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A NEWSLETTER

WRITTEN BY

VOLUNTEERS

FOR THE WELL-BEING

OF ADULTS WITH

CONGENITAL

HEART CONDITIONS

VISIT OUR WEBSITECACHNET.ORG

SUMMER 2005the BeatSUMMER 2005, VOL. 13, ISSUE 2

Marijuana and Marijuana and Congenital HeartsWynne Chiu, BSN, RN, CCN (C) andDoreen Fofonoff, MN, RN, CCN(C)

In low or medium doses, marijuana increases the heart rate, blood pressure and blood flow. It may cause a very fast heart rate called tachycardia. At high doses, the drug may cause a very slow heart rate (bradycardia) and low blood pressure (hypotension). With a low blood pressure and heart rate, the person may feel very weak, dizzy and generally unwell because the heart is pumping less blood to the brain and around the body. ECG changes have also been reported. It is unknown whether these changes are related to the drug itself or whether it is related to the rapid heart rate that the drug causes. The changes go away once the drug is out of the body. An increase in abnormal heart beats coming from the upper chambers (atria) or lower chambers (ventricles) of the heart can also occur. Marijuana may also increase angina (chest pain) in people with narrowed heart(coronary) arteries. Using marijuana with other drugs, such as ecstasy, crack or cocaine may cause life threatening abnormalheart rhythms and shock.

Marijuana has other physical health risks. The marijuana smoke has more than 400 chemicals that can affect the lungs.

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A recent Health Canada poll in 2003 showed that one third of all teens aged 12-19 had tried smoking marijuana more than once, with older teens using it more often.

For many young adults with congenital heart disease (CHD) smoking marijuana is as socially acceptable as smoking tobaccoor drinking alcohol. Many of these users assume that this drug is not harmful. However, marijuana does affect the heart by changing heart rate and blood pressure. These changes may have no effect on a person with a normal heart but could cause serious problems for someone with CHD.

Marijuana is most often smoked and its effects on the body last for four to six hours after a single dose. The drug starts to leave the body about 20-30 hours after it is smoked. It can be found in the urine for several days after it is used and up to two months in heavy users.

IN THIS ISSUE

Marijuana and Congenital Hearts 1Book Contest • Ebstein’s Anomaly 2How You Can Help 3Tribute to Dr. John Kirklin • Volunteer Work 4Annual Conference for the Doctors & Nurses 5Clinic Updates • Contributors

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SUMMER 2005

into the right lower chamber.

The valve leaflets close once the blood has flowed into the right lower chamber. This stops the blood from leaking back into the right upper chamber when the right lower chamber pumps blood into the lungs.

In Ebstein’s anomaly, the tricuspid valve is moved downward into the right lower chamber. This may cause the right upper chamber to be larger than usual and the right lower chamber to be smaller. As well, one or two of the three valve leaflets may be stuck to the wall of the heart. As a result, the leaflets do not close properly and may leak. When the valve leaks, some of the blood pumped by the right lower chamber flows backwards into the right upper chamber. As a result, the heart does not work as well. The right upper chamber of the heart may enlarge as a result of the increased blood that leaks back into it.

Over half of the people with Ebstein’s anomaly also have a hole in the wall between the heart’s two upper chambers (atria). This hole is called an atrial septal defect or ASD. The hole causes oxygen rich blood from the left upper chamber to blow into the right upper chamber with each heart beat. This decreases the amount of blood that would normally go forward to the body.

One in four patients with Ebstein’s anomalymay also have abnormal heart rhythms called arrhythmias. These rhythms are most often fast, but may also be slow.

Diagnosis of Ebstein’s Anomaly

Severe cases of Ebstein’s are oftendiagnosed shortly after birth. Ebstein’s may not be found until a person starts to have problems (symptoms) in their late teens or early adult life.

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It may c au s e a ch ron i c cough o rbronchitis. The drug impairs motor skills, lessens coordination and balance, and may decrease male sex hormones.

Marijuana may also have psychological health risks. These include impaired judgment, learning and memory, and difficulty concentrating. Marijuana can trigger psychotic episodes in people with schizophrenia or who have a family historyof schizophrenia. Recent studies have shown that frequent use of marijuana may cause depression and anxiety.

With all this in mind, it is easy to see why marijuana may worsen existing problems for people with CHD. Some of these problems are low heart rates, low blood pressure, high blood pressure, abnormalheart rhythms, heart failure, angina, depression and anxiety. It is important that patients tell their heart doctor they smoke marijuana. They should ask what effect it will have on their CHD. People born with heart defects are able to live healthy lives. They need to care for their hearts by making healthy lifestyle choices. Not taking drugs is a healthy lifestyle choice. •

Ebstein’s AnomalyDoreen Fofonoff, MN, RN, CCN(C)

Ebstein’s anomaly, also called Ebstein’s malformation, is a congenital heart defect in which the tricuspid valve is abnormally formed. The defect is present at birth. It occurs in about 1:20,000 live births. Ebstein’s anomaly occurs in less than 1% of all congenital heart defects. It affects both males and females equally.

