Adult Congenital Heart Disease and Pulmonary Arterial Hypertension. Patient Information

Transcription

1 Adult Congenital Heart Disease and Pulmonary Arterial Hypertension Patient Information

2 WHAT IS CONGENITAL HEART DISEASE? Congenital heart disease (CHD) refers to a defect of the heart or major blood vessels (aorta, pulmonary artery, systemic veins and pulmonary veins). The defect results when the heart or blood vessels do not form normally. This happens while the fetus is in the mother s uterus (womb). There are many types of heart defects. They include: Abnormal openings (communications) between heart chambers Abnormal openings (communications) between major blood vessels Reversed position of heart chambers resulting in abnormal connections between the upper and lower heart chambers Reversed position of major blood vessels resulting in abnormal connections between the major blood vessels and the heart chambers Abnormal or missing heart valves WHAT IS PULMONARY ARTERIAL HYPERTENSION (PAH)? PAH refers to high blood pressure in the arteries (blood vessels) of the lungs. This high pressure means that the right side of the heart must work harder to pump blood into the lungs. Many patients with CHD will develop some degree of PAH. Some types of CHD that can lead to PAH include: Atrial septal defect (ASD): a hole in the wall (septum) between the two upper chambers of the heart (atria) Ventricular septal defect (VSD): a hole in the wall (septum) between the two lower chambers of the heart (ventricles) Patent ductus arteriosus (PDA): a small blood vessel (ductus arteriosus) between the aorta and pulmonary artery that stays open instead of closing after birth. PAH may get worse over time. PAH can develop if you have an open communication between the heart chambers. It can also occur if you underwent repair of your CHD during childhood and adulthood. The most severe form of PAH due to CHD is called Eisenmenger syndrome.

3 HOW DOES EISENMENGER SYNDROME DEVELOP? The right side of the heart (an area of low blood pressure and low oxygen) gets blood from the body and pumps it to the lungs to pick up oxygen. The blood from the lungs then goes to the left side of the heart (an area of high blood pressure and high oxygen) and is pumped out to the body. Normally, the right side of the heart is separated from the left side by a wall called the septum.

4 Normal Heart and Arteries Ao Normal Heart and Arteries PA LA Ao RA PA LA LV RA/LA = Right & Left Atrium RV/LV = Right & Left Ventricle Ao = Aorta PA = Pulmonary Artery RA/LA = Right & Left Atrium RV/LV = Right & Left Ventricle Ao = Aorta PA = Pulmonary Artery RA RV RV LV there is a hole in the septum or an abnormal opening betw e major blood vessels (aorta and pulmonary artery) me blood will flow or shunt from the high pressure left the heart to the low pressure right side of the heart with e art beat. This is called a left-to-right shunt. If there is a hole in the septum or an abnormal opening between the major blood vessels (aorta and pulmonary artery) then some blood will flow or shunt from the high pressure left side of the heart to the low pressure right side of the heart with each heart beat. This is called a left-to-right shunt.

5 The shunt lets more blood than usual to flow into the blood vessels of the lungs. Over time, the increased blood flow causes a permanent change to the blood vessels in the lungs. The blood vessels become thick and stiff. These changes lead to: 1. Problems with the exchange of oxygen between the blood and the lungs 2. Even higher blood pressure in the lungs

6 As the pressure in the lungs increases, the right side of the heart has to work harder to get blood to the lungs. This causes the right side of the heart to become larger and the heart muscle to get thicker. Over time, the blood pressure on the right side of the heart gets higher than that on the left side and causes some oxygenpoor blood to flow to the left side of the heart across the Normal Heart and Arteries opening. Oxygen poor blood crosses the defect to enter the left side of the heart leading to lower oxygen levels in the blood going to the body Ao PA LA RA LV RA/LA = Right & Left Atrium RV/LV = Right & Left Ventricle Ao = Aorta PA = Pulmonary Artery RV This is called a right-left shunt and results in oxygen-poor blood mixing with oxygen-rich blood (Eisenmenger syndrome). As a result, blood pumped from the left side of the heart to the body has a lower than normal oxygen level. WHAT HAPPENS WHEN OXYGEN LEVELS ARE LOW? there is a hole in the septum or an abnormal opening between e major Your red blood blood vessels cells (aorta carry oxygen and pulmonary to all parts artery) of the then body. me The blood part will of flow the or red shunt blood from cell that the high carries pressure oxygen left is side called the hemoglobin. heart to the When low pressure oxygen levels right side are low, of the the heart body with adapts each by increasing the number of red blood cells to carry more oxygen. art beat. This is called a left-to-right shunt. Having more red blood cells than normal is called secondary erythrocytosis.

