EA: How your child's nurse can help

Analyze nursing care of individuals with your disease for the most current information. Identify key websites that you recommend as part of your blog in this area.

Nursing Plan for Ebstein’s Anomaly Treatment

Diagnosis 1.

Activity intolerance related to insufficient oxygenation secondary to inefficient blood flow through the pulmonary artery as evidenced by weakness, fatigue, shortness of breath, changes in pulse rate and changes in blood pressure.

Let’s break it down:

Problem	Related To	Plan	Outcome	Nursing Interventions
Activity intolerance	Insufficient oxygenation	To achieve optimal activity level	-To breathe easily during activity. -To maintain an adequate and acceptable blood pressure (age dependent) during activity. -To maintain and adequate and acceptable pulse rate (age dependent) during activity.	-Monitor pulse rate and blood pressure in response to activity. -Encourage alternating periods of rest and activity to conserve energy and decrease demands on the heart. -Encourage activities to gradually build endurance to increase cardiac tolerance. -Assist the patient and caregiver to establish realistic exercise goals to promote the patient’s health and sense of accomplishment.

Diagnosis 2.

Risk for excess fluid volume related to fluid retention secondary to tricuspid value induced right heart failure as evidenced by peripheral edema, weight gain, adventitious breath sounds, and/or jugular venous distension.

Diagnosis 3.

Decreased cardiac output related to tricuspid valve insufficiency as evidenced by murmurs, dyspnea, and peripheral edema.

Diagnosis 4.

Impaired gas exchange related to insufficient blood flow to the lungs through the pulmonary artery as evidenced by abnormal oxygen saturation, dyspnea, restlessness, or the statement “I feel short of breath.”

Diagnosis 5. 
Altered Nutrition: Less than body requirements related to difficulty breathing while eating as evidenced by prolonged feeding time, increased respiratory rate between latching or chewing and swallowing, weight loss.


References:
1. Lewis LL, Dirksen SR, Heitkemper MM, et al. Medical-Surgical Nursing: Assessment and Management of Clinical Problems. Edition 9. 2014. Elsevier Health Sciences. 

EA: What the nurse is looking for

EA:The Nurse's Exam and Diagnosis

The nurse will examine your child's heart in a similar fashion to the video above. In order to listen to the heart and to listen carefully for abnormal heart beats (arrhythmias). The nurse will do this while the child is calm and quiet. The nurse will also inspect the child for signs of difficulty breathing, and cyanosis (blue skin). Difficulty breathing is a common finding in children with congenital heart defects such as EA. This difficulty breathing can also lead to exercise intolerance (easy fatigue with activity) and even feeding problems. 

The American Heart Association Website discusses the evaluation of children with congenital heart defects, such as EA: 

Adults with a history of congenital heart defects may find more information about evaluation and medical care at the Adult Congenital Heart Association Website.

The following table shows common nursing diagnoses for EA, and what those diagnoses mean to the nurse[1]: 

Nursing Diagnosis	Explanation	Nursing Care
Impaired gas exchange	The lack of blood flowing to the lungs due to the decreased size of the right ventricle means that the blood which enters the whole body lacks the oxygen necessary to keep the cells working properly. This is commonly seen by cyanosis of the skin.	-        Elevate the head of the bed to 30 degrees to ease the fluid load in the lungs. -        Offer oxygen if indicated.
Activity intolerance	Difficulty maintaining exercise due to fatigue. The child may squat or bend over with hands on the knees to reduce the return of blood to and demand on the right side of the heart.	-        Place infant with knees flexed and HOB elevated or being held by caregiver to reduce blood return to the heart and ease the fluid load on the heart. -        Promote activity as tolerated with rest periods.
Altered Nutrition: Less than body requirements	Difficulty breathing can lead to problems with breast and bottle feeding in newborns and young children. This is a common problem with congenital heart disease. These children	-        Encourage patience with breastfeeding. May need to have more frequent feeds to get the necessary caloric intake for proper growth and development.
Risk for infection	Fluid buildup in the lungs increase the risk for pneumonia. If your child requires surgery, the nurse will be looking for signs of post-operative infection.	-        The nurse will be listening to the lungs using the stethoscope on the child’s chest and back. Fluid in the lungs causes crackle and wheezing sounds as the child breathes. -        The nurse will also be assessing for fever and a cough which produces a lot of fluid.
Risk for altered family processes	The amount of time and expense of hospital visits for treating EA children can be very stressful for families and disrupt normal patterns of living.	-        Assess family coping and encourage parents/caregivers to express their feelings. -        Suggestions about support groups. -        Encourage parent/caregivers to take time for themselves

