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Flashcards in Cardiology Deck (102):
1

To measure blood pressure, one must have a mercury sphygmomanometer with a cuff that covers

approximately two thirds of the upper part of the arm or leg.

2

The intensity of systolic murmurs is graded from I to VI:

I, barely audible;
II, medium intensity;
III, loud but no thrill;
IV, loud with a thrill;
V, very loud but still requiring positioning of the stethoscope at least partly on the chest;
VI, so loud that the murmur can be heard with the stethoscope off the chest

3

The heart is usually enlarged when the maximal cardiac width is

more than half the maximal chest width (cardiothoracic ratio >50%),

4

In the posteroanterior view, the left border of the cardiac shadow consists of three convex shadows produced, from above downward, by the

aortic knob, the main and left pulmonary arteries, and the left ventricle

5

Three structures contribute to the right border of the cardiac silhouette.

from above:
superior vena cava
ascending aorta
right atrium.

6

Lesions Resulting in Increased Volume Load

those that cause left-to-right shunting: atrial septal defect, ventricular septal defect (VSD), AV septal defects (AV canal), and patent ductus arteriosus

7

Heart Failure in left to right CHD

-tachypnea, chest retractions, nasal flaring, and wheezing.
-Sympathetic activation leads to sweating and irritability
-the imbalance between oxygen supply and demand lead to failure to thrive.

8

Lesions Resulting in Increased Pressure Load

due to obstruction to normal blood flow:
-valvular pulmonic stenosis, valvular aortic stenosis, and coarctation of the aorta
- tricuspid or mitral stenosis, cor triatriatum and obstruction of the pulmonary veins.

9

Characteristic of right-sided heart failure

-hepatomegaly
-peripheral edema

10

Characteristic of left-sided heart failure

-pulmonary edema
-poor perfusion

11

Cyanotic Lesions with Decreased Pulmonary Blood Flow

-tricuspid atresia
-tetralogy of Fallot
-various forms of single ventricle with pulmonary stenosis

These lesions must include both an obstruction to pulmonary blood flow (at the tricuspid valve or right ventricular or pulmonary valve level) and a pathway by which systemic venous blood can shunt from right to left and enter the systemic circulation (via a patent foramen ovale, atrial septal defect, or VSD)

12

Cyanotic Lesions with Increased Pulmonary Blood Flow

Cyanosis is caused by either
1) abnormal ventricular-arterial connections
-Transposition of the great vessels (TGA)
2) total mixing of systemic venous and pulmonary venous blood within the heart
-total anomalous pulmonary venous return (TAPVR)
-truncus arteriosus

13

Syndrome associated with ASD which includes a hypoplastic or absent radii and a 1st-degree heart block

Holt-Oram syndrome

14

most common form of ASD

Ostium Secundum Defect

15

Ostium Secundum Defect

-most often asymptomatic
-subtle failure to thrive
-mild left precordial valve
-enlargement of the RA and RV and dilatation of the PA
-2nd heart sound is widely split and fixed in its splitting
-ECG: NA or RAD, rsR' pattern in the precordial leads

16

In patients with Ostium Secundum ASD, surgical or transcatheter device closure is advised for

-all symptomatic patients
-for asymptomatic patients with a Qp : Qs ratio of at least 2 : 1 or those with right ventricular enlargement

17

Procedure of choice for ASD

percutaneous catheter device closure

18

In patients with ASD, closure is not required in

-patients with small secundum ASDs and minimal left-to-right shunts without right ventricular enlargement

19

True or False:
Small- to moderate-sized ASDs detected in term infants may close spontaneously.

True

20

True or False:
antibiotic prophylaxis for isolated secundum ASDs is recommended.

False
Infective endocarditis is extremely rare, and antibiotic prophylaxis for isolated secundum ASDs is NOT recommended.

21

AV septal defect, also known as an AV canal defect or an endocardial cushion defect, consists of

contiguous atrial and ventricular septal defects with markedly abnormal AV valves

22

Atrioventricular Septal Defects is commonly associated with what syndrome?