7 WHAT ARE THE SYMPTOMS OF PAH? Shortness of breath or difficulty breathing (dyspnea) Fatigue or feeling tired Swelling of the feet, ankles or abdomen (edema) Chest pain, heaviness, pressure, or tightness that may occur at rest or with activity Feeling dizzy, lightheaded or like passing out or actually losing consciousness Irregular, rapid or forceful heart beats (palpitations) Hoarseness of the voice Frequent cough Coughing up blood (hemoptysis) HOW IS PAH IN CHD DIAGNOSED? A cardiologist or a respirologist with expertise in CHD and PAH is the best person to diagnose PAH in persons with CHD. This requires a complete medical history and physical exam. Other tests which may be required before a diagnosis is made include: blood work, electrocardiogram (ECG), chest x-ray, echocardiogram, pulse oximetry, 6 minute walk test, Holter monitor, CT scan, lung scan, MRI, and/or heart catheterization. HOW IS PAH IN CHD TREATED? There is no way to reverse the damage that has been done to the blood vessels of the lungs. The goal of treatment is to manage symptoms, stop the PAH from getting worse and prevent further problems. Medications used in the management of PAH in CHD differ between patients. The medications used depend on the severity of symptoms or problems that occur because of low oxygen levels in the blood. Medications prescribed for PAH must be closely monitored by the PAH or CHD clinic.

8 Ao WHAT SHOULD I KNOW ABOUT LIVING WITH PAH IN CHD? REGULAR CHECK-UPS WITH THE SPECIALIST CLINIC ARE VERY IMPORTANT. PAH in CHD is a complex and serious disease. It is a life-long chronic condition that requires regular check-ups with a specialist in PAH and CHD. Even if you feel fine, it is important to go to all your check-ups. Problems may start even before you notice symptoms. Early RA treatment prevents more serious problems. KNOW YOUR HEART CONDITION. Learn all that you can about PAH and your CHD. Learn how to draw your CHD on a heart diagram. Know your usual oxygen saturation level and blood pressure. Know the name of the medications you are taking, why you are taking them, the dose and how often you take them. Many health care providers are not experts and have limited experience in PAH or CHD. Ask for a copy of your clinical report and carry it with you. This report provides very helpful information if a new doctor needs to take care of you in an emergency. Knowing your condition helps to ensure you receive proper care. RA/LA = Right & Left Atrium RV/LV = Right & Left Ventricle Ao = Aorta PA = Pulmonary Artery PA LA WEAR A MEDICAL ALERT BRACELET OR NECKLACE THAT LISTS PAH AND YOUR CHD. This ensures that emergency health care providers know right away that you have PAH and CHD even if you cannot speak for yourself. RV there is a hole in the septum or an abnormal ope KNOW THE NAME OF YOUR PAH AND CHD DOCTORS AND THEIR e major blood vessels (aorta and pulmonary CONTACT NUMBERS. Contact your PAH or CHD doctor or clinic if me you blood have a serious will illness flow or or injury, shunt go to an emergency from the department high pres or are admitted to hospital for any reason. If you cannot contact f the heart to the low pressure right side of the he the doctor or clinic yourself, have a family member or friend call on eart your beat. behalf. This is called a left-to-right shunt.

9 TELL YOUR DOCTOR ABOUT ANY OF THESE SYMPTOMS: Increasing shortness of breath (dyspnea) Dizziness, especially when standing or climbing stairs Fainting (syncope) Swollen ankles and legs (edema) Chest pain, especially with activity Frequent rapid or irregular heart beats (palpitations) Coughing up blood (hemoptysis) Fevers of unexplained origin WHAT FACTORS COULD IMPACT MY HEALTH? DEHYDRATION Dehydration is caused by a number of factors (e.g. diarrhea, vomiting, fasting, fever, hot climate, air travel, bleeding). It may limit exercise tolerance, increase the risk of stroke, or increase the risk of irregular heart rhythms. Most patients should drink at least 1 to 1.5 liters of fluid daily. You may need to drink more when you have a fever or when it is hot and humid. But you might also need to drink less if you have heart failure. Talk to your doctor or nurse about the right amount of fluid intake for you. LOW IRON LEVELS IN THE BLOOD Low iron levels in the blood may limit exercise tolerance and increase the risk of stroke. Your doctor may prescribe iron supplements or ask you to eat foods rich in iron if you are low in iron. BIRTH CONTROL AND PREGNANCY Some birth control pills may increase your risk of blood clots and stroke. Pregnancy in women with PAH and CHD can be very risky for both the mother and fetus. Talk to your CHD or PAH doctor about birth control and pregnancy.

