Term
| What is Patent Ductus Arteriosus (PDA)? |
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Definition
| When the fetal ductus between the descending thoracic aorta and the pulmonary arteries doesn't close. 5-10% of all infants with congenital heart disease. |
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Term
| How do you diagnose Patent Ductus Arteriosus (PDA)? |
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Definition
| A murmur is present, ECG and chest radiograph show ventricular hypertrophy. The patent ductus arteriosus can be visulized and the blood flow can be measured on an echocardiogram. |
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Term
| Why does Patent Ductus Arteriosus cause left ventricular hypertrophy and difficulty breathing? |
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Definition
| As oxygenated blood moves back into the lungs there is an overload of blood into the lungs and then into the left side of the heart. This reduces the compliance of the lungs and puts more pressure on the left ventricle to get blood out. |
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Term
| What is Atrial Septal Defect (ASD)? |
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Definition
| It's when the foramen ovale fails to close at birth. It may also occur when the atrial septum doesn't form fully or at all. Left to right shunting of blood overloads the right ventricle. |
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Term
| What medication treatment can be used to treat Patent Ductus Arteriosus (PDA)? |
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Definition
| Indomethacin or an NSAID may trigger the closure of the Patent Ductus Arteriosus. |
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Term
| What diagnostic tests can identify an atrial septal defect (ASD)? |
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Definition
| An echocardiogram can identify a dilated right ventricle due to the increased blood. It will also see the shunt size. ECG and radiograph does not reveal much unless the ASD is large. |
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Term
| What should you be alert for with unclosed Atrial Septal Defects (ASDs)? |
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Definition
| Sroke from clots that form in the septal opening. Also CHF |
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Term
| When is surgery recommended for correcting an Atrial Septal Defect (ASD)? |
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Definition
2 years if there is significant increase in pulmonary blood flow, causing CHF.
4 years if it has not closed on its own. |
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Term
| What is a Ventricular Septal Defect (VSD)? |
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Definition
| When there is an opening in the ventricular septum allowing more/oxygenated blood to enter the pulmonary artery. |
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Term
| What do you need to watch for in a Ventricular Septal Defect (VSD)? |
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Definition
| CHF, pulmonary infections and pulmonary hypertension |
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Term
| What tests can check for Ventricular Septal Defects? |
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Definition
| Echocardiograms show if shunting is present. Chest radiograph and ECG show right and left hypertrophy. |
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Term
| When should surgery be considered an option for Ventricular Septal Defects? |
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Definition
| If no CHF or pulmonary artery hypertension present, see if it closes in 6 months. Pulmonary artery banding can be used to reduce flow of blood into lungs till surgery. VSD patching at 3-12 months. |
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Term
| What is an Atrioventricular Canal (endocardial cushion) defect? |
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Definition
| One large atrioventricular valve is present and a large septal defect between both atria and ventricles. This causes left to right shunting of blood across the septum. |
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Term
| What diagnostic tests will show if an atrioventricular canal defect is present? |
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Definition
A chest radiograph - Shows cardiomegaly and pulmonary vascular markings
ECG - Shows atrial enlargement, right ventricular hypertorophy, incomplete right bundle branch block
Echocardiogram - Dilation of ventricles, septal defects and valve malformation details.
Cardiac catheterization: Increased O2 in right atrium, increased R ventricle and/or pulmonary artery pressure. |
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Term
| When is surgery an option for an Atrioventricular Canal defect? |
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Definition
| By 3 months of age to prevent pulmonary vascular diseas. Patches placed over defects and valve tissue used to form functioning valves. |
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Term
| What condition is linked with 70% of Atrioventricular canal defect? |
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Definition
| Trisomy 21, down syndrome. |
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Term
| What are the main concerns with defects that cause decreased pulmonary blood flow? |
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Definition
Right to left blood shunting which raises blood pressure on the right side of the heart, greater than that of the left.
Hypoxemia and cyanosis, clubbing of fingers. |
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Term
| Why does Polycythemia occur in decreased pulmonary blood flow conditions? |
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Definition
| Bone marrow stimulated to produce more red blood cells to carry oxygen. |
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Term
| What signs and symptoms do you expect to see with infants who have decreased pulmonary blood flow? |
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Definition
| Dyspnea, loud murmur, cyanosis or mottled skin shortly after birth |
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Term
| What is pulmonic stenosis? |
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Definition
| A narrowing of the pulmonic valve causing reduced blood flow into the pulmonary arteries. This decreases pressure into the left side of the heart. |
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Term
| What diagnostic tests help identify pulmonic stenosis? |
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Definition
Chest Radiograph - show an enlarged pulmonary artery with normal heart size and normal vascularity.
