Pediatric Cardiology (4%)

Mother brings in 1wk old NBN b/c of 1D history of difficulty breathing and discoloration of extremities. Appears ill, Temp = 97.5, Pulse = 160, Resp = 52, BP = 60/36 in upper extremities and unobtainable in lower extremities. Skin, mucous membranes, and nail beds are dusky. There is also mottled discoloration of the extremities. Moderate intercostal retractions and grunting. Lungs clear. Cardiac: Holosystolic murmur on left sternal border. Liver edge palpable 4cm below costal margin. ABG: pH = 7.15, CO2 = 28, O2 = 98
Intubation, mechanical ventilation, IVF started, but no improvement an 1hr later. XR shows cardiomegaly and pulmonary congestion. What is the most likely cause and Dx. in this NBN?
Hypoplastic Left Heart Syndrome
Cause: Closure of PDA
Poor Perfusion: lactic acidosis, HF, ❤️shock
Cyanosis w grayish color, poor pulses.
Hyperdynamic ❤️pulses
CXR: ❤️ Enlargement, prominent pulmonary vasculature
ECG: RA/RV hypertrophy
⭐️ECHO is Diagnostic
Use PGE1 to maintain PDA

This cardiac anomaly is associated with DiGeorge
Truncus Arteriosus

1 month old F seen by you in the office b/c of dyspnea, wheezing and FTT. The pt. is <3% for age and height but eating well up until this morning. On exam, There is is a harsh holosystolic murmur on the LUSB. Peripheral pulses are strong and bounding. What is the most likely Dx?
Truncus Arteriosus
Associated with VSD
Left-Right shunt ⬆, pt. present w Left ❤️ failure, dyspnea, wheezing, FTT.
Cyanosis not really there.
Heart is hyperdynamic, peripheral pulses strong, bounding.
CXR: Cardiomegaly, Pulmonary Edema
⭐️ECHO is Diagnostic
Tx: Medical management of HF, Surgery.
OR Death…between 3-12m of life
Note: Valved conduit (to fix the single arterial trunk) needs changing at 3-7y of life to allow for growth

At birth, an infant has a relatively thick right ventricle. What does this mean for ECG findings?
QRS axis points anteriorly and to the right. RAD, large R waves in right precordium

6 y/o Male sent to you b/c of HTN. On exam, you hear a short systolic murmur along the LSB of the 3,4th ICS. You also notice has weak, delayed femoral pulses. B/Ps are high. What finding would you see on CXR?
Coarctation of the Aorta
CXR: Notching of the ribs
CXR: Looks like a ⓷
⓷systers (Systolic ejection murmurs)
Do ECHO first!!!
CoA may present w HTN or Murmur. Epistaxis, Claudication-like symptoms in Lower Extremities w Exercise.

What are some of the complications of CoA
Untreated CoA complications include: HTN, Rupture of a berry aneurysm, HF, endocarditis and rupture of the Aorta (in Adults).

CoA in NBNs presents as HF or Cardiogenic shock.
CoA in infancy, like Severe Aortic Stenosis
Septic Shock appearance.
Murmurs not common. With Patent ductus, cont. murmur along LSB, mid-back
PGE1 is stabilizing

On CXR you see a water bottle like figure.
ECG shows Low voltage complexes w RBBB and RAD.
What Cardiac anomaly do you find this in?
Ebstein Anomaly
WATER BOTTLE
Peaked P waves II, VI = RAH
Downward displacement of Abnormal Tricuspic Valve into RV

What is the MC known cause of Ebstein Anomaly?
What is the MC known cause of Ebstein Anomaly?
Lithium (the med)

Upon examination of a 4y/o ♀, you hear a low pitched vibratory systolic ejection murmur Grade II in the LLSB. It has a musical Quality without radiation. The murmur get louder when your patient is supine and decreases when she is standing.
Still’s Murmur: Chorda-Tendinae Vibration

Musical Murmur at LLSB toward apex. It decreases when pt. sits up:
Stills Murmur (Vibratory, Musical)
Music be Still

FT NBN M noted to have cyanosis at approximately 40minutes of life. On exam, there's is no murmur. You order a CXR which shows a narrow mediastinum, egg on a string.
FT NBN M noted to have cyanosis at approximately 40minutes of life. On exam, there’s is no murmur. You order a CXR which shows a narrow mediastinum, egg on a string.
Transposition of Great Arteries.
Pulmonary venous return to RA, ASD.
Widely fixed S2 (single loud S2)
MC NO MURMUR
Can hear a soft-systolic ejection murmur @ mid-left sternal border

What changes do you see on EKG with Hypokalemia?
What changes do you see on EKG with Hypokalemia?
Prolonged QT, flattened T-waves, ST segment depression, U-waves, and PVCs

What is the Tx. for children with SVT if not ablated?
β-blocker (Propranalol) for 1y to prevent recurrence. If patient resistant to Tx, Then Radiofrequency ablation