References:

1. NANDA List of Nursing Diagnoses. Available from: http://nclex.ucoz.net/_ld/0/30_NANDALISTOFDIAG.pdf. Accessed February 11, 2015. 

EA: The Treatment Options

What are the Treatment Options for Ebstein's Anomaly (EA)?
If your child was recently diagnosed with EA, you are probably wondering what treatment options are available. 

Depending on the severity of  your child's symptoms, there are several options. Those children with mild EA who are free of symptoms require no medical treatment, although they should be closely monitored by the pediatric health provider. If your child is showing symptoms of EA, a healthcare provider may suggest medical treatments or surgery. 

Medical Treatments
The medical treatments for EA focus on treating the secondary effects - enlargement of the right side of the heart, the backflow of blood through the tricuspid valve, and right sided heart failure.[1] Issues on the right side of the heart result in a backup of blood in the rest of the body. This causes peripheral edema (swelling due to poor blood flow from the legs and arms back to the heart). Diuretic medications (those that help the body remove excess fluid in the urine) are a common treatment because they reduce the peripheral edema and the stress on the right side of the heart.[1] EA patients may also be given an anticoagulant medication to prevent clots of blood from forming.[1] Blood clots can be formed due to the slow flow of blood in the arms and legs, these clots can travel through the blood stream to the lungs causing a build up of fluid in the lungs and infection. 
Patients who are not showing symptoms should go to the doctor to followup every two or three years, but if symptoms arise they should go to the doctor and follow up every 6 months and discuss medical treatment options with their healthcare provider.[1]

The University of Washington has developed a model called the The Seattle Heart Failure Model that patients and their healthcare providers can use to identify patients who are at high risk for poor outcomes with EA and require more specialized treatment and intervention. Take this form, or the web address, to your child's doctor (or your doctor) to develop a treatment plan that fits your needs.[2]

Surgical Treatments
EA patients with arrhythmia of the heart (heart murmurs or abnormal heartbeats) usually must have surgery to repair the heart. Surgery will be performed on patients who have difficulty exercising due to the heart defect, cyanosis (blue tones to the skin), those who develop emboli (blood clots which travel through the blood stream), those with cardiomegaly (an increasing heart size), and those with right ventricular dilation (a size increase of the lower right heart chamber).[1] However, surgery is usually not performed on newborn babies due to immaturity of the respiratory system.[1]  These surgeries focus on rebuilding the tricuspid valve which is pushed downward into the right ventricle in EA.[1] The surgical techniques used depend on the patient and the severity of EA, but all aim to reduce the backflow of blood through the tricuspid valve, reduce arrhythmias, closing the foramen ovale, and placing a pacemaker (a device that controls and regulates heart beats) if necessary.[1] Your healthcare provider and heart surgeon will discuss these options with you. 
The following video is a surgical technique used to repair the tricupid valve - "Cone Reconstruction":

References
1. Arya P, Beroukhim R. Ebstein Anomaly: Assessment, Management, and Timing of Intervention. Curr Treat Options in Cardio Med. 2014. 16(10): 1-13. doi: 10.1007/s11936-014-0338-x. 
2. Seattle Heart Failure Model. University of Washington Web site. Available from: https://depts.washington.edu/shfm/. Accessed February 10, 2015.