In the normal heart, the tricuspid valve has three “flaps” or leaflets. The valve splits the top right heart chamber (right atrium) from the bottom right heart chamber (right ventricle). The leaflets of the valve act as one-way gates, letting blood flow from the right upper chamber

Marijuana and Congenital HeartsMarijuana and Congenital HeartsMarijuana continued from page 1

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SUMMER 2005 Surgery may be advised when the patient has severe symptoms, or when the heart starts to enlarge and the heart does not work as well. The surgeon may repair the tricuspid valve or replace it with a tissue or mechanical valve. If there is an ASD, it is usually closed at the same time. A Maze procedure may be done during surgery if the person has abnormal fast heart rhythms. During this procedure, the surgeon makes a series of cuts in the right upper chamber of the heart. These cuts interrupt the elec-trical pathways that cause the abnormal heart rhythm.

Ongoing Care:

Regular Check-Ups:Ebstein’s anomaly is uncommon so people with Ebstein’s anomaly should receive on-going care with regular check-ups at an adult congenital heart centre where the heart doctors have expertise in congenital heart defects. Regular check-ups allow the heart doctor to detect problems early so that the best care and treatments can be offered.

Report Symptoms:Symptoms such as decreased stamina, increased shortness of breath, fatigue, cyanosis or episodes of rapid heart rhythm, fainting, or swelling of the feet and legs should be reported to the family doctor or heart doctor. These problems may develop or worsen as the valve leaks more.

Activity Restrictions:Being physically active is good for the heart, so people with Ebstein’s should stay active. Ask the heart doctor which activities are safe. If the valve leakage is mild and tests show no abnormal heart rhythms, people can usually perform most physical activities. However, if the valve leakage is moderate or severe, the heart doctor may advise some physicalactivities be avoided.

Endocarditis Prevention: Endocarditis is an infection that can damage the inner lining of the heart or

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People with mild Ebstein’s may not have any problems at all and their defect may be found during routine exams.

The heart doctor (cardiologist) may confirmthe diagnosis of Ebstein’s anomaly by doing a full physical exam and several tests. These tests may include:

• An ECG (electrocardiogram) to look for an increase in size of the right upper chamber and abnormal heart rhythms.• An echocardiogram (ultrasound of the heart) to look for a leaky and abnormal tricuspid valve, a large right upper cham-ber, and a small right lower chamber. This test will also provide information about how well the heart pumps.• A chest x-ray to look for an increase in the size of the heart.• A Holter monitor (24 hour ECG) to look for abnormal heart rhythms.• An exercise stress test to see how much the person can exercise and to see if abnormal heart rhythms occur during exercise.

Symptoms of Ebstein’s Anomaly

People with mild Ebstein’s may not have any symptoms at all. However, those with moderate or severe Ebstein’s may have shortness of breath with exercise, decreased energy, dizziness, lightheaded-ness, feel tired, or have slow or very rapid heart rhythms (arrhythmias) or blueness of the lips, fingers or toes (cyanosis).

Treatment Options

Treatment of Ebstein’s depends on the severity of the defect and the person’s symptoms. The heart doctor may advise regular check-ups if the person has no symptoms or no abnormal heart rhythms. If symptoms are present, medication may be given to improve the work of the heart. Medicine may also be used to prevent fast abnormal heart rhythms. For somepeople, the source of the rapid heart rhythm may also be removed by a procedurecalled radiofrequency ablation. Theablation uses sound waves to destroy the tissue that is causing the abnormal rhythm.

HOW YOU CAN HELP

Make a difference, make a donation. The CACH Network and the Adult Congenital Heart Council are a group of lay and professional volunteers determined to promote the interests of Canadians born with heart defects. We need your help to accomplish our objectives.

Thousands of Canadians are now alive and well thanks to dramatic advances in the care of heart defects. Donations are needed to ensure that people with heart defects continue to enjoy a completelife, and to make sure that our children and adolescentswill be even healthier as they grow to adult life.

The CACH Networkc/o BB&C

6835 Century Ave., 2nd flr.Mississauga, Ontario,

Canada.L5N 2L2

Please make your cheque out to: The CACH Network.All donations are tax deductible and a receipt will be issued for tax purposes.

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SUMMER 2005 and praise to Dr. Kirklin for his expertise in saving my life even though he told me plainly that I would never walk out of the Mayo Clinic alive and it wasn’t he who saved my life.

Dr. Kirklin and I first met in late Octoberof 1957. He was young, stern and hand-some. I was a young teenager, shy, fright-ened and motionless as we set eyes on each other for the first time at St. Mary’s Hospital in Rochester, Minnesota.

