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Tetralogy of Fallot

Tetralogy of Fallot is the most common heart defect in children. The condition causes mixing of oxygen-poor blood with the oxygen-rich blood being pumped out of the heart and into the circulatory system of blood vessels.

ventricle pumps the blood out of the heart into the circulatory system via a large artery known as the aorta.

The blood leaving the heart has less oxygen than is needed by the organs and tissues of the body, a condition called hypoxemia. Chronic (ongoing, long-term) lack of oxygen causes cyanosis, a bluish color of the skin, lips, and membranes inside the mouth and nose.

The blood moves throughout the body, supplying oxygen and nutrients to organs and cells. Organs cannot work properly if they do not receive enough oxygen-rich blood.

The 4 abnormalities (tetralogy) of the heart described by Fallot include the following:

The normal heart works as follows:


The heart is made up of 4 chambers: 2 upper chambers called atria and 2 lower, largerchambers called ventricles. Each atrium is separated from its paired ventricle by a valve. The heart has a left and a right side. The left and right sides of the heart are separated by a septum (wall). The right side of the heart receives oxygen-depleted or blue blood returning by veins (superior vena cava and inferior vena cava) from the body. The blood flows from the right atrium through the tricuspid valve into the right ventricle, which pumps it through the pulmonic valve into the pulmonary artery, the main artery to the lungs. In the lungs, the blood absorbs oxygen and then returns to the left atrium through the pulmonary veins. From the left atrium, the blood is pumped through the mitral valve to the left ventricle. The left

Right ventricular hypertrophy: Narrowing or blockage of the pulmonaryvalve and/or muscle under the pulmonary valvecoming out of the right ventricle.This restriction to blood outflow causes an increase in right ventricular work and pressure, leading to right ventricular thickening or hypertrophy. Ventricular septal defect (VSD): This is a hole in the heart wall (septum) that separates the 2 ventricles. The hole is usually large and allows oxygen-poor blood in the right ventricle to pass through, mixing with oxygen-rich blood in the left ventricle. This poorly oxygenated blood is then pumped out of the left ventricle to the rest of the body. The body gets some oxygen, but not all that it needs. This lack of oxygen in the blood causes cyanosis. Abnormal position of the aorta: The aorta, the main artery carrying blood out of the heart and into the circulatory system, exits the heart from a position overriding the right and left ventricles. (In the normal heart, the aorta exits from

the left ventricle.) This is not of major importance in infants.


The following symptoms suggest tetralogy of Fallot:


Pulmonary valve stenosis (PS): The major issue with tetralogy of Fallot is the degree of pulmonary valve stenosis, since VSD is always present. If the stenosis is mild, minimal cyanosis occurs, since blood still mostly travels to the lungs. However, if the PS is moderate to severe, a smaller amount of blood reaches the lungs, since most is shunted right-to-left through the VSD.

Growth and development are slower, especially if the pulmonary stenosis is severe. Puberty may be delayed if the tetralogy is untreated. The child usually tires easily and begins panting with any form of exertion. He or she may play for only a short time before sitting or lying down. Once able to walk, the child often assumes a squatting position to catch his or her breath and then resumes physical activity. Squatting increases the pressure transiently in the aorta and left ventricle, causing less blood to move into the left ventricle, more out the pulmonary artery to the lungs.

Tetralogy of Fallot accounts for 10-15% of all congenital (newborn) heart defects. Infants with this abnormality develop signs of the condition very early in life. Tetralogy of Fallot Causes Tetralogy of Fallot occurs during development of the fetus, before birth, and is therefore termed a congenital birth defect. An error occurs as the fetal heart separates into the chambers, valves, and other structures that make up the normal human heart. No one is really sure why this error occurs. Tetralogy of Fallot Symptoms Most infants with tetralogy of Fallot develop cyanosis in the first year of life.

Episodes of extreme blue coloring (called hypercyanosis or simply "tet spells") occur in many children, usually in the first 2-3 years of life.

The child suddenly becomes blue, has difficulty breathing, and may become extremely irritable or even faint. Up to 20-70% of children with tetralogy of Fallot experience these spells. The spells often happen during feeding, crying, straining, or on awakening in the morning. Spells can last from a few minutes to a few hours.

The skin, lips, and mucous membranes inside the mouth and nose take on a noticeably dusky blue color. Only some infants with very severe obstruction of the right ventricle outflow turn blue at birth. A small number of children with tetralogy of Fallot never turn blue at all, especially if the pulmonary stenosis is mild, the ventricular septal defect is small, or both. In some children, the cyanosis is quite subtle and may go undetected for some time.

When to Seek Medical Care Sometimes tetralogy of Fallot goes undiagnosed for several months to a year. Diagnosing conditions such as tetralogy of Fallot is one of the goals of routine checkups with your doctor. Take your child to his or her health care provider if the child develops a bluish color, has breathing difficulties, seizures, fainting,

fatigue, slow growth, or developmental delay. A medical professional should establish the cause of these problems. If you cannot reach your child's health care provider or if the child develops any of the following symptoms, take the child to a hospital emergency department right away:
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Surgery The Blalock-Taussig operation: Apalliative procedure performed in smaller infants to increase blood flow to the lungs. This allows the child to grow big enough to have complete surgical repair. A connection is made between one of the majorarteries of the body, usually the right subclavian artery,and the right pulmonary artery, which increases the amount of red oxygenated blood reaching the lungs, relieving cyanosis with dramatic relief of the patient's symptoms. Total correction: The hole in the ventricular septum (between the ventricles) is closed with a patch and the obstruction to right ventricular outflow, pulmonic stenosis, isopened. These corrections allow blood flow to the lungs for oxygenation before being pumped out into the body. The timing of the operation depends on symptoms. Surgery usually is performed within the first 2 years of life. Operative mortality rates have dramatically dropped over the last 20 years. Still, about 1-5% of children who undergo complete correction die during or immediately after the procedure, secondary to other additional defects in the body and/or heart, and the heart lung bypass procedure itself.

