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Leading the way with heart
Leading the way with heart

SickKids eNewsletter February 2016

Welcome to the new SickKids eNewsletter. Distributed quarterly, this publication will focus on a topic and highlight where we’ve been, where we are and where we are going: the THEN, the NOW and the NEXT. Please enjoy our most recent publication, and if you like what you see, subscribe now!

Message from Dr. Michael Apkon | Then - 1963: A new future for blue babies | Now - A new dimension to heart surgery | Next - The genetic code behind heart disease


Mike Apkon


Every day, I find myself inspired by the heartbeat of SickKids: our amazing staff and volunteers, as well as the patients and families that we have the privilege to care for. I feel lucky to be part of this team and I am proud to say that I #HeartSK. This February, to mark heart month, we are celebrating our history of innovation in cardiac care, highlighting the groundbreaking work that is being done right now, and looking forward to the future of continued advancements that will ultimately lead to healthier hearts for our patients as they grow up to be successful adults.

Today, we are able to treat many heart conditions that were once considered untreatable. We celebrate those advances and at the same time work to continue to improve the quality of life for our patients born with heart disease. Through the integration of care, research and education, our exceptional teams of caregivers and scientists are at the forefront, developing more individualized treatments that are less invasive and more targeted. I am excited to see what the future holds and proud to share our stories with you in this edition of Then Now Next.

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Dr. William Mustard in surgery

Transposition of the greater arteries (TGA) is a relatively common heart defect, occurring in about one in every 5,000 live births. The greater vessels of the heart are reversed in babies born with this condition. Called ‘blue babies’ because of the blue tinge of their skin due to a lack of oxygen in their blood, it was not all that long ago that most of these children did not survive past infancy.

In 1963, a great advancement in treatment was made at SickKids by Dr. William Mustard. Now known throughout the world as the ‘Mustard Procedure’, this surgical intervention left the greater vessels in their transposed position but corrected the circulation in the infants by reversing the way in which the blood entered the heart. The problem was thus corrected, as the blood could be oxygenated.

While the Mustard Procedure is no longer the standard treatment for correcting this defect – it was replaced by a new intervention in the late 1980s – it saved many lives, improving an 80 per cent mortality rate in the first year of life to an 80 per cent survival at age 20. The Mustard Procedure has been described as one of the greatest achievements of modern cardiovascular medicine.

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SickKids is a leader in cardiac care

SickKids cardiac radiologist Dr. Shi-Joon Yoo, and MRI technologist Omar Thabit, had a revolutionary, and life-saving, application in mind for 3-D printing. They decided to 'print' babies' hearts, using diagnostic imaging as their blueprint, to give surgeons a new learning tool. At first, the hearts were hard plastic. Although each heart still takes a day to print, they're now soft. While not quite the same as real tissue, they're an effective medium in which surgeons can see, and operate on, a variety of congenital heart defects. 

When 'practice' surgery is performed on a printed heart, "If there's a problem, no problem," says Dr. Glen Van Arsdell, Head of Cardiovascular Surgery at SickKids, who, with Yoo, recently welcomed 11 surgeons from across Canada and from countries as far away as Chile and Norway, to SickKids for what they call 'HOST': Hands-On Surgical Training.

Launched less than a year ago, this new kind of training is a world first. Van Arsdell, also Professor of Surgery at the University of Toronto, defines one of the key advantages of this training when he speaks of his own experience. "I spent about 10 years getting to where I had the right to operate on children." Seeing, and operating on accurately modelled complex defects accelerates a surgeon's competency. "It's two years after a fully-trained surgeon joins my team that they can join me in operating," says Van Arsdell. He sees a time when, using this training, surgeons can be signed off on a 'menu' of operations by senior surgeons.

The detail of the hearts makes them profoundly realistic. As Yoo, also a Professor of Medical Imaging at the University of Toronto says, "The highest resolution current medical imaging can give surgeons is 0.3 - 0.7 mm. The printer we use delivers 0.3 mm resolution." At a recent training session in the Peter Gilgan Centre for Research and Learning at SickKids, one of the visiting surgeons put it well: "Usually, I have to assemble a picture of the heart in my mind from a series of 2-D images. This way, I can see it."

It’s clear that 3-D printing provides huge benefits to surgeons. But 3-D hearts will have a huge impact on patients and families, too. Traditionally, the discovery process happened in the operating room, with the child's chest open, creating the possibility of the negative impacts associated with major surgery on tiny patients. Now, the operation can be rehearsed, and assistants can be as familiar with the heart defect as the primary surgeon. Rehearsal will save surgical time, and therefore time under anesthesia, which both Yoo and Van Arsdell know leads to better outcomes.

In the recent training session Van Arsdell and Yoo 'HOST'ed, the surgeons worked with five models, representing a suite of five congenital heart defects. This initiative reinforces SickKids' commitment to training, and the hospital's well-earned reputation as a world leader in congenital heart disease. Van Arsdell and Yoo look forward to making this training standard for SickKids fellows further establishing SickKids as "the place to train."

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Dr. Seema Mital in her lab

“We are bringing the future of medicine to the patient’s bedside. Targeting health care to the unique genetic make-up of the child will make treatments safer, more effective and precise,” says Dr. Seema Mital, Head of Cardiovascular Research at The Hospital for Sick Children (SickKids) and Scientific Co-lead, Ted Rogers Centre for Heart Research.

Mital, who is also a Professor of Paediatrics at the University of Toronto, has launched the first Cardiac Precision Medicine Program in the country focused on deciphering the genetic code of heart disease in children. This knowledge will be used to develop targeted therapies that will help the heart adapt and prevent patients from developing heart failure in the future.

“While surgery is the cornerstone of treatment for congenital heart disease, we need to find ways to either prevent or treat heart failure in children that are more effective than the current ‘one size fits all’ approach,” says Mital.

Since the establishment of the Ted Rogers Centre for Heart Research, a collaborative initiative between SickKids, University Health Network (UHN) and the University of Toronto (U of T), one year ago, Mital and her team at the Cardiac Precision Medicine Program have been analyzing the genes that predispose patients to two conditions: right ventricle failure in children with Tetralogy of Fallot, which is the leading cause of “blue baby” syndrome, and cardiomyopathy, a disease that affects the heart’s ability to contract.

While “blue baby” syndrome can be treated early on with surgical intervention, these children live with leaky valves that eventually cause their heart to fail. SickKids researchers are exploring the genes that predispose infants to right ventricle failure, in hopes of developing new drugs to treat the condition before it becomes irreversible.

Cardiomyopathy (CMP) is a disease of the heart muscle that can cause dangerous forms of irregular heart rate and sudden cardiac death. There is currently no cure for CMP. Significant developments have been made in genetically diagnosing CMP, although the manner in which gene defects influence patient outcomes is still unknown. In order to find new therapies, Mital is working to generate heart cells from the skin or blood of patients with heart disease. These cells are used to screen drugs before they are tested with patients.

Precision medicine is beginning to change the way we treat patients. Treatment that is directed towards the underlying cause of disease rather than just the symptoms of disease will lead to more timely and effective cures, before the condition becomes irreversible.

“The Ted Rogers Centre is facilitating newer, faster and better ways of reaching our goals by catalyzing opportunities and galvanizing researchers to work together to try and find cures for heart failure,” says Mital.

The Ted Rogers Centre for Heart Research was enabled by a $130-million dollar donation from the Rogers Foundation. It is the largest monetary gift ever made to a Canadian health-care initiative and was matched by an additional $139-million in combined funds from the three partner institutions.

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