10 HIGH ALTITUDES Air at high levels contains less oxygen and you should avoid altitudes higher than 2,500 meters. However, commercial airplanes are usually safe because the air pressure in the cabin is controlled. Talk to your doctor regarding the need for oxygen during your flight. ALCOHOL AND STREET DRUGS You should avoid large amounts of alcohol. Do not take illicit street drugs (e.g. marijuana, cocaine, heroin, ecstasy). They can cause life threatening irregular heart rhythms. INFECTIONS You should avoid getting infections. To avoid infections you should: Brush and floss your teeth daily and see your dentist at least once a year. Ask your doctor if you need to take antibiotics to prevent endocarditis, an infection in the blood that could damage the heart. If you do, then you will need to take antibiotics before dental work or other invasive procedures. See your doctor right away if you get an upper or lower respiratory (lung) tract infection or have other signs of an infection (e.g. infected cut). Avoid or limit contact with people who are ill with a cold or flu. See your family doctor for the seasonal flu vaccine every fall. Make sure that you have had a pneumonia vaccine. Make sure your immunizations are up-to-date. EXERCISE In general, regular physical activity is good for people with PAH and CHD. However, strenuous activity should be avoided. Talk to your CHD or PAH doctor about which activities are safe for you. Get your doctor s approval before starting any exercise program.

11 SMOKING Smoking lowers the amount of available oxygen in the blood. It also damages the lungs and heart. If you smoke, talk to your doctor or nurse about ways to help you stop smoking. NON-CARDIAC SURGERY No surgical procedure is simple in a patient with PAH or Eisenmenger syndrome. Talk with your CHD or PAH doctor before having any surgery. INTRAVENOUS (IV) LINES If you have a shunt with PAH or have Eisenmenger syndrome you are at risk for air embolism with intravenous (IV) lines. Air embolism refers to an air bubble in the blood. Normally, air bubbles that accidentally get into an IV line are stopped in the lungs. However, in the presence of a shunt or Eisenmenger syndrome, the right to left shunt may allow the air embolism to bypass the lung and travel directly to the brain. If this happens, some areas of the brain may get less oxygen or a stroke may happen if an artery becomes totally blocked. Special care should be taken with IV lines to avoid air embolism. WHAT CAN I DO TO KEEP HEALTHY? Eat a heart healthy diet Maintain a healthy weight Stay active Have regular check-ups with your PAH and CHD clinics

12 WHAT WORK CAN I DO? Talk with your CHD or PAH doctor about the right type of work for you. It is best to avoid jobs that require physical labor, frequent traveling or long commutes. WHEN SHOULD I CALL MY PAH OR CHD DOCTOR OR CLINIC? If you develop worse or new symptoms. Before starting any new medication. This includes birth control pills, over-the-counter medications, herbal medications or dietary supplements. If you get a severe cold or respiratory tract (lung) infection. If you get a fever with no clear cause. Before having any kind of surgery. Before starting an exercise program. Before starting a new diet. Before traveling or moving outside of your province. FOCUS ON YOUR QUALITY OF LIFE It is important to know your condition and what you can do to keep yourself in the best health possible. Talk to your doctor or nurse if you have any questions. You can even ask them about what they think your health will be like in the longer term. Don t forget that life isn t only about your PAH and CHD. Focus on what you can do and what you like to do in life. The goal is to live as full and rich a life as possible.

13 NOTES

14 NOTES

15 This document was prepared by Doreen Fofonoff, MN, RN, CCN(C), Clinical Nurse Specialist, Pacific Adult Congenital Heart Clinic Vancouver, St-Paul s Hospital, Vancouver, BC With the valuable contribution of the following individuals: Pamela Heggie, RN, BN, Clinic Nurse Coordinator, Northern Alberta Adult Congenital Heart Clinic, University of Alberta Hospital, Edmonton, AB Yvonne Balon, RN, BN, MN, Nurse Clinician, Southern Alberta Adult Congenital Heart Clinic, Peter Lougheed Hospital, Calgary, AB Erwin Oechslin, MD, FRCPC, Director, Toronto Congenital Cardiac Centre for Adults, Toronto General Hospital, Toronto, ON Pictures courtesy of and used with permission of James L Wilkinson, FRACP, FRCP, FACC, FRCPCH, FCSANZ Professorial Fellow, Dept of Paediatrics, University of Melbourne Senior Cardiologist, Royal Children s Hospital, Melbourne, Australia

16 RESOURCES For more information about congenital heart defects go to: Canadian Adult Congenital Health Network Canadian Congenital Heart Alliance (CCHA) For more information about pulmonary arterial hypertension go to: Pulmonary Hypertension Association of Canada Sponsored by an unrestricted educational grant from Actelion Pharmaceuticals Canada Inc.