1.
A
4-week old girl presents with poor feeding and tachypnea. The infant was
born at term after a normal pregnancy. At 2 weeks of ago, a cardiac
murmur was noted, but the baby was otherwise well. However, over the last
week she has become more tachypneic and is feeding poorly. Her family is
concerned that she takes up to 50 minutes to feed and becomes sweaty and
tachypneic during feedings. There is no history of cough or fever.
What physiological change takes place at approximately 4 weeks of age
that could be important to consider in this case?
Correct Answer
D. Decline in pulmonary vascular resistance
Explanation
closing of foramen ovale occurs at, or shortly after, birth. Closing of ductus arteriosus and ductus venosus occur shortly after birth. The decline in pulmonary vascular resistance can take a few weeks.
2.
A 4-week old girl presents with poor
feeding and tachypnea. The infant was born at term after a normal
pregnancy. At 2 weeks of ago, a cardiac murmur was noted, but the baby
was otherwise well. However, over the last week she has become more
tachypneic and is feeding poorly. Her family is concerned that she takes
up to 50 minutes to feed and becomes sweaty and tachypneic during feedings.
There is no history of cough or fever. On physical exam, weight
3.2kg (>5th %ile), length 53cm (~25th %ile), temp 36.5, HR 160, RR 64, BP
(right arm) 80/40, O2 sat 92%. She is pale and diaphoretic. There
is subcostal indrawing. The cardiac apex is just lateral to the
mid-clavicular line at the 5th left intercostal space, and the precordial
activity is increased. The heart sounds are normal, a harsh 3/6
pansystolic murmur is present. The liver is 4cm below the right costal
margin. All of the following are causes of a
pansystolic murmur EXCEPT
Correct Answer
C. Pulmonary stenosis
3.
A 4-week old girl presents with poor feeding and tachypnea.
The infant was born at term after a normal pregnancy. At 2 weeks of
ago, a cardiac murmur was noted, but the baby was otherwise well.
However, over the last week she has become more tachypneic and is feeding
poorly. Her family is concerned that she takes up to 50 minutes to feed
and becomes sweaty and tachypneic during feedings. There is no history of
cough or fever. On physical exam, weight 3.2kg (>5th %ile), length
53cm (~25th %ile), temp 36.5, HR 160, RR 64, BP (right arm) 80/40, O2 sat 92%.
She is pale and diaphoretic. There is subcostal indrawing.
The cardiac apex is just lateral to the mid-clavicular line at the 5th
left intercostal space, and the precordial activity is increased. The
heart sounds are normal, a harsh 3/6 pansystolic murmur is present. The
liver is 4cm below the right costal margin.Where should the pediatric cardiac apex be?
Correct Answer
B. 4th intercostal space
4.
A 4-week old girl presents with poor feeding and tachypnea.
The infant was born at term after a normal pregnancy. At 2 weeks of
ago, a cardiac murmur was noted, but the baby was otherwise well.
However, over the last week she has become more tachypneic and is feeding
poorly. Her family is concerned that she takes up to 50 minutes to feed
and becomes sweaty and tachypneic during feedings. There is no history of
cough or fever. On physical exam, weight 3.2kg (>5th %ile), length
53cm (~25th %ile), temp 36.5, HR 160, RR 64, BP (right arm) 80/40, O2 sat 92%.
She is pale and diaphoretic. There is subcostal indrawing.
The cardiac apex is just lateral to the mid-clavicular line at the 5th
left intercostal space, and the precordial activity is increased. The
heart sounds are normal, a harsh 3/6 pansystolic murmur is present. The
liver is 4cm below the right costal margin. Are you concerned about hepatomegaly?
Correct Answer
A. Yes
Explanation
yes, 4cm below is ENORMOUS considering total torso size of a 4-week old baby!
5.
A 4-week
old girl presents with poor feeding and tachypnea. The infant was born at
term after a normal pregnancy. At 2 weeks of ago, a cardiac murmur was
noted, but the baby was otherwise well. However, over the last week she
has become more tachypneic and is feeding poorly. Her family is concerned
that she takes up to 50 minutes to feed and becomes sweaty and tachypneic
during feedings. There is no history of cough or fever. On physical
exam, weight 3.2kg (>5th %ile), length 53cm (~25th %ile), temp 36.5, HR 160,
RR 64, BP (right arm) 80/40, O2 sat 92%. She is pale and diaphoretic.
There is subcostal indrawing. The cardiac apex is just lateral to
the mid-clavicular line at the 5th left intercostal space, and the precordial
activity is increased. The heart sounds are normal, a harsh 3/6
pansystolic murmur is present. The liver is 4cm below the right costal
margin.
This
CXR shows all of the following EXCEPT
Correct Answer
E. Tracheal deviation
Explanation
the CXR shows mild cardiomegaly with increased pulmonary vascularity, and mild pulmonary edema. Bone and soft tissues are normal. There is a nasogastric tube in stomach.