Down Syndrome

23

most common cardiac malformation and accounts for 25% of congenital heart disease

VSD

24

Most common type of VSD

membranous type

25

characteristic murmur of a small VSD

-loud, harsh, or blowing holosystolic murmur over the lower left sternal border
-frequently accompanied by a thrill

26

characteristic of large VSD

-pulmonary blood flow and pulmonary hypertension result to dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections, and cardiac failure in early infancy.
-duskiness during infections or crying
-Prominent left precordium
-palpable parasternal lift, a laterally displaced apical impulse and apical thrust, and a systolic thrill.

27

Typical CXR of VSD

small VSD: normal
large VSD: cardiomegaly with prominence of both ventricles, the left atrium, and the pulmonary artery, increased pulmonary markings, pulmonary effusion, and pulmonary edema

28

Natural course of VSD:

-30-50% of small defects close spontaneously during the 1st 2 yr of life
- Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs (up to 35%)
-majority close before 4yo
- infective endocarditis is a long-term risk

29

True or False
All VSD should be surgically repaired.

False.
Surgical repair is currently not recommended for patients with small VSD.

30

what should you advise in patients with small VSD?

-should be encouraged to live a normal life, with no restrictions on physical activity
-integrity of primary and permanent teeth should be carefully maintained

31

Should patients with VSD receive antibiotic prophylaxis for dental and surgical procedures?

antibi otic prophylaxis is no longer recommended for dental visits or surgical procedures

32

Indications for surgical closure of a VSD include

-patients at any age with large defects in whom clinical symptoms and failure to thrive cannot be controlled medically;
-infants between 6 and 12 mo of age with large defects associated with pulmonary hypertension, even if the symptoms are controlled by medication; -patients older than 24 mo with a Qp : Qs ratio greater than 2 : 1
-patients with supracristal VSD of any size because of high risk for aortic valve regurgitation

33

Leading causative agents for endocarditis in pediatric patients

Viridans-type streptococci (α-hemolytic streptococci) and Staphylococcus aureus

- no underlying disease: Staph
- after dental procedure: viridans Strep
- after lower bowel/ GU manipulation: grp D enterococci
- IV drug user: Serratia marscesens, P. aeruginosa
- indwelling catheter: CoNS

34

Initiating lesion formed in infective endocarditis which becomes the substrate for the deposition of thrombin and fibrin

nonbacterial thrombotic embolus

- fungating masses >1 cm are at greatest risk for embolization

35

What criteria are used for the diagnosis of infective endocarditis?

Duke Criteria
2 major criteria, 1 major and 3 minor, or 5 minor criteria suggest definite endocarditis

Major: (+) blood CS, echo evidence of endocarditis (intracardiac mass)

Minor: predisposing conditions, fever, embolic-vascular signs, immune complex phenomena (glomerulonephritis, arthritis, rheumatoid factor, Osler nodes, Roth spots), a single positive blood culture or serologic evidence of infection, and echocardiographic signs not meeting the major criteria

additional minor criteria:
newly diagnosed clubbing, splenomegaly, splinter hemorrhages, and petechiae; a high erythrocyte sedimentation rate; a high C-reactive protein level; and the presence of central nonfeeding lines, peripheral lines, and microscopic hematuria

36

What is the treatment recommendation for IE?

Target most common organisms: viridans strep and Staphylococci

empiric: vanco+genta
culture-guided:
if highly susceptible, penG or ceftri+penG
or ceftri+genta/vanco

37

Routine IE prophylaxis for dental procedures is done for which high risk groups?

patients with cardiac conditions, pxs with prosthetic valves, pxs wit previous IE

- prophylaxis for gastrointestinal or genitourinary procedures is no longer recommended in the majority of cases. Prophylaxis for patients undergoing cardiac surgery with placement of prosthetic material is still recommended.

38

A 1 day old is noted to be cyanotic. PE reveals a grade 2-3/6 systolic murmur and a single loud second heart sound. CXR reveals a normal sized heart and decreased pulmonary vascular markings. ECG left ventricular dominance. The next step in management of this neonate is to administer
a. Sodium bicarbonate
b. Morphine
c. Prostaglandin E1
d. Digoxin
e. Positive pressure ventilation

C
The murmur may represent a PDA. If the PDA closed, marked cyanosis would supervene and result in acidosis, shock and death. PGE1 maintains patency of the ductus arterious between the pulmonary artery and the aorta.

