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Cyanotic Heart Disease research can help restructure health care, do better planning

11 July 2022 | Story Andre Damons | Photo Supplied

Prof Stephan Brown is a Principal Specialist and Head of the Division of Paediatric Cardiology in the Department of Paediatrics and Child Health in the Faculty of Health Sciences at the University of the Free State (UFS).

Paediatric heart specialists at the Universitas Academic Hospital and the University of the Free State (UFS) hope their research into the deadly Cyanotic Heart Disease amongst newborns will assist health authorities in central South Africa to restructure healthcare services and do better health-planning to save more lives.

Prof Stephen Brown, Principal Specialist and Head of the Division of Paediatric Cardiology in the Department of Paediatrics and Child Health in the Faculty of Health Sciences at the UFS, says children from poor and rural areas in central South Africa are dying of Cyanotic Heart Disease. One of the main contributors to these deaths is the distance patients have to travel to regional hospitals.

The research was done under the auspices of the Robert W M Frater Cardiovascular Research Centre in the department of cardiothoracic surgery in the UFS School of Medicine. The results are still in the preliminary stage as the final data is still being analysed. The Robert W M Frater Cardiovascular Research Centre (the Frater Centre) was established in 2015 under the leadership of Prof. Francis E Smit. This was made possible through donor funding, especially by Dr Robert W M Frater MD PhD (honoris causa, UFS), a South Africa-born New York-based cardiothoracic surgeon, researcher and innovator as infrastructure and project support by the UFS.

The vision of the Frater Centre is to be a leading cardiovascular research institution in South Africa and sub-Saharan Africa. It provides an interdisciplinary training and research platform for scientists and clinicians from different backgrounds to develop as researchers and collaborators in cardiovascular and thoracic surgery and related domains. Activities are focused on the development of African solutions for African problems.

According to Prof Brown, who is also a paediatric cardiologist at the Universitas Hospital, children with this disease present with a blueish colour because the oxygenated and desaturated blood mixes, leading to the blue discoloration. Prof Brown and his master’s degree researcher (Marius van Jaarsveld) focused on single ventricle physiologies; children who effectively have a single pumping chamber which means one of the chambers is underdeveloped or not developed at all. A normal person has two pumping chambers.

“With this study we looked over 20 years of cases. Over this period we saw 154 children. It is a retrospective study because we are fortunate to have a very extensive database dating back to 1987. One thing of concern is that we should have seen a lot more children if you look at the worldwide statistics,” says Prof Brown.

Treatment

According to him, 40 of these children never received any form of therapy for the simple reason that a lot of them presented too late while others had severe birth asphyxia when they got to the hospital.

Treatment for Cyanotic Heart Disease usually involves up to three operations before the children become pink again. “The first operation is called palliation to ensure we control the lung blood. That is usually in the first to two to six weeks after birth. The second operation is done between six months to a year of age when we do to what we call a bidirectional Glen – second-stage palliation. Also to improve general condition and take some of the volume off the heart. The last operation, called the Fontan operation, happens between six to seven years of age and that’s when they become pink,” explains Prof Brown.

Prof Brown says the results from the study compare favorably with the rest of South Africa and Africa but do not compare that well to high-income countries because they have more resources available.

They have seen children from Northern Cape, North West, some parts of the Eastern Cape and Lesotho. According to Prof Brown, once they looked closer, they discovered that the closer the patients are to the hospital, the sooner they present to hospital. The further away they are, the longer it takes them to present at a hospital with congenital cardiac facilities.

“In Mangaung we saw the kids when they were around about four days old. At Thabo Mofutsanyana district in Qwaqwa we saw them three to four days after birth. So they presented early. Lejweleputswa and Xhariep districts we saw the patients after they were one month old. In densely populated areas it is picked up early, as they are closer to the referral hospitals. The further, away from a hospital, the longer it takes to get to us. In Lesotho it takes up to six months [for them to get to us] and the Northern Cape up to two months of age,” explains Prof Brown.

This is most likely an indication that distance from the hospitals plays a major role in deaths.

How will the study help?

Though a part of the study is for epidemiological information, Prof Brown hopes that the health authorities will take stock of the findings. “These studies are important to make health authorities aware of the challenges and to assist in health planning. What can we do better for the people? We are doing clinical research. This is important because we are a mid- to low-income country with limited resources and it is important for the population we are dealing with.”

“Our prime aim is if one knows what is going on in your population you can restructure your health care accordingly. That is our ultimate aim. Get it published and talk to the authorities. Now we can scientifically prove instead of relying on perception.”

The solution

Prof Brown says this disease can potentially be prevented by doing foetal heart sonar scans. If there is a huge screening project, a large number of deaths can potentially be prevented. Maternal screening is very important. Early referrals are also a step in the right direction. “Our parents, caregivers, and nurses need to be educated. Another solution is to do a simple saturation screening monitor prior to discharge after birth. I have been advocating for this for years and hopefully, before I retire, it will become routine procedure. Obviously there will be a lot of false positives, but we can help our people by earlier recognition of cyanosis.”

• Prof Brown, who is passionate about the health of children, says a life-saving collaboration initiative between the UFS, the Mother and Child Academic Hospital (MACAH) Foundation, and the Discovery Fund started five years ago to help curb the death of young patients due to congenital heart disease, and to make services more accessible to rural communities. With this outreach initiative, Prof Brown travels to rural areas in the Free State to diagnose heart defects in babies early.