6.
Which of the following is seen in this ECG (check
all that apply)
Correct Answer(s)
A. Sinus tachycardia
B. Right ventricular hypertrophy
C. Left ventricular hypertrophy
E. Normal axis
Explanation
sinus tachycardia (HR 150-160 bpm) and biventricular hypertrophy (large voltages throughout the precordial leads). Upright T wave in v6 mandatory, inverted T waves in v1-v3 are appropriate. V1 will be the last one to flip upright, well into puberty. Normal baby axis between II and II (R heart dominant)
7.
A 4-week old girl presents with poor feeding and tachypnea.
The infant was born at term after a normal pregnancy. At 2 weeks of
ago, a cardiac murmur was noted, but the baby was otherwise well.
However, over the last week she has become more tachypneic and is feeding
poorly. Her family is concerned that she takes up to 50 minutes to feed
and becomes sweaty and tachypneic during feedings. There is no history of
cough or fever. On physical exam, weight 3.2kg (>5th %ile), length
53cm (~25th %ile), temp 36.5, HR 160, RR 64, BP (right arm) 80/40, O2 sat 92%.
She is pale and diaphoretic. There is subcostal indrawing.
The cardiac apex is just lateral to the mid-clavicular line at the 5th
left intercostal space, and the precordial activity is increased. The
heart sounds are normal, a harsh 3/6 pansystolic murmur is present. The
liver is 4cm below the right costal margin. Is this a cyanotic lesion?
Correct Answer
B. No
8.
A 4-week old girl presents with poor feeding and tachypnea.
The infant was born at term after a normal pregnancy. At 2 weeks of
ago, a cardiac murmur was noted, but the baby was otherwise well.
However, over the last week she has become more tachypneic and is feeding
poorly. Her family is concerned that she takes up to 50 minutes to feed
and becomes sweaty and tachypneic during feedings. There is no history of
cough or fever. On physical exam, weight 3.2kg (>5th %ile), length
53cm (~25th %ile), temp 36.5, HR 160, RR 64, BP (right arm) 80/40, O2 sat 92%.
She is pale and diaphoretic. There is subcostal indrawing.
The cardiac apex is just lateral to the mid-clavicular line at the 5th
left intercostal space, and the precordial activity is increased. The
heart sounds are normal, a harsh 3/6 pansystolic murmur is present. The
liver is 4cm below the right costal margin. What could be the cause of this baby's
problem?
Correct Answer
E. All of the above
Explanation
The broad differential diagnosis should include shunts (ASD, VSD, AVSD, large PDA, aortopulmonary window, arterio-venous fistula, etc.), left heart obstruction (coarctation, severe aortic stenosis, HLHS, etc.), arrhythamias (especially SVT), and myocardial disease (myocarditis, cardiomyopathy)
9.
Assessing
upper limb vs. femoral pulses and 4 limb BPs would help you steer away from
which cause of CHF?
Correct Answer
A. Left heart obstruction
10.
Echocardiogram shows large perimembranous VSD. Moderate
biventricular hypertrophy with normal function and mild RV/LV dilation.
Increased flow velocity within main pulmonary artery secondary to VSD
shunt, small PFO. When do most large VSDs require repair?
Correct Answer
B. 4-6 months of age
11.
Echocardiogram shows large perimembranous VSD. Moderate
biventricular hypertrophy with normal function and mild RV/LV dilation.
Increased flow velocity within main pulmonary artery secondary to VSD
shunt, small PFO. When do most small VSDs require repair?
Correct Answer
A. Never - often close spontaneously
Explanation
most large VSDs require repair around 4-6 months of age (definitely before 1 year of age) or around 5kg in weight. Medical management is used to control CHF to allow adequate weight gain pre-op. The latter includes the use of diuretics (lasix, aldactazide) to reduce pulmonary edema. NG tube feedings to optimize caloric intake and reduce the work of feeding. Some use digoxin as that may improve heart failure symptoms. Note: CHF in patients with L-R shunts differs from patients with pump failure, such as in the case of myocarditis and cardiomyopathy. The cardiac contractility in the former is usually normal. Small VSDs often close spontaneously and do not cause heart failure as the shunting is minimal. A murmur is heard until the VSD closes.
12.
4 hour old term male infant is seen because of "duskiness"
as well as a heart murmur. The infant's mother is 30 years of age and has
three other children who are healthy. This pregnancy was uncomplicated
and the baby was delivered at term with a birth weight of 3.9 kg.
Although pink and well-perfused at birth, over the last several hours the
infant has become cyanotic especially when crying. What is the significance of the heart
murmur?
Correct Answer
A. Suggests cardiac cause
13.
4 hour old term male infant is seen because of "duskiness"
as well as a heart murmur. The infant's mother is 30 years of age and has
three other children who are healthy. This pregnancy was uncomplicated
and the baby was delivered at term with a birth weight of 3.9 kg.