39

A 1 day old is noted to be cyanotic. PE reveals a grade 2-3/6 systolic murmur and a single loud second heart sound. CXR reveals a normal sized heart and decreased pulmonary vascular markings. ECG left ventricular dominance.
The most likely diagnosis in the patient:
a. PPHN
b. Transposition of the great arteries
c. Truncus arteriosus
d. Pulmonary atresia
e. Total anomalous venous return

D
Pulmonary atresia
Pulmonary atresia is manifested by a small RV, decreased pulmonary vascular markings, early marked cyanosis without heart failure, and ductul dependence to maintain some pulmonary blood flow.

Nelsons 19th Ed
As the ductus arteriosus closes in the 1st hours/days of life, infants with pulmonary atresia and an intact ventricular septum become markedly cyanotic since their only source of pulmonary blood flow is removed. Untreated, most patients die within the 1st wk of life. Physical examination reveals severe cyanosis and respiratory distress. The 2nd heart sound, representing only aortic closure, is single and loud. Often, no murmurs are audible; sometimes a systolic or continuous murmur can be heard secondary to ductal blood flow. A harsh holosytolic murmur may be heard at the lower left sternal border if there is significant tricuspid regurgitation.

40

3 An 18-month-old is noted to assume a squatting position frequently during playtime at the daycare center. The mother also notices occasional episodes of perioral cyanosis during some of these squatting periods. The day of admission, the child becomes restless, hyperpneic, and deeply cyanotic. Within 10 minutes, the child becomes unresponsive. The most likely underlying lesion is
a. Cardiomyopathy
b. Anomalous coronary artery
c. Tetralogy of fallot
d. Constipation
e. Breath holding spells

C
The child described has TOF with exercise induced cyanosis. The more serious episode is a cyanotic, blue or tet spell and may be due to decreased systemic vascular resistance, increased pulmonary artery pressure, or right ventricular outflow tract obstruction. The murmur of tetralogy (pulmonary stenosis) often disappears or lessens during a spell.

19th Ed
In older children with unrepaired tetralogy, dyspnea occurs on exertion. They may play actively for a short time and then sit or lie down. Children assume a squatting position for the relief of dyspnea caused by physical effort; the child is usually able to resume physical activity after a few minutes of squatting.
Paroxysmal hypercyanotic attacks (hypoxic, “blue,” or “tet” spells) are a particular problem during the 1st 2 yr of life. The infant becomes hyperpneic and restless, cyanosis increases, gasping respirations ensue, and syncope may follow. Temporary disappearance or a decrease in intensity of the systolic murmur is usual as flow across the right ventricular outflow tract diminishes. Severe spells may progress to unconsciousness and, occasionally, to convulsions or hemiparesis. Spells are associated with reduction of an already compromised pulmonary blood flow, which, when prolonged, results in severe systemic hypoxia and metabolic acidosis.

41

Therapy of a blue or tet spell could include all of the following EXCEPT
a. Epinephrine
b. Knee chest position
c. O2
d. Morphine
e. Sodium bicarbonate
f. phenylephrine

A
Epinephrine is potentially dangerous because it may exacerbate inotropy and contractile forces, which may obstruct the right ventricular infundibulum. Indeed, propranolol has been used to treat tet spells.

19th Ed
Treatment for Tet spells
(1) knee-chest position (2) oxygen (3) morphine subcutaneously in a dose not in excess of 0.2 mg/kg. 4) for metabolic acidosis, give IV sodium bicarbonate 5) For spells that are resistant to this therapy, intubation and sedation 6) Drugs that increase systemic vascular resistance, such as intravenous phenylephrine, can improve right ventricular outflow, decrease the right-to-left shunt, and improve the symptoms 7) β-Adrenergic blockade by the intravenous administration of propranolol (0.1 mg/kg given slowly to a maximum of 0.2 mg/kg) has also been used.

42

a 2-day-old experiences cyanosis, hypotension, metabolic acidosis. PE lethargic, tachycardic, gray-blue with hepatomegaly, grade 2-3/6 systolic murmur, poor radial and femoral pulses. CXR cardiomegaly ECG RV dominance with markedly reduced R waves in V5 and V6. The most likely diagnosis is
a. myocarditis
b. hypoplastic left heart syndrome
c. anomalous coronary arteries
d. total anomalous venous return
e. tetralogy of fallot

B
Poor pulses, reduced left ventricular forces on ECG, cardiogenic shock, and severe cyanosis are typical of hypoplastic left heart syndrome.

