Nursing Care of Children with Cyanotic Heart Diseases

CONGENITAL HEART DISEASES 

Congenital heart disease [CHD] is a structural malformation of heart or great vessels present at birth which may not necessarily be detected at time of birth. CHD can be grouped into three categories. 

1. Acyanotic congenital heart diseases 

2. Cyanotic heart diseases 

3. Obstructive lesions 

CYANOTIC CONGENITAL HEART DISEASE 

• There is a diminished pulmonary blood flow due to right to left shunt, it includes  
• Tetralogy of Fallot [TOF] 
• Tricuspid Atresia [TA] 
• Mixed blood flow with increased pulmonary blood flow 
• Transposition of great arteries [TGA] 
• Truncus Arteriosus 
• Total anomalous pulmonary venous return 

• TETRALOGY OF FALLOT  
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Tetralogy of fallot is the most common congenital defect, consists of four defects combindly which occur due to developmental abnormality of heart during the first 8 week of the pregnancy period. 

TOF is made up of four defects 

• Ventricular septal defect 
• Pulmonary stenosis  

• Right ventricular hypertrophy 
• An overriding of aorta 

PATHOPHYSIOLOGY 

• Due to pulmonary stenosis, there is an obstruction of blood flow from the right ventricle to pulmonary artery. 
• Pressure thus rises in the right ventricle resulting in its hypertrophy. 
• The pressure in the right ventricles rises so as to become equal or slightly more than the left ventricle. 
• Deoxygenated blood is shunted from the right ventricle through the VSD directly into aorta. [left to right shunt]. 

CLINICAL MANIFESTATIONS 

• Cyanosis during exertion and crying later at rest. 
• Hyper cyanotic spells or “tet” spells [hypoxic or anoxic spells] 
• Child assumes squatting position to increase pulmonary blood flow [knee chest position] 
• Slow weight gain 
• Failure to thrive 
• Systolic murmur heard in the pulmonic area 
• Polycythaemia 
• Metabolic acidosis 
• Clubbing 
• Exercise intolerance 

DIAGNOSTIC EVALUATION 

• Detail health history of illness 

• Through clinical examination  
• Chest X-ray   
• Cardiac catheterization 
• Echocardiogram  

THERAPEUTIC MANAGEMENT 

• Medical management consists of managing the hyper cyanotic spells on an emergency basis and correcting anaemia.  
• Surgical management may be palliative or definitive. 

• Palliative surgery consists of creating a communication between systemic and pulmonary circulation 
• Blalock –Taussig operation: creates an anastomosis between the right or left subclavian artery and the right pulmonary artery. 
• Waterson shunt: refers to an anastomosis between the ascending aorta and the right pulmonary artery. 
• Both operations result in increased blood supply to the pulmonary circulation through the anastomosis. 
• Total correction: VSD is repaired with patch closure and right ventricular outflow obstruction is relieved. 

• TRICUSPID ATRESIA [TA] 
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• It is characterised by absence of or complete closure of the tricuspid valve and therefore there is no correction between the RA and the RV. 
• Other associated defects include ASD, VSD and varying degrees of RV. hypoplasia 

PATHOPHYSIOLOGY 

• In infants with tricuspid atresia, there is a complete mixing of systemic and pulmonary venous return at the atrial level.  
• Systemic venous return enters the RA but cannot pass to the RV through the tricuspid valve and must flow through an intra atrial connection to the LA 
• Blood then flows to the LV and if there is a VSD, some blood flows through the VSD to the PA 

• The reminder of the blood in the LV will flow out the aorta  
• If there is no associated VSD, then blood flow to the lungs must be through a PDA 

CLINICAL MANIFESTATIONS 

• Cyanosis –blue colour of skin, lips and nail 
• Severe hypoxic spells 
• Tachycardia, tachypnea 
• Dyspnoea or laboured breathing  
• Clubbing, polycythaemia 
• Growth delay 
• Cool, clammy skin 
• Single s2 is heard in the aortic area 
• Harsh systolic murmur heard in the tricuspid area. 

THERAPEUTIC MANAGEMENT  

• Palliative procedures 

• Waterson shunt: anastomosis between the ascending aorta and right pulmonary artery. 
• Glenn procedure side to end anastomosis of the superior vena cava to the right pulmonary artery. 

• Complete correction 

• Fontan procedure involves placement of a tubular conduit with a valve between the right atrium and main pulmonary artery. the atrial defect is closed and the main pulmonary artery is ligated just above the pulmonary valve. 

• TRANSPOSITION OF THE GREAT ARTERIES[TGA] 

TGA is a defect in which the great vessels are transposed or reversed. The pulmonary artery originates from the LV and the aorta originates from the RV. 

