New lease of life for congenital heart disease patients

By Rashika Fazali

In Sri Lanka, around 2,500 to 3,000 babies are born with Congenital Heart Disease (CHD) yearly out of 340,000 to 400,000 births stated Children’s Heart project of Sri Lanka Chairman and Lady Ridgeway for Children Consultant Paediatric Dr. Duminda Samarasinghe. He mentioned that 40% of these babies suffer from Down’s syndrome.

However, looking at the worldwide figures, he mentioned that six to eight babies are born with CHD yearly in every 1,000 live births.

He stated that the figures don’t change over time, but other features in regard to this disease have improved. “The diagnosis is becoming better because you have more facilities, more doctors are available, there are early detections and patients and people are more aware so they bring their children to the doctor early.”

Nevertheless, Samarasinghe mentioned that today there is treatment and surgery for CHD in Sri Lanka at the Lady Ridgeway Hospital for Children (LRH) with a success rate of almost 100% - in fact more than 95%. He added: “Compared to the past we are at a very strong stage because now we can treat almost all even the most complex ones.”

Children’s Heart Project of Sri Lanka

Back in the 1990s, CHD was not treated in Sri Lanka due to the minimal facilities and technologies for interventions and surgery. The Children’s Heart Project of Sri Lanka (CHP) was established in 1993 by Dr. P.N. Thenabadu for this crisis - to support and provide surgery or interventions for children in whichever way and to improve the services in the country.

“The project is dedicated to children and adults who have CHD. But not for coronary diseases and diseases that occur later in adult life such as heart attacks,” stated Samarasinghe.

He also stated that the CHP has helped many patients by sending them abroad and providing them with overseas surgeons for operations. This project has also conducted training programs, cardio programs, and has provided money to purchase various items like surgical items, facilities and medical equipment.

“The CHP has provided LRC with an echocardiography machine which is around Rs. 5 to 6 million – US$ 50 000, “stated Samarasinghe. He added that there was a time when the Intensive Care Unit (ICU) and the High Dependency Unit (HDUs) at LRC could not be opened as certain amenities were not repaired. To help this case, CHP gave between Rs. 1.5 to 2 million.

He further revealed that surgeries at LRC were once on the brink of discontinuing due to the lack of surgical items. “We donated 50 of them which came up to around Rs. 1 million.”

He asserted that CHP plays an indirect role where they collect funds from donors. He noted that in the recent past there had been different people who collected money wrongfully saying that their children have heart disease. “We want to divert those donations to the best benefits of these patients – to the right patients and in the most cost effective manner,” stated Samarasinghe.

Future programs

He revealed that CHP is planning to have awareness programs, a fund raising musical show next year on 9 and 10 February at Bishop’s College Auditorium which will feature The Revelations, and to update their website with information about CHD and treatments and facilities available in Sri Lanka.

Samarasinghe asserted that CHP wants to start a program called the extracorporeal membrane oxygenation which is not offered in Sri Lanka. Extracorporeal membrane oxygenation is a medical procedure performed out of the body which takes over the heart and the lungs function for a couple of days so that they can rest. This takes place only if the heart and the lungs are severely deceased. However, this program is quite costly stated Samarasinghe.

“Our second major plan to have is a tissue bank where we use tissues of the deceased people – only if they are healthy and are after accidents like in the eye donation campaign,” said Samarasinghe, adding, “If you are going to replace a value or a blood vessel, the human tissue is the best for replacements. All the commercial available ones are inferior to them.”

Developed countries and developing countries like India have tissue banks as well, but having one in Sri Lanka and running them is quite pricey.

Their third plan is to provide accommodation – especially made houses for the parents of the patient who most often than not, journey from far places. Samarasinghe stated that in other countries this housing facility is available for parents: “McDonald’s is doing a chain of homes in other developed countries. We want to start something like that.”

What is CHD?

Congenital heart diseases are defects in the heart and the great vessels present from birth. These defects could be either an obstruction of blood flow or abnormal blood flow patterns due to leakage or ‘holes in the heart’.

Samarasinghe mentioned that you can’t find an exact reason to what triggers these defects. “It can be due to maternal drugs – like lithium and steroids can cause malformation in the foetus. It can happen due to maternal infections like Rubella. Drug abuse can also cause foetal malformations,” explained Samarasinghe. On the other hand, CHD could also come from genetics.

Types of CHD

Under simple shunt lesions, he explained that blood crosses from the left side to the right side through a hole in the heart or due to patent ductus arteriosus (PDA); a communication between the two great vessel – the aorta and pulmonary vessel.

He further explained that Atrial Septal Defect (ASD) which is a hole in the upper chambers of the heart is not generally bad adding: “We wait till four years of age to treat them.” In the case of a Ventricular Septal Defect (VSD) – a hole between the lower chambers of the heart, Samarasinghe stated that early interventions need to be done just like in PDA.

The second type of CHD is called obstructive lesions which blocks the blood from flowing. Cyanotic heart diseases - the complex lesions are the third type of CHD. Samarasinghe noted that most patients will have a shunt as well as an obstruction. He added: “This combination becomes more complex. There is always a crossing of blood from right side to the left side causing discolouration.”

Symptoms of CHD

Samarasinghe stated that there are few features which can pronounce if a baby has CHD. The main symptom is having a murmur in the heart. “Most references we get are due to murmurs,” revealed Samarasinghe.

He further stated that another symptom is the bluish discolouration in children.

This bluish discolouration can be seen in the fingers, fingernails, palms, lips and tongue. He noted that these happen when the value is narrow and contains a hole.

Difficulty in breathing is another symptom. He noted that babies would feel breathless most of the time because their heart is beating faster and their lungs are working harder to oxygenate more blood. He also explained that patients who have shunt lesions – a simple hole in the heart will have recurrent chest infections like pneumonia. Sometimes they may also get infections in the heart called endocarditis where they have prolonged fever, stated Samarasinghe.

Treatment

There are three types of patients – the ones who do not need any intervention, the treatable and the untreatable.  “For the ones who do not need interventions, we don’t do anything,” stated Samarasinghe.

Under the patients who are treatable, there are three kinds of treatment: medical drugs, interventions and surgery. For the patients who go for definite treatment, they are treated with drugs.

However, he noted that there are no heart diseases that can be treated by drugs, adding: “We can control some symptoms with certain drugs until surgery or until the hole(s) closes on its own. Certain holes can be closed depending on the location of the hole and the structure of it. If we decide that it might have a higher chance of closing on its own, then we tend to wait.”

Speaking about interventions, he noted that this is procedure that does not need to have the chest opened. The cathedrals, balloons, sheets and whatever that is needed surgically is passed from the femoral vein – the blood vessel which takes blue blood to the body. A device closure is used to close certain holes in the heart stated Samarasinghe.

As far as the untreatable is considered, he revealed that patients are left alone. “We support them medically with whatever drugs that will help.”

However, he mentioned that complex CHD’s patients are of less than 1%. Nevertheless, he firmly stated that without treatment, most complex CHD’s die within the first year of life.