19th Ed
Although cyanosis may not always be obvious in the 1st 48 hr of life, a grayish-blue color of the skin is soon apparent and denotes a mix of cyanosis and poor perfusion. The condition is diagnosed in most infants in the 1st few hours or days of life. Once the ductus arteriosus begins to closes, signs of poor systemic perfusion and shock predominate. All of the peripheral pulses may be weak or absent. A palpable right ventricular parasternal lift may be present along with a nondescript systolic murmur.

On the chest roentgenogram, the heart is variable in size in the 1st days of life, but cardiomegaly develops rapidly and is associated with increased pulmonary vascularity. The initial electrocardiogram may show only the normal neonatal pattern of right ventricular dominance, but later, P waves become prominent and right ventricular hypertrophy is usual with reduced left ventricular forces

43

6 A 3 day old presents with fussiness and poor feeding. PE HR 250 ECG HR 250 QRS 0.07 no visible P waves. Most likely diagnosis?
a. Ventricular tachycardia
b. Supraventricular tachycardia with aberrant conduction
c. Supraventricular tachycardia
d. Heart block
e. None of the above

C
SVT is a frequent cause of heart failure in this age group with structurally normal heart.

19th Ed
Re-entrant SVT is characterized by an abrupt onset and cessation; it usually occurs when the patient is at rest, although in infants it may be precipitated by an acute infection. Attacks may last only a few seconds or may persist for hours. The heart rate usually exceeds 180 beats/min and may occasionally be as rapid as 300 beats/min. In neonates, SVT is usually manifested as a narrow QRS complex (

44

the first approach to SVT is
a. Fluid challenge
b. Iced saline bag placed over the face
c. Carotid massage
d. Propranolol
e. Verapamil

B
Placing an iced saline bag over the neonates face is the vagal maneuver of choice to break SVT. Verapamil may decrease contractility and is dangerous in this age group.

19th Ed
Vagal stimulation by placing of the face in ice water (in older children) or by placing an ice bag over the face (in infants) may abort the attack. To terminate the attack, older children may be taught vagal maneuvers such as the Valsalva maneuver, straining, breath holding, or standing on their head. Ocular pressure must never be performed, and carotid sinus massage is very rarely effective.

In stable patients, adenosine by rapid intravenous push is the treatment of choice because of its rapid onset of action and minimal effects on cardiac contractility

Calcium channel blockers such as verapamil have also been used in the initial treatment of SVT in older children. Verapamil may reduce cardiac output and produce hypotension and cardiac arrest in infants younger than 1 yr; it is therefore contraindicated in this age group.

In urgent situations when symptoms of severe heart failure have already occurred, synchronized DC cardioversion (0.5-2 J/kg) is recommended as the initial management

45

What is the congenital heart disease associated with Trisomy 21

Endocardial cushing defect

46

What is the congenital heart disease associated with Turner syndrome

Bicuspid aortic valve

47

What is the congenital heart disease associated with Di George syndrome

Conotruncal anomalies

48

What is the congenital heart disease associated with Asplenia syndrome

Complex multiple cyanotic lesions

49

What is the congenital heart disease associated with Congenital rubella

PDA

50

What is the congenital heart disease associated with Kartagener syndrome

Dextrocardia

51

What is the congenital heart disease associated with Williams syndrome

Supravalvular stenosis

52

What heart disease is associated with juvenile rheumatoid arthritis

Pericarditis

53

What heart disease is associated with lyme disease

Acquired Heart block

54

What heart disease is associated with homocystinuria

Coronary thrombosis

55

What heart disease is associated with marfan syndrome

Aortic insufficiency

56

What heart disease is associated with tuberous sclerosis

Cardiac rhabdomyoma

57

What heart disease is associated with hypothyroidism

Bradycardia

58

What heart disease is associated with pompe disease

Short PR interval

59

What heart disease is associated with carnitine deficiency

Cardiomyopathy

60

What heart disease is associated with maternal SLE

Congenital heart block

61

A previously well 3 ½ month old presents with poor feeding and poor growth. VS RR 70 HR 173 BP 90/65 in the upper and lower extremities. Cardiac exam (+) palpable parasternal lift and a systolic thrill. A grade 4 holosystolic murmur and a mild diastolic rumble are noted. CXR reveals cardiomegaly. The most likely diagnosis is
a. Cardiomyopathy
b. Myocarditis
c. VSD
d. Coarctation of the aorta
e. Transposition of the great arteries

