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COUNSELING OF PARENTS OF CHILDREN WITH CONGENITAL AND ACQUIRED HEART DISEASE

Introduction:

Illness in a child is wont to give a lot of psychological stress to parents. This is more so if the parents come to know that their child has congenital heart disease (which afflicts 1%of the population at any given time). Initially parents are in a denial that such an ailment is present in their child. As doctors it is our duty to guide them through this stressful period and to show them that there is hope in case the ailment is curable. One has to handle such parents with compassion and should emphatically discuss with them the problems in their child and the fact that it is curable. In this the involvement of a pediatric cardiologist is a must so that details of the diagnosis could be discussed. This initial counseling will play a big role in preparing the parents for corrective surgery for their. We have below a brief description of all the congenital heart disease and what the parents have to know about this and what should they expect once the ailment is corrected.

Brief discussion of Congenital heart defects

As stated in the brief introduction, we will now discuss in brief about the relevant questions that parents may ask about congenital heart defects.

What are congenital heart defects?

Congenital heart defects (CHD) are problems with the heart’s structure that developed in the womb or early development and are present at birth or shortly thereafter.  Each year, about 1 percent or 30,000 babies are born with the defect, making it the most common birth defect in the U. S. The same trend holds true for our country. Though detailed census is not available for the rest of the world but the incidence is the same as reflected in the US. In our country with 30 million births per year the quantity of new population with congenital heart defect is quite large. Congenital heart defects vary widely in structure and severity.  Some defects can be fatal, but thanks largely to new treatments, most affected individuals survive their childhood and live relatively normal lives.  Over 1,000,000 adults are currently living with a congenital heart disease. They come under a totally different category that is Grown Up Congenital Heart Disease (GUCH).

How do congenital heart defects develop?

As a fetus’s heart develops from a simple tube to a four-chambered heart with associated veins and arteries, defects may develop.  Part of the heart may develop partially or not at all, a hole in the wall of the heart may form, or the heart arteries and veins may form abnormal connections with the heart.  Congenital heart defects are often thought to be genetic, but they can also be caused by illness or behavioral factors in the mother such as viral infections like German measles, certain prescription drugs and over-the-counter medicines, alcohol, or illegal drugs.  Some conditions like Down syndrome and Turner syndrome are associated with CHDs. 

What are the types of congenital heart defects?

Congenital heart defects vary widely in structure and severity.  CHDs usually involve one of the following: abnormal passages in the heart or between blood vessels, problems with the valves that control the emptying and filling of the heart chambers, mismatched or abnormally located or developed blood vessels near the heart, or structural or developmental malformations in the heart itself.  The details of these will be discussed later.

What are the risk factors for congenital heart defects?

A person with a congenital defect is more likely to have a child with a defect than the average person.  Some factors increase a mother’s chance of having a child with a defect including viral infections, diabetes, certain legal and illegal drugs, some chemicals or x-rays, and alcohol consumption during pregnancy. In addition age of mother is also associated with certain syndromes that have associated congenital heart defects (Down syndrome in elderly mothers > 37 yrs age).

A DESCRIPTION OF NORMAL HEART ANATOMY IS GIVEN BELOW

DISCUSSION OF NORMAL CARDIAC ANATOMY

For parents who have read life science in their 10th or 12th standard it will be easy to understand the normal cardiac anatomy.

The primary function of the heart is to pump blood to all parts of the body and to take in impure blood and pump it into the lungs where purification (i.e., oxygenation) takes place. The normal heart is composed of four chambers, 2 on the right side and two on the left side. Under normal circumstances the two sides are not connected to each other. Hence these are basically parallel to each other. In the schematic diagram below we show the normal structure of the heart.

NORMAL HEART STRUCTURE

With a weight between 7 and 15 ounces (200 to 425 grams) your heart is a little larger than the size of your fist. It is one of the most efficient pumps in nature and by the end of a long life; a person's heart may have beat (expanded and contracted) more than 3.5 billion times. In fact, each day, the average heart beats 100,000 times, pumping about 2,000 gallons (7,571 liters) of blood. The size of baby’s heart is much smaller. If you imagine as mentioned before that the size of the heart is a little larger than ones fist then imagine the fist of a small baby.

 

Your heart (see diagram above) is located between your lungs in the middle of your chest, behind and slightly to the left of your breastbone (sternum). A double-layered membrane called the pericardium surrounds your heart like a sac. The outer layer of the pericardium surrounds the roots of your heart's major blood vessels and is attached by ligaments to your spinal column, diaphragm, and other parts of your body. The inner layer of the pericardium is attached to the heart muscle. A coating of fluid separates the two layers of membrane, letting the heart move as it beats, yet still be attached to your body.