NBN Male born approximately 1 hour ago, with cyanosis. Upon exam you hear a fixed split S2 and short systolic murmur at the ULSB. You order an XR and there is a ground glass appearance of heart, like a snowman. What is this NBNs Dx?
Total Anomalous Pulmonary Venous return.
EKG shows: RAD, RVH
TAPV: O2 blood returns to RA, increases RV activity. Causes Pulmonary Edema, Normal size heard
WIDE Split Fixed S2

What is characteristic of a HARSH ejection murmur?
Harsh ejection murmur is characteristic of Pulmonary Stenosis

CXR of an infant with idiopathic persistent pulmonary hypertension. Note the clear lung fields with decreased vascularity. Similarly oligemic lung fields would also be expected in conditions with low pulmonary blood flow, such as pulmonary atresia

Initial Management of an Infant with Cyanosis in ER
Severe cyanosis requires urgent supportive tx IVF and NPO
Thermoneutral environment -radiant warmer. Hypoglycemia common-✔️ glucose levels Glucose infusions maintain a > 55 mg/dL
Airway/assisted ventilation considered for infants with respiratory distress, deferred for the comfortable infant
Severe acidosis correct with infusions of Sodium bicarbonate (do ABG first)
Use of 100% O2 should generally be avoided at the outset
Initiating oxygen therapy with 40-60% O2 will allow caregiver to provide support, assess for improvement, seek advice from a cardio

What is a common side effect of PGE1 when administered
Apnea
Also Flushing and Diarrhea

Tetralogy of Fallot
Tetralogy of Fallot

You hear a rumbling diastolic murmur in a 8y ♂along the left axillary line. What is this cardiac condition?
Mitral Stenosis
Present w MR in RF after 10Y

Examination of your pt. reveals an early systolic click in the LSB and RUSB. You can also view, palpate a thrill in the suprasternal notch. What do you find on EKG?
Aortic Stenosis
ECG shows LVH
AS – Early systolic click “SNAP” @ LSB, RUSB. Radiation to neck, Right infraclavicular region.

The primary developmental defect in Tetralogy
of Fallot that is responsible for the
characteristic four cardiac defects is what?
Anterior Displacement of the Infundibular Septum

You discover a heart murmur in a term, 3.1kg male infant
immediately after birth. The infant demonstrates cyanosis and
his oxygen saturation is 65%. His breathing is unlabored.
There is no hepatomegaly or splenomegaly, and the distal
pulses are excellent. A CXR demonstrates clear lung fields and
a normal cardiac silhouette but a narrowed mediastinal
shadow. You order an echocardiogram. Of the following the
most likely diagnosis is:
a. Coarctation of the aorta
b. Complete AV canal defect
c. Hypertrophic cardiomyopathy
d. Large VSD
Ⓔ. Transposition of the great arteries

You are evaluating a 6-hour-old male infant who was born after a term pregnancy and normal delivery and weighs 4 kg. The infant is
comfortable but exhibits mild tachypnea with a RR: 50 and HR: 150. Oxygen saturation is 60% in all extremities and does not increase
significantly with the administration of oxygen via facemask. His lungs are clear, and there are no murmurs, gallops, or rubs. You suspect
transposition of the great arteries. Of the following, the BEST management strategy is:
a. Diuretic therapy intravenously for pulmonary edema
Ⓑ. Increasing left to right (or aorta-pulmonary artery) shunt at the ductus
arteriosus
c. Increasing the right to left shunt at the foramen ovale
d. Increase the right to left (or pulmonary-aorta artery) shunt at the
ductus arteriosus
e. Intubation and mechanical ventilation with an Fi02 of 1.0

You are seeing a 4-week-old previously healthy infant in your office
because of concern about poor feeding. On questioning, the parents
report that the child has developed grunting respirations associated
with feedings, diaphoresis, pallor, and prolonged periods of sleep. On
physical exam, HR: 160, RR: 55, BP (R arm): 75/48, BP (L leg): 88/55,
oxygen sat: 95%. He exhibits tachypnea, rales and retractions, a II/VI
low-pitched holosystolic murmur across the precordium, and a palpable
liver 2 cm below the right costal margin. Of the following, the MOST
likely explanation of the child’s findings of congestive heart failure is:
a. Aortic valve stenosis
b. Coarctation of the aorta
c. Tetralogy of Fallot
d. Transposition of the Great Arteries
Ⓔ. Ventricular septal defect

A 6-year-old previously healthy boy presents with a recent development of nocturnal dyspnea. On questioning of his parents you discover that the child has experienced exercise intolerance, two episodes of syncope while running, poor appetite, and a cough without congestion for the past year. Exam reveals HR: 120, RR: 26, a gallop rhythm, a III/VI high pitched blowing systolic
murmur at the apex, hepatomegaly, and diminished pulses. Chest radiography
documents an enlarged cardiac silhouette with pulmonary vascular congestion.
An echo demonstrates a regurgitant mitral valve with a dilated left ventricle
and markedly reduced systolic contractility. Of the following, the most likely
cause of this child’s dilated cardiomyopathy:
Ⓐ. Congenital mitral valve abnormality
b. Duchenne’s muscular dystrophy
c. Freidreich’s ataxia
d. Rheumatic heart disease
e. Sickle cell disease

