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, RN for OPENPediatrics

Learn about the three different pathophysiological states that infants with congenital heart disease may display.
Direct links to chapters:
1:09 Chapter 2: Cardiac Assessment
2:12 Chapter 3: Low Cardiac Output
3:28 Chapter 4: Congestive Heart Failure
5:21 Chapter 5: Cyanosis

Initial publication: June 02, 2015.
Last reviewed: April 3, 2019.

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Content

13.99 -> Clinical Presentation of Congenital Heart Disease by Nancy Braudis.
19.95 -> My name is Nancy Braudis, and I am a clinical nurse specialist in the cardiac ICU at Children's
26.29 -> Hospital Boston.
29.759 -> Introduction.
32.89 -> Children with congenital heart disease often present in one of three pathophysiological
37.89 -> states.
39.39 -> Low cardiac output.
41.39 -> This occurs when there's a heart defect that obstructs the flow of blood from the heart,
46.81 -> or when the heart muscle is unable to pump effectively.
51.66 -> Congestive heart failure.
52.8 -> This often occurs when there is a heart defect that causes an increase in blood flow to the
58.71 -> lungs.
60.489 -> Cyanosis.
62.269 -> This occurs when there is a heart defect that causes a decrease in blood flow to the lungs.
69.55 -> Cardiac Assessment.
73.119 -> On admission, a full cardiac assessment should be completed.
77.46 -> This includes a comprehensive history that should identify if there was a prenatal diagnosis
83.67 -> or any significant birth and genetic history.
87.84 -> A complete set of vital signs should be done as a baseline, and a set of blood pressures
93.21 -> in all four extremities should be done to evaluate for any gradient within the aorta.
99.6 -> The physical exam includes evaluation of general color, body temperature, evaluation of heart
107.009 -> sounds, and the presence of any murmurs, assessment of the liver will indicate if there is an
112.819 -> overload of fluid on the right side of the heart, and the quality and strength of pulses.
120.249 -> Diagnostic tests should include a chest radiograph, an electrocardiogram.
124.729 -> Other tests may include an echocardiogram or a cardiac catheterization.
132.69 -> Low Cardiac Output.
136.959 -> Signs of low cardiac output in infants and children include pale or mottled skin color,
142.26 -> irritability, cool and clammy skin, or decreased level of consciousness, decreased urine output,
150.43 -> capillary refill greater than three seconds, metabolic acidosis, hypoglycemia, and increased
159.16 -> serum lactate.
161.69 -> Other signs include weak pulses, temperature instability, or apnea.
169.51 -> There may also be evidence of organ dysfunction such as kidney or liver failure.
175.89 -> Late signs include hypotension, cyanosis, anuria, and altered mental status.
185.04 -> Interventions to improve low cardiac output include correcting the heart rate and restoring
190.3 -> normal conduction within the heart, administration of fluids, correcting acid-base imbalance
197.6 -> and electrolyte abnormalities, and using medications to improve the function of the heart and to
204.1 -> reduce the stress on the body.
209.23 -> Congestive Heart Failure.
213.33 -> Congestive heart failure occurs when the heart cannot deliver enough blood to meet the demands
217.72 -> of the body.
219.34 -> The heart attempts to compensate by increasing the heart rate, thickening the walls of the
224.78 -> heart to contract more effectively, or dilating the heart to increase the volume of blood
230.19 -> within the heart to improve cardiac output.
234.48 -> Congestive heart failure occurs in 30% of infants and children with congenital heart
239.5 -> disease and occurs in over 75% of children with complex heart disease.
248.22 -> The causes of congestive heart failure include volume overload of the heart, pressure overload
254.22 -> of the heart, and a heart muscle dysfunction.
258.91 -> Some of the clinical signs of congestive heart failure include tachypnea, retractions, nasal
266.04 -> flaring, diaphoresis, pulmonary edema, irritability, change in responsiveness, fatigue, poor feeding,
276.699 -> failure to thrive, tachycardia, cool skin, decreased urine output, an enlarged liver,
291.81 -> periorbital edema, pulmonary effusions, and ascites.
298.949 -> Interventions for congestive heart failure include medications, such as diuretics to
