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CARDIOVASCULAR
Ten times more common in men. Other risk factors are hypertension, atherosclerosis, diabetes,
hyperlipidemia, smoking, syphilis, Marfan's disease, and Ehlers-Danlos disease.
Urgent conversion requires DC cardioversion. A patient may be started on digoxin (slows the ventricular
response by prolonging conduction time through the AV node) followed by an agent such as quinidine or
procainamide (to suppress the ar r hythmia).
This is most often the result of a chronically stretched atrial myocardium. Atrial fibrillation occurs most
commonly in older children with rheumatic mitral valve disease.
Re-entrant tachycardias using an accessory pathway in infants, though AV node re-entry becomes more
common in childhood.
q q What is the treatment for a stable SVT that is not caused by digitalis toxicity or WPW
syndrome?
Vagal stimulation (submersion of face in iced saline or ice bag to the face); vagotonic maneurens (valsalva,
straining, breath-holding); rapid intravenous push of adenosine; quinidine, procainamide or propranolol; or
verapamil.
A progressive prolongation of the PR interval until the atrial impulse is no longer conducted. If
symptomatic, atropine and transcutaneous/transvenous pacing is required.
A constant PR interval in which one or more beats fail to conduct. Treat with atropine and
transcutaneous/transvenous pacing.
q q A patient presents to the hospital 1 month after placement of a mechanical prosthetic valve
with fever, chills, and a leukocytosis. Endocarditis is suspected. Which type of bacterium is most
common?
q q Documentation of three BP results in a fully grown adolescent obtained at one week intervals
must show an average greater than 140 mm Hg systolic or 90 mm Hg diastolic for the correct
diagnosis of hypertension. What single reading can be considered sufficient for the diagnosis of
hypertension without necessitating further investigations?
When a patient’s single reading is greater than 110 mm Hg diastolic, a diagnosis can be made. Another
conclusive indicator of hypertension is when end organ damage occurs with a lower reading.
q q A patient presents with a history of episodic elevations in BP. She complains of headache,
diarrhea, and skin flushing. What is the diagnosis?
Pheochromocytoma.
Sodium nitroprusside (not drug of choice for eclampsia). Sodium nitroprusside assists in relaxing smooth
muscle through the production of cGMP. As a result, there is decreased preload and afterload, decreased
oxygen demand, and a slightly increased heart rate with no change in myocardial blood flow, cardiac
output, or renal blood flow. The duration of action is 1–2 minutes. Sometimes, a ß-blockade is required to
treat rebound tachycardia.
Hypotension. Thiocyanate toxicity accompanied by blur r ed vision, tinnitus, change in mental status,
muscle weakness, and seizures is more prevalent in patients with renal failure and after prolonged
infusions. Cyanide toxicity is uncommon. However, this type of toxicity may occur with hepatic
dysfunction, after prolonged infusions, and in rates greater than 10 mg/kg per minute.
An elevated diastolic blood pressure that is > 115 mm Hg with associated end organ dysfunction or
damage.
The BP should be lowered gradually over 2-3 hours to a systolic of 140-160 mm Hg and a diastolic of 90-
110 mm Hg diastolic. To prevent cerebral hypoperfusion, the BP should not be decreased more than 25%
of the mean arterial pressure.
A diastolic blood pressure that is < 115 mm Hg with no symptoms of end organ damage. Uncomplicated
hypertension does not require acute treatment.
Nausea, vomiting, headache, lethargy, coma, blindness, nerve palsies, hemiparesis, aphasia, retinal
hemor r hage, cotton wool spots, exudates, sausage linking, and papilledema.
Viruses (especially adenovirus and Coxsackie virus group B). Other causes include bacteria (diphtheria,
meningococcus and tuberculosis), fungi, protozoa (Chagas' disease), spirochetes (Lyme disease), and
Kawasaki disease.
Females have a stronger genetic link to the disease. 2-5% of the population has MVP.
A midsystolic click sometimes accompanied by a late systolic murmur. MVP is largely a clinical
diagnosis. An ECG is performed to assess the degree of prolapse. Other clinical findings include a
laterally displaced diffuse apical pulse, decreased S1, split S2, and a holosystolic murmur radiating to the
axilla.
Pericardial friction rub. The rub is best heard at the left sternal border or apex with the patient in a forward
sitting position. Other findings include fever and tachycardia.
q q When diagnosing pericardial effusion, how much fluid has to be present in the pericardial sac
for visualization by an echocardiography and by an x-ray?