The symptom MOST suggestive of early heart failure in a 2-month-old infant is:
a. Apnea
b. Clubbing
c. Pedal Edema
d. Seizure
Ⓔ. Slowed feeding

Included in your rounds today is a 36-hour-old boy who was born at term by normal spontaneous vaginal delivery. His RR 80, HR 168. He has easily palpable bounding pulses in all 4 extremities. His BP 72/30. Precordial examination reveals a
lift and a 3/6 systolic ejection murmur at the upper left sternal border. You also note a murmur over the anterior fontanelle. Of the following, the most likely diagnosis is:
a. Aortic coarctation with congestive heart failure
b. Aortic insufficiency
c. Large VSD with congestive heart failure
Ⓓ. Left-to-right extracardiac shunting with congestive heart failure
e. Right-to-left extracardiac shunting with right heart failure

You are seeing a 6-week-old infant who was born with Trisomy 21 and a large AV septal defect. Over the previous week, she has tired with feeding and has not gained weight. RR: 60, HR 150. Auscultation
reveals mild retractions and a II/VI systolic murmur with a gallop rhythm. The liver is palpable at 2-cm below the costal margin, and the perfusion is good. You decide to increase the caloric content of the formula to 24 kcal/oz, and you contact her pediatric cardiologist to discuss referral for surgical repair. Of the following, the best
therapeutic option while awaiting surgical repair is:
a. Captopril
Ⓑ Furosemide
c. Hydralazine
d. Propranolol
e. Verapamil

You are caring for a 2-year-old girl who has cardiomyopathy and is awaiting cardiac transplantation. She is receiving a continuous infusion of milrinone at 0.5 mcg/kg/min, IV furosemide three times a day, and 2L/min of oxygen administered via nasal cannula. On physical exam,Temperature: 39°C, HR: 130, RR: 30, BP: 80/40, 02 sat: 92%. An arterial blood gas shows a pH 7.35, PaCO2 40, Pa02 50 with a hemoglobin of 8. Of the following, the treatment that can BEST increase her tissue oxygen delivery is:
a. Administration of 10 mg/kg acetaminophen
b. Increased furosemide administration to four times a day
c. Increased oxygen flow to achieve an oxygen sat of 95%
d. Reduction of the milrinone infusion to 0.25 mcg/kg/min
Ⓔ Transfusion with 15 ml/kg of packed red blood cells

Yesterday you received a call from the newborn nursery that they were
referring to you a term infant who was being discharged at 4 days of age.
The female newborn’s birth weight was 3 kg, and the delivery was by repeat
C-Section. Findings on examination at discharge include heart rate,
respiratory, and blood pressures were normal. Her lungs were clear and no murmurs were noted. She was breastfeeding without difficulty. Today, her
mother calls to tell you that she is difficult to awaken, pale, and breathing much more rapidly than she was in the hospital nursery. She has had one wet diaper in the past 12 hours. When you meet them in the emergency department, you note that the infant has cool extremities, weak pulses, and lethargy. Of the following, the most likely cause of this newborn’s condition is:
Ⓐ. Aortic coarctation
b. Atroventricular septal defect
c. Tetralogy of Fallot
d. Transposition of the Great Arteries
e. Ventricular septal defect
** Know that shock maybe the initial finding in a newborn infant with congenital heart disease

A 3-year-old girl presents for a health supervision visit. At birth, she
was diagnosed with hypoplastic left heart syndrome and underwent
uncomplicated, staged surgical palliation. Today, her mother asks you
if her daughter’s heart disease could affect her development. Of the
following, you are MOST likely to advise the mother that:
a. A small percentage of children who undergo neonatal heart surgery
may develop transient motor delay
b. Cognitive problems are rare with neonatal heart surgery
c. Evaluation for developmental delay should wait until after
kindergarten
Ⓓ Speech and behavioral disorders are common among those who
undergo neonatal heart surgery
e. The only children who suffer developmental delay after neonatal
heart surgery are those who have pre- or post-operative
complications

What happens to the peripheral circulation in Hypoplastic Left Heart syndrome?
⬇ Peripheral Circulation

Name 3 Congenital Heart Diseases that you will prescribe PGE1 for
Hypoplastic Left Heart Syndrome
Pulmonary Atresia
Tricuspid Atresia

Pre-ductal
Ⓡ Hand

Post-Ductal
Ⓛ Hand

PPHN direction of shunting
Right ➡ Left Shunting

Examples of CHD that have same Pre-Post Ductal %
Tricuspid Atresia
TAPVR
In TOGV Preductal ⬇ by 10 points

Pulmonary Stenosis
SEM, LUSB
Thrill Common @ 1-2m
Variable click to back and axilla

Critical Aortic Stenosis 3-6% of all cases of CHD Males 4x>females Aortic valve obstruction bicuspid or unicuspid aortic valve Interrupted Aortic Arch 1% CHD ^ assoc. with 22q11 complete discontinuity of proximal and distal portions of aortic arch R->L shunt …

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