PATHOPHYSIOLOGY 

• In infants with TGA, unoxygenated blood entries the RA to RV then flows out the aorta to the body. 
• Oxygenated blood from the lungs enters the LA to the LV and then flows out the pulmonary artery back to the lungs. 
• Therefore, there is a parallel circulation with no oxygenated blood getting to the systemic circulation.  

CLINICAL MANIFESTATIONS 

• Cyanosis with first few hours of birth  
• Clubbing of fingers and toes 
• Congestive heart failure 
• Easy fatigability 
• Slow weight gain 
• Failure to thrive 
• Frequent chest infections 
• Tachypnea 

THERAPEUTIC MANAGEMENT 

• Medical management 
• A continuous infusion of PGE1 is begun to maintain ductal patency and support mixing of oxygenated and unoxygenated blood at the level of the ductus.  
• A Rashkind balloon atrial septostomy by interventional cardiac catheterization may be performed on some infants to enhance mixing of blood if there is not adequate intra atrial mixing. 
• Surgical management 

Surgical management may be palliative by balloon atrial septostomy or definite by redirecting the blood flow through mustard or senning procedure. 

• TRUNCUS ARTERIOSUS 

A single large vessel empties both ventricle and provides circulation for the pulmonary, systemic and coronary circulations AVSD is usually present. 

PATHOPHYSIOLOGY  

• Blood ejected from the left and right ventricles enters the common trunk mixing pulmonary and systemic circulations. 
• The amount of pulmonary blood flows depends on the vascular resistance and the size of the pulmonary arteries. 
• Generally, the pulmonic resistance is less than the systemic resistance resulting in preferential blood flow to the lungs. 
• Pulmonary vascular disease develops at an early age in patients with truncus arteriosus. 

CLINICAL MANIFESTATIONS 

• Cyanosis develop soon after the birth 
• Poor growth and feeding 
• Severe CHF 
• Dyspnoea 
• Retractions 
• Clubbing 
• Increased pulse pressure  
• Cardiomegaly 
• Frequent respiratory infections  

MANAGEMENT 

Medical management 

• Medical management is aimed at reducing the effects of CHF and preventing polycythaemia. 

• CHF is treated with digoxin and diuretics. surgical repair is recommended in the neonatal period. 

Surgical management 

• A Rastelli procedure is performed to close the VSD and create a passage to the pulmonary arteries. repeated surgeries is necessary to enlarge the pulmonary artery conduit as the child grows. 
• TOTAL ANOMALOUS PULMONARY VENOUS RETURN  

• The pulmonary veins empty into the right atrium or veins leading to the right atrium rather than into the left atrium. 
• The foramen ovale remains patent for mixed blood from the right atrium to pass to the systemic circulation. 

CLINICAL MANIFESTATIONS 

• Mild cyanosis 
• Frequent respiratory infections 
• S2 has wide, fixed split 
• Ejection murmur and gallop rhythm heard in the pulmonic area  

MANAGEMENT  

• Prostaglandin E1 is given to maintain patent ductus area 
• Hypoxia and CHF are treated 
• Balloon atrial septostomy may be performed to promote better mixing of blood 

Surgery to reconnect the pulmonary veins to the left atrium is performed, the ASD is closed 

NURSING CARE OF CHILDREN WITH CYANOTIC HEART DISEASES. 

ASSESSMENT  

The nurse must know the source of pulmonary blood flow when caring for a child with cyanotic heart diseases. Infant and children who are shunt dependent for pulmonary blood flow are at risk for shunt thrombosis. In infants with right to left shunts are at risk for air embolus in IV lines. 

• Evaluation of the child with cyanotic heart disease includes an assessment of baseline cyanotic heart disease includes an assessment of baseline cyanosis and general appearance. 

• Assess the level of activity, including irritability. 
• Visible cyanosis is most easily seen in natural light and is evaluated by observing the skin of the central mucous membrane of mouth and conjunctiva and the nail beds. 
• Cyanotic children may be smaller than their peers. 
•  Clubbing, thickening and flattening of the fingertips and toes as a result of polycythaemia. 
• The child may become dyspnoeic during feeding, crying and the other exertional activities. 
• Children with cyanosis may have frequent respiratory infections.They are also risk at greater risk for development of infective endocarditis and may need continuing antibiotic prophylaxis. 
• In addition to assess the child and assisting with diagnostic procedures, nursing management of an infant or child with congenital heart disease includes helping family members to adjust to the child’s care and providing both preoperative and post-operative care. 