80-200 mL. However, the rate of accumulation is more important than the amount of accumulation. If
accumulated slowly the pericardium can tolerate up to 2000 mL of fluid.
A 2 week treatment of aspirin q 4 hours is recommended if no contradictions exist and if effusion is not
present. Ibuprofen, indomethacin, or colchicine are other alternatives. The use of corticosteroids is
controversial, as recur r ent pericarditis is common when doses are tapered.
q q What are the most common signs and symptoms of a pulmonary emoblus?
Tachypnea (92%), CP (88%), dyspnea (84%), anxiety (59%), tachycardia (44%), fever (43%), DVT (32%),
hypotension (25%), and syncope (13%).
PEDIATRIC PEARLS OF WISDOM, 2e 59
Elevated dome of one hemidiaphragm. This finding is caused by decreased lung volume, which occurs in
50% of patients with PEs. Other common findings include lack of lung markings in the area perfused by
the occluded artery, pleural effusions, atelectasis, and pulmonary infiltrates.
q q Which test is considered the gold standard for a diagnosis of deep vein thrombosis (DVT)? Of
pulmonary embolies (PE)?
The gold standard for DVT is venography. The gold standard for the diagnosis of PE is the pulmonary
angiography.
q q Rheumatic heart disease is the most common cause of stenosis of what 3 heart valves?
An S4.
q q When measuring a pulsus paradoxus, what is considered a normal difference in systolic blood
pressures (with inspiration versus expiration)?
Up to 8-10 mmHg.
q q Which congenital heart lesions commonly present with cyanosis in an infant’s first day of life?
All the abnormalities that start with a T. Tetralogy of Fallot, Transposition of the great vessels, Total
anomalous pulmonary circulation, Truncus arteriosus, and Tricuspid valve atresia (and Ebstein’s
malformation of the Tricuspid valve).
A systolic murmur in the first days of life with no signs related to their condition. They usually resolve
spontaneously.
The majority of these children are totally asymptomatic, and the defect usually is not diagnosed until they
are school age.
q q A short PR interval and a delta wave are characteristic of what cardiac anomaly?
Wolff-Parkinson-White (WPW) syndrome. This is due to a reentry phenomenon along the Bundle of Kent.
q q Which patients with mitral valve prolapse (MVP) should receive prophylactic antibiotics to
protect against subacute bacterial endocarditis?
q q How does the size of a VSD correlate with the risk of infective endocarditis?
q q T/F: You can rule out subacute bacterial endocarditis (SBE) after you have obtained two
negative blood cultures?
False. False negatives occur 10-30% of the time in two cultures. For this reason, three blood cultures are
recommended.
Not very common. These manifestations are seen late in the disease, so they are not commonly seen in the
appropriately treated patient.
q q A 16-year-old boy comes to your office for a pre-participation sports physical. What three
findings during the physical exam can help you identify those patients at risk for sudden cardiac
arrest?
q q What test can be used to identify vasovagal neuroregulatory syncope as the cause of syncope in
children with a history of "falling out"?
q q What test can be used to differentiate congenital heart disease from primary pulmonary
parenchymal disease in a newborn with cyanosis?
The hyperoxia test. There is little to no difference in arterial oxygen content between breathing room air
and 100% oxygen with cyanotic congenital heart disease
q q On a chest x-ray of a child, what bony abnormality would lead you to suspect coarctation of the
aorta?
q q What bony abnormalities are seen in the chest x-rays of children with Down syndrome?
Aortic and mitral insufficiency, mitral valve prolapse (MVP), and dissecting aortic aneurysm.
q q What congenital cardiac diseases will present with increased pulmonary markings on a chest x-
ray?
Transposition of the great vessels, total anomalous pulmonary venous return (and truncus arteriosus).
PEDIATRIC PEARLS OF WISDOM, 2e 61
q q An "egg shaped" heart is a characteristic x-ray finding for what congenital heart
malformation?
ASD, tetralogy of Fallot (TOF), pulmonary stenosis, right bundle branch block (RBBB), total anomalous
pulmonary venous return.
A loud first heart sound, sometimes a pulmonic ejection click, a second heart sound that is widely split and
fixed in all phases of respiration, and a systolic ejection murmur (best heard at the left middle and upper
sternal border). A short, rumbling mid-diastolic murmur may be heard at the left lower sternal border.
Medium-pitched, "musical" or vibratory systolic ejection murmur best heard at LLSB without radiation.