303.46 -> eliminate excess fluid, limiting fluid administration, and improved nutrition to maximize calories.
312.56 -> Other interventions include respiratory support to reduce the work of breathing and temperature
318.659 -> control.
322.96 -> Cyanosis.
327.27 -> Cyanosis occurs when neonates are unable to tolerate the changes that occur when the blood
332.289 -> flow within the heart changes after birth.
336.49 -> Cyanosis may also occur in infants and children with congenital heart defects that cause a
341.889 -> decrease in blood flow to the lungs.
346.05 -> Cyanosis: respiratory or cardiac?
350.18 -> When cyanosis occurs with respiratory disease, the cyanosis usually decreases with crying,
357.37 -> improves with oxygen administration, and there are usually signs of respiratory distress.
363.569 -> When cyanosis occurs with cardiac disease, the cyanosis usually increases with crying
369.509 -> because the demand of oxygen increases, but the body is unable to deliver sufficient oxygen.
377.74 -> Cyanosis with cardiac disease does not usually improve significantly with the administration
382.68 -> of supplemental oxygen, and the infants usually breathe fast but do not have evidence of respiratory
389.68 -> distress.
391.479 -> Tests that can help in the diagnosis of respiratory or cardiac disease include a 12-lead electrocardiogram,
400.13 -> a chest radiograph, and a hyperoxia test that involves evaluating the oxygen content of
407.15 -> the blood with and without supplemental oxygen.
413.12 -> Point of clarification.
418.669 -> Hyperoxia test.
420.379 -> The basic physiology of the hyperoxia test is that you are going to use supplemental
424.199 -> oxygen to distinguish between cardiac and pulmonary causes of cyanosis.
429.02 -> Specifically, the way that the test is done is that you obtain a preductal arterial blood
437.909 -> gas in the cyanotic patient.
439.289 -> So that is typically a radial arterial blood gas to measure the oxygen tension in arterial
444.87 -> blood on room air.
447.36 -> You then place the patient in an oxygen-rich environment.
450.3 -> So that's at 100% oxygen for 10 minutes.
453.529 -> That can be either via ventilator or via an oxy hood.
457.569 -> After 10 minutes of breathing in oxygen-rich environment, you repeat the blood gas preductally--
465.05 -> so the radial arterial blood gas-- and remeasure the partial pressure of oxygen in arterial
470.419 -> blood.
471.699 -> Typically, the cutoffs that we use here at Children's Hospital Boston is a PaO2, in your
477.67 -> arterial blood of greater than 150 indicates a high likelihood of a known cardiac cause
483.24 -> of cyanosis, such as pulmonary venous desaturation.
486.86 -> A PaO2 of less than 150 is very suspicious for a cardiac cause of cyanosis as a right-to-left
497.49 -> shunt cannot be overcome by the provision of supplemental oxygen.
504.28 -> Degraded further, we consider a PaO2 of less than 100 very suspicious for cyanotic congenital
510.61 -> heart disease, and this should trigger cardiac consultation.
515.44 -> Signs of cyanosis with congenital heart disease include bluish discoloration of skin and mucous
521.56 -> membranes, increased respiratory rate and effort, irritability, or lethargy.
530.94 -> Other signs include decreased blood flow to the body, decreased urine output, and metabolic
537.51 -> acidosis.
538.66 -> Long-term effects of chronic cyanosis include an increased hematocrit, thickening of the
544.69 -> blood, increased risk of clot formation, clubbing of fingers, and an increase in the release
552.79 -> of oxygen to the tissues.
557.529 -> Interventions to reduce cyanosis include maintaining a high hematocrit to maximize oxygen-carrying
563.579 -> capacity and fluid administration to reduce the thickness of the blood.
569.84 -> Other interventions include the administration of supplemental oxygen-- close monitoring
575.399 -> for air in intravenous infusion lines is essential to reduce the risk of air traveling to the
581.57 -> brain in an infant or child with cyanotic heart disease-- and using alprostodil in neonates
589.97 -> to keep the ductus arteriosus open to allow mixing of blood within the heart.
597.68 -> Please help us improve the content by providing us with some feedback.

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