Your heart has 4 chambers. The upper chambers are called the left and right atria, and the lower chambers are called the left and right ventricles. A wall of muscle called the septum separates the left and right atria and the left and right ventricles. The left ventricle is the largest and strongest chamber in your heart. The left ventricle's chamber walls are only about a half-inch thick, but they have enough force to push blood through the aortic valve and into your body. In view of its responsibility to pump blood into the whole body it is stronger than the right ventricle. All the four chambers of the heart and their out flow tracts are separated by stoppers known as valves which have the function of allowing blood to flow in one direction but not in the reverse. If these allow the blood to flow in the reverse direction then they are said to be defective. The discussion of these so called valves is given below.

The Heart Valves

DIAGRAM OF HEART SHOWING THE VALVES IN CARDIAC CYACLE

Four valves regulate flow through your heart:

· The tricuspid valve regulates blood flow between the right atrium and right ventricle. It has three leaflets (cusps) and hence is called tricuspid. During diastole, when the heart relaxes, this valve opens up and allows impure blood to flow into the right ventricle from the right atrium.

· The pulmonary valve controls blood flow from the right ventricle into the pulmonary arteries, which carry blood to your lungs to pick up oxygen.
 This valve also has three leaflets and hence is also a tricuspid valve. It opens during systole (heart contraction phase) and allows impure blood to flow from the right ventricle into the lung for purification.

· The mitral valve lets oxygen-rich blood from your lungs pass from the left atrium into the left ventricle. It is a bicuspid valve (having only two cusps) and allows the flow of pure blood from the lungs into the left ventricle during ventricular relaxation phase (diastole).

· The aortic valve opens the way for oxygen rich blood to pass from the left ventricle into the aorta, your body’s largest artery, where it is delivered to the rest of the body. It is also a tricuspid valve.

The Conduction System(see diagram below)

The daily activity of the heart is controlled by a series specialized muscle tissue that is collectively called the conduction tissue. It is this system that determines the regularity of the “lup” and “dup” sounds that you hear when you listen to your heart. The activity of this system is mentioned as follows. Electrical impulses from your heart muscle (the myocardium) cause your heart to beat (contract). This electrical signal begins in the sinoatrial (SA) node, located at the top of the right atrium (see diagram). The SA node is sometimes called the heart's "natural pacemaker." When an electrical impulse is released from this natural pacemaker, it causes the atria to contract. The signal then passes through the atrioventricular (AV) node. The AV node checks the signal and sends it through the muscle fibers of the ventricles, causing them to contract.

The SA node sends electrical impulses at a certain rate, but your heart rate may still change depending on physical demands, stress, or hormonal factors. All these factors influence you nervous system (autonomic nervous system) and in turn influences the SA node which sends impulses that determine whether the heart rate has to be fast. Imagine all this happens in a matter of seconds and you feel either your heart increasing or decreasing. The regulator of the heart is indeed remarkable. However in certain disease states the work of the regulator may be overcome or suppressed due to some unknown electrical signal. This dominance or suppression of the regular heart rhythm leads to a rhythm that may be chaotic/too fast. Such abnormal rhythms are called arrhythmias (abnormal rhythms). Detailed discussion on this topic will be done later.

The Cardiovascular System

Your heart and circulatory system make up your cardiovascular system. Your heart works as a pump that pushes blood to the organs, tissues, and cells of your body. Blood delivers oxygen and nutrients to every cell and removes the carbon dioxide and waste products made by those cells. Blood is carried from your heart to the rest of your body through a complex network of arteries, arterioles, and capillaries. Blood is returned to your heart through venules and veins. If all the vessels of this network in your body were laid end-to-end, they would extend for about 60,000 miles (more than 96,500 kilometers), which is far enough to circle the earth more than twice!

This one-way circulatory system carries blood to all parts of your body. This process of blood flow within your body is called Circulation. Arteries carry oxygen-rich blood away from your heart, and veins carry oxygen-poor blood back to your heart.

In pulmonary circulation, though, the roles are switched. It is the pulmonary artery that brings oxygen-poor blood into your lungs and the pulmonary vein that brings oxygen-rich blood back to your heart.

In the diagram, the vessels that carry oxygen-rich blood are colored red, and the vessels that carry oxygen-poor blood are colored blue. This is a standard medical code for veins and arteries.

Twenty major arteries make a path through your tissues, where they branch into smaller vessels called arterioles. Arterioles further branch into capillaries, the true deliverers of oxygen and nutrients to your cells. Most capillaries are thinner than a hair. In fact, many of these are so tiny, only one blood cell can move through them at a time. Once the capillaries deliver oxygen and nutrients and pick up carbon dioxide and other waste, they move the blood back through wider vessels called venules. Venules eventually join to form veins, which deliver the blood back to your heart to pick up oxygen.

