wcpccs2013 day 1

Scientists at the Sixth World Congress of Paediatric Cardiology and Cardiac Surgery being held this week in

Cape Town spoke of promising new developments in their field. “It is the best of times – we are achieving amazing new insights in cardiac development,” said Dudley Woodrow Benson, cardiology department researcher at Cincinnati Children’s Hospital Medical Centre.

The congress, which is hosting over 2 400 delegates from around the world, is being held in Africa for the first time. Chairman of the organising committee, Christopher Hugo-Hamman, said that they were hoping the congress would raise awareness of the plight of children born with congenital heart disease (CHD) in developing countries who have limited access to medical treatment. He said that 80 out of 1 000, or almost 1%, of live births have CHD.

At a talk entitled “Emerging concepts in cardiac embryology in the 21st century”, Benson said that the post-genomic era was going well and that the development of induced pluripotent stem cells (iPSC) provided a new tool for the scientist and clinician. “The 1 000 Genomes Project has revolutionised our thinking of the human genome,” he said. “It shows the great variation of the human genome.”

But Benson said that challenges remain. “To date, new genetic taxonomy does not precisely align with clinical taxonomy used by anatomists, cardiologists and surgeons,” he commented. “Our capability to discover genetic variants has outpaced our ability to analyse them.

Benson said that there are overlapping elements when it comes to identifying the causes of and treating CHD, including human genetics, cardiac development and the new science of pluripotent stem cells. “The early teaching was that

genetic causes were rare,” he said. “But subsequent studies have supported genetic origin above other reasons, such as environmental factors. There is a close association of CHD and other defects with chromosomal abnormalities.”

Despite the progress being made in this field, Benson said that most CHD causes remain unknown. Another speaker, Robert Anderson, a retired professor of paediatric cardiac morphology, said that until the 1990s, embryology was potentially a hindrance rather than a help, since concepts tended to originate from the armchair, rather than the workbench.

“Since then, the advances in genetic and molecular research have been amazing,” Anderson explained, but he cautioned that the challenge of embracing new ideas regarding embryology was to escape the old ones.

New research promises brave New world for paediatric cardiology

Loyiso Bala entertains delegates at the opening ceremony

NEWSLETTER - DAY 01MONDAY 18th FEBRUARY

By KATHERINE GRAHAM

Dudley Woodrow Benson

Every person born with congenital heart disease deserves to live for

a lifetime, not just a childhood – so it is crucial for the medical community to plan for the future of all congenital heart patients.

This was the impassioned message put forward by c, president and CEO of the Adult Congenital Heart Association (ACHA).

Verstappen (51), who herself has a complex congenital heart disease, used the Plenary on A Lifetime with Congenital Heart Disease, to call on paediatric cardiologists and specialists in adult congenital heart disease to work together to take account of and plan for the futures of people with congenital heart disease.

Born in 1961, Verstappen was one of the earliest group of patients who were treated for congenital heart disease.

“I am very lucky to have lived as long as I have,” she said. “What we have found out

is that children who have had operations for heart defects

usually do very well as children, but as people

age, their chances of developing new, life threatening problems, increase dramatically.”

Verstappen started her organisation in 1998, the same year that she was fi tted with a pacemaker. She and her team aim to assist the millions of congenital heart patients in the United States.

In an upbeat address, she described how she has managed to live a life which includes swimming, yoga, a two mile walk every day and a full time job with lots of travel. She said she fi rmly intends to dance at not only her daughter’s wedding, but also at the wedding of her grand-daughter.

Verstappen stressed that congenital heart disease is a lifelong chronic disease.

“It is not a childhood condition and there is no cure for congenital heart disease.

“The goal for every person born with congenital heart disease should be to live to age 80 and not to age 18. We all need to work together to achieve that future for everybody so each of us can thrive throughout the lifespan.”

According to Verstappen, when paediatric cardiologists fi rst started treating children with congenital heart disease, this was seen as a

simple problem.“If the child was blue,

you did an operation and they turned pink - and they fi gured the

problem was solved. It was all seen as very straight-forward.

