“Clinical Presentation of Congenital Heart Disease” by Nancy Braudis, RN for OPENPediatrics

“Clinical Presentation of Congenital Heart Disease” by Nancy Braudis, RN for OPENPediatrics


Clinical Presentation of Congenital Heart
Disease by Nancy Braudis. My name is Nancy Braudis, and I am a clinical
nurse specialist in the cardiac ICU at Children’s Hospital Boston. Introduction. Children with congenital heart disease often
present in one of three pathophysiological states. Low cardiac output. This occurs when
there’s a heart defect that obstructs the flow of blood from the heart, or when the
heart muscle is unable to pump effectively. Congestive heart failure. This often occurs
when there is a heart defect that causes an increase in blood flow to the lungs. Cyanosis. This occurs when there is a heart
defect that causes a decrease in blood flow to the lungs. Cardiac Assessment. On admission, a full cardiac assessment should
be completed. This includes a comprehensive history that should identify if there was
a prenatal diagnosis or any significant birth and genetic history. A complete set of vital
signs should be done as a baseline, and a set of blood pressures in all four extremities
should be done to evaluate for any gradient within the aorta. The physical exam includes evaluation of general
color, body temperature, evaluation of heart sounds, and the presence of any murmurs, assessment
of the liver will indicate if there is an overload of fluid on the right side of the
heart, and the quality and strength of pulses. Diagnostic tests should include a chest radiograph,
an electrocardiogram. Other tests may include an echocardiogram or a cardiac catheterization. Low Cardiac Output. Signs of low cardiac output in infants and
children include pale or mottled skin color, irritability, cool and clammy skin, or decreased
level of consciousness, decreased urine output, capillary refill greater than three seconds,
metabolic acidosis, hypoglycemia, and increased serum lactate. Other signs include weak pulses,
temperature instability, or apnea. There may also be evidence of organ dysfunction
such as kidney or liver failure. Late signs include hypotension, cyanosis, anuria, and
altered mental status. Interventions to improve low cardiac output include correcting the
heart rate and restoring normal conduction within the heart, administration of fluids,
correcting acid-base imbalance and electrolyte abnormalities, and using medications to improve
the function of the heart and to reduce the stress on the body. Congestive Heart Failure. Congestive heart failure occurs when the heart
cannot deliver enough blood to meet the demands of the body. The heart attempts to compensate
by increasing the heart rate, thickening the walls of the heart to contract more effectively,
or dilating the heart to increase the volume of blood within the heart to improve cardiac
output. Congestive heart failure occurs in 30% of infants and children with congenital
heart disease and occurs in over 75% of children with complex heart disease. The causes of congestive heart failure include
volume overload of the heart, pressure overload of the heart, and a heart muscle dysfunction.
Some of the clinical signs of congestive heart failure include tachypnea, retractions, nasal
flaring, diaphoresis, pulmonary edema, irritability, change in responsiveness, fatigue, poor feeding,
failure to thrive, tachycardia, cool skin, decreased urine output, an enlarged liver,
periorbital edema, pulmonary effusions, and ascites. Interventions for congestive heart
failure include medications, such as diuretics to eliminate excess fluid, limiting fluid
administration, and improved nutrition to maximize calories. Other interventions include
respiratory support to reduce the work of breathing and temperature control. Cyanosis. Cyanosis occurs when neonates are
unable to tolerate the changes that occur when the blood flow within the heart changes
after birth. Cyanosis may also occur in infants and children with congenital heart defects
that cause a decrease in blood flow to the lungs. Cyanosis: respiratory or cardiac? When cyanosis
occurs with respiratory disease, the cyanosis usually decreases with crying, improves with
oxygen administration, and there are usually signs of respiratory distress. When cyanosis
occurs with cardiac disease, the cyanosis usually increases with crying because the
demand of oxygen increases, but the body is unable to deliver sufficient oxygen. Cyanosis
with cardiac disease does not usually improve significantly with the administration of supplemental
oxygen, and the infants usually breathe fast but do not have evidence of respiratory distress. Tests that can help in the diagnosis of respiratory
or cardiac disease include a 12-lead electrocardiogram, a chest radiograph, and a hyperoxia test that
involves evaluating the oxygen content of the blood with and without supplemental oxygen. Point of clarification. Hyperoxia test. The basic physiology of the
hyperoxia test is that you are going to use supplemental oxygen to distinguish between
cardiac and pulmonary causes of cyanosis. Specifically, the way that the test is done
is that you obtain a preductal arterial blood gas in the cyanotic patient. So that is typically
a radial arterial blood gas to measure the oxygen tension in arterial blood on room air. You then place the patient in an oxygen-rich
environment. So that’s at 100% oxygen for 10 minutes. That can be either via ventilator
or via an oxy hood. After 10 minutes of breathing in oxygen-rich environment, you repeat the
blood gas preductally– so the radial arterial blood gas– and remeasure the partial pressure
of oxygen in arterial blood. Typically, the cutoffs that we use here at
Children’s Hospital Boston is a PaO2, in your arterial blood of greater than 150 indicates
a high likelihood of a known cardiac cause of cyanosis, such as pulmonary venous desaturation.
A PaO2 of less than 150 is very suspicious for a cardiac cause of cyanosis as a right-to-left
shunt cannot be overcome by the provision of supplemental oxygen. Degraded further,
we consider a PaO2 of less than 100 very suspicious for cyanotic congenital heart disease, and
this should trigger cardiac consultation. Signs of cyanosis with congenital heart disease
include bluish discoloration of skin and mucous membranes, increased respiratory rate and
effort, irritability, or lethargy. Other signs include decreased blood flow to the body,
decreased urine output, and metabolic acidosis. Long-term effects of chronic cyanosis include
an increased hematocrit, thickening of the blood, increased risk of clot formation, clubbing
of fingers, and an increase in the release of oxygen to the tissues. Interventions to reduce cyanosis include maintaining
a high hematocrit to maximize oxygen-carrying capacity and fluid administration to reduce
the thickness of the blood. Other interventions include the administration of supplemental
oxygen– close monitoring for air in intravenous infusion lines is essential to reduce the
risk of air traveling to the brain in an infant or child with cyanotic heart disease– and
using alprostodil in neonates to keep the ductus arteriosus open to allow mixing of
blood within the heart. Please help us improve the content by providing
us with some feedback.

5 Replies to ““Clinical Presentation of Congenital Heart Disease” by Nancy Braudis, RN for OPENPediatrics”

  1. I am 32 week pregnant in scanning report they have mentioned that outflow of blood arising from right ventricle. Is it fine please reply I'm worried about my baby

  2. @Menaka , @Prakash @Anba Krishnan – you all need immediately to consult your Pediatric Doctor or better yet Congential Heart Disease specialist at hospitals such as http://www.amritahospitals.org/ . Please do not delay , do it as soon as possible so your baby is safe and healthy

  3. My son is now 4 months old. He has congenital heart disease, Sinus Tachycardia. He's on duretics but it is not helping. His coughing is deep and phlemy and has diaphragm retraction. What do you recommend?

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