Objectives:
To know:
Remember- Normal ECG and normal pulmonary vascular markings on chest x-ray
films do not rule out CHD .
Presentations of CHD:
Findings that should alert to the possibility of serious CHD in a newborn include:
N.B.
Other:
I. Chest radiograph:
1. Heart size
Determined using the cardiothoracic (CT) ratio.
CT ratio = (A + B) ÷ C
Where A and B are maximal cardiac dimensions to the right and left of the
midline, respectively, and C is the widest internal diameter of the chest .
Note,
A. B.
Figure 7- 4. A Posteroanterior view of the chest roentgenogram from a 2-month-
old infant with complete transposition of the great arteries. Note cardiomegaly
(cardiothoracic ratio of 0.7), “egg-shaped” heart with narrow waist, and increased
pulmonary vascular markings, which are characteristic of this condition . B. a
diagram of “Egg-shaped” cardiac silhouettes TGA.
A. B.
a) Tricuspid atresia: Normal size or mild cardiomegaly with straight right heart
border with decreased pulmonary vascular markings
Total anomalous pulmonary venous return: Cardiomegaly with right atrial and
ventricular enlargement and increased pulmonary vascular markings. "Snowman"
heart (or figure-of-8 configuration) can be seen with supracardiac, unobstructed
veins
Figure 7-8. (A) Posteroanterior view of plain chest film demonstrating the
“snowman” sign (B) Angiocardiogram demonstrating anatomic structures that
participate in the formation of the snowman sign; the vertical vein (left superior
vena cava), the dilated left innominate vein, and the right superior vena cava are
opacified. (C) A diagram of snowman” sign of cardiac silhouette).
o VSD: 2-6%.
Figure 7-10. An x-ray film of the chest and upper abdomen of a newborn infant
with polysplenia syndrome. Note a symmetrical liver (“midline liver”), a
stomach bubble in the midline, dextrocardia, and increased pulmonary
vascularity.
II. Electrocardiogram:
It is essential to refer to age-specific charts of normal values for most ECG
parameters.
III. Monitoring:
O2 saturation by pulse oximetry, both pre-ductal (right hand) and post-ductal
(Right foot).
In contrast to older children and adults, CO2 may be elevated in newborn infants
with congestive heart failure
1. Echocardiography
Providing information about the structure and function of the heart and great
vessels in a timely fashion.
As the procedure may take an hour or more to perform, and may therefore not
be well tolerated by a sick and/or premature newborn, pay attention to
Truncus arteriosus
Management:
In some cases, the hyperoxia test must be done with the administration of
positive-pressure ventilation to expand atelectatic lung adequately to
exchange gas.
5. Assess right and left sided SaO2 for any ductal difference (Bowman and Fraser,
2006).
Arterial PO2 from the right radial artery that is 10 to 15 mm Hg higher than
that from an umbilical artery catheter is significant.
In severe cases of right-to-left ductal shunt, differential cyanosis may be
noticeable, with a pink upper and a cyanotic lower body. Such a right-to-left
ductal shunt is caused by
1. Persistent pulmonary hypertension of the newborn (PPHN)
2. Aortic obstructive lesions (such as interrupted aortic arch, coarctation of
the aorta [COA]).
6. CXR and ECG, if possible, are often useful in reducing the diagnostic possibilities
7. Intubation and paralysis if significant distress.
8. Two IV lines ideally or UVC.
9. Parental antibiotics (preferably after blood cultures taken).
10. Prostaglandin E1 (Prostin VR ®) infusion if a cyanotic congenital heart defect or
a ductus-dependent cardiac defect is suspected . (See below for duct
dependent lesions).
Note,
Differential cyanosis (i.e. pink upper body and blue lower body):
In these situations, the descending aorta is filled with oxygenated blood from the
pulmonary system because of right-to-left shunting through the ductus arteriosus.
The most common causes are right-sided obstructive lesions that result in
inadequate blood flow to the lungs.
Tetralogy of Fallot (TOF) (with or without pulmonic atresia). In mild cases,
cyanosis may not be present at birth (acyanotic Fallot, “pink tet”).
Tricuspid atresia, pulmonic atresia with intact ventricular septum, severe
pulmonic stenosis.
