largest killers among diseases of children. As per the studies, 8 out of every 1000 kids suffer from a rheumatic heart disorder. Early symptoms of rheumatic heart disease in children include fatigue, shortness of breath, palpitation, chest pains and in many cases congestive heart failure.

Heart Failure
The term ‘heart failure’ describes a situation when the heart is not functioning properly. Like adults, heart failure can also occur newborns, infants, toddlers and teenagers but for the reasons very different from those that cause heart failure in adults. It can also occur in newborns, infants, toddlers and teenagers for other reasons.  Since heart failure has different causes and outcomes, it's important to recognize how it's diagnosed, treated and even cured in younger children.  Heart failure in children occurs mainly because of the these reasons:

• Over circulation Failure: This defect occurs when blood mixes inside the heart due to a congenital heart defect. 
• Pump Failure: Pump Failure occurs when the heart muscle becomes damaged and no longer contracts normally.

HOW PARENTS CAN HELP:

Look out for symptoms of congenital heart defects:

At birth

• Blueness at birth or immediately after birth
• Murmur of the heart
• Accelerated breathing
• High pressure in the lungs
• Low blood pressure

At Two to six months

• Difficulty in feeding – baby is unable to suck properly, sweats or starts breathing fast while feeding
• Blue nails and toes. Fainting spells
• Inadequate weight gain
• Recurrent chest infection

First three years

• Fainting spells (which may sometimes be fatal)
• Abnormal heartbeats
• Avoid rigorous activity for the child. Ensure the child does not get infected, too. Maintain the salt balance in the child’s body.

 Drugs to avoid during pregnancy 

• A strict no to isoretinoin, thalidomide, estrogens, oral contraceptives, chloramphenicol, chlorpropamide, erythromycin, tetracycline and haloperidol.
• Anti-cancer drugs and phenytoin are harmful but the benefits of these drugs outweigh the side effects
• Epinephrine, ephedrine, b-blockers and promethazine do not pose any significant risk, though research is inadequate.
• Multivitamins can be safely taken

Common Heart Defects that appear at birth:

• Atrial septal defect: A hole in heart between the upper chambers-the right and left atrium- causes blood to flow from the left chamber to the right. 

• Transposition of the greater arteries: The two major arteries leaving the heart- aorta carrying oxygen- rich blood to the rest of the body and pulmonary artery carrying impure blood to the lungs- arise from the wrong chamber.

• Single Ventricle Cardiac Anomaly: A group of different cardiac defects where only one ventricle is of adequate functional size.

• Patent Ductus Arteriosus: In the womb the fetal lungs are not in use. Blood therefore flows through a passageway called ductus arterious which normally closes soon after birth. If it does not usually in premature babies, blood does not flow correctly.

• Hypoplastic left heart syndrome: Malformation of the left side of the heart-the side which receives oxygen –rich blood from the lungs and pumps it to the body.

• Ebstein Anomaly: Two leaflets of the triscupid valve between the right atrium and the right ventricle are displaced downward and the third is elongated and stuck to the chamber wall. Blood flows back into the atrium when the ventricle contracts.

• Interrupted aortic arch: Absence of a portion of the aortic arch; in normal cases, the aorta , after it leaves the heart, branches off into blood vessels to the arms and head and arches down towards the lower half of the body.

• Truncus arterious: Instead of two, there is only one great blood vessel leaving the heart, which then branches into vessels that go to the lungs and the body.

• Tetralogy of Fallot –Blue Baby: A combination of four related defects; pulmonary stenosis; ventricular septal defect; enlarged aortic valve ;thickening of the walls of the right ventricle, impure blood flows to the body so that babies with the problem appear blue.

• Coarctation of the aorta: A defect in which the part of the aorta is too narrow and affects the blood flow.