36 Questions
What percentage of congenital heart diseases are caused by chromosomal genetic defects?
10%-12%
What type of lesion is characterized by a blockage or narrowing of a blood vessel?
Obstructive lesion
What is the name of the defect in which there is a hole in the wall separating the two upper chambers of the heart?
Atrial septal defect
What is the term for a heart defect in which the pulmonary artery and aorta are reversed?
Transposition of great arteries
What is the term for a heart defect in which there is a hole in the wall separating the two lower chambers of the heart?
Ventricular septal defect
What is the primary purpose of an echocardiogram in the diagnosis of a congenital heart defect?
To visualize the heart and its blood vessels
What is the benefit of early use of indomethacin to close a PDA soon after birth?
It decreases the risk for pulmonary hemorrhage and IVH
Where is the incision made during PDA surgery?
On the side of the child's chest between the ribs
What is the normal course of development for the hole between the two atria in infants?
It closes within the first few weeks of life
What is the result of oxygen-rich blood flowing through the hole in the septum into the right ventricle?
The oxygen-rich blood mixes with the oxygen-poor blood and goes back to the lungs
What is the term for the extra noise created by blood flowing through the hole in the septum?
Heart murmur
What is the treatment for a child with a small VSD that causes no symptoms?
Regular visits to a cardiologist to monitor for any problems
What is a common lung problem associated with Tetralogy of Fallot?
Pneumonia
What is the purpose of a prostaglandin infusion in Tetralogy of Fallot treatment?
To dilate the ductus arteriosus and increase pulmonary blood flow
What is the term for the sudden drop in arterial blood gases in Tetralogy of Fallot?
Tetralogy spell
What is the purpose of a synthetic Dacron patch in Tetralogy of Fallot surgery?
To close the ventricular septal defect
What is the term for the narrowing of the pulmonary valve and outflow tract in Tetralogy of Fallot?
Pulmonary stenosis
What is the result of right ventricular hypertrophy in Tetralogy of Fallot?
Thickening of the right ventricular wall
What is the term for the loud murmur or blue lips and skin in Tetralogy of Fallot?
Cyanosis
What is the primary goal of corrective repair of Tetralogy of Fallot?
To increase pulmonary blood flow
What is a common symptom of mitral valve prolapse?
Difficulty breathing or shortness of breath
What is a risk factor for mitral valve prolapse?
Marfan syndrome
What is the purpose of beta blockers in managing mitral valve prolapse?
To lower blood pressure
What is the name of the diagnostic test that uses X-rays to produce images of the chest?
Chest X-ray
What is the purpose of diuretics in managing mitral valve prolapse?
To reduce sodium reabsorption
What is the name of the procedure that involves repairing or replacing the mitral valve?
Valve repair and replacement
What is myocarditis?
An inflammation of the heart muscle
What is the purpose of aspirin in managing mitral valve prolapse?
Antithrombotic therapy
What is the primary site of attachment for the organisms involved in GABHS infection?
Epithelial cells of the upper respiratory tract
What is the typical duration of an incubation period in GABHS infection?
2-4 days
What is the term for the inflammation of the pericardium, myocardium, or endocardium?
Carditis
What is the characteristic of subcutaneous nodules?
They are non-tender and firm to touch
What is the term for a neurological disorder characterized by spasmodic involuntary movements of the limbs or facial muscles?
Chorea
What is the characteristic of erythema marginatum?
It is a temporary skin lesion that comes and goes
What is the term for joint pain affecting five or more joints?
Polyarthritis
What is the effect of molecular mimicry in GABHS infection?
It causes an immune response against human proteins
Study Notes
Fetal Circulation
- Before birth, the fetus receives oxygenated blood from the mother's placenta
- The oxygenated blood flows through the umbilical vein to the heart, where it bypasses the lungs and goes directly to the body
Fetal Circulation Right After Birth
- After birth, the infant's lungs expand and begin to breathe in oxygen
- The bypasses that were present in the fetal circulation now close, and the lungs take over the oxygenation of blood
Causes of Congenital Heart Diseases
- Chromosomal/genetic: 10%-12% (e.g., Down syndrome)
- Maternal or environmental: 1%-2% (e.g., maternal alcohol consumption, maternal illness)
- Infections: CMV, toxoplasmosis, other viral illnesses
- Infants of Diabetic Mothers (IDMs): 10% risk of CHD (e.g., VSD, cardiomyopathy, TGA)
- Multifactorial: 85%
Etiology of Congenital Heart Disease
- Lesions: obstructive, acyanotic, and cyanotic
- Obstructive lesions: Coarctation of aorta, pulmonary stenosis
- Acyanotic lesions: Ventricular septal defect, atrial septal defect, patent ductus arteriosus
- Cyanotic lesions: Tetralogy of Fallot, transposition of great arteries, truncus arteriosus, total anomalous pulmonary venous return
Increased Pulmonary Blood Flow Defects
- Abnormal connection between two sides of the heart
- Increased blood volume on the right side of the heart
- Increased pulmonary blood flow: atrial septal defect, ventricular septal defect
- Diagnostic tests: echocardiogram, chest X-ray, electrocardiogram, cardiac catheterization
Management of Congenital Heart Diseases
- Watchful waiting
- Medications: indomethacin to close PDA soon after birth
- Surgical closure: PDA surgery is done on the blood vessel, not the heart
Ventricular Septal Defect (VSD)
- A hole between the two ventricles
- Oxygen-rich blood from the left ventricle flows into the right ventricle
- Creates a heart murmur
- Treatment: depends on the size, location, and severity of the VSD
Tetralogy of Fallot
- Four defects: VSD, overriding aorta, pulmonary stenosis, right ventricular hypertrophy
- Signs and symptoms: loud murmur, cyanosis, rapid breathing, sudden drop in arterial blood gases
- Treatment: prostaglandin infusion, surgery (corrective repair of tetralogy of Fallot)
Diagnostic Tests
- Chest X-ray
- Electrocardiogram
- Stress test
- Coronary angiogram
Management of Heart Disease
- Medications: beta blockers, diuretics, heart rhythm medication, aspirin
- Surgery: valve repair and replacement, minimally invasive surgery
Myocarditis
- Inflammation of the heart muscle
- Causes: GABHS infection, molecular mimicry, streptococcal antigens, decrease in regulatory T-cells
- Signs and symptoms: rapid or abnormal heart rhythms, fever, malaise, headache, elevated leukocyte count
Rheumatic Fever
- Causes: GABHS infection, molecular mimicry
- Signs and symptoms: carditis, polyarthritis, chorea, subcutaneous nodules, erythema marginatum
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