Tetralogy of Fallot: Difference between revisions
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== Medical Management (current best evidence) == | == Medical Management (current best evidence) == | ||
Surgery is the only corrective measures for children born with TOF. There are two different surgical routes the infant may undergo: early/temporary correction, or complete correction. First though, the neonate may be injected with prostaglandin in order to preserve ductal patency and provide a stable blood source to the lungs. Early or temporary correction is done to increase blood flow to the lungs for a period of time until the child is big enough and ready for complete corrective surgery. In this surgery, surgeons place a shunt between the systemic and pulmonary artery in order for the subclavian and pulmonary artery on the same side to communicate. <br>In a complete repair, surgeons close the hole between the ventricles with a patch, re-channel the aorta to connect with the left ventricle instead of the right, and widen the narrowed pulmonary tract. This surgery is usually performed somewhere between 4-6 months, but is beginning to be introduced in as young as 3 months. A complete repair provides relief of the volume and pressure overload on the right ventricle, minimizing cyanosis (and thus reducing secondary damage to organs), decreasing right ventricular hypertrophy, and reducing the incidence of arrhythmias. Patients who have a successful surgery usually do not need to have another heart surgery in their first year. Longer follow-up is usually necessary with children undergoing a complete correction. There is however, a risk of surgery using the patch between ventricles at such a young age. The patch can create a state of chronic pulmonary regurgitation, which increases morbidity in young adults. This can lead to exercise intolerance and ventricular arrhythmias. The mortality rate of this surgery is less than 3%, and more children are surviving into adulthood than ever before, living normal, healthy lives. <br><br> | Surgery is the only corrective measures for children born with TOF. There are two different surgical routes the infant may undergo: early/temporary correction, or complete correction. First though, the neonate may be injected with prostaglandin in order to preserve ductal patency and provide a stable blood source to the lungs. Early or temporary correction is done to increase blood flow to the lungs for a period of time until the child is big enough and ready for complete corrective surgery. In this surgery, surgeons place a shunt between the systemic and pulmonary artery in order for the subclavian and pulmonary artery on the same side to communicate. | ||
<br>In a complete repair, surgeons close the hole between the ventricles with a patch, re-channel the aorta to connect with the left ventricle instead of the right, and widen the narrowed pulmonary tract. This surgery is usually performed somewhere between 4-6 months, but is beginning to be introduced in as young as 3 months. A complete repair provides relief of the volume and pressure overload on the right ventricle, minimizing cyanosis (and thus reducing secondary damage to organs), decreasing right ventricular hypertrophy, and reducing the incidence of arrhythmias. Patients who have a successful surgery usually do not need to have another heart surgery in their first year. Longer follow-up is usually necessary with children undergoing a complete correction. There is however, a risk of surgery using the patch between ventricles at such a young age. The patch can create a state of chronic pulmonary regurgitation, which increases morbidity in young adults. This can lead to exercise intolerance and ventricular arrhythmias. The mortality rate of this surgery is less than 3%, and more children are surviving into adulthood than ever before, living normal, healthy lives. <br><br> | |||
== Physical Therapy Management (current best evidence) == | == Physical Therapy Management (current best evidence) == | ||
Revision as of 00:17, 3 April 2012
Original Editors - Megan Clayton & Jaclyn Muenchow from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Lead Editors - Your name will be added here if you are a lead editor on this page. Read more.
Definition/Description[edit | edit source]
Tetralogy of Fallot is a congenital heart defect which presents with 4 conditions: a ventricular septal defect (a hole between the right and left ventricles), a narrowing of the pulmonary outflow tract (the vessel between the heart and lungs), an overriding aorta (the aorta is shifted to the right instead of being connected strictly to the left side), and hypertrophy of the right ventricle. Each of these components can vary in severity. These defects cause there to be lower oxygen levels in the blood, leading to cyanosis. It is the most common cause of cyanotic cardiac disease in neonates.
http://www.youtube.com/watch?v=yePivAlbR4A.
Prevalence[edit | edit source]
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Characteristics/Clinical Presentation[edit | edit source]
Most babies present with mild to moderate cyanosis, but do not typically present with signs of respiratory distress or heart failure. Symptoms of TOF include:
• cyanosis, which gets worse when the baby is upset
• clubbing of fingers
• fainting
• heart murmurs
• poor appetite and/or slow growth
• tires easily
• has trouble breathing when eating or crying
• squatting episodes during cyanosis
Patients with TOF may experience a hypercanotic spell. They are not common but they can occur in as young as 2 months of age. These spells are characterized by a sudden decrease in oxygen due to acute, almost complete obstruction of subpulmonary outflow. These spells are a medical emergency and can result in death if not treated.
If the child is having a hypercyanotic spell, put his/her knees to chest in order to try to increase venous return to the right side of the heart. Call 911 immediately.
Associated Co-morbidities[edit | edit source]
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Medications[edit | edit source]
Surgery is the first line of defense in children with TOF. Afterwards, they may or may not be on various medications to help control arrhythmias (if they have one) or to help their heart beat more forcefully. Children after TOF surgery may be required to take antibiotics when going to the dentist to get their teeth cleaned, or if they are around someone that has an infection as an act of prophylactic care.
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
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Etiology/Causes[edit | edit source]
The cause of TOF is unknown, but there are some risk factors:
• chromosomal abnormalities
• gestational diabetes
• untreated phenylketonuria
• excessive alcohol consumption while pregnant
• malnutrition while pregnant
• mother over 40 years old
• rubella or another virus during pregnancy
Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
Surgery is the only corrective measures for children born with TOF. There are two different surgical routes the infant may undergo: early/temporary correction, or complete correction. First though, the neonate may be injected with prostaglandin in order to preserve ductal patency and provide a stable blood source to the lungs. Early or temporary correction is done to increase blood flow to the lungs for a period of time until the child is big enough and ready for complete corrective surgery. In this surgery, surgeons place a shunt between the systemic and pulmonary artery in order for the subclavian and pulmonary artery on the same side to communicate.
In a complete repair, surgeons close the hole between the ventricles with a patch, re-channel the aorta to connect with the left ventricle instead of the right, and widen the narrowed pulmonary tract. This surgery is usually performed somewhere between 4-6 months, but is beginning to be introduced in as young as 3 months. A complete repair provides relief of the volume and pressure overload on the right ventricle, minimizing cyanosis (and thus reducing secondary damage to organs), decreasing right ventricular hypertrophy, and reducing the incidence of arrhythmias. Patients who have a successful surgery usually do not need to have another heart surgery in their first year. Longer follow-up is usually necessary with children undergoing a complete correction. There is however, a risk of surgery using the patch between ventricles at such a young age. The patch can create a state of chronic pulmonary regurgitation, which increases morbidity in young adults. This can lead to exercise intolerance and ventricular arrhythmias. The mortality rate of this surgery is less than 3%, and more children are surviving into adulthood than ever before, living normal, healthy lives.
Physical Therapy Management (current best evidence)[edit | edit source]
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Alternative/Holistic Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
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Case Reports/ Case Studies[edit | edit source]
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Resources
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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