CATEGORISATION OF CONGENITAL HEART DISEASE BASED ON AGE AT PRESENTATION

From the discussion above it is clear that congenital heart disease can present at any age group but the type of disease presenting in a particular age group is different. Some of these defects present in the 1st week of life (Early presentation), some between 7th day of life to 30 days. Some of these may present after 30 days of life. Any defect that presents within the first few days after birth is usually life threatening and needs early intervention to save the child’s life.

Congenital Heart Disease presenting in the first week of life(within 7 days)

These include:

1) Transposition of Great Arteries with Intact Ventricular Septum

2) Pulmonary atresia with Intact Ventricular septum

3) Critical Pulmonary stenosis

4) Critical Aortic stenosis

5) Tricuspid Atresia with Restrictive PFO

6) Tricuspid Atresia with Pulmonary atresia

7) Transposition of Great Arteries with VSD(Restrictive)

8) Interrupted Aortic Arch

9) Tetralogy of Fallot with Critical pulmonary stenosis

10) Pulmonary atresia with Ventricular Septal Defect

11) Critical Coarctation of Aorta

12) Hypoplastic Left Heart Syndrome(HLHS)

Congenital Heart Disease presenting in the 2nd to 4th week of life

1) Truncus Arteriosus Type 1

2) Coarctation of Aorta

3) Large Non restrictive Ventricular Septal defect

4) Transposition of Great arteries with VSD

5) Coarctation of Aorta with VSD

6) Large Patent Ductus Arteriosus

7) Double outlet right ventricle with No Pulmonary stenosis(Tausig Bing Anomaly)

8) Multiple VSD

9) Persistent Pulmonary Hypertension of the New born

10) Isolated Severe Pulmonary stenosis

11) Severe Aortic stenosis

 

Congenital Heart Disease Presenting between 1month and 6months age

1) Ventricular Septal Defect

2) Complete AV canal defect

3) Multiple VSD

4) Large PDA

5) Large ASD

6) Coarctation of Aorta

7) Truncus Arteriosus Type I

8) Anomalous Left Coronary Artery from Pulmonary Artery(ALCAPA)

9) Tetralogy of Fallot

10) Pulmonary Atresia with VSD with PDA(Restrictive)

11) Tricuspid Atresia with ASD with No Pulmonary stenosis

12) Tricuspid Atresia with restrictive ASD with Severe Pulmonary stenosis

13) Ebstein’s Anomaly of tricuspid Valve with Pulmonary stenosis

14) Isolated Pulmonary stenosis

Congenital Heart Defect that present between 6months to 2 yrs of age

1) Atrial Septal defect

2) Moderate sized Ventricular Septal Defect

3) Isolated Pulmonary stenosis

4) Tetralogy of Fallot

5) Partial AV canal defect with Mitral regurgitation

6) Eisenmenger Syndrome

Congenital Heart disease presenting beyond 2 yrs of age

Congenital heart defects usually do not present for the first time after 2 yrs of age. If a defect presents after 2 yrs of age then it is only because it was not detected earlier due to lack of medical attention.

ACQUIRED HEART PROBLEMS

Compared to congenital heart problems, acquired heart problems are not many in numbers but they are equally dangerous in severity if not treated in time. Acquired heart defects are usually found more in the older pediatric age groups. Based on the cause of the heart problems these are categorized into:

Causes of Acquired Heart disease according to Etiology.

A. Infectious causes

a) Neonatal age group (1st 30 days)

I) Bacterial cause

i) Sepsis syndrome

ii) Endocarditis (streptococcal, E.coli, Klebsiella, Staphylococcal)

iii) Myocarditis (Streptococcal, Staphylococcal)

II) Viral cause

i) TORCH infections

ii) Coxsackie A and B

iii) Adenovirus

iv) Cytomegalovirus

v) HIV

vi) Congenital Rubella syndrome

III) Fungal Infection

i) Fulminant Candidial infection

ii) Histoplasmosis

iii) Aspergillosis

b.) Infantile age group(1mo to 1yr)

I) Bacterial Cause

i) Staphylococcal infection

ii) Pneumococcal infection

iii) H. Influenzae infection

iv) Salmonella Typhi infection

v) Tuberculosis

vi) Sepsis syndrome

II) Viral Cause

i) Adenovirus

ii) Enterovirus

iii) Coxsackie A & B virus

iv) HIV

v) Cytomegalovirus

vi) Mumps virus

vii) Echovirus

III) Fungal Infection

i) Candida

ii) Histoplasmosis

iii) Aspergillosis

iv) Actinomycosis

IV) Protozoal infection

i) Malaria

ii) Toxoplasmosis

V) Spirochaetal Infection

i) Leptospirosis

c) Older children and Adults (1y to adults)