ECG - May show right atrial enlargement, right ventricular hypertrophy.
Echocardiogram - Shows the pressure gradient across the valve and size of the valve ring
Cardiac catheterization - Finds increased right ventricular pressure and normal or lower pulmonary artery pressure. |
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Term
| What kind of treatments do you expect for pulmonic stenosis? |
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Definition
1) None if it is not severe. Monitor and treat prophylactically.
2) Balloon dilation of the valve
3) Valvotemy may occur with other defect corrections |
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Term
| What is the Tetralogy of Fallot (TOF)? |
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Definition
4 defects in the heart:
Pulmonic stenosis, right ventricular hypertrophy, ventricular septal defect, overriding of aorta. (occasionally an atrial septal defect.) |
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Term
| How do you recognize tetralogy of Fallot in infants? |
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Definition
| When the ductus arteriosus closes, infant is hypoxic and cyanotic. Murmer and thrill may show. Polycythemia, hypercaynotic spells, metabolic acidosis, clubbing. |
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Term
| When should surgeries take place? |
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Definition
| 4-6 months unless a hypercyanotic spell occurs. Palliative may be done first to delay corrective surgery. |
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Term
| What can you expect after tetralogy of Fallot surgery? |
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Definition
| Not always a cure, but improved quality of life. Ventricular arrhythmias can cause sudden death later, right bundle branch rhythm pattern may result. |
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Term
| What is Pulmonary or Tricuspid atresia? |
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Definition
| A severe form of pulmonary stenosis. No valve opens to allow blood between the right atrium and right ventricle. Pulmonary atresia affects blood to pulmonary arteries, Tricuspid halts blood between the right atrium and ventricle. |
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Term
| What diagnostic tests can be used to identify pulmonary or tricuspid atresia? |
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Definition
Chest radiograph may show a slightly enlarged heart.
ECG may show right atrial hypertrophy
Echocardiogram shows a small hypoplastic right ventricular cavity and tricuspid valve, absent right ventricular outflow tract, dilated right atrium and right to left shunting across the atrial septum. |
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Term
| What medications are used to treat pulmonary or tricuspid atresia? |
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Definition
Digoxin and diuretics
Prostaglandin E1 to maintain a patent ductus arteriosus (normally should be closed)
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Term
| When presented with a child who has severe hypoxemia with bradycardia, what should you do to reduce risk from both? |
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Definition
| Treat hypoxemia. This will reverse bradycardia and prevent cardiac arrest. |
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Term
| For transposition of the great arteries, truncus arteriosus and total anomalous pulmonary venous return, what is the usual surgical response? |
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Definition
| To perform a palliative procedure first to allow the child to survive until they grow, increasing chances of survival. |
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Term
| What is transposition of the great arteries? |
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Definition
| The aorta comes out of the right ventricle, the pulmonary artery is the outlet for the left ventricle. Life threatening at birth. |
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Term
| What situation allows for some survival at birth with transposition of the great arteries? |
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Definition
| An open ductus arteriosus and foramen ovale. |
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Term
| When is corrective surgery for transposition of the great arteries attempted? |
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Definition
| Before one week of age (arterial switch). Prostaglandin E1 is used to maintain a patent ductus arteriosus, and balloon atrial septostomy is performed to permit oxygenated and unoxygenated blood to mix until the surgyer. |
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Term
| What diagnostic tests will show transposition of the great arteries? |
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Definition
Chest radiograph may show a classic egg-shaped heart on a string.
ECG shows right ventricular hypertrophy
Echocardiogram shows abnormal position of the great arteries
Blood tests show hematocrit and hemoglobin, or polycythemia. |
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Term
| What is truncus Arteriosus? |
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Definition
| A single vessel (Truncus Arteriosus) empties mixed blood to both the aorta and pulmonary arteries. |
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Term
| What physiological conditions occur with truncus Arteriosus? |
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Definition
| With increased pulmonary blood flow (too much blood pumped to the heart on each beat), CHF, dyspnea, poor feeding occur. |
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Term
| What medications are given with surgical treatment for Truncus Arteriosus? |
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Definition
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Term
| What restrictions are there for a child who receives the Ratelli procedure to fix Truncus Arteriosus? |
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Definition
| Should not participate in competitive sports. Ventricular arrhythmias may develop. |
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Term
| What is total anomalous pulmonary venous return? |
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Definition
| THe pulmonary veins empty into the right atrium, or veins leading to the right atrium. Mixed blood passes through the foramen ovale or an atrial septal defect to provide circulation. |
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Term
| What diagnostic tests show a total anomalous pulmonary venous return? |
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Definition
Chest radiograph shows cardiac enlargement, large pulmonary artery and increased pulmonary blood flow.
