Huge challenges are faced by children born with ‘half a heart’—a condition known as hypoplastic left heart syndrome.
The syndrome is a rare inherited disorder in which the left half of the heart is undersized and can’t perform its function of pumping blood to the body. Without surgery, it is fatal.
The good news is that surgical procedures have been developed over the past 40 years, and recent improvements are seeing more and more people with the syndrome live into adulthood.
As numbers of adult survivors increase, there is a need for medical information to help patients, doctors and parents of children with the syndrome to make informed decisions.
To address this problem, Dr. Rachel Wald, a Clinical Researcher at the Toronto General Hospital Research Institute, led a study that compiled existing data from seven health centres from around the world to shed light on the health of adults with hypoplastic left heart syndrome for the first time.
Her team looked specifically at one of the most common surgical treatments for the syndrome, known as the Fontan procedure. The procedure works by redirecting blood flow so that the heart is able to pump blood to the body.
Dr. Wald explains, “Our international study focuses on the outcomes of patients older than 18 years who received the Fontan procedure in childhood but are now in adult care for a mean duration of 3 years. We were concerned to see that around a quarter of those who received the procedure faced major health complications at various points during the study period.”
The study also revealed that common risk factors associated with heart disease, such as lower oxygen levels in the blood and exercise intolerance, were also associated with complications after the Fontan procedure.
By providing the first description of health outcomes of adults who have received the Fontan procedure, this study represents an important first step to providing individuals and families affected by hypoplastic left heart syndrome with the tools they need to make informed treatment decisions.
This work was supported by the Canadian Institutes of Health Research, the University of Toronto Dean’s Fund and the Toronto General & Western Hospital Foundation.
Wilson WM, Valente AM, Hickey EJ, Clift P, Burchill L, Emmanuel Y, Gibson P, Greutmann M, Grewal J, Grigg LE, Gurvitz M, Hickey K, Khairy P, Mayer JE Jr, Teo E, Vonder Muhll I, Roche SL, Silversides CK, Wald RM. Outcomes of Patients With Hypoplastic Left Heart Syndrome Reaching Adulthood After Fontan Palliation: Multicenter Study. Circulation. 2018 Feb 27. doi: 10.1161/CIRCULATIONAHA.117.031282.
Princess Margaret Cancer Centre Scientist Dr. Trevor Pugh has received one of five 2018 Phillip A. Sharp Innovation in Collaboration Awards from Stand Up To Cancer. The US $250,000 grant, awarded to Dr. Pugh and his collaborator Dr. David Barrett from the Children’s Hospital of Philadelphia, aims to promote collaboration among cancer researchers.
Drs. Pugh and Barrett will use the funds to develop personalized medicine tests for children with cancer. Specifically, they will examine the genetic makeup of patients’ immune cells involved in mounting anti-cancer responses. The researchers will develop ways to use this genetic information to match patients with clinical trials of drugs that are designed to boost the immune system’s anti-cancer response. This treatment strategy, called immunotherapy, is one of today’s most promising approaches to treat cancer.
One unique aspect of this award is how quickly projects are selected, approved and funded: the entire process takes just a few weeks. This is done so that the research can begin right away. This year, the selection committee placed particular emphasis on teams of researchers who had not worked together in the past—with the aim of bringing the researchers closer together so that they can better leverage and share resources and expertise.
The Sharp Awards are sponsored by Stand Up To Cancer, a charitable organization that raises funds “to accelerate the pace of groundbreaking translational research that can get new therapies to patients quickly and save lives now.” They are named after Dr. Phillip A. Sharp, winner of the prestigious Nobel Prize in Physiology or Medicine in 1993 and chair of Stand Up To Cancer’s Scientific Advisory Committee.
Dr. Pugh and Dr. Barrett’s specific Sharp Award was co-sponsored by the Emily Whitehead Foundation, whose mission is “to raise awareness and funding for innovative childhood cancer treatments, such as immunotherapy, that will improve survival rates and quality of life.”
The Canadian Science Policy Centre (CSPC) is hosting an event at MaRS on Tuesday April 17 starting at 4:00pm. The event is open to the public and will bring together science policy stakeholders to participate in an interactive and engaging panel session on the 2018 federal budget and its implications for science, innovation and society.