EPISODES OF CYANOSIS OR DYSPNOEA 

• Parents usually very frightened when their child becomes cyanotic and or dyspnoeic, if they know how to handle the situation, they can feel more in control and can convey this confidence to the child, reducing the child’s anxiety. 
• Management of paroxysmal dyspnoeic attacks consists in placing the infant or child on the abdomen in a knee chest position or on the side with knees flexed and the chest and head elevated. 
• Any clothing that restricts respirations should be loosened. 

NEED FOR COMFORT AND REST  

• with cyanosis and dyspnoea. The infants or child’s expenditure of energy should not exceed the ability of the body to oxygenate the blood.  
• A quiet area should be provided for sleep. Crying should be prevented as much as possible. 
• In order to prevent crying parents can give more frequent feedings, can have the feedings ready before the usual time when hunger becomes evident, can change diapers as soon as they are solid. 
• Rest period must be included in the daily schedule, after period of activity. 
• Older children learn to limit their own activities and to prevent problems 

NUTRITIONAL NEEDS 

• Possibly cyanotic and dyspnoeic if feeding is prolonged, small amount of formula and food should be given frequently. 
• They should be fed slowly and bubbled as often as necessary in order to prevent distension of the stomach. 

• Older children who may not feel hungry should be encouraged to participate in planning their meals, increasing their interest in the food that is served. 
• If a cyanotic heart defect is present, the child should be provided with an adequate fluid intake to prevent dehydration and decrease the danger of polycythaemia. 

MEDICATIONS 

• Parents are often anxious about administering medications to their children, especially if they know that they will affect the heart. 
• The nurse can help to alleviate this anxiety by explaining the name, and purpose of the medication. 
• The dose and times it is to be given, its expected effects, its potential side effects and the method of administration. 

PSYCHOSOCIAL NEEDS 

• Activities to encourage normal growth and development should be provided considering their limitation. 
• Experiences in which infants cannot participate should be replaced by experiences of comparable learning value. For example, infants should be given toys that are light weight and easily handled. 
• Young children with heart defects who have limited exercise tolerance restrict their own activities to a level of comfort. 
• Although they may not be able to complete they learn that solitary activities, such as reading or handicrafts. 

CONTINUING CARE 

• The frequently with which children having congenital heart defects are seen by a cardiologist or paediatrician often depends on whether the parents can provide adequate care at home and whether they are observant enough to promptly report changes in the child’s condition 
• The nurse should teach parents that respiratory infections should be prevented and exposure to others who have active infection should be avoided. 
• If they do occur, they should be treated immediately. 
• If minor surgical procedures are necessary, prophylactic antibiotics may be indicated. 
• If the parents continue to be anxious about the care of their child, the community or public health nurse and the nurse in the hospital or clinic may be able to provide continuing support and guidance. 

FAMILY RELATIONSHIPS 

• It is important that they accept the fact that these discrepancies are due to the child’s physical status and not to deficiencies in their care. 
• When the mother is the primary caregiver, other family members may feel deprived of her attention as she cares for the ill child. 

• Other parents of children with cardiac problems may provide a support system, with suggestions for care. 
• Although parents of children with cardiac problems are often reluctant to discipline their children, this is an important part of parenting. 
• When encouraged to depend completely on their care givers children may become over dependent and difficult to control. 
• The nurse who aware of overprotectiveness should encourage professional referral, to assist the child and other family members. 

FINANCIAL RESPONSIBILITIES 

• The care of a chronically ill child pot only may be physically and emotionally exhausting for family members but also may drain their financial resources. 

PROVIDING PREOPERATIVE CARE 

Admission to the hospital unit for palliative or correction of a cardiac defect is generally scheduled several days before surgery in order to complete the preoperative assessment and to familiarize the child and family with the environment. 

PREOPERATIVE ASSESSMENT  

The preoperative assessment includes the history and physical examination, preoperative studies, baseline vital signs, measurement of height and weight and additional nursing observations.  

PREOPERATIVE STUDIES 

• This include chest radiography, EKGs, laboratory tests and other necessary procedures. 
• If a fluid and electrolyte imbalance is found, it is corrected. 
• The blood is typed and cross matched for intraoperative and postoperative replacement. 
• If evidence of infection is found the child is treated appropriately, but the surgery is postponed because of the risk of infective endocarditis. 

BASELINE VITALSIGNS 

• A series of baseline vital signs are obtained so that the child intraoperative and postoperative status can be evaluated. 
• Any changes in vital signs should be reported to the physician immediately as they could indicate infection or congestive heart failure. 

HEIGHT AND WEIGHT MEASUREMENT 

• These measurements are obtained because they form the basis for calculating fluid replacement volumes and medication dosages throughout the hospitalization. 

