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CoNGeNiTAL firsts
Mustard Baby has twice Congenital Cardiac Centre.
defied the odds “We have a good rapport,”
Marina operator Maria Willing pays careful attention to says Maria, a mother of three.
her heart health with the help of congenital heart disease specialists “The joke is, [Dr. Oechslin] always
wants me to have the stress
Maria Willing There is little doubt that Maria She had developed atrial test, and I always refuse.”
remembers sitting Willing has lived up to her fibrillation and was experiencing
in her Renfrew, Ont., name throughout her life. irregular heart rhythms. She knew “We see Maria once a year
kitchen at age 30 something was terribly wrong. to monitor her heart function
thinking, “I’m going to Born with “blue baby and heart rhythm,” Dr. Oechslin
die”. As a child, her syndrome” in June 1961, Maria Maria vividly recalls sitting in says. “Her lifestyle is healthy,
life was saved by a was given six months to live. her Renfrew kitchen: “I just sat she is physically active and she
surgical procedure The syndrome is also known as there thinking, ‘I’m going to die.’” watches her diet and her weight.
performed by Dr. the transposition of the great She also has good genes.”
William Mustard. arteries – a form of cyanotic heart Not one to give up, but feeling
She has since been disease where the pulmonary she had nowhere to turn, she FeeLiNG TRuLy BLeSSeD
followed by adult and systemic circulation run called the Hospital for Sick Today, Maria and her husband,
congenital heart parallel instead of in a series, Children. “I told them I had had Brad Willing, own and operate a
specialists at the Peter resulting in a low blood-oxygen the Mustard Procedure. They marina in Haliburton, Ont. Open
Munk Cardiac Centre. level. She had two holes made immediately said, ‘Can you get from April to October, Maria
into her heart at only three here tomorrow?’” Maria drove to manages a busy convenience
weeks of age to allow mixing Toronto, only to realize they were store situated in the marina.
of the blue (non-oxygenated) referring to the nearest children’s
and pink (oxygenated) blood. hospital – the Children’s Hospital She attributes her youthfulness
of Eastern Ontario in Ottawa. to the careful attention she
A MuSTARD BABy pays to maintaining her health.
Blue babies had a less than THe WRoNG WAy, BuT “In August 2014 I got sick, and
20 per cent survival rate back THe RiGHT CHoiCe I thought I was going to have a
then, but Maria defied all odds, “I went the wrong way, but heart attack,” she says. “I didn’t
growing stronger each day. made the right choice,” says want to take the chance, so my
On May 16, 1963, Maria made Maria. “They saw me at Sick friends graciously offered to
medical history and became the Kids and immediately referred lend their time to help out.”
poster girl for the groundbreaking me to Dr. Peter McLaughlin at
surgical procedure performed by Toronto General Hospital.” Maria and Brad often sneak in
Dr. William Mustard at Toronto’s an evening cruise aboard their
Hospital for Sick Children. A Maria had to be cardioverted favourite pontoon in the summer.
three times, which involved
heroic repair to the converting an abnormally “I like the calmness the water
transposition of the great fast heart rate (arrhythmia) makes me feel,” she says. “I have
arteries that allows to a normal rhythm through all the time in the world to enjoy
oxygenation of the blood electricity or medication. the water. We are truly blessed.”
in the lungs, it became
known worldwide as the Then she had a stroke, SPeCiAL exPeRTiSe
Mustard Procedure. but within a week she was In the 1980s, the Mustard
fitted with a pacemaker. Procedure was replaced by the
By 1987, the procedure Jatene Procedure, the arterial
had saved more than 500 Maria attributes her survival switch procedure to repair
babies at the Hospital to the fine work of the doctors complete transposition of the
for Sick Children and at the Toronto General Hospital. great arteries. All survivors of the
thousands worldwide. Now 54 years old, she attends Mustard (Jatene) Procedure –
Survival to adulthood annual follow-ups at the Toronto like all patients born with
increased to 80 per cent Congenital Cardiac Centre congenital heart disease of
for these “Mustard babies.” at the Peter Munk Cardiac moderate or great complexity –
Centre (PMCC). “I always take had to be transferred to adult
The hospital lost a few days off and go on a congenital heart disease
touch with Maria over mini vacation to Toronto with centres with special expertise.
the years – her family my girlfriends,” she says.
lived in Whitby, Ont., According to Dr. Oechslin,
then Renfrew, Ont. It Maria has developed a “patients with a Mustard
wasn’t until she was 30 strong relationship with her Procedure are at risk for long-term
years old, facing another former cardiologist, Dr. Vera complications and require long-
complication, that she was Rose, who retired about three term follow-up in a specialized
drawn back to Toronto. years ago, and now she’s centre. Fifty per cent of patients
under the care of Dr. Erwin with a Mustard Procedure who
Oechslin, director of the Toronto graduated from the Hospital for
Sick Children are not associated
with an adult congenital heart
disease program and are lost
to follow-up. We don’t know
where they are and how they
are doing. Only an emergency
brings some of them back to
the health-care system.”
Winter 2016 9