Decoding the Federal Budget 2018 for Science and Innovation
Location: MaRS Discovery District, 101 College Street, Room CR-3
Date: April 17, 2018
Time: 4:00 – 6:00pm
The session will be led by David Watters, who has worked for 30 years in the Public Service of Canada. His past roles include a dozen years as an Assistant Deputy Minister in Industry Canada, Treasury Board Canada and Finance Canada, where he was responsible for overseeing federal economic development, budgets and corporate finance policies.
CSPC is committed to keeping the community up to date and informed regarding science, technology, and innovation policy issues. Regarding the Federal Budget 2018, CSPC has published featured editorials on sciencepolicy.ca, including an exclusive interview with the Federal Minister of Science, Hon. Kirsty Duncan.
Welcome to the April issue of Research Spotlight.
This newsletter highlights top research advancements from the five research institutes and over 450 appointed researchers at UHN. As Canada’s largest research hospital, UHN is a national and international source for discovery, education and patient care.
Stories in this month’s issue:
● FEELING GOOD AFTER CANCER: New eight-week program enhances body image and well-being of breast cancer survivors.
● A RISK UNCOVERED: Researchers have uncovered why only a subset of diabetic patients develop kidney disease.
● CAN THE FLU TRIGGER A HEART ATTACK? Study reveals new strategy to help prevent heart attacks: stop the spread of the flu.
● BUT WHY DOES IT WORK? While deep brain stimulation is effective for controlling tremor in Parkinson Disease, a new study helps us understand why.
Also included is information about UHN’s participation in the upcoming March for Science Toronto, which is happening on Saturday April 14.
Congratulations to the following four UHN researchers, who recently received prestigious national and international honours.
Dr. Joanne Bargman, staff nephrologist in the Division of Nephorology at UHN, is this year’s recipient for the Gabor Zellerman Lectureship given at the North American Chapter of the International Society of Peritoneal Dialysis meeting. The Gabor Zellerman Award was established in the memory of Dr. Gabor Zellerman by his wife, Lisa Hoffman, and recognizes lifetime contributions to the science and practice of peritoneal dialysis. Dr. Bargman is also a Professor of Medicine at the University of Toronto. She received her MD cum laude from the University of Toronto and pursued nephrology training at Stanford University.
Dr. Andrea Furlan received the Mayday Pain & Society Fellowship from the Mayday Fund. Dr. Furlan is a staff physician and Senior Scientist at Toronto Rehab and an Associate Professor in the Department of Medicine at the University of Toronto. Her main area of research focuses on the appropriate use of prescription medications for pain management, which include opioids, benzodiazepines, antidepressants, anticonvulsants and cannabinoids. She is one of 12 pain experts from across North America selected for the prestigious Fellowship program, which is focused on communications and advocacy for improved pain care. Fellows will learn skills to effectively communicate and advocate for the translation of scientific research and evidence-based best practices in pain care and management. Following the workshop, the fellows will receive follow-up coaching support to pursue their advocacy goals.
Dr. Dafna Gladman was awarded the 2018 Carol Nachman Prize for outstanding innovative research activities in the field of rheumatology. This award will be presented to her in Wiesbaden, Germany, in June. Dr. Gladman is Director of the Psoriatic Arthritis Program at UHN, co-Director of the University of Toronto Lupus Clinic and Senior Scientist at the Krembil Research Institute. The Carol Nachman award is considered one of the most prestigious international awards for research in rheumatology and one of Germany’s highest medical honours.
Dr. Tak Mak is one of five members elected to serve on the American Association for Cancer Research (AACR) Board of Directors for the 2018-2021 term. Dr. Mak is a Senior Scientist and Director of the Campbell Family Institute for Breast Cancer Research at UHN’s Princess Margaret Cancer Centre. He is a member of the Institute of Medical Science and Professor in the Departments of Medical Biophysics and Immunology at the University of Toronto. Dr. Mak will join the AACR Board of Directors, along with other esteemed cancer researchers, at the AACR Annual Meeting 2018, to be held in Chicago, April 14-18. The AACR is the world’s first and largest professional organization dedicated to advancing cancer research and its mission to prevent and cure cancer.