C
A large VSD with a large left to right shunt produces significant heart failure. The age of onset usually corresponds to the time when the normally high fetal pulmonary vascular resistance declines in the first 1-3 months of life. With decreasing pulmonary artery pressure, the left to right shunt increases.

19th Ed
Large VSDs with excessive pulmonary blood flow and pulmonary hypertension are responsible for dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections, and cardiac failure in early infancy. Cyanosis is usually absent, but duskiness is sometimes noted during infections or crying. Prominence of the left precordium is common, as are a palpable parasternal lift, a laterally displaced apical impulse and apical thrust, and a systolic thrill. The holosystolic murmur of a large VSD is generally less harsh than that of a small VSD and more blowing in nature because of the absence of a significant pressure gradient across the defect. It is even less likely to be prominent in the newborn period. The pulmonic component of the 2nd heart sound may be increased as a result of pulmonary hypertension. The presence of a mid-diastolic, low-pitched rumble at the apex is caused by increased blood flow across the mitral valve and indicates a Qp : Qs ratio of ≥2 : 1. This murmur is best appreciated with the bell of the stethoscope.

62

the initial treatment of choice for a symptomatic patient with isolated pulmonic stenosis is
a. Closed surgical blade valvotomy
b. Open surgical valvotomy
c. Balloon catheter valvuloplasty
d. Blalock taussig shunt
e. Valve replacement

C
Balloon valvuloplasty has greatly improved the management of stenotic lesions of the pulmonic and aortic valves.

19th Ed
Patients with moderate or severe isolated pulmonary stenosis require relief of the obstruction. Balloon valvuloplasty is the initial treatment of choice for the majority of patients

63

pulsus paradoxus is associated with
a. Pericarditis
b. Endocarditis
c. Rheumatic fever
d. Myocarditis
e. Postperfusion syndrome

A
Pericarditis

19th Ed
Acute Pericarditis
Muffled or distant heart sounds, tachycardia, narrow pulse pressure, jugular venous distension, and a pericardial friction rub provide clues to the diagnosis of acute pericarditis. Cardiac tamponade is recognized by the excessive fall of systolic blood pressure (>10 mm Hg) with inspiration. This pulsus paradoxus can be assessed by careful auscultatory blood pressure determination (automated blood pressure cuffs are inadequate), arterial pressure line wave form, or pulse oximeter tracing inspection. Conditions other than cardiac tamponade, which may result in pulsus paradoxus include severe dyspnea, obesity, and positive pressure ventilator support.

64

The radiographic finding of notching of the ribs is associated with
a. Pulmonary hypertension
b. Anomalous pulmonary venous return above the diaphragm
c. Coarctation of the aorta
d. Systemic hypertension
e. Aortic insufficiency

C
Rib notching is caused by increased collateral arteries trying to supply the lower trunk and extremities and bypass the aortic coarctation.

19th Ed
The enlarged left subclavian artery commonly produces a prominent shadow in the left superior mediastinum. Notching of the inferior border of the ribs from pressure erosion by enlarged collateral vessels is common by late childhood. In most instances, the descending aorta has an area of poststenotic dilatation.

65

What is the syndrome associated with hypoplasia of the right lung and anomalous pulmonary venous return

Scimitar

66

What is the syndrome associated with biliary atresia

Polysplenia

67

What is the syndrome associated with pulmonary stenosis

Noonan

68

What is the syndrome associated with VSD

Fetal alcohol

69

What is cardiac finding is associated with fenfluramine

Aortic stenosis

70

What is cardiac finding is associated with Kearns-Sayre syndrome

Heart block

71

What is cardiac finding is associated with hemochromatosis

cardiomyopathy

72

What is cardiac finding is associated with tuberous sclerosis

Rhabdomyoma

73

All of the following are true about an ostium secundum defect (ASD) EXCEPT
a. Females outnumber male
b. Symptoms usually benign in infancy
c. Wide and fixed splitting of the second heart sound is present
d. Right ventricular volume overload is present
e. Cardiac catheterization is unnecessary for diagnosis

B
Most pediatric patients with an ASD are asymptomatic. Heart failure is rare in childhood.