“But as these children aged it turned out that they are at very high risk for developing new problems and

this is for two reasons: Firstly they still don’t have a normal heart and as their hearts age, they have all sorts of risks that are inborn in that anatomy.

“Secondly, the surgeries themselves cause problems. These are new technologies and we had no idea how people were going to age with them.”

Verstappen said in countries which started early doing congenital heart care, like the US, Canada and Europe, no effort was made to track patients, “because we thought the surgery was curative”.

There was also no planning ahead to take care of them.

“We are left in a situation where we have large numbers of aging congenital heart patients and no system to take care of them.

“So the good news now, at least in the developed world, is that most children born with complex heart problems will live to age 18. The bad news is that once they get over 18, many of them are not living a normal lifespan and many are developing new life-threatening problems.

“So, we actually haven’t solved the problem of congenital heart disease. We’ve really just delayed the problem.”

For this reason, the medical community, must collaborate on working together to plan for the futures of their patients, Verstappen said.

The countries that did not have well-developed paediatric cardiology systems had a “tremendous opportunity”, because they could learn from the lessons which the more developed countries had learnt “the hard way”, she said.

ageiNg briNgs problems to chd patieNtsBy SUE SEGAR

Children who have operations do well, but develop problems in adulthood

Amy Verstappen

Our challenge is to become better diagnosticians, according to Robert Campbell MD, Professor of

Paediatrics at the Emory University School of Medicine during the plenary on The Epidemic of Sudden Cardiac Athletic Death.

Campbell, who is also chief of Children’s Healthcare of Atlanta Sibley Heart Centre, said an awareness of the risk factors associated with sudden cardiac arrest was crucial in preventing it. This awareness was needed among doctors and families too.

Contrary to popular belief, he did not believe there was an “epidemic” of Sudden Cardiac Arrest. However, there was heightened awareness of its incidence because of a changed world in which there was instant communication.

”What we really need to do is find the patients who are affected by certain disorders before they have the arrest - and to move them out of harms way,” Campbell said.

A key way of doing this was through the use of a cardio-vascular risk assessment form aimed at gathering information on patients’ and families’ possible risk factors for cardiac arrest.

The Paediatric Sudden Cardiac Death Risk Assessment Form should be completed by parents periodically during “well” visits to the doctor, such as neonatal and preschool visits, as well as during middle school and before or during high school.

The form includes questions like: “Has your child fainted or passed out during exercise, emotion or startle?”; “Has your child fainted or passed out after exercise” and “Has your child ever had extreme shortness of breath during exercise?”

Key Sudden Cardiac Arrest warning signs and symptoms included:

• Exercise, emotion or startle-induced seizure or loss of consciousness;

• Exercise chest pain or shortness of breath; • Sudden, unexpected, unexplained death of

any family member before the age of 50 years; and

• A family history of known genetic heart muscle disorder or heart electrical disorder.

“You see what you know,” Campbell said – in a bid to stress the need for medical practitioners and parents and other interested parties to arm themselves with knowledge of the disorder.

“If we know what those warning signs and symptoms are, we can get them to an appropriate cardiac specialist so we can rule that in or out.

“It is important to make sure everybody recognises those warning signs and can act on them.”

Campbell added that anybody who has children needs to know what an abnormal family history looks like and what warning signs they should be aware of - and bring it up with their physician.

Furthermore, he said, people should become skilled at the life-saving techniques of cardiopulmonary resuscitation (CPR).

“Specifically, do they recognize a cardiac arrest when it occurs and do they activate the emergency medical system, can they and will they begin effective chest compressions and do they use appropriately an automatic external defibrillator.

“It is a life skill you need to know. Anybody can be the first respondent to a cardiac arrest. If you don’t know what to do, you won’t do anything.

“How many of us know what cardiac arrest looks like? How many of us know how to respond effectively? You have about five minutes before it’s a lost cause. You don’t have time to figure it out.”

