Living with Half a Heart

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Study provides new insights for adults living with hypoplastic left heart syndrome.
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Blood is circulated by only half of the heart’s chambers in people who have been treated with the Fontan procedure.

Huge challenges are faced by children born with ‘half a heart’—a condition known as hypoplastic left heart syndrome.

The syndrome is a rare congenital disorder in which the left half of the heart is undersized and cannot 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.

The lead author of the study, Dr. Rachel Wald, is a staff cardiologist at UHN’s Peter Munk Cardiac Centre’s Adult Congenital Cardiac Clinic and a Clinical Researcher at the Toronto General Hospital Research Institute.