ADDITIONAL NURSING OBSERVATIONS 

• The child’s normal cycle of activity and rest are determined from the nursing history and are observed during the preoperative period. 
• The nurse should also validate the information on 

•  the nursing history concerning the child’s likes and dislikes, as well as observe the routine the child establishes in the hospital setting. 
• Fluids can be given at preferred times, and provisions can be made for monitoring toilet habits so that postoperative constipation can be prevented. 

PRREOPERATIVE TEACHING  

• The goals of preoperative preparation include helping the parent and child deal with an unfamiliar situation, reducing the trauma of the experience and promoting growth and coping abilities. 
• Teaching of parents and child is done throughout the preoperative period so that information can be given gradually. 
• If the child is fatigued, as after receiving a great deal of information, anxiety producing details can be shared at another time. 
• The nurse continually evaluates the response to teaching to determine whether further clarification is necessary.  

INTRODUCTION TO THE ENVIRONMENT 

• Because of the seriousness of the diagnosis and the intensity of feeling tones surrounding the surgery, the child should be cared for preoperatively by one nurse on each shift. 
• If possible the nurse who will be responsible for postoperative care should become familiar with the child. 
• The child and parents are taken on a tour of the recovery room or intensive care unit the day prior to surgery at a time when these units are relatively quiet. 
• Depending on the child level of understanding, the equipment that will be used can be explained, with emphasis placed on its purpose, the noises that will be heard during its use, and any sensory changes it will create. 
• If an oxygen tent is to be used after surgery, the child may be given the experience of looking through the plastic film to see how it distorts vision so this will not be frightening postoperatively.   
• If a television set is available for viewing, the child will feel more comfortable because this is probably part of a more familiar environment –home. 
• The child and family members should show the area where the family can wait during surgery and be told that the family can receive information about their child during and after surgery. 

INTRODUCTION TO THE EQUIPMENT 

• The parents and child should be permitted to see and possibly handle the equipment that will be used in postoperative care. 
• The nurse can show the parents and child the actual equipment or pictures of the equipment and child the actual equipment or pictures of the equipment and can role play its use through dolls or puppets. 
• The equipment can be shown being used on the another child on the unit or a child in the recovery or intensive care area. 
• The nurse can locate on the drawing the area of the incision and the size and type of dressing or adhesive strips that will be applied after surgery. 

INTRODUCTION TO POSTOPERATIVE PROCEDURES 

• The nurse can teach and help the child practice the various procedures that will be necessary postoperatively. These procedures may be practiced as a game with young children. 
• The older child should be told the reasons why turning deep breathing, coughing, postural drainage and percussion, and intermittent positive pressure are needed following surgery. 
• The nurse shows the child when in semi fowlers position how to turn from one side to side by flexing the knees and holding the side rail. 
• The nurse can demonstrate the procedure of deep breathing to the child and then together can practice deep inspiration and expiration. 
• The child should be told that coughing and deep breathing will cause some discomfort after surgery. 
• Depending on the child’s age the skin is prepared by shaving or by the use of a topical depilatory for adolescents. 
• Preoperative skin preparation also includes an antibacterial bath, perhaps in a tub or shower. 
• Loose teeth of older children are reported in order to prevent aspiration during intubation. 
• Cleansing enemas may be used to prevent abdominal distension and staining of defecation during the early postoperative period. 
• Throughout these teaching sessions the nurse must be acutely aware of the child’s level of anxiety and the cues that may be used to signal that the discussion should end. 

PROVIDING POSTOPERATIVE CARE 

• The child is transferred to the intensive care unit or the recovery for 24 -48 hours or longer until the vital signs and all systems of the body are stabilized. 
• Adequate rest is essential in order to minimize the demands on the heart and to promote healing of the operative area. 
• If the parents are involved in the plan they may able to make helpful suggestions concerning their child’s care. 
• Their involvement can increase their level of cooperation with visiting and assisting with their child’s care. 

• Postoperatively prophylactic antibiotics may be administered for several days to prevent infection, and electrolyte therapy such as calcium and potassium may be given to promote optimal cardiac functioning. 

NURSING DIAGNOSIS   

• Ineffective tissue perfusion related to hyper cyanotic episodes. 
• Deficient knowledge related to inexperience with the management of a child with a life threatening illness. 
• Interrupted family process related to impact of an acute, chronic, or life threatening disease. 
• Delayed growth and development related to altered oxygenation or inadequate cardiac output to meet metabolic needs. 
• Risk for infection related to the presence of infection promoting conditions created by the underlying defect. 
• Deficient knowledge related to unfamiliarity with the systemic complications from increased risk of clotting.