Case 18

Question 1

What are key findings from history in a child with a VSD and CHF?

Answer 1

Poor feeding, respiratory distress, diaphoresis

Question 2

What are key physical exam findings from a child with VSD?

Answer 2

Tachypnea, Murmur, hepatomegaly

Question 3

What is on the differential diagnosis for VSD/CHF?

Answer 3

Bronchiolitis, Pneumonia, GERD, Metabolic disorder

Question 4

What is seen in a CXR of VSD?

Answer 4

Cardiomegaly, increased pulmonary vascular markings, and pulmonary edema (hallmark findings of a large left-to-right shunt due to congenital heart defect)

Question 5

What is seen on electrocardiogram of VSD?

Answer 5

Prominent biventricular forces (high voltage QRS complexes in leads V1 and V2), suggesting both LV volume overload and RV pressure overload

Question 6

What is seen on an echocardiogram of VSD?

Answer 6

2D ECHO demonstrates a large perimembranous ventricular septal defect in the sub-aortic region

Question 7

Definition of a VSD?

Answer 7

Any persistent communication between the ventricles occurring in isolation or as part of a more complex defect.

Question 8

What is the anatomy associated with VSD?

Answer 8

Defect of variable size located along embryologic lines of fusion in the ventricular septum.

Question 9

What is the physiology associated with VSD?

Answer 9

• Left-to-right shunting of blood during ventricular systole causes increased pulmonary blood flow, increased pulmonary venous return, and resultant left ventricular volume overload.
• Magnitude of the shunt depends on size of defect and difference between systemic and pulmonary vascular resistance.
• Newborns have elevated pulmonary vascular resistance. Because the systemic and pulmonary vascular resistances are nearly equal, there is no reason for blood to shunt through the VSD. The murmur of a VSD will appear when the PVR drops, usually at a few days to weeks of age.

Question 10

What is the presentation of VSD?

Answer 10

• Murmur and clinical signs usually not present in the newborn nursery but are noted from several days to weeks of age.
• Age of presentation and symptoms related to magnitude of left to right shunt.
• Large defects often present with CHF as pulmonary resistance falls in the first weeks of life.
• Small defects usually cause no symptoms
• VSDs tend to diminish in size with time. There is spontaneous closure of approximately 75 percent of small defects and between 25-50 percent of all defects.
• Hyperactive precordium frequently with a thrill at the lower left sternal border.

Question 11

Etiology of CHF in infancy:

Answer 11

• Heart defects that present with a murmur and signs of CHF in infancy include VSD, severe aortic stenosis or coarctation of the aorta, and a large patent ductus arteriosus (PDA).
• Many other congenital heart defects, such as ASD, do not cause CHF. Most cyanotic heart defects, like tetralogy of Fallot, cause decreased pulmonary blood flow and therefore doe not cause CHF.

Question 12

How do CHF present in infancy?

Answer 12

• Infants with heart disease are generally otherwise healthy.
• Typically presents with predominantly respiratory symptoms (tachypnea) and feeding difficulties
• The history will often reveal that infant is feeding for longer periods of time than norma, and that feedings are terminated due to respiratory distress.
• Infants frequently become diaphoretic with feedings. Diaphoresis suggests adrenergic activation and is a major sign of CHF in infants.
• Ultimately, due to poor feeding and increased caloric expenditure, poor weight gain ensues (Failure to thrive)
• Hepatomegaly is a reliable finding in infants with heart failure:
• -Decreased renal blood flow via activation of renin-angiotensin system leads to fluid retention, systemic venous congestion and hepatomegaly

Question 13

How often do breastfeeding and bottle feeding infants usually nurse?

Answer 13

• Young, breastfeeding infants usually nurse for 20-30 min, as often as every 1-2 hours.
• Bottle-fed infants often take more per feed and feed a little less often

Question 14

How would a problem with maternal breast milk production present?

Answer 14

It would be more likely to present with the infant trying to feed more frequently and continually acting hungry.