Campbell concluded: “The devil is in the detail. We have to go out and inform ourselves … ask the questions about the genetic disorders that run through families. We have to do a better job. The objective is to find them before they have the arrest. It’s not easy but we can make it better.”

call for ‘better diagNosticiaNs’

By SUE SEGAR

Robert Campbell

Assessing risk is vital

As a young medical student in Cape Town in the 1970s, Derek Yach

was one of a group of students who were invited to visit the offices of cigarette manufacturers Rembrandt - and soon afterwards became involved in a campaign aimed at the company.

What he saw and heard during this visit had a profound effect and Yach, a former Executive Director at the World Health Organisation who is now works for The Vitality Group, became a leading international campaigner against smoking.

For him not smoking is a simple, life saving issue: he says quitting makes absolute sense – and it’s never too late to stop.

“If you quit smoking by before the age of 40, you can extend your life by 90 percent,” he says simply.

Tobacco smoke, including secondary smoke, is one of the highest causes of death in the world today.

And, while in the past more men than women smoked, the ratio of girls to boys and women to men who smoke is growing and it is estimated that the number of girls and women who will die from smoking will rise to between three

to four million in the next 25 years. He advocates a number of

solutions to arrest the growth of smoking including higher taxation and better border controls , “changing the media discourse “ and ensuring people had access smoke-free zones, both at home and in public.

Yach also took aim at the medical profession and the number of physicians who smoked and, as a result, were “not effective as advocates for tobacco control. Cardiologists need to change their private practice and become activists,” he says.

But quitting smoking was not easy and even though the number of smokers had dropped by between 15 and 18 percent, there were still 1.3 billion smokers worldwide, says Yach who argued, for the use of nicotine replacements like electronic cigarettes.

“I support it, I’d rather see nicotine replacement than people smoking.”

spotlight oN doctors who smoKeBy RAY JOSEPH

A new vaccine development initiative brings hope for

the estimated 34 million people worldwide who suffer from rheumatic heart disease (RHD).

The announcement of the NZ $3m investment by the New Zealand and Australian governments was made during the 6th World Congress of Paediatric Cardiology and Cardiac Surgery, currently on in Cape Town.

Rheumatic heart disease kills an estimated 320 000 children and young adults each year. It is a disease which is caused by the streptococcus virus and begins with a seemingly innocuous sore throat. A simple penicillin treatment can prevent damage to the heart, and if damage has already occurred, a series of monthly injections will prevent further damage.

“The message is that sore throats matter, and treatment is possible even in a low

resourced system,” said Diana Lennon, from the Department of Clinical Paediatrics at Auckland University.

With such an easy treatment, the need for a vaccine may seem less acute, but according to Lennon and Jonathon Carapetis, Director of the Telethon Institute for Child Health Research in Australia, vaccines are the best way to reduce disparities in health care.

“We have been trying to develop a vaccine for RHD for the past 100 years but haven’t got very far because it is a disease of the poor, so market forces haven’t stepped in.”

The commitment from the two governments means that if all goes well we could see a new vaccine in as few as five years,” Lennon said. “There are some very good candidate vaccines, which will be developed now that the funding is available.”

JoiNt iNitiative briNgs hope of rhd vacciNeBy LYNNE SMIT

3D image of rheumatic heart disease

Heart disease is not just the scourge of modern man. Researchers have discovered that 22 out of

44 Egyptian mummies in a recent study of people who had died under the age of 50 had suffered from cardiac issues.

This is according to Professor Sivasankaran Sivasubramonian, who also highlighted the fact that over the past three decades hypertension had increased to become the world’s number one cardiac risk factor. Sivasubramonian works for the Sree Chitra Tirunal Institute for Medical Sciences and Technology in India.

“We are experiencing a tsunami of obesity and of hypertension cases,” Sivasubramonian said, adding that it was important to recognise and treat high blood pressure and hypertension among children.

“The advent of high BP and hypertension preceded other problems,” he said.

Brazil had dealt with this

by making children physically active, while targeting women and children had also helped combat the onset of other diseases.

Atherosclerosis - the build up of a waxy plaque on the inside of blood vessels – begins in the

foetus, and left untreated can lead to death in middle age,” said David Celemajer of

Sydney University.Atherosclerosis was an important

public health issue and can be wiped out through intervention, but early detection was vital.