I) Bacterial cause

i) Diphtheria

ii) Pertusis

iii) Streptococcus

iv) Staphylococcus

v) Tuberculosis

vi) Typhoid

vii) Gonococcal

viii) Tetanus

ix) Brucellosis

II) Viral cause

i) Coxsackie A,B

ii) Echovirus

iii) Adenovirus

iv) Epstein Barr Virus

v) Influenza virus

vi) Human Immunodeficiency Virus

vii) Cytomegalovirus

viii) Rubella virus

ix) Measles

x) Mumps

xi) Parvovirus

III) Fungal Cause

NEONATES

Any child in the age group of 0 days to 30 days is labeled as a neonate in medical terminology. Neonates as such are fragile and give parents enough psychological stress even if they are normal. Parents waste no time in contacting a pediatrician in case they feel that the child is ill. If such a fragile and small baby is diagnosed with a cardiac problem then it further compounds the existing psychological stress of the parents. Parents often go into self denial that their child has such a problem. They may try to seek a second opinion without realizing that they are wasting precious time and any hope for their child will reduce with delay. It is hence imperative on the part of the primary doctor to refer the parents at the earliest to a center where facilities for detailed evaluation of cardiac problems in children is available and so also the presence of a pediatric cardiologist is present. In India there are a number of centers having facilities for pediatric cardiology and pediatric cardiac surgery. These will be mentioned later on in the discussion.

It is important for the primary pediatrician to be as transparent as possible to the parents as this is usually a life and death situation and any misinformation will only agitate the parents in case the results are opposite to what is told to them initially. In case the primary pediatrician does not know then he should refrain from making any statement and should refer the child and the parents as early as possible for pediatric cardiology check up and care. If the child is sick then the same should be informed to the parents and the need for early treatment stressed. Along with this the primary pediatrician should facilitate the transfer/referral of this child to a higher center for further care. Parents on their part have the right to ask the following questions:

a) What is wrong with my child?

b) What is the heart problem?

c) Is it curable?

d) What is the future of my child with this heart problem?

e) What is the risk to life?

f) Can I wait for the child to grow up?

g) What is the best possible treatment for my child?

h) What are the centers dealing with this problem?

i) What is the nearest center to my house?

j) Is there a good surgeon/cardiologist available?

k) What is the cost involved?

 

It is easy to answer all the questions up to( i) but the last question is a delicate one. In our country majority of children afflicted with cardiac problem come from economically weaker sections of the society. Further, we have only a few government centers that cater to pediatric cardiac surgery and their work load is already high. A list of common disorders presenting in the neonatal period is given above. So it is also imperative that the parents know about the cost of surgery and the future of their child in case they plan to get their child treated for such a problem.

CARDIAC PROBLEMS FOUND IN THE NEONATES

These are further categorized into two:

1) Cardiac problems associated with reduced flow into the body

2) Cardiac problems associated with reduced flow into the lungs(less or no oxygenation of blood).

3) Cardiac Problems associated with abonormality of heart rhythm

4) Cardiac problems due to presence of abnormal mass in the heart (Tumor).

A) Cardiac Problems in Neonates Associated with reduced flow into the body: These are defects that result in inadequate or absent blood flow into the lower half of the body. If not detected early then they could be lethal. Early sign of refusal of feeds, vomiting, cold peripheries, respiratory distress and tachycardia should be evaluated in detail in a previously well neonate.

a) Hypoplastic Left Heart Syndrome

b) Interruption of the Aortic Arch

c) Severe Coarctation of the Aorta

d) Severe Aortic Stenosis

e) Double aortic arch

f) Aortic Atresia

B) Cardiac Problems in Neonates Associated with reduced flow into the lungs: These are defects that are associated with reduced/absent blood flow into the lungs. As a result the entire body is exposed to hypo oxygenated/unoxygenated blood which if not treated in time will lead to multi organ dysfunction, neurological sequelae and death!!! Some of these defects are given below.

a) Hypoplastic right heart syndrome

b) Transposition of great arteries with intact ventricular septum and Restricted PDA

c) Pulmonary atresia with restricted PDA

d) Critical pulmonary stenosis

e) Pulmonary atresia with VSD with restricted PDA

f) Tricuspid Atresia with ASD with VSD with Pulmonary atresia

g) Tetralogy of Fallot with Severe Pulmonary stenosis