Question 15

Weight loss in a newborn:

Answer 15

• Newborns typically lose between 5-10 percent of their birth weight in the first few days after birth. Most will be back to birth weight by 10-14 days.
• Failure to gain weight adequately in infancy or childhood is referred to as “failure to thrive”

Question 16

What is a strong indicator of coarctation of the aorta?

Answer 16

A discrepancy in the strength of the brachial and femoral pulses.

Question 17

What is a simple way to grade murmur intensity?

Answer 17

Grade I - faint and easily missed.
Grade II - obvious
Grade III - loud
Grade IV - Associated with a thrill

Question 18

What grade of murmur is likely to be pathologic?

Answer 18

Any grade III or IV murmur is likely to be pathologic, and probably should be evaluated by a cardiologist.

Question 19

What are other pathologic murmurs?

Answer 19

• Diastolic murmurs are always pathologic
• Congenital heart defects must always be considered when evaluating a child with a murmur
• Acute rheumatic fever, endocarditis, and cardiomyopathies are uncommon causes of heart murmurs in children, but also must be considered, particularly in a child with a murmur and fever.

Question 20

What are the common congenital heart defects?

Answer 20

Atrial septal defect, Aortic stenosis, Coarctation of the aorta, patent ductus arteriosus, pulmonic stenosis, ventricular septal defect.

Question 21

Atrial septal defect (ASD):

Answer 21

The pathognomonic physical finding of an ASD is a widely split, fixed S2. The systolic murmur is due to increased flow across a normal pulmonic valve. Listening for wide splitting of the second heart sound is the most helpful way to distinguish an ASD from an innocent murmur. ASDs are often first detected at 3-5 years of age.

Question 22

Aortic stenosis:

Answer 22

A systolic ejection murmur often followed by an early diastolic murmur of aortic insufficiency. Aortic stenosis most commonly presents in infancy.

Question 23

Coarctation of the aorta:

Answer 23

It can present in infancy or at any age beyond because it tends to be a progressive problem, growing more severe over years. Coarctation presents with a murmur, HTN in the upper extremities, and a discrepancy between the upper and lower extremity blood pressures.

Question 24

Patent ductus arteriosus (PDA):

Answer 24

A continuous murmur, but a bit louder in systole. PDAs most commonly present in infancy.

Question 25

Pulmonic Stenosis:

Answer 25

Typically a prominent systolic ejection click just after S1, and a harsh systolic ejection murmur. Pulmonic stenosis most commonly presents in infancy.

Question 26

Ventricular septal defect (VSD):

Answer 26

This murmur is holosystolic, starting with S1. Its intensity is not reliable related to defect size. The murmur can have different qualities, from soft and blowing to harsh and loud. VSDs most commonly present in infancy.

Question 27

Innocent murmurs:

Answer 27

• By far the most common cause of a murmur in children, particularly at 3-7 years of age.
• An innocent murmur is by definition normal, and is not due to any heart abnormality.
• A murmur heard in a young infant or child who is not otherwise healthy is less likely to be innocent.
• The most commonly heard innocent murmur is the Still’s murmur.

Question 28

What is the Still’s murmur?

Answer 28

The most commonly heard innocent murmur. This is often described as musical or vibratory, and is heard best at the left lower sternal border in the supine position. The vibratory quality is the most characteristic feature of an innocent murmur.

Question 29

In deciding if a murmur is innocent, it is important to make sure there are no other signs of significant heart disease. Questions to ask yourself:

Answer 29

-Is the child otherwise well?
-Is the precordial activity normal?
-Is the second heart sound normally split?
-Is the murmur more than grade II/VI?
-Is the oxygen saturation normal?
If the answer to any of these questions is “no,” the murmur should not be considered innocent.

Question 30

What is a Gallop?

Answer 30

A gallop is an extra heart sound, quite different in character from a murmur, and very uncommonly heard in infants and children. A gallop rhythm is noted more often with primary cardiac contractile dysfunction than with volume overload lesions.

Question 31

What is on the differential diagnosis for CHF?

Answer 31

Metabolic disorders, Bronchiolitis or pneumonia, sepsis, GERD.