19th Ed
a. Females outnumber males 3 : 1 in incidence.
b. A child with an ostium secundum ASD is most often asymptomatic; the lesion is often discovered inadvertently during physical examination. Even an extremely large secundum ASD rarely produces clinically evident heart failure in childhood.
c. the characteristic finding is that the 2nd heart sound is widely split and fixed in its splitting during all phases of respiration.
d. ECG and echocardiogram shows findings characteristic of right ventricular volume overload.
e.?

74

An atrioventricular septal defect is different from an ostium secundum ASD because the AV septal defect
a. Does not manifest heart failure
b. Does not create volume overload
c. Has the same ECG findings
d. Produces an early tendency for pulmonary hypertension
e. Creates an atrial level shunt

D
The atrial and/or the atrial-ventricular defects both have a higher tendency for an earlier and more rapid onset of pulmonary hypertension. The ECG of an AV septal defect is characteristic because of the superior left deviation of the QRS axis.

75

A 5 month old previously well infant is found to have a loud holosystolic murmur 4/6 at the left sternal border. The first and second heart sounds are normal, there is no tachycardia, rumble, or gallop, and hepatomegaly is not noted. The child feeds well and has grown adequately. You suspect
a. Restrictive VSD
b. Anomalous left coronary artery
c. VSD with a 4:1 shunt
d. Tetralogy of fallot
e. Single ventricle

A
A small VSD

76

All of the following are true regarding small VSD EXCEPT
a. Spontaneous closure is more common in muscular vs membranous
b. Closure usually occurs in the first 2 years of life
c. Antibiotic prophylaxis is recommended for dental visits
d. Pulmonary pressures are normal
e. Surgical repair is not recommended

C
As protection against infective endocarditis, the integrity of primary and permanent teeth should be carefully maintained; with the latest revision of the American Heart Association guidelines, antibiotic prophylaxis is no longer recommended for dental visits or surgical procedures
19th Ed
a. Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs (up to 35%).
b. A significant number (30-50%) of small defects close spontaneously, most frequently during the 1st 2 yr of life.
c. As above
d. Small, restrictive VSDs are associated with normal right heart pressures and pulmonary vascular resistance.
e. In patients with small VSDs, surgical repair is currently not recommended. Parents should be reassured of the relatively benign nature of the lesion, and the child should be encouraged to live a normal life, with no restrictions on physical activity.

77

A 6 month old presents with tachycardia, tachypnea, and poor feeding for 3 months. PE continuous machinery murmur and a wide pulse pressure with a prominent apical impulse. The most likely diagnosis is
a. Pulmonic stenosis
b. Aortic stenosis
c. VSD
d. PDA
e. Anomalous coronary artery

D
A PDA often presents like a large VSD except that there is a continuous murmur and the diastolic run off, which produces the wide pulse pressure and bounding pulses

19th Ed
A small PDA is usually asymptomatic. A large PDA will result in heart failure similar to that encountered in infants with a large VSD. Retardation of physical growth may be a major manifestation in infants with large shunts. A small PDA is associated with normal peripheral pulses, and a large PDA results in bounding peripheral arterial pulses and a wide pulse pressure, due to runoff of blood into the pulmonary artery during diastole. The classic continuous murmur is described as being like machinery in quality.