Ebstein’s anomaly of the tricuspid valve (Bartman and Teitel, 2003).
Age of presentation varies depending on the lesion.
For example, Tricuspid atresia presents rapidly immediately after birth, mild
forms of Ebstein’s anomaly may not present until later in life.
For these infants, oxygen saturations in the 75-85% range are adequate.
Infants with severe right ventricular outflow tract (RVOT) obstruction can become
significantly cyanotic when PDA closes.
1. 100% Oxygen.
2. Positioning- knee-chest position.
3. Sedation with midazolam 0.05–0.1 mg/kg (IV, intranasal or intrarectal)
fentanyl 1–2 mg/kg (IV), or morphine 0.1 mg/kg (IM, IV, SC) .
4. Beta-blockers: Propranolol.
Propranolol is given by mouth for preventing cyanotic spells.
Dose:
By mouth; 0.25–1 mg/kg 2–3 times daily, max. 2 mg/kg 3 times daily.
The onset of signs relates to closure of the ductus arteriosus which may not occur
until a few days after birth.
The most common types of CHD that cause shock are left-sided obstructive
lesions.
Inotropes:
Postoperative management:
I. Ductal-dependent abnormalities
1. Hypoplastic left heart syndrome with a large PDA.
2. Truncus arteriosus.
3. Interrupted aortic arch type B (i.e., interruption between the left common
carotid artery and the left subclavian artery).
4. Coarctation of the aorta with a VSD.
5. Critical aortic stenosis.
II. Non-ductal-dependent abnormalities
TAPVR.
Endocardial cushion defect.
Single ventricle complex.
Myocardial dysfunction (cardiomyopathy).
Supraventricular tachycardia/arrhythmia.
Diagnosis:
CHF is a clinical diagnosis, made on the basis of the existence of certain signs and
symptoms rather than on radiographic or laboratory findings, although these may
be supportive evidence for the diagnosis
Presentation:
o Early:
Tachypneia and tachycardia with an increased respiratory effort and rales.
Cardiomegaly.
Hepatomegaly.
Delayed capillary refill.
Weak peripheral pulses.
Diaphoresis.
Feeding difficulties.
Edema is rarely seen.
Growth failure (Wechsler and Wernovsky, 2008).
o Late: Cyanosis may also occur, making diagnosis more difficult.
o Finally, CHF may present acutely with cardiorespiratory collapse, particularly in
“left-sided” lesions.
o Hydrops fetalis is an extreme form of intrauterine CHF.
Investigations: (Only as a supportive evidence for the diagnosis).
Laboratory Studies
Cardiomegaly and alveolar edema with pleural effusions and bilateral infiltrates
in a butterfly pattern.
Other signs are haziness of hilar shadows, vascular redistribution and
thickening of interlobular septa
Echocardiography
Table 7-7. Doses and effect of inotrops (Wechsler and Wernovsky, 2008).
HR = heart rate; BP = blood pressure; SVR = systemic vascular resistance; PVR = pulmonary vascular
resistance
A combination of low-dose dopamine (up to 5 µg/kg/minute) and dobutamine
may be used to minimize the potential peripheral vasoconstriction induced by
high doses of dopamine while maximizing the dopaminergic effects on the
renal circulation.
Care should be taken to repletion of intravascular volume before institution
of vasoactive agents (Wechsler and Wernovsky, 2008).
Inotropic support must be used judiciously and with caution in the setting of
patients with heart failure (Satou and Halnon, 2009).
Note, Although increased inotropy results in improved cardiac output and blood
pressure, it comes at the expense of increased myocardial oxygen consumption
and demand.
Digoxin
Milrinone:
Loading: 75 mcg/kg IV over 60 min, immediately followed by
Adjunctive therapy:
Correct acidosis with the administration of fluid and/or bicarbonate.
Calcium should be administered when hypocalcemia is documented
V.Heart murmurs:
Not all newborns with murmurs have CHD.
Cyanosis.
Tachypnea without respiratory distress.
Adequate perfusion initially.
Lesions:
Prostavasin® 20 μg /ampoule.
Dose:
Apnea (12%) is the most common complication and is due to a direct effect
of PGE1 on the CNS.
The vast majority of infants who require PGE1 will also need assisted
ventilation, either for the severity of their CHD or because of apnea.