Although pink and well-perfused at birth, over the last several hours the
infant has become cyanotic especially when crying. What is the significance of the timing of
the cyanosis?
Correct Answer
D. All of the above
14.
4 hour old term male infant is seen because of "duskiness"
as well as a heart murmur. The infant's mother is 30 years of age and has
three other children who are healthy. This pregnancy was uncomplicated
and the baby was delivered at term with a birth weight of 3.9 kg.
Although pink and well-perfused at birth, over the last several hours the
infant has become cyanotic especially when crying. Why does he become more cyanotic when
crying?
Correct Answer
B. Increased oxygen demand
15.
4 hour old
term male infant is seen because of "duskiness" as well as a heart
murmur. The infant's mother is 30 years of age and has three other
children who are healthy. This pregnancy was uncomplicated and the baby
was delivered at term with a birth weight of 3.9 kg. Although pink and
well-perfused at birth, over the last several hours the infant has become
cyanotic especially when crying. Weight 3.6kg, RR 55, HR 140. BP 73/41,
O2 sat on room air 74%. Pulses are normal. The precordium is quiet.
The right ventricular activity is increased. There is a harsh grade
3/6 systolic ejection murmur along the upper left sternal border. The
second heart sound appears single. There are no crackles or wheezes.
all of the following are seen on the CXR EXCEPT
Correct Answer
B. Edema
16.
This
ECG shows which of the following?
Correct Answer
A. Right atrial enlargement
Explanation
right atrial enlargement and right ventricular hypertrophy with strain.
17.
You
do a hyperoxic test: on 100% oxygen, O2 sat goes from 74% on room air to 80%.
What is the significance of this result?
Correct Answer
B. Cardiac cause of cyanosis
Explanation
In a cardiac R-L shunt, systemic venous blood bypasses the lung and is not oxygenated no matter how much inspired oxygen is given to teh patient, so there is little or no increase in PaO2 with 100% oxygen.
1. Measure Pa)2 in room air (21% O2)
2. Give 100% O2 for 10 minutes
3. Re-measure PaO2.
Interpretation: Normal before 90, after >500,
lung disease before 60-90, after >150,
R-L shunt before
18.
Match the findings with the appropriate cardiac disorder: Normal S1, fixed split S2, grade 2-3/6 systolic ejection murmur, on ECG normal rhythm, normal axis, incomplete right bundle branch block
Correct Answer
A. ASD
19.
Match the findings with the appropriate cardiac disorder: Normal S1 and S2, harsh grade 2-4/6 pansystolic murmur, ECG normal rhythm, normal axis, biventricular enlargement/hypertrophy
Correct Answer
B. VSD
20.
Match the findings with the appropriate cardiac disorder: Normal S1 and S2, harsh continuous "machinery" type murmur, ECG: normal rhythm and axis, left atrium and/or ventricular enlargement/hypertrophy
Correct Answer
D. PDA and AP window
21.
Match the findings with the appropriate cardiac disorder: Normal S1 and S2, loud grade 3-4/6 systolic ejection murmur (may also be holosystolic murmur if significant valve regurgitation). ECG: normal rhythm, left axis deviation, biventricular enlargement/hypertrophy
Correct Answer
C. AVSD
22.
Match the findings with the appropriate cardiac disorder: Normal S1, single or narrowly split S2, harsh grade 3-4/6 systolic ejection murmur. ECG: normal rhythm, right axis deviation, right ventricular hypertrophy.
Correct Answer
A. Tetralogy of Fallot
Explanation
Repair: If very cyanotic, will need a BT shunt to augment pulmonary blood flow in first few days of life, with prostin to maintain ductal patency until surgery. Some centers do complete repair as a neonate even if the baby is tiny. Otherwise, get them to a good size, 4-6 months ideally, and then do complete repair (close VSD with a patch, and place patch over the R ventricular outflow tract to open up the pulm artery)
23.
Match the findings with the appropriate cardiac disorder: Normal S1, single S2, no murmur. ECG: normal rhythm and axis, may see predominant right-sided forces.
Correct Answer
C. Transposition of Great Arteries
Explanation
cyanotic shortly after birth as systemic and pulm circulation in parallel. Need mixing of blue and red blood to survive initially, in the form of a big patent foramen ovale (PFO). If the PFO has closed or is very small, the baby will need an urgent balloon septostomy to open up a good sized interatrial communication to stabilize the baby and increase the saturations (often in the 50-60% at birth, stabilize in 70-80% range). Complete repair with an arterial switch procedure in the first 2 weeks of life.
24.
Match the findings with the appropriate cardiac disorder: Normal S1, single S2, no murmur or soft systolic ejection murmur. ECG: normal rhythm and axis, may be left ventricular enlargement
Correct Answer
B. Truncus
Explanation
Repair: develop heart failure early as high pressure flow to pulmonary arteries. Repair early (close VSD, create a conduit from the RV to the pulm arteries using a goretex tube)