78

The differential diagnosis of PDA (6 month old presents with tachycardia, tachypnea, and poor feeding for 3 months. PE continuous machinery murmur and a wide pulse pressure with a prominent apical impulse) includes all of the following EXCEPT
a. Truncus arteriosus
b. Aorticopulmonary window
c. Sinus of valsalva aneurysm rupture
d. Aortic valve insufficiency
e. Critical aortic stenosis from a bicuspid valve

E
Aortic stenosis without significant aortic regurgitation (insufficiency) produces an inactive precordium, normal or weak pulses, and pressure overload of the left ventricle
19th Ed
a- Truncus arteriosus with torrential pulmonary flow also has an “aortic runoff” physiology.
b- An aorticopulmonary window defect may rarely be clinically indistinguishable from a patent ductus
c-A sinus of Valsalva aneurysm that has ruptured into the right side of the heart or pulmonary artery, coronary arteriovenous fistulas, and an aberrant left coronary artery with massive collaterals from the right coronary display dynamics similar to that of a PDA with a continuous murmur and a wide pulse pressure.
d- VSD with aortic insufficiency, repaired tetralogy of Fallot, and combined aortic and mitral insufficiency (usually due to rheumatic fever) may be confused with a PDA

79

The treatment of a patient with PDA ( 6 month old presents with tachycardia, tachypnea, and poor feeding for 3 months. PE continuous machinery murmur and a wide pulse pressure with a prominent apical impulse) is best performed by which methods (may choose more than 1)?
a. IV indomethacin
b. Surgical closure
c. Catheter coil closure
d. Digoxin until PDA closes spontaneously
e. ACE inhibitor

B and C
A PDA manifesting in any patient other than a premature infant does not close spontaneously. Surgery carries low morbidity and even lower mortality. However, transcatheter coil closure or umbrellas has reduced the need for surgery in many patients.
19th Ed
b- Irrespective of age, patients with PDA require surgical or catheter closure. In patients with a small PDA, the rationale for closure is prevention of bacterial endarteritis or other late complications. In patients with a moderate to large PDA, closure is accomplished to treat heart failure or prevent the development of pulmonary vascular disease, or both. Once the diagnosis of a moderate to large PDA is made, treatment should not be unduly postponed after adequate medical therapy for cardiac failure has been instituted.
c- Small PDAs are generally closed with intravascular coils. Moderate to large PDAs may be closed with an umbrella-like device or with a catheter-introduced sac into which several coils are released

80

A neonate manifests cyanosis and hepatomegaly. There is a grade 4/6 systolic ejection murmur without an audible ejection click. ECG tall, spiked P waves and RVH. Best method to evaluate patient is to perform:
a. CXR
b. Vector cardiogram
c. Immediate cardiac catheterization
d. MRI
e. Echocardiogram

E
Echocardiography has replaced most methods of visualizing the anatomy of congenital and other heart lesions. In many circumstances, the echo diagnosis is all that is needed before surgery or other therapies.

81

The most likely diagnosis in the patient in q23 (A neonate manifests cyanosis and hepatomegaly. There is a grade 4/6 systolic ejection murmur without an audible ejection click. ECG tall, spiked P waves and RVH.) is
a. PDA
b. Critical aortic stenosis
c. Critical pulmonic stenosis
d. TOF
e. Truncus arteriosus

C
Critical pulmonic stenosis often presents in the neonate. Cyanosis is due to elevated right sided pressures and right to left shunting at the patent foramen ovale

19th Ed
When severe pulmonic stenosis occurs in a neonate, decreased right ventricular compliance often leads to cyanosis due to right-to-left shunting through a patent foramen ovale, a condition termed critical pulmonic stenosis.

A loud, long, and harsh systolic ejection murmur, usually accompanied by a thrill, is maximally audible in the pulmonic area and may radiate over the entire precordium, to both lung fields, into the neck, and to the back.

ECG: gross right ventricular hypertrophy, frequently accompanied by a tall, spiked P wave.

82

25 The treatment of choice for the lesion of a neonate with critical PS
a. Digoxin
b. Propranolol
c. Surgical shunt
d. Balloon valvuloplasty
e. Valve replacement

D
Balloon valvuloplasty is a remarkable nonsurgical method to dilate the valve and partially relieve the obstruction to right ventricular outflow.

