Radiological Category: Pediatrics
Principal Modality (1): General Radiography
Principal Modality (2): MRI
Case Report
Submitted by:
Todd Danziger, MS IV
Faculty reviewer:
Sandra Oldham, M.D.
Date accepted:
28 September 2011
Case History
Infant History: 37 week premature girl born at OSH via repeat caesarian section
with birth weight 2.645 kg and APGARs 8 @ 1 min and 9 @ 5 min. Developed
respiratory distress and hypoxia after birth and was transferred to NICU, where
she was initially placed on NCPAP and then eventually intubated. ECHO was
obtained at OSH and patient was transferred to MHCH via air transport for
higher-level of care.
Maternal History: 22 yo G2P1
Prenatal Labs: Blood: O+, Ab neg, RPRNR, HepB neg, HIV neg, GBS neg, rubella
equivocal, UDS neg
36 week Ultrasound: AFI 7.2 cm, hydronephrosis of right kidney
Case History
P.E.: T 99.6
BP 46/32
MAP 36
P 148
R 19-66
SpO2 86
FiO2 21%
Gen: NAD, sedated, under radiant warmer
HEENT: Normocephalic, oral mucosa moist, ears normally set and rotated
Resp: Lungs clear to auscultation
CV: Normal rate, regular rhythm, systolic murmur 2/6 best heard over LUSB, good
pulses in all extremities, normal peripheral perfusion
GI: soft, non-distended, no organomegaly, anus patent
GU: normal female
Integument: warm, pink, no rash
Neurologic: No focal defects, sedated
Radiological Presentations
Chest/Abdomen
Pediogram
Radiological Presentations
A/P Portable
Test Your Diagnosis
Which one of the following is your choice for the appropriate diagnosis? After
your selection, go to next page.
• Meconium Aspiration
• Neonatal Respiratory Distress Syndrome
• Transient Tachypnea of the Newborn
• Persistent Pulmonary Hypertension of the Newborn
• Congenital Heart Disease
Radiological Presentations
MRI heart
Radiological Presentations
MRI heart
Radiological Presentations
MRI heart
Radiological Presentations
MRI heart
Findings and Differentials
Findings:
Initial CXR: normal-sized heart with normal pulmonary vascularity with right
descending aorta
Later CXR: upturn of the cardiac apex . . . Boot-shaped heart
ECHO: moderate VSD, moderate aortic override, pulmonary atresia with a hypoplastic
main pulmonary artery and RV outflow tract, and mild biventricular hypertrophy. Arch
sidedness could not be determined. Repeat ECHO suggested a double aortic arch.
Cardiac MRI: TOF (VSD, pulmonary atresia, overriding aorta, RVH); double aortic arch
with descending aorta on the right; PDA
Differentials:
• Congenital Heart Disease (Tetralogy of Fallot)
•Meconium Aspiration Syndrome
• Neonatal Respiratory Distress Syndrome
• Transient tachypnea of the newborn
• Persistent Pulmonary Hypertension of the Newborn
Discussion
Meconium Aspiration Syndrome
Clinical History:
-Meconium-stained amniotic fluid in a term or post-term infant
-Respiratory distress: tachypnea, hypoxia, and hypercapnia
-May develop pulmonary hypertension and require positive pressure ventilation
Radiographic finding: Air trapping and
patchy atelectasis
Wiedemann J R, A M Saugstad, L BarnesPowell, and K Duran. Meconium Aspiration
Syndrome. Neonatal Network 2008, 27: 81-87.
Discussion
Neonatal Respiratory Distress Syndrome or Hyaline Membrane Disease
Clinical history: Premature infant with tachypnea, grunting, nasal flaring, chest
wall retractions, and cyanosis in first few hours of life
Pathogenesis:
-Surfactant deficiency
-Poor compliance
-Atelectasis, intrapulmonary shunting,
hypoxemia, and cyanosis
-Hyaline membrane: mechanical ventilation,
oxygen exposure, and alveolar
capillary leak
Radiographic findings: Uniform
reticulonodular or ground-glass
pattern and air bronchograms
Treatment: surfactant
Hermansen CL, Lorah KN. Respiratory Distress
in the Newborn. American Family Physician
2007, 76: 987-994
Discussion
Transient tachypnea of the newborn
Most common etiology of neonatal respiratory distress (>40%)
Secondary to residual pulmonary fluid remaining in the fetal lung
Risk factors: maternal asthma, maternal diabetes, macrosomia, and cesarean
delivery
Tachypnea begins soon after birth
and can last several hours to days
Radiographic findings:
-diffuse parenchymal infiltrates
-“wet silhouette”
-intralobar fluid accumulation and
fluid in fissures
Hermansen CL, Lorah KN. Respiratory Distress
in the Newborn. American Family Physician
2007, 76: 987-994
Discussion
Persistent Pulmonary Hypertension of the Newborn
Also called persistent fetal circulation
Pathogenesis:
-Acute or chronic hypoxia in utero
-Failure of pulmonary vascular resistance to fall with postnatal lung expansion and
oxygenation
-Worsening right-to-left shunt
Present with hypoxemia, cyanosis, and acidosis
Radiographic findings: depend on cause
ECHO: absence of structural heart disease, evidence of increased pulmonary
vascular resistance, and right-to-left shunt at foramen ovale and/or ductus
arteriosus
Treatment: Inhaled nitric oxide; ECMO for most severely ill
Marino BS Fine KS. Blueprints Pediatrics, 5th
ed. Baltimore: Lippincott Williams & Wilkins,
2009.
Discussion
Tetralogy of Fallot
Most common cyanotic heart disease
1.
2.
3.
4.
Obstruction of right ventricular outflow tract
Ventricular septal defect (VSD)
Overriding of the aorta above the VSD
Right ventricular hypertrophy
5. Right aortic arch in 20-30%
“Tet spells”: periodic episodes of cyanosis, rapid and deep breathing, agitation
from increase in right ventricular outflow tract resistance
Haider EA. The Boot-shaped Heart Sign.
Radiology 2008, 246: 328-9
Discussion
Pathogenesis
Anterosuperior displacement of the infundibular septum
Aortic valve forms superior border of VSD
Subpulmonic stenosis and right-to-left shunt
Right ventricular hypertrophy
Pulmonary arteries become hypoplastic and the aorta grows larger
Kumar et al. Robbins and Cotran Pathologic
Basis of Disease, 8th ed. Philadelphia:
Saunders, 2010: 542-3.
Photos from Yale Medical Group
Discussion
Boot-shaped heart sign (65%)
Sign of decreased pulmonary
vasculature
Upturned cardiac apex from right
ventricular hypertrophy
Small or absent main pulmonary
artery
Accentuated by a small thymus
Only seen in infants with
moderate to severe cases
Upturn of the apex increases with
severity of RV outflow tract
obstruction
RV failure is rare
Haider EA. The Boot-shaped Heart Sign.
Radiology 2008, 246: 328-9
Diagnosis
Tetralogy of Fallot
References
Haider EA. The Boot-shaped Heart Sign. Radiology 2008, 246: 328-9
Hermansen CL, Lorah KN. Respiratory Distress in the Newborn. American Family
Physician 2007, 76: 987-994.
Kumar et al. Robbins and Cotran Pathologic Basis of Disease, 8th ed. Philadelphia:
Saunders, 2010: 542-3.
Marino BS Fine KS. Blueprints Pediatrics, 5th ed. Baltimore: Lippincott Williams &
Wilkins, 2009.
Wiedemann J R, A M Saugstad, L Barnes-Powell, and K Duran. Meconium Aspiration
Syndrome. Neonatal Network 2008, 27: 81-87.