“If you were a very small baby you have enhanced risk factors and

your chances of cardiac issues in later life are enhanced,” he said.

But the good news is that it can be reversed - if detected early enough - with diet, exercise and non-pharmaceutical means, including the use of anti-oxidants, as well as pharmaceutical means like the use of gene or cell therapy.

a tsuNami of hyperteNsioNBy RAY JOSEPH

Exercise has no part in weight control, says Professor Tim Noakes,

the often controversial professor of Exercise and Sports Science at the University of Cape Town.

“My colleagues say I’m mad, that I have lost it,” says Noakes, who blames the United States Drug Administration (USDA) for the increase in obesity worldwide, after it changed its recommended diets some years ago.

Man’s ancestor had developed by “hunting the fattest animals, devouring the fat” but then everything changed when a high carbohydrate diet was recommended

and calorie consumption increased.“But what we want is less

carbs, more fat...Coke and Pepsi are weapons of mass destruction. “

Before 1978 the medical attitude exercise was “antagonistic”, says Noakes, whose no carbohydrate, no sugar and high fat regimen has turned the conventional wisdom on exercise and dieting on its head.

“Diet is more important for body weight than physical activity. You need to remove food addictions.”

But ground-breaking research in that year showed how exercise lowered the mortality rate, he says, adding that to be physically active required passion.

“One consequence of being fat is you are always hungry and you need to remove the addiction to carbohydrates”.

Advocating a diet of meat, fish, dried beans, eggs and certain nuts, Noakes said that human beings had evolved over thousands of year on a high fat diet and the problem of obesity had only spun out of control in the past few decades since carbohydrates became parts of peoples’ diets.

No liNK betweeN exercise aNd weight lossBy RAY JOSEPH

Tim Noakes Excercise: is it the answer?

Pluripotent stem cells and their implications for paediatric

cardiology proved to be a hot topic of discussion at the Sixth World Congress of Paediatric Cardiology and Cardiac Surgery, currently underway at the Cape Town International Convention Centre. The congress is being attended by over 2 400 scientists from around the world and aims to raise awareness of the plight of children with congenital heart disease in the developing world.

Professor Lior Gepstein of the Technion-Israel Institute of Technology in Haifa, Israel, said that regenerative

medicine, including cell therapy and tissue engineering, was an exciting new field in medical development. Gepstein won fame as the lead researcher in a groundbreaking study published last year which succeeded in taking skin cells from heart failure patients and transforming them into new healthy heart muscle cells that are capable of integrating with existing heart tissue.

The research opens the way for treating heart failure patients with their own, human-induced pluripotent stem cells (hiPSCs) to repair their damaged hearts. Until the study was published, scientists had not been able to show that heart cells created from hiPSCs could integrate with existing heart tissue.

At the time, Gepstein was quoted as saying: “What is new and exciting about our research is that we have shown that it’s possible to take skin cells from an elderly patient with advanced heart failure and end up with his own beating cells in a laboratory dish that are healthy and young – the equivalent to the stage of his heart cells when he was just born.”

The researchers were able to differentiate the hiPSCs to become heart muscle cells (cardiomyocytes) and then developed the cardiomyocytes into heart muscle tissue, which they cultured together with pre-existing cardiac tissue. The new tissue was transplanted into healthy rat hearts and the grafted tissue started to establish connections with the cells in the host tissue. Finally the rat cells and human cells were beating together in unison.

But more research needs to be conducted before these results can aid treatment for heart failure patients. “There are several obstacles to clinical translation,” said Gepstein. “These include scaling up to derive a clinically relevant number of cells; developing transplantation strategies that will increase cell graft survival, maturation, integration and regenerative potential; developing safe procedures to eliminate the risks for causing cancer or problems with the heart’s normal rhythm; further tests on animals; and large industry funding since it is likely to be a very expensive endeavour.”

He estimates that it will take at least five to ten years before this research reaches clinical trials.

repairiNg a damaged heartBy KATHERINE GRAHAM

Stem cell

Lior Gepstein

This newsletter was produced by the team at HIPPO. www.hippocommunications.com