Very. Up to 30% of children have one, with peak incidence between 3-7 years of age.
V3R or V4R. These are right precordial leads which evaluate the extent of right ventricular hypertrophy.
q q What is the test of choice to examine for the presence of vegetations in a child with
endocarditis?
Transesophageal echocardiography.
q q An ECG shows the following findings: RSR' in V3R and V1, prolonged P-R interval, tall P
waves, a QRS axis of -60 degrees and signs of RVH. What is your diagnosis, doc?
VSD. This accounts for about 25% of all heart diseases. Prognosis depends largely on size of the defect- if
small it may close spontaneously (usually before 4 years of age).
q q A 10-year-old child with a history of VSD is about to have an impacted tooth removed. What
antibiotic should he be given prophylactically to prevent endocarditis?
Amoxicillin 50 mg/kg one hour before the procedure and 25 mg/kg six hours after the first dose. If the
patient has a penicillin allergy, use clindamycin 20 mg/kg one hour before the procedure and 10 mg/kg six
hours after the first dose.
q q What compound causes the ductus arteriosus to constrict right after birth?
During the first 24-48 hours of life, with a second and third dose at 12 and 36 hours, following the initial
dose.
q q What percentage of patent ductus arteriosi that were initially closed with indomethacin
reopen?
25%. It is more likely to reopen if therapy is started beyond the first week of life.
q q What is the treatment of choice for a child with severe aortic stenosis?
Balloon valvuloplasty if peak systolic gradient between the LV and aorta is > 60 mmHg.
q q How does squatting help children with hypoxia secondary to their tetralogy of Fallot (TOF)?
q q You are on call in the NICU when a newborn with severe coarctation of the aorta suddenly
starts deteriorating and is acidotic. What do you do initially?
q q A 3-year-old child present with premature atrial contractions (PAC's). Should this be a cause
of concern?
They are usually benign, except in children less than 1-year-old, or those on digitalis.
Pulmonary stenosis, VSD, dextroposition of aorta with septal over r ide and RVH.
q q What is the shunting procedure currently employed to correct the cyanosis in TOF?
PEDIATRIC PEARLS OF WISDOM, 2e 63
The modified Blalock-Taussig shunt. This aortopulmonary shunt is a Gore-Tex conduit which
anastomoses the subclavian and pulmonary arteries (homolateral branch).
q q What therapy can be instituted to decrease the incidence of coronary abnormalities in children
with Kawasaki syndrome?
IV immune globulin.
q q What medication has been shown to decrease the fever and discomfort associated with an acute
attack of Kawasaki syndrome?
Children with Kawasaki syndrome tend to have an increased WBC count, elevated ESR, elevated liver
function and pyuria.
q q What congenital cardiac defects are commonly seen in children with Down syndrome?
q q What congenital cardiac defects are commonly seen in infants with fetal alcohol syndrome?
ASD, VSD.
q q What congenital cardiac defects are commonly seen in patient with autosomal dominant
polycystic kidney disease?
q q What is the procedure of choice for treatment of transposition of the great arteries?
q q What is the 5 year survival rate of infants and children who undergo a complete heart
transplantation?
q q What is the primary cause of congestive heart failure (CHF) in infancy and childhood?
q q A patient with congenital heart disease taking digoxin and Lasix, presents in CHF; what are
the potential causes?
Circulatory compromise.
Positional sharp substernal chest pain, abdominal pain, tachycardia, tachypnea, dyspnea, fever, friction rub.
35%
Dysrhythmias. Treat ventricular ar r hythmias with lidocaine, supraventricular tachyar r hythmias with
digoxin, and complete atrioventricular block with a temporary pacemaker.
Treat the CHF, restrict fluids, diuresis, oxygen, strict bed rest, and inotropic support if needed.
q q What are the ECG findings in a patient with a significant pericardial effusion?
1. Low voltage
2. Electrical alternans
1. Ir r itability 6. Tachypnea
2. Poor feeding 7. Hyperactive precordium
3. Lethargy 8. Gallop rhythm
4. Failure to thrive 9. Rales
5. Tachycardia 10.Hepatomegaly
Peripheral edema and neck vein distention are noted only in older children.
q q What are the most common bacteria seen in a purulent pericardial effusion?
1. Staphylococcus aureus
2. Streptococcus pneumoniae
3. Haemophilus influenza
q q A patient is diagnosed with infective endocarditis. She has a history of recent dental surgery.
What is the most likely organism?
q q A child with congenital heart disease develops infective endocarditis postoperatively, what is
the most likely organism?