News Archive

Inaugural lecture: Prof Robert Bragg, Dept. of Microbial, Biochemical and Food Biotechnology

2006-05-17

Attending the inaugural lecture were in front from the left Prof Robert Bragg (lecturer at the Department of Microbial, Biochemical and Food Biotechnology) and Frederick Fourie (Rector and Vice-Chancellor). At the back from the left were Prof James du Preez (Departmental Chairperson: Department of Microbial, Biochemical and Food Biotechnology) and Prof Herman van Schalkwyk (Dean: Faculty of Natural and Agricultural Sciences). Photo: Stephen Collett

A summary of an inaugural lecture delivered by Prof Robert Bragg at the University of the Free State:

CONTROL OF INFECTIOUS AVIAN DISEASES – LESSONS FOR MAN?

Prof Robert R Bragg
Department of Microbial, Biochemical and Food Biotechnology
University of the Free State

“Many of the lessons learnt in disease control in poultry will have application on human medicine,” said Prof Robert Bragg, lecturer at the University of the Free State’s (UFS) Department of Microbial, Biochemical and Food Biotechnology during his inaugural lecture.

Prof Bragg said the development of vaccines remains the main stay of disease control in humans as well as in avian species. Disease control can not rely on vaccination alone and other disease-control options must be examined.

“With the increasing problems of antibiotic resistance, the use of disinfection and bio security are becoming more important,” he said.

“Avian influenza (AI) is an example of a disease which can spread from birds to humans. Hopefully this virus will not develop human to human transmission,” said Prof Bragg.

According to Prof Bragg, South Africa is not on the migration route of water birds, which are the main transmitters of AI. “This makes South Africa one of the countries less likely to get the disease,” he said.

If the AI virus does develop human to human transmission, it could make the 1918 flu pandemic pale into insignificance. During the 1918 flu pandemic, the virus had a mortality rate of only 3%, yet more than 50 million people died.

Although the AI virus has not developed human-to-human transmission, all human cases have been related to direct contact with infected birds. The mortality rate in humans who have contracted this virus is 67%.

“Apart from the obvious fears for the human population, this virus is a very serious poultry pathogen and can cause 100% mortality in poultry populations. Poultry meat and egg production is the staple protein source in most countries around the world. The virus is currently devastating the poultry industry world-wide,” said Prof Bragg.

Prof Bragg’s research activities on avian diseases started off with the investigation of diseases in poultry. “The average life cycle of a broiler chicken is 42 days. After this short time, they are slaughtered. As a result of the short generation time in poultry, one can observe changes in microbial populations as a result of the use of vaccines, antibiotics and disinfectants,” said Prof Bragg.

“Much of my research effort has been directed towards the control of infectious coryza in layers, which is caused by the bacterium Avibacterium paragallinarum. This disease is a type of sinusitis in the layer chickens and can cause a drop in egg product of up to 40%,” said Prof Bragg.

The vaccines used around the world in an attempt to control this disease are all inactivated vaccines. One of the most important points is the selection of the correct strains of the bacterium to use in the vaccine.

Prof Bragg established that in South Africa, there are four different serovars of the bacterium and one of these, the serovar C-3 strain, was believed to be unique to Southern Africa. He also recently discovered this serovar for the first time in Israel, thus indicating that this serovar might have a wider distribution than originally believed.

Vaccines used in this country did not contain this serovar. Prof Bragg established that the long term use of vaccines not containing the local South African strain resulted in a shift in the population distribution of the pathogen.

Prof Bragg’s research activities also include disease control in parrots and pigeons. “One of the main research projects in my group is on the disease in parrots caused by the circovirus Beak and Feather Disease virus. This virus causes serious problems in the parrot breeding industry in this country. This virus is also threatening the highly endangered and endemic Cape Parrot,” said Prof Bragg.

Prof Bragg’s research group is currently working on the development of a DNA vaccine which will assist in the control of the disease, not only in the parrot breeding industry, but also to help the highly endangered Cape Parrot in its battle for survival.

“Not all of our research efforts are directed towards infectious coryza or the Beak and Feather Disease virus. One of my Masters students is currently investigating the cell receptors involved in the binding of Newcastle Disease virus to cancerous cells and normal cells of humans. This work will also eventually lead to a possible treatment of cancer in humans and will assist with the development of a recombinant vaccine for Newcastle disease virus,” said Prof Bragg.

We are also currently investigating an “unknown” virus which causes disease problems in poultry in the Western Cape,” said Prof Bragg.

“Although disinfection has been extensively used in the poultry industry, it has only been done at the pre-placement stage. In other words, disinfectants are used before the birds are placed into the house. Once the birds are placed, all use of disinfectants stops,” said Prof Bragg.

“Disinfection and bio security can be seen as the ‘Cinderella’ of disease control in poultry. This is also true for human medicine. One just has to look at the high numbers of people who die from hospital-acquired infections to realise that disinfection is not a concept which is really clear in human health care,” said Prof Bragg.

Much research has been done in the control of diseases through vaccination and through the use of antibiotics. “These pillars of disease control are, however, starting to crumble and more effort is needed on disinfection and bio security,” said Prof Bragg.

Prof Bragg has been working in close co-operation with a chemical manufacturing company in Stellenbosch to develop a unique disinfectant which his highly effective yet not toxic to the birds.

As a result of this unique product, he has developed the continual disinfection program for use in poultry. In this program the disinfectant is used throughout the production cycle of the birds. It is also used to ensure that there is excellent pre-placement disinfection.

“The program is extensively used for the control of infectious diseases in the parrot-breeding industry in South Africa and the product has been registered in 15 countries around the world with registration in the USA in the final process,” said Prof Bragg.

“Although the problem of plasmid mediated resistance to disinfectants is starting to rear its ugly head, this has allowed for the opening of a new research field which my group will hopefully exploit in the near future,” he said.

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