My world was certainly going nowhere. The principal of my high school asked mother to no longer send me to school because of heart episodes I was having during class as he told her not only was I disrupting classes, but I was upsetting students as well. The principal requested that until I had proper medical attention, I was not to return to school. This was heartbreaking news for me as I was just 15 years old and a grade ten student. Lucky for me; however, I had just had a visit with my cardiologist in Regina, Saskatchewan about 10 days prior to all this drama unfolding. He insisted strongly that I must again return to the Mayo Clinic and strongly insisted that no one other than Dr. John Kirklin would be allowed to perform my surgery. Dr. Israels, my cardiologist, had just returned from the United States where he had spent considerable time viewing surgeons perform cardiac surgery. Dr. Israels told mother that if Dr. Kirklin would not do my surgery, then I was to return home. There would be no surgery.

How could I ever forget Dr. Kirklin telling me that there was no surgical technique developed yet for opening the pulmonary artery? But while on theoperating table, he made the decision instead of stitching me up and sending me home to die, to proceed with hisinspiration to try and open my pulmonaryartery. Whatever decision he made, he felt he would still have to send me home to die. I am forever grateful for hiswisdom, fortitude and deep involvement in going the extra mile to save my live. It is hard for one to imagine the drama that unfolded while I was under his surgical

the heart valves. People with Ebstein’s anomaly are at risk of endocarditis. They usually need antibiotics before certain dental or surgical procedures to prevent endocarditis. As well, they should also look after their teeth and gums and avoid behaviors that may increase the risk of endocarditis (intravenous drug use, body piercing and tattooing).

Pregnancy:Pregnancy puts many demands on the normal heart. Patients with Ebstein’s should consult with their heart doctor about the safety of pregnancy, before they become pregnant. The heart doctor will assess what effect the pregnancy will have on the heart valve and the work of the heart. The heart doctor will decide what care is required before, during and after the pregnancy.

Tribute to Dr. John KirklinCarol (Hyman) Allan, "A Grateful Patient"

Today was a most unusual day for me. My husband Ray left yesterday for a golf week in Ireland and my twin sister Charlotte is visiting with family in Toronto. My stom-ach had been in knots all week with the anxiety over the temporary absence of the two most important people in my life.

This morning for no reason, I decided to search the internet for information about the Kirklin Clinic at UAB, which I had found a picture of a few years ago. I was not prepared to read that Dr. Kirklin had passed away on April 21, 2004. This date has a huge impact on me for not only did I have my first heart surgery at the Mayo Clinic on April 21, 1949, but my father passed away on April 21, 1965. Much significance and good in my life surrounds the date of April 21.

It is ironic for me to believe that on April 19 of 2004, the Canadian Adult Congenital Heart Network in Toronto posted my story entitled Courage to Heal a Broken Heart on their internet site under their Newsletter the beat at www.cachnet.org. In my story I give gratitude

VOLUNTEER WORK

Spending your time helpingothers can be very rewarding.We need you!

We're looking for people to help with advocacy to provincial governments and hospitals to improve the services available to adul t pat ients with congenital heart defects across Canada.

We're looking for people to join our mentorship program - program - program patients supportingpatients - friendship, and patients - friendship, and patientsencourage patients who would welcome a friendly would welcome a friendly would welcomevoice, and perhaps speak with someone who has gone through a similar challenge around the time of surgery or special procedures.

Becoming involved in the Becoming involved in the Becoming involvededucational evenings for patients and families would also be greatly appreciated.

W e ' r e l o o k i n g f o rpeople to help organize and work on our fund-raising program, to offer names of potential donors, and celebrities that may be wil l ing to par t ic ipate in a fundraising event.

We need people to contributearticles or reviews for u p c o m i n g i s s u e s o f "The Beat".

If you feel you can help in any other way we gladly encourage your participation. Go to cachnet.org and click on “how you can help”. •

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SUMMER 2005 Annual Conference for the Doctors and NursesMay 26 - 28th was the American College of Cardiology – International Symposium on Congenital Heart Disease in the Adult - Toronto 2005

This conference was the 15th annualconference. The course provided adultcongenital specialists with specific talks and work shops on the management o fcongenital patients. The speakers came from all over the world including, United Kingdom, Netherlands, Denmark, Switzerland, USA and Canada.

It was an excellent conference where the doctors and nurses were able to discuss how to best care for their patients to keep them heart healthy. •

Tribute continued from page 4

care. Little is even known to my close family members about that traumatic day back in January 1958.

Following the surgery I continued to bleed profusely and because I was still alive, Dr. Kirklin made the decision to give me one more chance and return me to surgery in the wee hours of the morningto try and stop the hemorrhaging. When I was about to return home from the Mayo Clinic following my surgery, Dr. Kirklin asked me to always continue to write him and let him know how I was doing. I feel this is a very special person who cared beyond the operating room.

