Jolo Part

Case 5.
NEONATE with CYANOTIC HEART DISEASE and its MANAGEMENT
A male infant was born via spontaneous vaginal delivery at 39 3/7 weeks’ gestation to
a 24-year-old G2 P0 woman with reassuring prenatal screens, including negative
Group B Streptococcus status. The membranes had spontaneously ruptured 4 hours
prior to delivery and the mother did not have a fever. The infant’s APGAR scores
were 8 and 9 at 1 and 5 minutes, respectively. The infant stayed with his mother and
attempted breastfeeding. During the second breastfeeding attempt at 4 hours of age,
the nurse noted that he had tachypnea and cyanosis.
1. Of the following, the preferred initial assessment to determine whether this

infant’s cyanosis results from a respiratory or cardiac cause is:
a. Complete blood count, blood culture, and arterial blood gas (ABG)
b. Echocardiography
c. Family history
d. Physical examination, chest radiograph, electrocardiogram, hyperoxia test.
This infant’s hyperoxia test results were consistent with cyanotic heart
disease. All of the following exams are needed not just for initial but also for
confirmatory diagnostic test
2. Of the following, the technique that best describes the hyperoxia test is:
a. Assessment of the amount of supplemental oxygen required to obtain an

oxygen saturation ≥95%
b. Measurement of the infant’s arterial Pao2 in room air
c. Measurement of the infant’s arterial Pao2 while receiving 50% Fio2
d. Comparison of the infant’s arterial Pao2 in room air with the infant’s Pao2
while receiving 100% Fio2. In patients with pulmonary disease, PaO2
generally increased greater than or equal to 100 mm Hg with 100% oxygen
as ventilation-perfusion discrepancies are overcome. A positive result
indicates the cardiac origin and further cardiac workup is indicated to rule
out CCHD.
3. Of the following, the congenital cardiac lesions that present with cyanosis are:
a. Atrial septal defect (ASD), ventricular septal defect (VSD), aortic stenosis
(AS), coarctation of the aorta.
b. Critical pulmonary stenosis (PS), truncus arteriosus, pulmonary atresia (PA)
with intact ventricular septum, Ebstein anomaly, total anomalous pulmonary
venous return (TAPVR) with obstruction
c. Hypoplastic left heart syndrome (HLHS), transposition of the great arteries
(TGA), tricuspid atresia, TOF with PA, TAPVR without obstruction, single
ventricle
d. B and C. These cardiac anomalies shunts the blood from right to left, that is
why cyanosis is the usual indication of these anomalies. In these process,
deoxygenated blood shunts to the left part of the heart and go with the
systemic circulation, which makes the infant cyanotic in color.
4. Of the following, the congenital cardiac lesions associated with decreased
pulmonary blood flow are:
a. ASD, VSD, AS, coarctation of the aorta, atrioventricular canal (AVC)
without PS
b. Critical PS, tricuspid atresia, PA, TOF, Ebstein anomaly, TAPVR with
obstruction
c. HLHS, TGA, truncus arteriosus, TOF with PA, TAPVR without obstruction,
single ventricle
d. B and C. The anomalies mentioned above are the types of congenital health
diseases that lessen the flow of the blood to the lungs
The infant’s examination was notable for severe cyanosis, nondysmorphic features,
and absence of murmur. He had a preductal oxygen saturation (right arm) that was
20% lower than his postductal oxygen saturation (leg). The infant’s chest radiograph
is shown in Figure 1. His electrocardiogram had a normal QRS axis.
Echocardiography revealed a cyanotic heart defect.
FIGURE 1. Eisenberg L. An Atlas of Differential Diagnosis. 4th ed.

Philadelphia, PA: Lippincott Williams & Wilkins; 2003. Figure 10.2
5. Of the following, the most appropriate next step(s) in the management of this
infant is (are):
a. Consult the cardiology service

b. Educate parents about the lesion and discuss that a Rashkind procedure may
be needed to increase the size of the foramen ovale
c. Initiate an intravenous prostaglandin (PGE1) infusion to maintain patency of
the ductus arteriosus
d. All of the above. All of the steps above are important steps and should be
done after the assessment because the infant needs immediate response to
address the condition. It should be done as soon as possible with help of the
mentioned steps above tp0 give cure and increase life expectancy.
6. Of the following, the possible side effects of an intravenous PGE1 infusion is

(are):
a. Apnea
b. Flushing
c. Hypotension
d. All of the above. Prostaglandin E1 is a potent vasodilator of all arterioles.
Other effects include inhibition of platelet aggregation, and stimulation of
uterine and intestinal small muscle. Alprostadil (Prostaglandin E1) is rapidly
cleared by metabolism, primarily occurring in the lungs, and excretion via
the kidney.Maximal drug effect usually seen within 30 minutes in cyanotic
lesion: may take several hours in acyanotic lesions.

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Case 5.

NEONATE with CYANOTIC HEART DISEASE and its MANAGEMENT

1. Of the following, the preferred initial assessment to determine whether this

a. Assessment of the amount of supplemental oxygen required to obtain an

FIGURE 1. Eisenberg L. An Atlas of Differential Diagnosis. 4th ed.

a. Consult the cardiology service

6. Of the following, the possible side effects of an intravenous PGE1 infusion is

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