Case Report

Submitted by: Todd Danziger, MS IV

Faculty reviewer: Sandra Oldham, M.D.

Date accepted: 28 September 2011

Radiological Category: Principal Modality (1):

Principal Modality (2):

Pediatrics

MRI

General Radiography

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

Chest/Abdomen

Pediogram

Radiological Presentations

A/P PortableRadiological Presentations

• Meconium Aspiration

• Neonatal Respiratory Distress Syndrome

• Transient Tachypnea of the Newborn

• Persistent Pulmonary Hypertension of the Newborn

• Congenital Heart Disease

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MRI heart Radiological Presentations

MRI heart Radiological Presentations

MRI heart Radiological Presentations

MRI heart Radiological Presentations

Initial CXR: normal-sized heart with normal pulmonary vascularity with right descending aortaLater CXR: upturn of the cardiac apex . . . Boot-shaped heartECHO: 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

• Congenital Heart Disease (Tetralogy of Fallot)

•Meconium Aspiration Syndrome

• Neonatal Respiratory Distress Syndrome

• Transient tachypnea of the newborn

• Persistent Pulmonary Hypertension of the Newborn

Findings:

Differentials:

Findings and Differentials

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 Barnes-

Powell, 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.Obstruction of right ventricular outflow tract

2.Ventricular septal defect (VSD)

3.Overriding of the aorta above the VSD

4.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

Discussion

Kumar et al. Robbins and Cotran Pathologic Basis of Disease, 8th ed. Philadelphia: Saunders, 2010: 542-3. Photos from Yale Medical Group

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

Discussion

Tetralogy of Fallot

Diagnosis

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.

References