Question 32

Congestive heart failure (CHF):

Answer 32

CHF often presents with a history of respiratory distress, difficulty feeding, and poor weight gain (also called failure to thrive). There are many potential causes of CHF in infancy.

Question 33

Metabolic disorders:

Answer 33

Many metabolic disorders could potentially cause poor feeding. A negative newborn screen makes this less likely, but still lan important possibility. Hepatomegaly can be found in many metabolic disorders (although jaundice might also be expected), and tachypnea may be a sign of metabolic acidosis.

Question 34

Bronchiolitis and pneumonia:

Answer 34

These present with respiratory distress, pneumonia typically also with fever. Bronchiolitis is very common, and presents with predominantly respiratory distress. Anything causing respiratory difficulty (increased work of respiration) in infants can also cause difficulty with feedings. Tachypnea is more likely to indicate a pulmonary process than heart disease.

Question 35

Gastroesophageal reflux:

Answer 35

Poor feeding may be a sign of gastrointestinal disease, but the absence of spitting up or emesis makes this less likely.

Question 36

CXR with CHF:

Answer 36

Hallmark radiologic findings of a large left to right shunt due to a congenital heart defect are cardiomegaly, increased pulmonary vascular markings, and pulmonary edema.

Question 37

ECG in patient with large VSD:

Answer 37

Shows prominent biventricular forces - high voltage QRS complexes in leads V1 and V2 - suggesting both LV volume overload and RV pressure overload (from pulmonary HTN).

Question 38

ECHO in patient with large VSD:

Answer 38

Use of echocardiography in teh primary pediatric care setting is controversial, and most pediatric cardiologists agree that it is not appropriate as first-line testing. An ECHO costs about $1500, a consult with a pediatric cardiologist, about $200. An echo is more informative when combined with a cardiology consultation.

Question 39

Cardiac magnetic resonance imaging (MRI):

Answer 39

Cardiac MRI is an emerging tool for evaluating congenital heart defects, but is available only in tertiary centers. A young infant would require anesthesia to obtain adequate images.

Question 40

Cardiac computed tomography (CT):

Answer 40

Cardiac CT is not as good as echocardiography for intracardiac anatomy and requires a high dose of radiation.

Question 41

Initial management of congenital heart disease:

Answer 41

• Not all children with CHD need to be admitted to the hospital; many may be seen by a pediatric cardiologist as an outpatient.
• Children with significant congenital heart defects presenting with cyanosis or CHF need to be admitted to the hospital for management
• All children in shock need to be admitted urgently to an ICU.
• For other patients, the decision to admit is based on the severity of illness and the tempo with which the patient’s symptoms are evolving:
• -Findings such as increased respiratory effort, lethargy/decreased level of alertness, feeding difficulty, or cyanosis merit referral for admission for evaluation and management of symptomatic congenital heart disease.

Question 42

Diuretics to treat congestive heart failure in kids:

Answer 42

• Effective at relieving symptoms of CHF.
• Counteract the fluid retention that occurs as a result of activation of the renin-angiotensin system
• Furosemide (lasix) is the diuretic used most commonly for CHF in infants.

Question 43

Digoxin to treat congestive heart failure in kids:

Answer 43

• Shown in some studies to improve symptoms in infants with CHF due to VSD
• Mechanism of the improvement is not altogether clear, as infants with a VSD do not have impaired contractility.

Question 44

Nutrition for CHF in kids:

Answer 44

-High-calorie formula (30 calories/ounce) may be considered if patient fails to gain adequate weight.

Question 45

Treatment of pulmonary HTN in a child with CHF:

Answer 45

• If pulmonary HTN is left untreated for several years, pulmonary vascular obstructive disease (also known as Eisenmenger’s disease) may develop.
• The decision about whether to proceed with surgery in an infant with a VSD should be made by 6 months of age to eliminate the risk of Eisenmenger’s disease.

Question 46

Management of Ventricular septal defect (VSD) via surgical closure:

Answer 46

Results of surgical closure (by patching) of a VSD are excellent, even in small infants.