How could I ever imagine back then in January 1958 that I would soon start to walk for the first time without gasping for air, turning bluish black and experiencing constant pain in my chest? I had been diagnosed with Tetralogy of Fallot. In April I would be 16 years old.

Throughout my lifetime, Dr. Kirklin has been my one and only hero. Dr. Kirklin was above and beyond what I could only imagine in a person. His memory will always live on through me. How could I ever forget the man who gave me a second chance at life? I truly think of him every day.

And to his family I say a special thank you. Thank you for giving this wonderfulhuman being an opportunity to pursue such human being an opportunity to pursue such human being an opportunity to pursuewisdom and courage. He has done so much good for young congenital heart patients like me. Words cannot say enough about my gratitude for him. Thank you for allowing him to come into my life at a time when I was most desperate. I would like to close by saying, that since my surgery in 1958, I have had no further cardiac surgery nor taken any cardiac medication. I can’t tell you how lucky I feel. For me, this is the greatest gift of all mankind. •

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SUMMER 2005

Pacific Adult Congenital Heart (PACH) Clinic

Wynne Chiu, BSN, RN, CCN(C) has joined the PACH Clinic as the new Patient Educator. Wynne most recently worked as a staff nurse on the Cardiac Surgery ward at St. Paul’s Hospital. Her position is part-time and she will be working be working Tuesday, Wednesday and Friday. She joins Doreen Fofonoff, who is the Clinical Nurse Specialist.

A round of applause to Cari Olson, a PACH p a t i e n t , w h o h a s o r g a n i z e d t h ePaddling with Heart Paddlethon incollaboration with St. Paul’s Hospital Foundation. On Saturday, June 4, 2005, the Women’s Racing Team paddled from Burrard Marina in Vancouver to Nanaimo on Vancouver Island, a distance of 56 km which will take anywhere from 5-10 hours. If extreme ocean conditions prevent a safe crossing the paddlethon will take place in local waters. Fifty percent of the money raised will be donated to the PACH Clinic. For more information contact St. Paul’s Hospital Foundation at 604.682.8206 or 1.800.720.2983.

- Doreen Fofonoff

Clinic UpdatesCalgary Adult Congenital Heart Clinic

Our 2005 Calgary Marfan Syndrome Information Day was held on June 11, 2005 at the Peter Lougheed Centre Auditorium. This event is held in conjunction with the Canadian Marfan Association and the Calgary Adult Congenital Heart Clinic. Topics to be covered include genetics, cardiac surgery, pregnancy, living with chronic disease, ophthalmology, exercise, orthotics, orthodontics, and exercise. We are looking forward to providing our patients and their f ami l i e s w i th the be s t in format ionavailable. Registration information isavailable at www.marfan.ca

Congratulations to our clinic social worker, June Yee and Nurse Clinician, Yvonne Balon. They have had their abstract “Predictors of Psychosocial Well-Being in Adults with Congenital Heart Defects” accepted at the international conference on Global Perspectives in Chronic Disease Management. This conference wil ltake place in Calgary at the end of September.

- Lori Newman

Toronto Congenital Cardiac Centrefor Adults (TCCCA)

It is summer time in Toronto again. Lots of good things happening here. The Fellowship program is expanding again with Fellows from Australia and Saudi Arabia arriving soon. We are also in the process of interviewing for the Director of the Toronto Congenital Program at the University Health Network. The dedicated staff Cardiologists at UHN are verycommitted to making the Toronto program a strong program with a patient centred approach and world leading congenitalcardiac care. The clinic is busy as usual and patients are certainly being patient (no pun) with the potential delays in their yearly appointments as we absorb Dr Gary Webb’s patients into the other cardiologist’s sched-ules. If patients have any concerns about their up coming appointment please call the clinic at 416-340-4206.

- Jeanine Allen

CONTRIBUTORS

EDITOR:

Lori Newman, RNNurse Clinician Calgary Adult

Congenital Clinic,Calgary Hypertrophic Cardiomyopathy Clinic

WRITERS:

Jeanine Allen,MN/ACNP

Acute CareNurse Practitioner,Toronto Congenital

Cardiac Centre for Adults (TCCCA)

Wynne Chiu,BSN, RN, CCN (C)Patient Educator,

Pacific Adult Congenital Heart Clinic (PACH)

Doreen FofonoffMN, RN, CCN(C)

Clinical Nurse Specialist, Pacific Adult Congenital

Heart Clinic (PACH)

Carol (Hyman) Allan,Volunteer

DESIGN & LAYOUT:

Ray GaneshCanadian Adult Congenital

Heart Network

www.cachnet.org