ECG shows hypertrophy of right atrium and ventricle
Echocardiogram shows dilated right atrium and ventricle, smaller left sided chambers, dilated pulmonary arteries, patent foramen ovale. |
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Term
| What medication is used along with surgery is used for total anomalous pulmonary venous return? |
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Definition
Prostaglandin E1 given to maintain patent ductus arteriosus.
Digoxin and diuretics treat CHF. |
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Term
| What are side effects of giving Prostaglandin E1 to reopen the ductus arteriosus? |
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Definition
| Respiratory depression and apnea. |
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Term
| What medications are used to treat Hypercyanotic episodes? |
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Definition
Morphine
Fluids to increase central venous pressure
Beta blocker (Reduce heart rate)
Dopamine or Phenylephrine (increase systemic vascular resistance)
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Term
| What are CHF symptoms in an infant? |
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Definition
| Tachycardia, tachypnea, crackles, frothy secretions, low urine output, pulmonary edema. |
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Term
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Definition
| A narrowing of the aortic valve, obstrucing blood flow to systemic circulation. A bicuspid valve instead of a tricuspid. |
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Term
| What diagnostic test will show aortic stenosis? |
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Definition
An echocardiogram shows the number of valve leaflets, pressure gradient across the valve and the size of the aorta.
ECG may show mild left ventricular hypertrophy and inverted T waves. |
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Term
| What can you expect from therapy with aortic stenosis? |
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Definition
PGE1 may be given to maintain a patent ductus arteriosus until dilation can occur.
Baloon dilation or valvotomy may take place.
Valve replacement may occur if stenosis is severe enough. |
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Term
| What kind of treatments can you expect after treatment of aortic stenosis? |
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Definition
Stenosis may re-occur
Calcification ma begin
Valve replacement and anticoagulant therapy during adulthood. |
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Term
| What is coarctation of the Aorta? |
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Definition
| Narrowing of the descending aorta, near the ductus arteriosus or left subclavian artery. |
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Term
| How does coarctation of the aorta affect systemic blood flow? |
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Definition
| Increased pressure in the upper extremities, poor perfusion and pulse in lower extremities. |
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Term
| What kind of tests can be used to show coarctation of the aorta? |
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Definition
Chest radiograph may show cardiomegaly, pulmonary venous congestion
CT and MRI show the aortic arch and site of coarctation
ECG shows left ventricular hypertrophy and right hypertrophy.
Echocardiogram shows the size of the aorta and coarctation. |
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Term
| What medications and treatments are given for coarctation of the aorta? |
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Definition
PGE1 to reopen the ductus arteriosus and promote blood flow.
CHF rated with diuretics and oxygen
Surgical resection
Some balloon angioplasty and stent placement |
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Term
| How often do people with treatments with coarctation of the aorta need follow up treatments? |
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Definition
| Lifelong, the treatments are palliative, the coarctation may reoccur. |
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Term
| What is hypoplastic left heart syndrome? (HLHS) |
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Definition
| THe mitral and aortic valves are absent or stenosed. Small left ventricle and small aorta. |
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Term
| What diagnostic procedures help identify hypoplastic left heart syndrome? |
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Definition
Chest radiograph shows cardiomegaly and increased pulmonary vascularity.
Echocardiogram shows a small left ventricle. This may be diagnosed prenatally. |
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Term
| What treatments can you expect for hypoplastic left heart syndrome? |
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Definition
Prostaglandin E1 to maintain a patent ductus arteriosus.
No supplemental O2
Surgery has 3 stages:
Norwood at week 1, aorta reconstructed and the right ventricle is committed to pumping blood through the pulmonary valve to the aorta.
Stage 2 (3-6 months) connects the superior vena cava to the pulmonary artery
Stage 3 at 2-3 years, connects the inferior vena cava to the pulmonary circulation |
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