Congenital Heart Disease: The silent killer

Lack of access to medical treatment, soaring cost of surgery and subsequent medication are the primary reasons of increased mortality among children with congenital heart diseases, writes Dr Gopichand Mannam.

Every year more than two lakh neonates are diagnosed with congenital heart diseases (CHD) in India. That means eight out of every 1000 newborn babies suffer from CHD. But sadly, on an average only 6000 to 8000 of the two lakh affected children receive treatment. Nearly 80 percent of the infants die due to their parents’ inability to afford treatment. The rest continue to live with the disease depending upon its condition. Shocking, isn’t it?

On an average, a patient spends Rs 75,000 to Rs 2,00,000 for the operation, depending on the severity of the complication. In India, where those below or near the poverty line often don’t earn this enormous amount even in a couple of years, this is definitely out of the reach for the poor. Tragically, it is they who can ill afford treatment who are most afflicted by CHD. Malnutrition in pregnant mothers and diseases like rubella infection during pregnancy are known causes of children being born with heart diseases.

What is CHD?

Congenital heart disease is a term used to describe heart defects that develop before birth. These defects result from a failure of the heart or the blood vessels near the heart to develop normally. Even as the child grows inside his or her mother, changes occur which cause an abnormal development of the heart. Depending on the time at which this change happens, different kinds of abnormalities may be seen. Each type of defect has a different severity level on the child’s health. Some of these may be so severe that the child may not be able to live, or needs early and major operations to help it survive.

On the other hand, in some cases an affected child may continue living a normal life with the defect getting detected accidentally much later. Take the case of a young girl in her early twenties from a poor family in Andhra Pradesh, whom I treated recently. The defect in her heart was detected rather late in her life and her family could not afford treatment. The poor parents would have lost their grown up daughter, but for the timely help from the state government and intervention of Care hospital. However, by the time she was brought to the hospital she had turned completely blue due to bad blood being mixed with the good blood in her heart, a defect that had been growing since her birth. An emergency operation of the heart did save the girl’s life, but had this been done long before, she would have been a much healthier person now.

Causes of CHD

In spite of identifying so many cases of CHD, the exact cause of the defects cannot be ascertained. Research has shown that heredity factors, certain drug/medicine intake during pregnancy, an infection to mother, especially from Fetal Rubella virus, or usage of retinoic acid (for acne) during pregnancy increases the incidence of heart defects in the feotus. One more possibility that has been identified is genetic aberration. However, in most cases, the cause of the heart defect is not known. Though one cannot term it as disease prevalent among the poorer sections, the unhygienic living conditions and lack of proper nutrition for the mother during pregnancy are also responsible for causing heart defects in children.

As the disease affects the new born babies, children particularly during the initial few years after birth are extremely vulnerable. The magnitude of the problem caused by CHD can be gauged by the fact that out of the newly born in a year, 65 per cent die before one year of age and only 15 per cent survive beyond five years.

Identifying defective hearts

Depending upon the visible symptoms, CHDs are often divided into two types:

• Those with cyanosis i.e. blue babies and
• Those without cyanosis

In the first type, blood pumped to the body contains less oxygen than normal resulting in a condition called cyanosis, a bluish discoloration of the skin.  Infants with cyanosis are often called “blue babies.” 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 cyanotic heart defects may need an operation to give temporary relief by increasing blood flow to the lungs with a shunt. This reduces the cyanosis and allows children to grow and develop until the problem can be fixed when they are older. In some of these defects impure blood is mixed with the pure blood supplied to the body organs.

In the second type, the defect allows blood to flow between the heart’s left and right 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.” A majority of the CHD defects fall under this category. Some such defects cause blood to take an abnormal route through the heart while some obstruct blood flow within the heart or the great vessels near it. ‘Hypoplastic’ is another defect affecting children where part of the heart may be selectively under-developed. Depending on the child’s development, these defects may occur as single abnormalities or in a various combinations. The CHD defects that sometimes occur are association of other diseases.

Anticipating inherent CHD risk

Ideally, some preventive measures before birth could to a certain extent help avoid the risk of a child getting CHD. Firstly, women should inform their physicians about the pregnancy before medication is prescribed to them. Secondly, the woman’s immunity status of rubella should be evaluated early in the pregnancy. If the mother is not immune, she must avoid any possible exposure to rubella and should be immunised immediately following delivery. Further, if a defect is found during the pregnancy stage, it is of the utmost importance that expectant mothers receive good prenatal care. Many of these defects can be discovered on routine ultrasound examinations performed by an obstetrician. Anticipating the delivery date and the consultation with medical personnel such as pediatric cardiologists, cardiothoracic surgeons, and neonatalogist allows them to intervene as the need arises. This can make the difference between life and death for many babies.

Treatment

Once a CHD is detected in a new born, treatment could vary depending upon the severity of the defect. While some heal over time, some require medication and others require one or multiple surgical interventions. However, not all people have access to timely treatment primarily because of two reasons. Firstly, due to lack of awareness among the public about the disease and the treatment options available. Secondly, due to the high cost of surgeries, medication and hospital stay which sometimes is extended because of complications. Thirdly, not all hospitals are equipped with the technology, which is expensive. Last but not the least, delayed referrals due to late detection of CHD in children. It is very important that referral doctors are aware of the natural course of heart defects in their patients and the appropriate timing of surgical intervention. Delayed referral may result in inoperability or increased post- operative mortality.

Addressing the issue

In all fairness to the advancements in medical science, there has been an improvement in the risk of death from congenital heart disease surgery, from approximately 30 per cent in the 1960s and 1970s to approximately five per cent today. But still, a lot of people in India are deprived of basic primary and secondary healthcare, and we are not even talking about tertiary medical care. It is time that respective state governments and private sector actively and seriously participate in funding and investing in healthcare especially tertiary medical care aimed at the poor.

Government funding can be directed towards providing tertiary care for the CHD affected poor children through existing hospitals providing the required medical services.

One of the top priority tasks is definitely awareness generation about congenital heart diseases among general public and medical practitioners through seminars and continuous medical education programmes. Society on its part should join hands in this collective and collaborative effort by generating funds that could be used to provide treatment to the CHD affected underprivileged children.

The writer is chief cardiothoracic surgeon at Care Hospital, Hyderabad.