Bluish discoloration Trouble breathing Seizures Fainting Extreme fatigue or weakness

Medical Treatment Surgery is the primary way to correct the heart problem. Your child may be prescribed medication for tet spells. You will also be given information for dealing with future tet spells.

The child will be placed on his or her back in the knee-to-chest position to increaseaortic resistance. The increased aortic and left ventricular pressure reduces the rush of blood through the septal hole from the right ventricle and improves blood circulation to the lungs, so more red blood reaches the tissues. The child may be given oxygen through a face mask to increase the amount of oxygen in the blood. The child may be given morphine, propranolol (or metoprolol), or, in extreme cases, phenylephrine (Alconefrin, Vicks Sinex). These medications decrease the frequency and severity of tet spells.

Overview and Summary: Environmental Health: Important Choices for a Greener World
By: Susan Wilburn, M.P.H., B.S.N., R.N.

How many "Green' magazine covers have you seen lately? Likely you've seen a number of these Green covers since there have indeed been an increase in the number of such covers recently. This increase is not just because of the annual Earth Day events celebrated in April, or the visibility of Al Gore and his movie, "An Inconvenient Truth." This increase is also a result of the growing recognition of the connection between health and the environment, a recognition which has changed the nature, the visibility, and the critical importance of this topic for public health. This topic takes a look at environmental hazards, particularly chemical hazards, and considers the health effects of these hazards and the role of nurses in protecting and promoting health through healthier environments. Since Florence Nightingale recognized and statistically analyzed the impact of clean air and water on the survival rates of wounded soldiers, nurses have incorporated principles of environmental health into their practice (Butterfield, 2002; McDonald, 1987). Indeed the holistic approach to health embodied in the nursing process recognizes and acts on all determinants of health and disease, including those related to environmental conditions. In 1995 the Institute of Medicine report, Nursing, Health and the Environment, called upon nurses to be prepared to integrate environmental health into nursing practice, research, education, and advocacy (Pope, Snyder, & Mood, 1995). This report is credited with revitalizing and reemphasizing environmental health in nursing. This new era of environmental health activity in nursing moved beyond recognizing environmental factors that contribute to disease to more upstream thinking about prevention and precaution related to these diseases (Butterfield, 2002). Prevention now includes not only preventing of exposure to environmental contaminants, through the application of the hierarchy of controls in the workplace, but also to practicing source reduction, which involves replacing products and processes that create pollution with more environmentally healthy ones. Thus nurses and nursing have emerged at the forefront of the Green revolution in health care and are active in creating environmentally sustainable, health care practices. Terms, such as Green and sustainable development are becoming more common in the nursing literature. According to Wikipedia, Green is the color symbolizing earth, nature, and in a broader sense, life. The term Green is used to mean environmentally friendly. For example, green cars are vehicles that have extremely low emissions that are harmful to the environment (Green, 2007). The term sustainable development was defined in a United Nations (UN) report titled Our Common Future, by what became known as the Brundtland Commi ssion, which concluded that a global goal should be to make social and economic development sustainable, meaning that it meets the needs of the present without compromising the ability of future generations to meet their own needs

. In the ensuing decade, science has documented these toxic health effects. As American Nurses Association President Becky Patton said in her 2007 Earth Day editorial in The American Nurse,

Ignorance is bliss, but today we know the truth about the hazards of mercury and disinfectant use in health care and the need to substitute less hazardous substances for those currently in use . I first saw the film An Inconvenient Truth in France; and the French translation of the movie title: Une Vrit qui Drang , means a truth that co nfuses and disturbs. The truth, as Becky has written, should cause cognitive dissonance and spur us into action. Although progress has been made in improving the health of our environment in the last ten years, much work remains to be done. According to the World Health Organization (WHO) report, Preventing Disease through Healthy Environments: Towards an Estimate of the Environmental Burden of Disease, approximately one -fourth of the global burden of disease is attributable to environmental exposures and 23% of all deaths can be attributed to environmental factors.

REACTION: This topics introductory articles cover a broad range of issues in environmental health .nursing designed to inform and engage nurses on the subject. A predominate theme in all the articles is that the environment is a primary determinant of health, and environmental health hazards affect all aspects of life and all areas of nursing practice. This series of articles begins by highlighting the responsibility of nurses and the health care industry to first clean up our own house by demonstrating environmentally responsible practices, products, and policies in our hospitals and other health care settings. Three articles deal with environmental health in health care settings from the design, building, and operations of hospitals to the procurement, use, and disposal of the products used for caring. It is essential that nurses be aware of these and other common environmental health hazards so that they can include this teaching into nursing care for their patient populations, particularly those with baseline vulnerabilities, such as infants and children, pregnant mothers, the elderly, and people with disabilities. It is also necessary for nurses to have access to evidence-based information, tools, and resources so that they can incorporate environmental health knowledge into everyday practice and plan interventions for their patients and communities that protect health.