Staphylococcal species.
q q In a child previously healthy child without congenital heart disease, what is the most common
cause of infective endocarditis.
Staphylococcus aureus.
90%.
q q If antibiotics are required before the organism has been identified, which agents should be
used?
q q A child presents with chest pain and a new murmur. Which cardiac conditions should be
suspected?
q q What are the five major Jones criteria for diagnosing rheumatic fever?
Polyarthritis.
Non-pruritic, fine, lacey in appearance with central blanching and a serpiginous pattern.
q q What is the most common cardiac defect in children and adolescents with rheumatic heart
disease?
Mitral insufficiency.
q q What percent of untreated patients with Kawasaki’s disease will develop coronary artery
aneurysms?
15% to 25%. The coronary artery damage can lead to thrombosis or sudden death after acute symptoms
have resolved.
66 PEDIATRIC PEARLS OF WISDOM, 2e
Myocarditis, endocarditis, rheumatic fever, cardiac muscle disease, cardiac tumors, and postoperative
cardiac surgery.
q q What is the most common dysrhythmia seen in the pediatric age group?
Congenital heart disease (30%). Other causes include fever, infection, or drug exposure (20%), and
unknown etiology (50%).
Wolf-Parkinson-White.
q q What problem can occur by using digoxin to treat SVT in a patient with the WPW syndrome?
Digoxin can shorten the refractory period in the bypass tract and enhance conduction in the accessory
pathway, leading to a rapid ventricular response and ventricular fibrillation.
q q What are the most common causes of atrial fibrillation and flutter?
q q What is the treatment of choice in an unstable patient with atrial fibrillation and flutter?
Digitalization.
q q Why should atrial flutter be treated in a patient with congenital heart disease?
Because this patient’s risk of sudden death is four times higher than normal.
80%.
Electrolyte imbalance, metabolic disturbances, cardiac tumors, drugs, cardiac catheterization or surgery,
congenital heart disease, cardiomyopathies prolonged QT syndrome, and idiopathic.
PEDIATRIC PEARLS OF WISDOM, 2e 67
No treatment is required in an asymptomatic patient but frequent PACs should be evaluated to rule out
myocarditis.
q q What is the initial treatment for symptomatic second and third degree heart block?
Atropine 0.01 to 0.03 mg/kg. A minimum dose of 0.1 mg should be used in children to avoid atropine-
induced paradoxical bradycardia.
Episodes of paroxysmal hypoxemia which are usually self-limited and last less than 15 to 30 minutes,
although they may last longer. The spells are seen more often in the morning but may occur during the day
and may be precipitated by activity, a sudden fright, or injury or may occur spontaneously without apparent
cause. The tachypnea and cyanosis are due to an increase in right-to-left shunting and concomitant
decrease in pulmonary blood flow. The exact mechanism by which this occurs is unknown.
Place in the knee-chest position which increases peripheral resistance in the lower extremities, which in
turn, promotes increased pulmonary blood flow. Place the patient on oxygen and give morphine sulfate 0.1
mg/kg IV. If these measures fail, use propranolol to a maximum total dose of 0.1 mg/kg.
q q In the pre-term infant, what is the most common cause of congestive heart failure?
q q What is the most common cause of cardiac chest pain in an infant or child?
q q What is the most common atrial septal defect seen and what does it involve anatomically?
Most often involves the fossa ovalis, is midseptal, and is of the ostium secundum type.
q q What are the most notable cardiac auscultatory findings in children with atrial septal defects?
Normal or split first heart sound, accentuation of the tricuspid valve closure sound, a midsystolic
pulmonary ejection murmur and a wide, fixed split second heart sound.
68 PEDIATRIC PEARLS OF WISDOM, 2e
q q What is the typical CXR findings of a patient with atrial septal defect?
Enlargement of the right atrium and ventricle, dilated pulmonary artery and its branches, and increased
pulmonary vascular markings.
q q When should surgical repair be considered for patients with atrial septal defect and when is the
optimal timing for such treatment?
Surgical repair should be carried out in all patients with atrial septal defect who have evidence of a left-to-
right shunt where there is a pulmonary-systemic flow ratio equal to or greater than 1.5:1.0 found on cardiac
catheterization, ideally carried out at while the patient is between ages 2 – 4. Surgical therapy should not
be carried out in patients with small defects and trivial left-to-right shunts (less than 1.5:1.0) or in patients
with severe pulmonary hypertension without a significant left-to-right shunt.