19th Ed
Balloon valvuloplasty is the initial treatment of choice for the majority of patients

83

A 12yo male tries out for a middle school hockey team. He has a history of a heart murmur as an infant, but the doctor thought it would go away. During the tryout, he experiences severe dyspnea and becomes light headed. At your office, he has a normal rhythm, pulse, and blood pressure, and is no longer dizzy. There is a grade 4/6 systolic ejection murmur that radiates to the neck. There is also an ejection click. An ECG reveals LVH. The next approach to his management includes
a. CXR
b. Exercise test
c. Digitalization
d. Echocardiography
e. Tilt table testing

D
Echocardiography is essential to identify valve disease, estimate gradients, and detect signs of endocardial fibroelastosis

84

A 12yo male tries out for a middle school hockey team. He has a history of a heart murmur as an infant, but the doctor thought it would go away. During the tryout, he experiences severe dyspnea and becomes light headed. At your office, he has a normal rhythm, pulse, and blood pressure, and is no longer dizzy. There is a grade 4/6 systolic ejection murmur that radiates to the neck. There is also an ejection click. An ECG reveals LVH.
The patient most probably has
a. Pulmonic stenosis
b. Mild aortic stenosis
c. Severe aortic stenosis
d. PDA
e. Williams syndrome

C
Severe aortic stenosis may develop gradually, progressing over 5-10 years.

85

Wide pulse pressure, with bounding peripheral pulses. Cardiomegaly with LV apical heave. Diastolic thrill may be present. Murmur begins at S2 and continues until late in diastole; heard best on the upper and midleft sternal border, radiates to the apex and upper right sternal border. Murmur is high pitched blowing quality, easily heard on full expiration with the diaphragm of steth and patient leaning forward. Austin-Flint murmur (apical presystolic murmur) may be heard, which resembles MS.
What is the cardiac lesion?

aortic insufficiency

86

What are the cardiac lesions found in RHD?

Mitral insufficiency
Mitral stenosis
Aortic insufficiency

- it takes 10 yrs or more for MS to develop

Tricuspid insufficiency - may occur secondary to left sided lesions
Pulmonary insufficiency may occur with pulmonary hypertension - a late finding in severe MS

Murmur of pulmonary insufficiency is called Graham Steell murmur, similar to AI but without bounding pulses.

87

What are the most common causes of myocarditis in the pediatric population?

enteroviruses (coxsackie and echovirus), adenovirus, EBV, parvovirus and CMV

88

What is the most common form of cardiomyopathy in children?

dilated cardiomyopathy

* etiologies are diverse but ischemic etiology is rare in children
* commonly familial with autosomal dominance eg. Duchenne and Becker muscular dystrophies
* also the most common cause of cardiac transplantation

89

Which vasculitis predominantly affects the medium-sized arteries, with a striking predilection for the coronary arteries

Kawasaki Disease

90

What are the five principal criteria of Kawasaki Disease?

1. bilateral nonexudative bulbar conjunctival injection with limbal sparing;
2. erythema of the oral and pharyngeal mucosa with strawberry tongue and dry, cracked lips;
3. edema and erythema of the hands and feet;
4. rash of various forms (maculopapular, erythema multiforme, or scarlatiniform) with accentuation in the groin area; and
5. nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm

- Perineal desquamation is common in the acute phase. Periungual desquamation of the fingers and toes begins 1-3 wk after the onset of illness and may progress to involve the entire hand and foot

91

in clara's heart, who's mr right?

mr. joren

92

What is the heart lesion?

Loud, harsh, blowing holosystolic murmur at the lower, left sternal border

VSD

93

What is the lesion?

Continuous, machinery like murmur at the second left ICS, radiating to the left sternal border or left clavicle

PDA

94

What is the lesion?

Second heart sound widely split, fixed splitting systolic ejection murmur, left middle and upper sternal border

ASD

95

What is the lesion?

Systolic murmur, loud, harsh, most intense at the left sternal border

TOF

96

What is the heart lesion corresponding this/these syndrome?

Noonan syndrome
Alagille syndrome

Pulmonary stenosis

97

What is the heart lesion corresponding this/these syndrome?

William syndrome

Aortic Stenosis

98

What is the heart lesion corresponding this/these syndrome?

Turner Syndrome

Coarctation of the Aorta

99

What is the heart lesion corresponding this/these syndrome?

DiGeorge Syndrome

TAPVR

100

What is the characteristic radiographic finding in TOF?

Boot or wooden shoe appearance (couer en sabot)

101

What is the radiographic finding in TGA?

Egg shaped heart

102

What is the radiographic finding in TAPVR?

Snowman appearance in older children